Research Updates
Sickle Cell News from Allan Platt
Sickle Cell News for May 2013
June 19 World Sickle Cell Day http://worldsicklecellday.
The World Health Organization (WHO) has started work to promote a world wide agenda to address hemoglobin dysfunctions.
WHO has made a commitment to:
- Recognize that sickle cell disease is a major health issue.
- Increase awareness of the world community regarding sickle cell disease.
- Eliminate harmful and wrong prejudices associated with sickle cell disease.
- Urges member countries where sickle cell disease is a public health problem to establish health programs at the national level and operate specialized centers for sickle cell disease and facilitate access to treatment.
- Promote satisfactory access to medical services to people affected with sickle cell disease.
- Provide technical support to all countries to prevent and manage sickle cell disease.
- Promote and help research to improve the lives of people affected with sickle cell disease.
The World Sickle Cell day is celebrated across the globe with special emphasis in African Nations and Asia. The celebrations include a press, media campaigns, music shows, cultural activities, and talk shows.
The main emphasis is hence on educating medical professionals, care givers, and associated personnel about prevention, research, and resources to minimize the complications due to sickle cell disease. Hence June 19th is devoted mainly to spread awareness, through talks, seminars, pamphlets, literature and consultations.
SCDAA Announces Kier "Junior" Spates as Ambassador http://www.sicklecelldisease.
Comedian Kier "Junior" Spates of the Steve Harvey Morning Show is joining the fight against sickle cell disease. Spates signed on as the national celebrity ambassador for the Sickle Cell Disease Association of America, Inc. (SCDAA) after recently speaking out about his struggle with the disease. Together, SCDAA and Spates will launch the "Rise Above" initiative. The campaign will educate and raise awareness about the blood disease across the nation.
Spates, a native of Houston, Texas, boasts an impressive resume that includes television spots on Black Entertainment Television, collaborative efforts on the "Rickey Smiley and Friends" show and his role in Hinton Battle's "Love Lies" stage play. He is most notably known today for his hilarious and high energy commentary on the Steve Harvey Morning Show. "We are excited to work with such a bright talent as Kier. He is the perfect choice for raising awareness about sickle cell disease in addition to spreading joy to those who support our cause," says SCDAA President Sonja Banks.
Spates is also living with sickle cell disease. He hopes to inspire others and increase the support and visibility of the disease through this recent partnership with the SCDAA. Please visitwww.facebook.com/
New Video
New Parents hand book and Children’s DVD produced in the UK
The 3rd Edition of, 'A Parents guide to managing Sickle Cell Disease', in English is available on the UK national screening web site www.sct.screening.nhs.ukand on the Brent Centre web site www.sickle-thal.nwlh.nhs.uk. It is being translated into French also and will be available in the summer. A new children's DVD, 'My Sickle Cell Disease and Me', funded by Roald Dhal, aimed at children aged 5 - 11years is available on the Brent Centre web site www.sickle-thal.nwlh.nhs.uk
SAVE THE DATES
Fellow colleagues, you are invited to participate in the“Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, CDC
4th Thursday of every month from 2:00PM – 3:00PM EST
6/27: Beta-globin Haplotype Analysis in Children with Sickle Cell Anemia
Dr. Christopher Bean, National Center on Birth Defects and Developmental Disabilities, CDC
7/25: Nurses’ Impact on the Stigmatization of Individuals with Sickle Cell: Challenges and Recommendations
Dr. Coretta Jenerette, International Association of Sickle Cell Nurses and Physician Assistants
8/22: Mental Health and Learning Needs in children with Sickle Cell Disease
Dr. Patricia Kavanagh, Boston University School of Medicine/Boston Medical Center
9/26: NHLBI Sickle Cell Disease Guidelines
Dr. Joylene John-Sowah, National Heart Lung and Blood Institute, NIH
10/24: Current Data on Efficacy and Effectiveness of Hydroxyurea in Children with Sickle Cell Disease
Dr. Winfred Wang, St. Jude Children’s Research Hospital
November/December: --- No Webinars---
If you have ideas on topics for future webinars, or have any questions or comments about this series please submit them to Shae Pope spope@cdc.gov .
The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
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CDC Web based Sickle Cell Resources CDC’s YouTube Link: http://www.youtube.com/watch? CDC Video Archive (http://scinfo.org/world-wide- CDC Sickle Cell Disease Webpage: http://wwwdev.cdc.gov/NCBDDD/ Articles in the Medical Literature for May
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Sickle Cell News for April 2013
Anti-sickling therapies should be focus for sickle cell science http://www.eurekalert.org/pub_
Pain is an undeniable focal point for patients with sickle cell disease but it's not the best focus for drug development, says one of the dying breed of physicians specializing in the condition.
Rather scientists need to get back to the crux of the disease affecting 1 in 500 black Americans and find better ways to prevent the hallmark sickling that impedes red blood cells' oxygen delivery, damaging blood vessel walls and organs along the way, said Dr. Abdullah Kutlar, Director of the Sickle Cell Center at the Medical College of Georgia at Georgia Regents University.
"We have one drug that reduces sickling and we need more," said Kutlar, the 2013 Roland B. Scott, M.D., Lecturer for the 7th Annual Sickle Cell Disease Research and Educational Symposium and National Sickle Cell Disease Scientific Meeting April 14-17 in Miami.
"Pain is very important to someone who is suffering, but by using pain as an end point, we are missing opportunities and wasting drugs that could be very helpful," he said. "Moving forward, I suggest we develop new combination therapies that have anti-sickling capabilities at their center," said Kutlar, noting such cocktail approaches have worked well for cancer and HIV.
Kutlar completed an extensive historical review of patient and study outcomes in preparation for the lecture honoring the late Howard University physician who made it his mission to improve the lives of children with sickle cell disease. Scott's contributions include prompting the National Sickle Cell Control Act of 1972, which established the first federally-funded comprehensive sickle cell centers, including the one at MCG led by Dr. Titus H.J. Huisman.
No doubt Scott, Huisman and others have made a tremendous difference to patients, whose average life expectancy has gone from the teens to the 50s in the past 30 years, Kutlar said. Much of that progress grew out of focusing on the basics, including developing hydroxyurea, still the only Food and Drug Administration-approved drug that targets sickling.
Approved 15 years ago, hydroxyurea works by increasing expression of fetal hemoglobin, which can't sickle. However, it's still not widely used – about 35 percent of Kutlar's adult patients take it, for example – probably for a combination of reasons that include a side effect of weight gain and unsubstantiated concerns that it increases cancer risk. He and his colleagues need to do a better job communicating the benefits of this drug in addition to finding new ones, Kutlar said. Reduced sickling means less damage to blood vessels and organs, he said, noting that the major cause of death in adults and children is acute chest syndrome, a severe pneumonia resulting from cumulative lung damage. A handful of new anti-sickling drugs are in various stages of development, including a thalidomide- derivative pioneered by MCG researchers that also enhances fetal hemoglobin expression.
Other good endpoints for drug development include downstream effects of sickling, such as the unnatural adhesion of red blood cells to blood vessel walls, Kutlar said. Unfortunately work was recently halted on a drug that reduced adhesion but not pain, Kutlar said.
Pain needs to be the primary endpoint only for pain medications, he noted. The good news is that many new pain medications are available for these patients, whose pain crises can be severe enough to require hospitalization and whose chronic pain can impair daily living. However, that circles back to the complex causes of pain. The pain initially likely results from tissues crying out for more oxygen and later from nerve and organ damage resulting from ongoing impaired oxygen supplies. Pain control can get even more complex and difficult because regular use of opiates, a common analgesic for sickle cell patients, actually increases pain sensitivity, Kutlar said.
In addition to finding better therapies, physicians who treat sickle cell patients need to help cultivate the next generation of caregivers, Kutlar said. He's in the minority in that he opted to take care of patients with sickle cell disease rather than pursue the more common – and generally more professionally lucrative – hematology path: treating cancer. "We don't have enough hematologists, period," said Kutlar. The problem does have a good cause: the reality that more patients are living longer. However, the number of physicians to treat adult patients is dismal. Helping cultivate the next generation is a focus of a study led by Kutlar and Dr. Robert W. Gibson, a GRU occupational therapist and medical anthropologist. They are reaching out to primary care physicians – who also are in short supply in this country – as a permanent medical home for patients as they reach adulthood. Kutlar and Gibson are co-principal investigators on $7 million, five-year grant from the National Center on Minority Health and Health Disparities of the National Institutes of Health supporting this initiative as well as the search for new drugs and more.
MCG physicians follow about 1,500 adults and children with sickle cell disease.
Clinical Research Forum selects sickle cell project among 'Top 10' clinical research achievements of 2012http://www.news-medical.net/
Pioneering research led by Johns Hopkins scientists on the use of partially matched bone marrow transplants to wipe out sickle cell disease has been selected as one of the Top 10 Clinical Research Achievements of 2012 by the Clinical Research Forum. The success of a preliminary clinical trial of the so-called haploidentical transplants has the potential to bring curative transplants to a majority of sickle cell patients who need them, eliminating painful and debilitating symptoms and the need for a lifetime of pain medications and blood transfusions.
On behalf of the research team, Robert A. Brodsky, M.D., the Johns Hopkins Family Professor of Medicine in Oncologyand director of the Division of Hematology at the Johns Hopkins University School of Medicine, will receive the award and an additional honor, the Distinguished Clinical Research Achievement Award, at a ceremony on April 18 during the Clinical Research Forum annual meeting in Washington, D.C.
New Web article Sickle Cell Disease Review
Advances in disease management and new treatment models help patients live longer. 1 contact hour of CEU for nurses:
http://nursing.advanceweb.com/
New Video - Strategies to Improve Implementation of Hydroxyurea
Dr. Courtney Thornburg, Duke Pediatric Sickle Cell Clinic
mms://realaudio.service.emory.
SAVE THE DATES
Fellow colleagues, you are invited to participate in the“Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, CDC
4th Thursday of every month from 2:00PM – 3:00PM EST
5/23: Baby on Board: What You Need to Know about Pregnancy in the Hemoglobinopathies
Dr. Sophie Lanzkron, Sickle Cell Center for Adults at Johns Hopkins Hospital
6/27: Beta-globin Haplotype Analysis in Children with Sickle Cell Anemia
Dr. Christopher Bean, National Center on Birth Defects and Developmental Disabilities, CDC
7/25: Nurses’ Impact on the Stigmatization of Individuals with Sickle Cell: Challenges and Recommendations
Dr. Coretta Jenerette, International Association of Sickle Cell Nurses and Physician Assistants
8/22: Mental Health and Learning Needs in children with Sickle Cell Disease
Dr. Patricia Kavanagh, Boston University School of Medicine/Boston Medical Center
9/26: NHLBI Sickle Cell Disease Guidelines
Dr. Joylene John-Sowah, National Heart Lung and Blood Institute, NIH
10/24: Current Data on Efficacy and Effectiveness of Hydroxyurea in Children with Sickle Cell Disease
Dr. Winfred Wang, St. Jude Children’s Research Hospital
November/December: --- No Webinars---
If you have ideas on topics for future webinars, or have any questions or comments about this series please submit them to Shae Pope spope@cdc.gov .
The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
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CDC Web based Sickle Cell Resources CDC’s YouTube Link: http://www.youtube.com/watch? CDC Video Archive (http://scinfo.org/world-wide- CDC Sickle Cell Disease Webpage: http://wwwdev.cdc.gov/NCBDDD/ Articles in the Medical Literature for April
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News for March 2013
New Treatment for Sickle Cell Brings Hope and a Cure to Chicago Area Patients
Source Newsroom: University of Illinois at Chicago
Newswise — Two brothers have been cured of their sickle cell disease at the University of Illinois Hospital & Health Sciences System using a relatively uncommon type of stem cell transplant that is performed without chemotherapy.
Their transplants were possible thanks to a third brother who was a match for both, against long odds.
Julius and Desmond Means never imagined life without sickle cell. The brothers, ages 25 and 19, have spent their lives in and out of hospitals, each suffering from different complications of the disease.
Growing up, they tired easily and couldn't keep up with their friends. As they grew older, the disease caused bone damage and affected Julius's lungs. Desmond's organs were also being damaged, and he was jaundiced.
For either young man to receive a transplant, a healthy sibling who is a compatible donor was needed. An acceptable match requires that at least eight of 10 known human leukocyte antigen (H.L.A.) genes be identical between donor and recipient.
Julius and Desmond's healthy older brother Clifford, 27, matched 10 of 10 H.L.A. genes with both of them -- an occurrence of "extremely low" likelihood, according to Dr. Damiano Rondelli, director of stem-cell transplantation at UI Health. The men's mother, Beverly Means, also noted the good fortune.
"I had won the lottery of health," she said.
In preparation for the transplant, Clifford was given medication to increase the number of stems cells released from the bone marrow into the bloodstream. His blood was then processed through a machine that collects white cells, including stem cells. The stem cells were frozen until the transplants.
Last May, Julius received his transplant at UI Hospital. He was given medication to suppress his immune system and one small dose of total body radiation right before the transplant. Then the frozen bags of stem cells were thawed and hung by IV pole for infusion into him.
Then in September, Desmond received his stem cell transplant.
Now several months since the transplants, both Julius and Desmond no longer have sickle cell disease. Their bone marrow is producing healthy red blood cells.
"Being cured, I feel like we can do anything," says Julius, who composed a rap about his transplant while recovering in the hospital. The brothers are pursuing their interests in rap music and dance and plan to attend college.
The chemotherapy-free stem cell transplant is a new procedure and is much better-tolerated by patients with aggressive sickle cell disease, who often have underlying organ damage and other complications.
UI Health is the first center in the Chicago area to offer this treatment, and one other patient has been successfully transplanted here. The efficacy and safety of the pre-transplant medication regimen are currently being studied at UI Health.
About 30 adults have received chemotherapy-free stem cell transplants for sickle cell disease at the National Institutes of Health in Bethesda, Md. Approximately 85 percent have been cured.
NSIGHT: Cyclists for sickle cell unite in Dar es Salaam Tanzania
http://thecitizen.co.tz/
In one of the biggest cycling charity events the city has yet seen, around 400 people, of an astonishing range of ages and physical abilities, donned orange reflector vests emblazoned with ‘Cycle for Sickle Cell’, to join the event to raise funds for a new Sickle Cell Centre at Muhimbili National Hospital, (MNH) and awareness into a disease that is one of the biggest causes of childhood mortality in Tanzania.
Sickle cell disease is an inherited blood disorder that causes significant illness and death and Tanzania’s burden of the disease ranks fourth in the world, with the highest number of SCD births a year (up to 11,000), after Nigeria, India and Democratic Republic of Congo. If untreated, up to 90 per cent of these children will die in childhood. One of the main challenges is that awareness of this inherited disease is considerably low in Tanzania. Many children go undiagnosed and lives are lost. Research scientists, doctors and other healthcare workers are working hard, hand in hand, to change this.
The Government of Tanzania has recognized the public health burden of sickle cell disease and dedicated sickle cell services have been provided at Muhimbili National Hospital since the 1980s.
In 2004, a systematic framework for comprehensive healthcare was established at Muhimbili (http://www.muhas.ac.tz and http://www.mnh.or.tz/), which is the main clinical, academic and research centre in the country.
In 2009, the ministry of Health and Social Welfare included sickle cell disease as a priority condition in the national strategy for non-communicable diseases and Tanzania intends to introduce new-born screening and strengthen sickle cell services in health facilities at primary, secondary and tertiary level.
In order to develop a platform for advocacy, the Sickle Cell Foundation of Tanzania was launched in 2010. Tanzania has established a biomedical research programme in SCD to find interventions that are locally appropriate and have global significance.
With local and global partnerships, it has developed a systematic framework for comprehensive research with prospective surveillance of over 2,500 SCD patients.
Tech update – Novartis develops two Smartphone apps for Android and iPhone
The Sickle Cell Disease Resource Locator uses your location to connect you with health resources relevant to sickle cell disease and a Sickle Cell Disease Tracker to help with iron overload.
New Video 2/28: Disparities in Sickle Cell Disease Care: Disentangling the Roles of Race, Place, and Disease State Dr. Carlton Haywood Jr., Johns Hopkins University
mms://realaudio.service.emory.
SAVE THE DATES
Fellow colleagues, you are invited to participate in the“Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, CDC
4th Thursday of every month from 2:00PM – 3:00PM EST
3/28: Strategies to Improve Implementation of Hydroxyurea
Dr. Courtney Thornburg, Duke Pediatric Sickle Cell Clinic
4/25: Building Behavioral and Social Science Databases for the Hemoglobinopathies: Lessons from the Study of Thalassemia
Dr. Robert Yamashita, California State University San Marcos
5/23: Baby on Board: What You Need to Know about Pregnancy in the Hemoglobinopathies
Dr. Sophie Lanzkron, Sickle Cell Center for Adults at Johns Hopkins Hospital
6/27: Beta-globin Haplotype Analysis in Children with Sickle Cell Anemia
Dr. Christopher Bean, National Center on Birth Defects and Developmental Disabilities, CDC
7/25: Nurses’ Impact on the Stigmatization of Individuals with Sickle Cell: Challenges and Recommendations
Dr. Coretta Jenerette, International Association of Sickle Cell Nurses and Physician Assistants
8/22: Mental Health and Learning Needs in children with Sickle Cell Disease
Dr. Patricia Kavanagh, Boston University School of Medicine/Boston Medical Center
9/26: NHLBI Sickle Cell Disease Guidelines
Dr. Joylene John-Sowah, National Heart Lung and Blood Institute, NIH
10/24: Current Data on Efficacy and Effectiveness of Hydroxyurea in Children with Sickle Cell Disease
Dr. Winfred Wang, St. Jude Children’s Research Hospital
November/December: --- No Webinars---
If you have ideas on topics for future webinars, or have any questions or comments about this series please submit them to Shae Pope spope@cdc.gov .
The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
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CDC Web based Sickle Cell Resources CDC’s YouTube Link: http://www.youtube.com/watch? CDC Video Archive (http://scinfo.org/world-wide- CDC Sickle Cell Disease Webpage: http://wwwdev.cdc.gov/NCBDDD/ Articles in the Medical Literature for March
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Sickle Cell News for February 2013
Could an old antidepressant treat sickle cell disease?
Tests in mice & human blood cells look promisinghttp://www.eurekalert.org/pub_
An antidepressant drug used since the 1960s may also hold promise for treating sickle cell disease, according to a surprising new finding made in mice and human red blood cells by a team from the University of Michigan Medical School.
The discovery that tranylcypromine, or TCP, can essentially reverse the effects of sickle cell disease was made by U-M scientists who have spent more than three decades studying the basic biology of the condition, with funding from the National Institutes of Health.
Their findings, published in Nature Medicine, pave the way for a clinical trial now being planned for adult patients who have the life-threatening condition. The discovery may also lead to other treatments for the disease, which leads misshapen red blood cells to cause vascular damage and premature death.
But the researchers caution it is too soon for the drug to be used in routine treatment of sickle cell anemia, an inherited genetic disease that affects tens of thousands of Americans and millions of others worldwide.
The climax of a decade of discovery
In the new paper, the researchers describe a painstaking effort to test TCP's effect on the body's production of a particular form of hemoglobin – the key protein that allows red blood cells to carry oxygen.
The drug acts on a molecule inside red blood cells called LSD1, which is involved in blocking the production of the fetal form of hemoglobin. The U-M team zeroed in on the importance of LSD1 as a drug target after many years of research.
Then, they literally did a Google search to find drugs that act on LSD1. That's how they found TCP, which since 1960 has been used to treat severe depression.
In the new paper, they describe how TCP blocked LSD1 and boosted the production of fetal hemoglobin -- offsetting the devastating impact of the abnormal "adult" form of hemoglobin that sickle cell patients make.
"This is the first time that fetal hemoglobin synthesis was re-activated both in human blood cells and in mice to such a high level using a drug, and it demonstrates that once you understand the basic biological mechanism underlying a disease, you can develop drugs to treat it," says Doug Engel, Ph.D., senior author of the study and chair of U-M's Department of Cell & Developmental Biology. "This grew out of an effort to discover the details of how hemoglobin is made during development, not with an immediate focus on curing sickle cell anemia, but just toward understanding it."
Engel credits the dedication and persistence of his team, including a former research assistant professor, Osamu Tanabe, M.D., Ph.D., now at Japan's Tohoku University, U-M postdoctoral fellow, Lihong Shi, Ph.D., first author of the study, and research instructor Shuaiying Cui, Ph.D..
Together, they have identified LSD1's crucial role, and its epigenetic interaction with two nuclear receptors in the nuclei of red blood cell precursors called TR2 and TR4. Working in tandem, they repress the expression of the gene that makes fetal hemoglobin – an effect called gene silencing. So, interfering with this repression allows the fetal hemoglobin subunits to be made.
Treatment with TCP caused fetal hemoglobin to be produced at such high abundance that it made up 30 percent of all hemoglobin in cultured human blood cells – a finding Engel called "startling." TCP is FDA-approved, though patients taking it need to follow strict dietary guidelines to avoid drug interactions with certain naturally occurring chemicals in some foods.
Reference: Nature Medicine, http://dx.doi.org/
NFL Twins to Tackle Sickle Cell Diseasehttp://newbrunswick.patch.com/
NFL defensive backs and twin brothers Jason McCourty of the Tennessee Titans and Devin McCourty of the New England Patriots will visit Robert Wood Johnson University Hospital on Saturday, Feb. 23 for a campaign against sickle cell disease.
The McCourtys, along with the Embrace Kids Foundation, Robert Wood Johnson University Hospital, and the New York Blood Center are working together on the “Tackle Sickle Cell” campaign, which intends to educate and raise monetary and blood donors to fight against the disease.
Anyone who registers through TackleSickleCell.org a
For more information, visit TackleSickleCell.org or
Treatment of sickle cell disease largely involves long-term disease management.http://myfox8.com/2013/02/22/
Proper lifestyle modifications are essential to avoiding sickle cell disease-related symptomatic episodes and health conditions, which involve maintaining a well-balanced diet, avoiding smoking and alcohol consumption, limiting caffeine intake, and staying hydrated. Long-term sickle cell disease management also involves proper dental, foot, eye and skin/wound care.
Sickle news from Tanzania. Sickle Cell Disease: What can Africa contribute? 13 February 2013. http://videocast.nih.gov/
Improving Sickle Cell Disease Care Beyond the Clinic
From text reminders to self-monitoring pill bottles, healthcare providers in the Working to Improve Sickle Cell Healthcare (WISCH) project using technology to improve care for patients with sickle cell disease when they leave the doctor’s office.
http://www.nichq.org/
Books
Telfair, J. and Crosby, L. (2013) Disparities in the delivery of health care and pain management for persons with sickle cell disease. In Incayawar, M and Todd, K (EDs) CULTURE, BRAIN AND ANALGESIA: Understanding and Managing Pain in Diverse Populations New York, NY: Oxford University Press (Chapter 17), 198-204.
Sickle Cell Art by Hertz Nazaire
World famous sickle cell artist Hertz Nazaire is selling his art to raise sickle cell awareness. Much of his work goes unpaid, but he needs support also:
“I don't mind not getting paid for my work as long as it helps others understand our pain but I don't think the community knows how hard my life has been while these images have been on slides and spread out all over the world.”
“I only sold 20 items in 6 years online mostly the postage stamps that canvas is new, just created this week. I turn 40 years old this year my pain is still very annoying but I am glad that my art has been loved by so many. Thank You for your support”
His latest work, avery large wrapped canvas print ready to hang for an office is available at: http://www.zazzle.com/need_
Hertz online store address is http://www.zazzle.com/
Happy birthday Hertz and many more
Video Resources
# 505 LIVING WITH ILLNESS ( with Discussion Guide)
In the Mix is the Emmy award winning PBS series for young adults. One program addresses sickle cell anemia and is available as an educational DVD. A special discount of $20 off the usual $70 can be received by using the code 20Off and listing that on the P.O. For more information and a transcript, visit www.inthemix.org
This program addresses the most critical issues and problems concerning school, friends and family that challenge young people who are coping with serious and/or chronic conditions. Teens speak frankly from their experiences, sharing their concerns and advice with insight and humor. A boy describes his ways of dealing with Crohn’s; a girl copes with Juvenile Diabetes; and several teens who are living with sickle cell anemia describe their conditions and dispel misconceptions. They all stress that they want to be treated as normal teenagers.Young Adult Library Services Association “Selected Videos” list. Winner of the CINE Golden Eagle Award
“The well-spoken adolescents, who represent a variety of backgrounds, openly share their experiences and discuss the impact of their afflictions on their lives. Including information on treatments and side effects, this video takes an honest and insightful look at a topic not often discussed among teens.” – Booklist
New Video Posted Managing and Monitoring Transfusional Iron Overload by Dr. Thomas Coates on 1/25/13
mms://realaudio.service.emory.
SAVE THE DATES
Fellow colleagues, you are invited to participate in the“Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, CDC
4th Thursday of every month from 2:00PM – 3:00PM EST
2/28: Disparities in Sickle Cell Disease Care: Disentangling the Roles of Race, Place, and Disease State
Dr. Carlton Haywood Jr., Johns Hopkins University
3/28: Strategies to Improve Implementation of Hydroxyurea
Dr. Courtney Thornburg, Duke Pediatric Sickle Cell Clinic
4/25: Building Behavioral and Social Science Databases for the Hemoglobinopathies: Lessons from the Study of Thalassemia
Dr. Robert Yamashita, California State University San Marcos
5/23: Baby on Board: What You Need to Know about Pregnancy in the Hemoglobinopathies
Dr. Sophie Lanzkron, Sickle Cell Center for Adults at Johns Hopkins Hospital
6/27: Beta-globin Haplotype Analysis in Children with Sickle Cell Anemia
Dr. Christopher Bean, National Center on Birth Defects and Developmental Disabilities, CDC
7/25: Nurses’ Impact on the Stigmatization of Individuals with Sickle Cell: Challenges and Recommendations
Dr. Coretta Jenerette, International Association of Sickle Cell Nurses and Physician Assistants
8/22: Mental Health and Learning Needs in children with Sickle Cell Disease
Dr. Patricia Kavanagh, Boston University School of Medicine/Boston Medical Center
9/26: NHLBI Sickle Cell Disease Guidelines
Dr. Joylene John-Sowah, National Heart Lung and Blood Institute, NIH
10/24: Current Data on Efficacy and Effectiveness of Hydroxyurea in Children with Sickle Cell Disease
Dr. Winfred Wang, St. Jude Children’s Research Hospital
November/December: --- No Webinars---
If you have ideas on topics for future webinars, or have any questions or comments about this series please submit them to Shae Pope spope@cdc.gov .
The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
|
|
CDC Web based Sickle Cell Resources CDC’s YouTube Link: http://www.youtube.com/watch? CDC Video Archive (http://scinfo.org/world-wide- CDC Sickle Cell Disease Webpage:http://wwwdev.cdc.gov/NCBDDD/ Articles in the Medical Literature for January
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Lab Chip. 2013 Feb 22. [Epub ahead of print] A simple, rapid, low-cost diagnostic test for sickle cell disease. Yang X, Kanter J, Piety NZ, Benton MS, Vignes SM, Shevkoplyas SS. Department of Biomedical Engineering, Tulane University, New Orleans, LA 70118. shevkop@tulane.edu. Abstract This communication describes a very simple, rapid and inexpensive point-of-care diagnostic test for sickle cell disease (SCD) that can conclusively differentiate between blood samples from normal healthy individuals, sickle cell trait carriers and SCD patients using the characteristic blood stain patterns produced by each sample on paper. |
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PMID: 23429713 [PubMed - as supplied by publisher] |
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Pediatr Neurol. 2013 Mar;48(3):188-99. doi: 10.1016/j.pediatrneurol.2012. Cerebral blood flow abnormalities in children with sickle cell disease: a systematic review. Behpour AM, Shah PS, Mikulis DJ, Kassner A. Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada. Abstract A systematic review was performed to assess whether cerebral blood flow with different imaging modalities could identify brain abnormalities in children with sickle cell disease where structural magnetic resonance imaging and transcranial Doppler velocity appeared normal. A total of 11 studies were identified which reported cerebral blood flow abnormalities alongside structural magnetic resonance imaging or transcranial Doppler velocity abnormalities in patients with sickle cell disease. Potential for bias was assessed with the quality assessment of diagnostic accuracy studies scale in addition to treatment bias. Subjects of each study were categorized into patients with and without stroke. The prevalence of abnormalities for each modality was then separately calculated in each group. The included studies had mostly moderate degrees of bias. The prevalence of blood flow abnormalities compared with structural magnetic resonance imaging abnormalities was equal to or lower in patients with stroke and equal to or greater in patients without stroke. Blood flow abnormalities were more prevalent than transcranial Doppler abnormalities in four studies of patients without stroke and in one study of patients with stroke. The studies suggest that the assessment of cerebral blood flow in sickle cell disease can be of potential value in addressing brain abnormalities at the tissue level; however, further studies are warranted. Copyright © 2013 Elsevier Inc. All rights reserved. |
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PMID: 23419469 [PubMed - in process] |
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Pediatr Blood Cancer. 2013 Feb 15. doi: 10.1002/pbc.24486. [Epub ahead of print] Lebensburger JD, Sidonio RF, Debaun MR, Safford MM, Howard TH, Scarinci IC. Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama. jlebensburger@peds.uab.edu. Abstract BACKGROUND: Several sickle cell clinical trials have closed due to inability to enroll patients. To limit the early cessation of a proposed clinical trial due to low accrual rates, we sought to better understand barriers and facilitators to enrolling parents of children with sickle cell anemia (SCD) into clinical trials. PROCEDURE: Focus groups (n = 3) were conducted with parents/guardians (n = 14) who had not previously been recruited for a clinical trial and were not administering hydroxyurea to their children. RESULTS: Three main themes related to barriers to clinical trial enrollment were identified during analysis of focus groups: general barriers to health related research (general mistrust of research studies, emotional and practical concerns), barriers to trial design (randomization), and barriers to hydroxyurea (long term unknown risks, cancer, myelosuppressive effects). Facilitators identified were need for more education, including request for peer education, and improved explanation of clinical trials or study rationale. CONCLUSION: Engagement of parents/guardians of children with SCD in identifying barriers and facilitators to clinical trial enrollment may be critical to the development of strategies to enhance SCD trial completion. Pediatr Blood Cancer © 2013 Wiley Periodicals, Inc. Copyright © 2013 Wiley Periodicals, Inc. |
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PMID: 23418000 [PubMed - as supplied by publisher] |
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Biol Blood Marrow Transplant. 2013 Feb 14. pii: S1083-8791(13)00082-7. doi: 10.1016/j.bbmt.2013.02.010. [Epub ahead of print] Dallas M, Triplett B, Shook D, Hartford C, Srinivasan A, Laver J,Ware R, Leung W. Departmentof BoneMarrowTransplantationand CellularTherapy,St. JudeChildren's Research Hospital; Departmentof Pediatrics,Universityof TennesseeHealthScienceCenter, College of Medicine, Memphis, Tennessee. Electronic address: mari.dallas@stjude.org. Abstract Human leukocyte antigen (HLA) matched related donor (MRD) hematopoietic stem cell transplant (HSCT) for patients with sickle cell disease (SCD) has been well established, however experience using alternative donors, including haploidentical donors for treatment of SCD is limited. We report the long-term outcome of 22 pediatric patients who underwent a related donor HSCT for SCD at St. Jude Children's Research Hospital. Patients received a myeloablative MRD from a sibling (14) or reduced intensity parental haploidentical (8) HSCT. The medianageforthe patients who underwent a MRD and haploidentical donor HSCT were11.0±3.9 yrs. and9.0±5.0 yrs., respectively. The median time to follow up for the MRD cohort was 9.0 ± 2.3 yrs. withan overallsurvival (OS) of93%andrecurrence/ Copyright © 2013 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved. |
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PMID: 23416852 [PubMed - as supplied by publisher] |
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J Hematol Oncol. 2013 Feb 17;6(1):17. [Epub ahead of print] Wun T, Soulieres D, Frelinger AL, Krishnamurti L, Novelli EM,Kutlar A, Ataga KI, Knupp CL, McMahon LE, Strouse JJ, Zhou C,Heath LE, Nwachuku CE, Jakubowski JA, Riesmeyer JS, Winters KJ. Abstract ABSTRACT: BACKGROUND: Platelet activation has been implicated in the pathogenesis of sickle cell disease (SCD) suggesting antiplatelet agents may be therapeutic. To evaluate the safety of prasugrel, a thienopyridine antiplatelet agent, in adult patients with SCD, we conducted a double-blind, randomized, placebo-controlled study. METHODS: The primary endpoint, safety, was measured by hemorrhagic events requiring medical intervention. Patients were randomized to prasugrel 5 mg daily (n = 41) or placebo (n = 21) for 30 days. Platelet function by VerifyNow(R) P2Y12 and vasodilator-stimulated phosphoprotein assays at days 10 and 30 were significantly inhibited in prasugrel- compared with placebo-treated SCD patients. RESULTS: There were no hemorrhagic events requiring medical intervention in either study arm. Mean pain rate (percentage of days with pain) and intensity in the prasugrel arm were decreased compared with placebo. However, these decreases did not reach statistical significance. Platelet surface P-selectin and plasma soluble P-selectin, biomarkers of in vivo platelet activation, were significantly reduced in SCD patients receiving prasugrel compared with placebo. In sum, prasugrel was well tolerated and not associated with serious hemorrhagic events. CONCLUSIONS: Despite the small size and short duration of this study, there was a decrease in platelet activation biomarkers and a trend toward decreased pain. Free Article |
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PMID: 23414938 [PubMed - as supplied by publisher] |
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J Health Psychol. 2013 Feb 13. [Epub ahead of print] Psychological aspects and hospitalization for pain crises. Tsao J, Jacob E, Seidman L, Lewis MA, Zeltzer L. University of California, Los Angeles, USA. Abstract Sickle-cell disease is a genetic disorder characterized by severe pain episodes or "vaso-occlusive crises" that may require hospitalization. This study examined the associations among emotion regulation, somatization, positive and negative affect, and hospitalizations for pain crises in youth with sickle-cell disease. Multivariate analyses indicated that emotional suppression and somatization were significantly associated with more frequent hospitalizations for pain crises in the previous year after controlling for sickle-cell disease type and pain. These results suggest that efforts to reduce emotional suppression and somatization may assist in decreasing the frequency of hospitalizations for pain crises among youth with sickle-cell disease. |
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PMID: 23407129 [PubMed - as supplied by publisher] |
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Am J Emerg Med. 2013 Feb 1. pii: S0735-6757(12)00568-2. doi: 10.1016/j.ajem.2012.11.005. [Epub ahead of print] The impact of race and disease on sickle cell patient wait times in the emergency department. Haywood C Jr, Tanabe P, Naik R, Beach MC, Lanzkron S. Department of Medicine, Division of Hematology, The Johns Hopkins School of Medicine, Baltimore, MD 21205, USA; The Johns Hopkins Berman Institute of Bioethics, Baltimore, MD 21205, USA. Electronic address: chaywoodjr@jhu.edu. Abstract STUDY OBJECTIVE: To determine whether patients with sickle cell disease (SCD) experience longer wait times to see a physician after arrival to an emergency department (ED) compared to patients with long bone fracture and patients presenting with all other possible conditions (General Patient Sample), and to attempt to disentangle the effects of race and disease status on any observed differences. METHODS: A cross-sectional, comparative analysis of year 2003 through 2008 data from the National Hospital Ambulatory Medical Care Survey, a nationally representative sample of nonfederal emergency department visits in the United States. Our primary outcome was wait time (in minutes) to see a physician after arrival to an ED. A generalized linear model was used to examine ratios of wait times comparing SCD visits to the two comparison groups. RESULTS: SCD patients experienced wait times 25% longer than the General Patient Sample, though this difference was explained by the African-American race of the SCD patients. SCD patients waited 50% longer than did patients with long bone fracture even after accounting for race and assigned triage priority. CONCLUSIONS: Patients with SCD presenting to an ED for care experience longer wait times than other groups, even after accounting for assigned triage level. The African-American race of the SCD patients, and their status as having SCD itself, both appear to contribute to longer wait times for these pati |
Sickle Cell News for January 2013
Sickle Cell Test Gets NCAA OK Despite Docs
The National Collegiate Athletic Association (NCAA) has approved mandatory confirmation of sickle cell trait status in Division III student athletes, despite the objections of the American Society of Hematology (ASH).
NCAA delegates voted 254 to 200 in favor of the measure at the 2013 NCAA convention over the weekend. Confirmation of sickle cell status will be required of all incoming student athletes in the 2013-2014 school year and for all athletes by 2014-2015. Mandatory sickle cell screening is already required by the NCAA in Division I and Division II athletes.
Last year, ASH challenged the NCAA, declaring that athletes need not be tested for or disclose sickle cell trait status before participating in sporting events. In a statement released over the weekend, ASH said the "NCAA policy is medically groundless – perhaps even dangerous – and is focused more on protecting the NCAA from legal liability than protecting the health of student athletes." http://www.medpagetoday.com/
Fighting Painful Misconceptions About Sickle Cell Disease In The ER
When sickle cell patients arrive at emergency rooms, they often have great difficulty getting the treatment they need. Paula Tanabe, an associate professor at the Duke University School of Nursing, is making it her mission to change that. http://www.kaiserhealthnews.
Bahrain to Host International Conference on Sickle Cell Disease, Management and Prevention http://www.bna.bh/portal/en/
Bahrain is hoping to benefit from international experience when it hosts a global conference about sickle cell disease. Hundreds of patients and doctors are expected to attend the event, taking place at the Ritz-Carlton Bahrain, Hotel and Spa from February 5 to 7. The International Conference on Sickle Cell Disease, Management and Prevention is being organised by the Health Ministry and held under the patronage of His Royal Highness Prime Minister Prince Khalifa bin Salman Al Khalifa.
"Prominent speakers from around the world, the Middle East and GCC as well as from Bahrain have already confirmed their participation," said National Committee to Combat Genetic Diseases and students screening programme head Dr Shaikha Al Arrayed.
"The conference will also provide an interactive forum for participants to discuss important and emerging health issues," she said. "The event will be an opportunity to strengthen communication and networking and to share best practices and improve the health of blood disorder populations."This is the time to unite to fight these diseases and protect the new generation." Dr Al Arrayed said emerging health issues, protocols for pain management, prevention and treatment of opiate addiction, avoidance of causes of death in patients and avoidance of complications such as acute chest syndrome, stroke, renal failure and vascular necrosis would be discussed at the event. Participants will also learn about alternative forms of treatment.
Sickle Cells Show Potential to Attack Aggressive Cancer Tumors
Reporting in the Jan. 9, 2013, edition of the on-line journal PLOS ONE, the researchers describe a process of exploiting sickle-shaped red blood cells to selectively target oxygen deprived cancer tumors in mice and block the blood vessels that surround them.
"Sickle cells appear to be a potent way to attack hypoxic (oxygen-starved) solid tumors, which are notable for their resistance to existing cancer chemotherapy agents and radiation," said senior author Mark W. Dewhirst, DVM, PhD, a radiation oncologist and director of Duke's Tumor Microcirculation Laboratory. "This is an exciting finding that suggests a potential new approach to fighting tumors that are currently associated with aggressive disease." http://www.sciencedaily.com/
Greensboro's Cone Health To Open Sickle Cell Center
The center will be the second round-the-clock sickle cell center in the southeast.http://www.digtriad.com/video/
5-year-old twin rappers battling sickle cell anemia
Marcus and Marlon Davis are identical 5-year-old twins who could be the next big rappers to hail from Houston. But first, they'll have to overcome an obstacle bigger than those typically faced by singers seeking fame in the rap game. Their group -- The Official Two Times Two -- is out with their new single called, "I'ma Act Bad."The twins were four when they started rapping, but they're moving up quickly.
Marcus and Marlon recently had their first live performance at a small downtown venue. "It was fantastic. I thought they were going to be nervous but they weren't," said their mother, Felicia Pollard. Their mother is nervous for another reason.The twins have a severe case of sickle cell anemia.
"They've been hospitalized 36 times, both of them," Pollard said. "Marlon, we almost lost him before Thanksgiving,” said their aunt and manager, Linda Marshall. Doctors have told the family that the twins may not live to see the age of 21. Sickle cell causes the boys' red blood cells to collapse into a crescent shape. This can lead to organ damage. It always leads to excruciating pain. In between hospital stays, Marcus and Marlon enjoy sessions in the studio. "It keeps 'em focused, you know, keeps their mind off their pain," Marshall said. Their next goal is to appear on "The Ellen DeGeneres Show." http://www.khou.com/news/
Video Resources
SAVE THE DATES
Fellow colleagues, you are invited to participate in the“Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, CDC
4th Thursday of every month from 2:00PM – 3:00PM EST
2013 Dates: 1/24, 2/28, 3/28, 4/25, 5/23, 6/27, 7/25, 8/22, 9/26, 10/24
The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
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CDC Web based Sickle Cell Resources CDC’s YouTube Link: http://www.youtube.com/watch? CDC Video Archive (http://scinfo.org/world-wide- CDC Sickle Cell Disease Webpage: http://wwwdev.cdc.gov/NCBDDD/ Articles in the Medical Literature for January
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Sickle Cell News for December 2012
Q&A with Dr. Kwaku Ohene-Frempong about the importance of sickle cell screening and disparities between the US and abroad To mark the 50th anniversary of newborn screening, we did an interview with Dr. Kwaku Ohene-Frempong about screening for SCD and trait. Dr. Ohene-Frempong is the Director Emeritus of the Comprehensive Sickle Cell Center at Children’s Hospital of Philadelphia, President of the Sickle Cell Foundation of Ghana, and a NICHQ faculty member. Link http://www.nichq.org/resources/Sickle-Cell-Frempong-QA-Dec2012.html
Into Adulthood, Sickle Cell Patients Rely on ER
Patients with sickle cell disease rely more on the emergency room as they move from pediatric to adult health care, according to researchers at Washington University School of Medicine in St. Louis. Link http://www.infozine.com/news/stories/op/storiesView/sid/54200/
Sickle cell patient refuses to let disease define her
Marquita Gaines is a college student living with sickle cell disease. She was diagnosed at birth and first presented symptoms at a young age. She currently receives regular blood cell transfusions administered by registered nurses from the American Red Cross to treat and prevent complications from the disease Link http://www.cnn.com/2012/12/13/us/iyw-blood-donor-gaines/
Ask The Experts
Q: What is the best diet to give a 8 year old with sickle cell disease (Hb SS)
A: Thank you for your question. Diet in sickle cell disease is the subject of active research, but the studies are far from complete or conclusive. Researchers have not tried to demonstrate a what a "Sickle Cell Diet" should be. Here are some bits of information:
(1) Sickle hemoglobin and breakdown of sickle red blood cells place the body under high oxidant stress, so that taking anti-oxidants supplements seem like a good idea.
(2) Blood chemistries in people with sickle cell are slightly abnormal in multiple different ways. Supplementing for the deficiencies seem like a good idea.
(3) Studies have been designed to examine single-ingredient supplements such as vitamins ( folic acid, tetrahydrobiopterin, vitamin D, vitamin C, vitamin E), minerals ( zinc, magnesium), amino acids ( glutamine, arginine, citrulline), anti-oxidants, nitrite, nitrate, or fish oil. I don't remember seeing studies designed as head-to-head comparions to show what ingredient(s) are the most important.
(4) We do think that extra iron supplements are not necessary for sickle cell disease, unless somebody has lost a lot of blood or has demonstrated unusual barriers to iron absorption.
(5) Probably the only advice we can give is to eat a generally balanced diet as recommended for most American families in the general population: plenty of fruits & vegetables, balanced protein intake (meats, dairy, nuts & beans), drink plenty of fluids, eat dietary fiber, and avoid excessive amounts of sugary or fried snacks. This nutritional advice is based on dietary research on reduce the risks of diabetes, heart attacks, stroke, and some cancers.
Sincerely,
Lewis Hsu, MD
Pediatric Hematologist
Video Resources
SAVE THE DATESFellow colleagues, you are invited to participate in the“Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, CDC
4th Thursday of every month from 2:00PM – 3:00PM EST2013 Dates: 1/24, 2/28, 3/28, 4/25, 5/23, 6/27, 7/25, 8/22, 9/26, 10/24
The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
January’s webinar will take place on Thursday, January 24th from 2:00pm – 3:00pm EST, featuring
Dr. Thomas Coates’ presentation on “Monitoring and Management of Transfusional Iron Overload”If you have ideas on topics for future webinars, or have any questions or comments about this series please submit them to Shae Pope spope@cdc.gov.
CDC would appreciate your feedback on this year’s “Public Health Webinar Series on Hemoglobinopathies” hosted by the Division of Blood Disorders, CDCby completing a brief online survey: http://www.surveymonkey.com/s/M986NWM Please forward the survey link to colleagues who participated on this webinar series as a group.Your feedback is valuable and will help us improve the series for 2013.
Thank you for your participation!
CDC Web based Sickle Cell Resources
CDC’s YouTube Link: http://www.youtube.com/watch?v=gnrvYsZWR1c&list=UUiMg06DjcUk5FRiM3g5sqoQ&index=2&feature=plpp_video
CDC Video Archive (you can get the video code from this site and embed the video on another webpage, or download it): http://www.cdc.gov/NCBDDD/video/SickleCell/index.html CDC Sickle Cell Disease Webpage: http://wwwdev.cdc.gov/NCBDDD/sicklecell/video.html Articles in the Medical Literature for November
1. Blood. 2012 Dec 20. [Epub ahead of print]
Hemolysis and free hemoglobin revisited: exploring hemoglobin and hemin scavengers as a novel class of therapeutic proteins.
Schaer DJ, Buehler PW, Alayash AI, Belcher JD, Vercellotti GM.
Division of Internal Medicine, University Hospital, Zurich, Switzerland;
Abstract
Hemolysis occurs in many hematologic and non-hematologic diseases. Extracellular hemoglobin (Hb) has been recognized to trigger specific pathophysiologies that are associated with ad
verse clinical outcomes in patients with hemolysis, such as acute and chronic vascular disease, inflammation, thrombosis and renal impairment. Among the molecular characteristics of extracellular Hb, translocation of the molecule into the extravascular space, oxidative and nitric oxide reactions, hemin release and molecular signaling effects of hemin appear to be the most critical. Limited clinical experience with a plasma-derived haptoglobin product in Japan and more recent preclinical animal studies suggest that the natural Hb and hemin scavenger proteins haptoglobin (Hp) and hemopexin (Hpx) have a strong potential to neutralize the adverse physiologic effects of Hb and hemin. This includes conditions that are as diverse as red blood cell transfusion, sickle cell disease, sepsis and extracorporeal circulation. This perspective reviews the principal mechanisms of Hb and hemin toxicity in different disease states, updates how the natural scavengers efficiently control these toxic moieties, and explores critical issues in the development of human plasma-derived Hp and Hpx as therapeutics for patients with excessive intravascular hemolysis.
PMID: 23264591 [PubMed - as supplied by publisher]
2. Transfus Apher Sci. 2012 Dec 17. pii: S1473-0502(12)00234-0. doi: 10.1016/j.transci.2012.09.002. [Epub ahead of print]
Long-term red blood cell exchange in children with sickle cell disease: Manual or automatic?
Duclos C, Merlin E, Paillard C, Thuret I, Demeocq F, Michel G, Kanold J.
CHU Bordeaux Hôpital Haut-Lévêque, Service hématologie, 33600 Pessac, France. Electronic address: cedric.duclos@chu-bordeaux.fr.
Abstract
Little information is available on erythrocytapheresis in children with sickle cell disease, and no comparison has ever been made with manual exchanges in a long-term blood exchange program. We matched a historical cohort of five patients who received 60 erythrocytapheresis procedures with five who received 124 manual exchanges. Long-term erythrocytapheresis was feasible and well-tolerated even in children of low weight. In a long-term approach, automated exchanges were more efficient in maintaining a low HbS level, and exchanges could be spaced out. This approach appears especially useful in the cases where the HbS level must be maintained below 30%.
Copyright © 2012 Elsevier Ltd. All rights reserved.
PMID: 23257506 [PubMed - as supplied by publisher]
3. Am J Hematol. 2012 Nov 17. doi: 10.1002/ajh.23365. [Epub ahead of print]
Hydroxyurea treatment decreases glomerular hyperfiltration in children with sickle cell anemia.
Aygun B, Mortier NA, Smeltzer MP, Shulkin BL, Hankins JS, Ware RE.
Department of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee. baygun@nshs.edu.
Abstract
Glomerular hyperfiltration and microalbuminuria/proteinuria are early manifestations of sickle nephropathy. The effects of hydroxyurea therapy on these renal manifestations of sickle cell anemia (SCA) are not well defined. Our objective was to investigate the effects of hydroxyurea on glomerular filtration rate (GFR) measured by (99m) Tc-DTPA clearance, and on microalbuminuria/proteinuria in children with SCA. Hydroxyurea study of long-term effects (HUSTLE) is a prospective study (NCT00305175) with the goal of describing the long-term cellular, molecular, and clinical effects of hydroxyurea therapy in SCA. Glomerular filtration rate, urine microalbumin, and serum cystatin C were measured before initiating hydroxyurea therapy and then repeated after 3 years. Baseline and Year 3 values for HUSTLE subjects were compared using the Wilcoxon Signed Rank test. Associations between continuous variables were evaluated using Spearman correlation coefficient. Twenty-three children with SCA (median age 7.5 years, range, 2.5-14.0 years) received hydroxyurea at maximum tolerated dose (MTD, 24.4 ± 4.5 mg/kg/day, range, 15.3-30.6 mg/kg/day). After 3 years of treatment, GFR measured by (99m) Tc-DTPA decreased significantly from 167 ± 46 mL/min/1.73 m(2) to 145 ± 27 mL/min/1.73 m(2) (P = 0.016). This decrease in GFR was significantly associated with increase in fetal hemoglobin (P = 0.042) and decrease in lactate dehydrogenase levels (P = 0.035). Urine microalbumin and cystatin C levels did not change significantly. Hydroxyurea at MTD is associated with a decrease in hyperfiltration in young children with SCA.Am. J. Hematol., 2012. © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
PMID: 23255310 [PubMed - as supplied by publisher]
4. Pediatr Blood Cancer. 2012 Dec 19. doi: 10.1002/pbc.24413. [Epub ahead of print]
High rates of recurrent biliary tract obstruction in children with sickle cell disease.
Amoako MO, Casella JF, Strouse JJ.
Johns Hopkins University School of Medicine, Baltimore, Maryland.
Abstract
BACKGROUND:
Individuals with sickle cell disease (SCD) have an increased risk of cholelithiasis from bilirubin stones. Symptomatic biliary tract disease (BTD) includes acute and chronic cholecystitis, obstruction of the common bile duct (CBD), cholangitis, and gallstone pancreatitis. Cholecystectomy is the main treatment strategy for symptomatic patients; however, the prevalence of recurrent BTD following cholecystectomy has not been systematically evaluated. We conducted a retrospective cohort study to describe the recurrence of BTD after cholecystectomy and characterize risk factors for recurrent disease.
PROCEDURE:
We identified patients <22 years of age who presented to the Johns Hopkins Children Center with symptomatic BTD from July 1993 to June 2008.
RESULTS:
We identified 56 patients with a total of 76 episodes of symptomatic BTD (median age at first event 15.9, range 4.6-21.5 years). Eleven of the 56 patients (19.6%) had at least one episode of recurrent symptomatic BTD (median follow-up of 5.3 years). Baseline characteristics were similar between the patients with a single episode of BTD and those with recurrent BTD.
CONCLUSIONS:
These results demonstrate that recurrent BTD is a frequent complication of SCD (20% by age 4 years) and often presents as CBD obstruction by stone, despite cholecystectomy. In our cohort, recurrence was not associated with age at first episode, baseline total bilirubin, gender, or genotype of SCD. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
PMID: 23255346 [PubMed - as supplied by publisher]
5. Clin Transl Sci. 2012 Dec;5(6):437-44. doi: 10.1111/cts.12005. Epub 2012 Oct 17.
Effect of propranolol as antiadhesive therapy in sickle cell disease.
De Castro LM, Zennadi R, Jonassaint JC, Batchvarova M, Telen MJ.
Duke Comprehensive Sickle Cell Center, Division of Hematology, Department of Medicine, Duke University, Durham, North Carolina, USA.
Abstract
Sickle red blood cells (SSRBCs) adhere to both endothelial cells (ECs) and the extracellular matrix. Epinephrine elevates cyclic adenosine monophosphate in SSRBCs and increases adhesion of SSRBCs to ECs in a β-adrenergic receptor and protein kinase A-dependent manner. Studies in vitro as well as in vivo have suggested that adrenergic stimuli like epinephrine may contribute to vaso-occlusion associated with physiologic stress. We conducted both animal studies and a Phase I dose-escalation study in sickle cell disease (SCD) patients to investigate whether systemically administered propranolol inhibits SSRBC adhesion and to document the safety of propranolol in SCD. Systemically administered propranolol prevented SSRBC adhesion and associated vaso-occlusion in a mouse model. In patients receiving a single oral dose of 10, 20, or 40 mg propranolol, SSRBC adhesion to ECs was studied before and after propranolol, with and without stimulation with epinephrine. Propranolol administration significantly reduced epinephrine-stimulated SSRBC adhesion in a dose dependent manner (p = 0.03), with maximum inhibition achieved at 40 mg. Adverse events were not severe, did not show dose dependence, and were likely unrelated to drug. No significant heart rate changes occurred. These results imply that β-blockers may have a role as antiadhesive therapy for SCD. Clin Trans Sci 2012; Volume 5: 437-444.
© 2012 Wiley Periodicals, Inc.
PMID: 23253664 [PubMed - in process]
6. Platelets. 2012 Dec 18. [Epub ahead of print]
Circulating platelet and erythrocyte microparticles in young children and adolescents with sickle cell disease: Relation to cardiovascular complications.
Tantawy AA, Adly AA, Ismail EA, Habeeb NM, Farouk A.
Pediatrics Department, Faculty of Medicine, Ain Shams University , Cairo , Egypt.
Abstract
Sickle cell disease (SCD) is characterized by a complex vasculopathy, consisting of endothelial dysfunction and increased arterial stiffness, with a global effect on cardiovascular function. The hypercoagulable state may result from chronic hemolysis and circulating cell-derived microparticles (MPs) originating mainly from activated platelets and erythrocytes. We measured the levels of platelet and erythrocyte-derived MPs (PMPs and ErMPs) in 50 young SCD patients compared with 40 age- and sex-matched healthy controls and assessed their relation to clinicopathological characteristics and aortic elastic properties. Patients were studied stressing on the occurrence of sickling crisis, transfusion history, hydroxyurea therapy, hematological, and coagulation profile as well as flow cytometric expression of PMPs (CD41b(+)) and ErMPs (glycophorin A(+)). Echocardiography was performed to assess aortic stiffness and distensibility, left ventricular function and pulmonary artery pressure. Both PMPs and ErMPs were significantly elevated in SCD patients compared with control group (p < 0.001). SCD patients had significantly elevated D-dimer and von Willebrand factor antigen (vWF Ag) levels with lower antithrombin III compared with controls (p < 0.001). Aortic stiffness index and pulmonary artery pressure were significantly higher in SCD (p < 0.001), whereas aortic strain and aortic distensibility were significantly lower (p < 0.001) compared with controls. MPs levels were significantly increased in SCD patients with pulmonary hypertension, acute chest syndrome, and stroke as well as those who had history of thrombosis or splenectomy (p < 0.001). Also, patients in sickling crisis during the study had higher PMPs and ErMPs levels than those in steady state (p < 0.001). Patients on hydroxyurea therapy had lower MPs levels than untreated patients (p < 0.001). PMPs and ErMPs were positively correlated with disease duration, transfusion index, white blood cell count, HbS, markers of hemolysis, serum ferritin, D-dimer, and vWF Ag, whereas negatively correlated with hemoglobin and HbF levels (p < 0.05). Both PMPs and ErMPs levels were positively correlated with aortic stiffness, pulmonary artery pressure, and tricuspid regurgitant velocity (p < 0.05) while negatively correlated with aortic distensibility. We suggest that PMPs and ErMPs overproduction may be considered a potential biological marker for vascular dysfunction and disease severity in SCD and may be implicated in the pathogenesis of coagulation abnormalities encountered in those patients. Their levels are closely related to sickling crisis, pulmonary hypertension, markers of hemolysis, fibrinolysis, and iron overload. Therefore, quantification of MPs in SCD may provide utility for identifying patients who are at increased risk of thrombotic events or cardiovascular abnormalities and would help to monitor response to hydroxyurea therapy.
PMID: 23249216 [PubMed - as supplied by publisher]
7. Clin J Pain. 2012 Dec 14. [Epub ahead of print]
A Preliminary Study of Psychiatric, Familial, and Medical Characteristics of High-utilizing Sickle Cell Disease Patients.
Carroll PC, Haywood C Jr, Hoot MR, Lanzkron S.
*Department of Psychiatry and Behavioral Sciences †Department of Medicine, Division of Hematology, The Johns Hopkins School of Medicine ‡The Johns Hopkins Berman Institute of Bioethics, Baltimore, MA.
Abstract
OBJECTIVES:: To identify demographic, medical, and psychosocial characteristics that distinguished sickle cell disease (SCD) patients who were frequent utilizers of urgent or emergent care resources from low-utilizing patients. METHODS:: Patients at a large urban comprehensive SCD treatment center were recruited from clinic or during urgent care visits. Participants who were high utilizers, defined as having >4 acute or emergency care visits in the prior 12 months, were compared with patients with more typical utilization patterns on lifetime complications of SCD, family background, psychiatric history, occupational function, coping, depressive symptoms, and personality. RESULTS:: High utilizers were nearly a decade younger on average; despite this they had a similar lifetime history of SCD complications. High-utilizing patients' parents seemed to have greater educational achievement overall. High utilizers reported a nearly 3-fold greater prevalence of psychiatric illness in family members than low utilizers. On other measures, including coping strategies, social support, and personality, the 2 groups were comparable. DISCUSSION:: The study strengthens emerging evidence that disease severity, familial factors related to greater parental education, and psychiatric illness are important factors in high care utilization in patients with SCD.
PMID: 23246997 [PubMed - as supplied by publisher]
8. Cochrane Database Syst Rev. 2012 Dec 12;12:CD007175. doi: 10.1002/14651858.CD007175.pub3.
Antibiotics for treating osteomyelitis in people with sickle cell disease.
Martí-Carvajal AJ, Agreda-Pérez LH.
Facultad de Ciencias de la Salud Eugenio Espejo, Universidad Tecnológica Equinoccial, Quito, Ecuador.
Abstract
BACKGROUND:
Osteomyelitis (both acute and chronic) is one of the most common infectious complications in people with sickle cell disease. There is no standardized approach to antibiotic therapy and treatment is likely to vary from country to country. Thus, there is a need to identify the efficacy and safety of different antibiotic treatment approaches for people with sickle cell disease suffering from osteomyelitis.
OBJECTIVES:
To determine whether an empirical antibiotic treatment approach (monotherapy or combination therapy) is effective and safe as compared to pathogen-directed antibiotic treatment and whether this effectiveness and safety is dependent on different treatment regimens, age or setting.
SEARCH METHODS:
We searched The Group's Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of conference proceedings. We also searched the LILACS database (1982 to 2 November 2012), African Index Medicus (3 November 2012), ISI Web of Knowledge (3 November 2012) and World Health Organization International Clinical Trials Registry Platform (3 November 2012).Date of most recent search of the Group's Haemoglobinopathies Trials Register: 29 October 2012.
SELECTION CRITERIA:
We searched for published or unpublished randomised and quasi-randomised controlled trials.
DATA COLLECTION AND ANALYSIS:
Each author intended to independently extract data and assess trial quality by standard Cochrane Collaboration methodologies, but no eligible randomised controlled trials were identified.
MAIN RESULTS:
This update was unable to find any randomised or quasi-randomised controlled trials on antibiotic treatment approaches for osteomyelitis in people with sickle cell disease.
AUTHORS' CONCLUSIONS:
We were unable to identify any relevant trials on the efficacy and safety of the antibiotic treatment approaches for people with sickle cell disease suffering from osteomyelitis. Randomised controlled trials are needed to establish the optimum antibiotic treatment for this condition.
PMID: 23235640 [PubMed - in process]
9. Cochrane Database Syst Rev. 2012 Dec 12;12:CD003968. doi: 10.1002/14651858.CD003968.pub3.
Psychological therapies for the management of chronic and recurrent pain in children and adolescents.
Eccleston C, Palermo TM, Williams AC, Lewandowski A, Morley S, Fisher E, Law E.
Centre for Pain Research, The University of Bath, Claverton Down, Bath, UK, BA2 7AY.
Abstract
BACKGROUND:
Chronic pain affects many children, who report severe pain, distressed mood, and disability. Psychological therapies are emerging as effective interventions to treat children with chronic or recurrent pain. This update adds recently published randomised controlled trials (RCTs) to the review published in 2009.
OBJECTIVES:
To assess the effectiveness of psychological therapies, principally cognitive behavioural therapy and behavioural therapy, for reducing pain, disability, and improving mood in children and adolescents with recurrent, episodic, or persistent pain. We also assessed the risk of bias and methodological quality of the included studies.
SEARCH METHODS:
Searches were undertaken of MEDLINE, EMBASE, and PsycLIT. We searched for RCTs in references of all identified studies, meta-analyses and reviews. Date of most recent search: March 2012.
SELECTION CRITERIA:
RCTs with at least 10 participants in each arm post-treatment comparing psychological therapies with active treatment were eligible for inclusion (waiting list or standard medical care) for children or adolescents with episodic, recurrent or persistent pain.
DATA COLLECTION AND ANALYSIS:
All included studies were analysed and the quality of the studies recorded. All treatments were combined into one class: psychological treatments; headache and non-headache outcomes were separately analysed on three outcomes: pain, disability, and mood. Data were extracted at two time points; post-treatment (immediately or the earliest data available following end of treatment) and at follow-up (at least three months after the post-treatment assessment point, but not more than 12 months).
MAIN RESULTS:
Eight studies were added in this update of the review, giving a total of 37 studies. The total number of participants completing treatments was 1938. Twenty-one studies addressed treatments for headache (including migraine); seven for abdominal pain; four included mixed pain conditions including headache pain, two for fibromyalgia, two for pain associated with sickle cell disease, and one for juvenile idiopathic arthritis. Analyses revealed five significant effects. Pain was found to improve for headache and non-headache groups at post-treatment, and for the headache group at follow-up. Mood significantly improved for the headache group at follow-up, although, this should be interpreted with caution as there were only two small studies entered into the analysis. Finally, disability significantly improved in the non-headache group at post-treatment. There were no other significant effects.
AUTHORS' CONCLUSIONS:
Psychological treatments are effective in reducing pain intensity for children and adolescents (<18 years) with headache and benefits from therapy appear to be maintained. Psychological treatments also improve pain and disability for children with non-headache pain. There is limited evidence available to estimate the effects of psychological therapies on mood for children and adolescents with headache and non-headache pain. There is also limited evidence to estimate the effects on disability in children with headache. These conclusions replicate and add to those of the previous review which found psychological therapies were effective in reducing pain intensity for children with headache and non-headache pain conditions, and these effects were maintained at follow-up.
PMID: 23235601 [PubMed - in process]
10. Hematology Am Soc Hematol Educ Program. 2012;2012:290-1. doi: 10.1182/asheducation-2012.1.290.
What is the evidence that hydroxyurea improves health-related quality of life in patients with sickle cell disease?
Darbari DS, Panepinto JA.
1Division of Hematology, Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC; and.
Abstract
A 10-year-old male patient with homozygous sickle cell disease presents for a follow-up clinic visit after a recent hospitalization for a painful vasoocclusive event. His parents mention that in the past year he has had 4 hospitalizations for vasoocclusive events, 2 of which were complicated by the development of acute chest syndrome that resulted in transfer to the intensive care unit. He has missed many school days and may be retained a grade this year. He feels particularly sad about missing the school field trip that occurred during his last hospitalization. He also reports that he is not able to keep up with his friends when participating in physical activities at school. The child's parents are worried that he may be depressed. You as the provider discuss the option of hydroxyurea therapy. His parents ask if hydroxyurea would improve his overall well-being and functioning.
Free Article
PMID: 23233594 [PubMed - in process]
11. Hematology Am Soc Hematol Educ Program. 2012;2012:284-9. doi: 10.1182/asheducation-2012.1.284.
Health-related quality of life in patients with hemoglobinopathies.
Panepinto JA.
1Department of Pediatrics, Section of Hematology/Oncology/Bone Marrow Transplantation, Children's Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, WI.
Abstract
The use of patient-reported outcomes to measure the health and well-being of patients from their perspective has become an acceptable method to determine the impact of a disease and its treatment on patients. In patients with hemoglobinopathies, prior work has demonstrated that patients experience significant impairment in health-related quality of life (HRQL, a type of patient-reported outcome). This work has provided a better understanding of the burden that these patients experience and the factors that are associated with worse HRQL. The recent development of disease-specific HRQL instruments in sickle cell disease heralds new opportunities to explore the impact of the disease and its treatment on patients. The standards necessary to incorporate the measurement of HRQL into clinical trials are now well outlined by regulatory agencies. Measuring HRQL within a clinical practice setting and outside of the healthcare setting while the patient is at home are now possible and present new opportunities to understand the health and well-being of patients with hemoglobinopathies.
Free Article
PMID: 23233593 [PubMed - in process]
12. Hematology Am Soc Hematol Educ Program. 2012;2012:276-83. doi: 10.1182/asheducation-2012.1.276.
Advances in stem cell transplantation and gene therapy in the β-hemoglobinopathies.
Payen E, Leboulch P.
1Commissariat a l'Energie Atomique, Institute of Emerging Diseases and Innovative Therapies, Fontenay aux Roses, France;
Abstract
High-level production of β-globin, γ-globin, or therapeutic mutant globins in the RBC lineage by hematopoietic stem cell gene therapy ameliorates or cures the hemoglobinopathies sickle cell disease and beta thalassemia, which are major causes of morbidity and mortality worldwide. Considerable efforts have been made in the last 2 decades in devising suitable gene-transfer vectors and protocols to achieve this goal. Five years ago, the first β(E)/β(0)-thalassemia major (transfusion-dependent) patient was treated by globin lentiviral gene therapy without injection of backup cells. This patient has become completely transfusion independent for the past 4 years and has global amelioration of the thalassemic phenotype. Partial clonal dominance for an intragenic site (HMGA2) of chromosomal integration of the vector was observed in this patient without a loss of hematopoietic homeostasis. Other patients are now receiving transplantations while researchers are carefully weighing the benefit/risk ratio and continuing the development of further modified vectors and protocols to improve outcomes further with respect to safety and efficacy.
Free Article
PMID: 23233592 [PubMed - in process]
13. Hematology Am Soc Hematol Educ Program. 2012;2012:271-5. doi: 10.1182/asheducation-2012.1.271.
Emerging 'A' therapies in hemoglobinopathies: agonists, antagonists, antioxidants, and arginine.
Vichinsky E.
1Hematology/Oncology, Children's Hospital and Research Center Oakland, Oakland, CA.
Abstract
Sickle cell disease and thalassemia have distinctly different mutations, but both share common complications from a chronic vasculopathy. In the past, fetal hemoglobin-modulating drugs have been the main focus of new therapy, but the increased understanding of the complex pathophysiology of these diseases has led to the development of novel agents targeting multiple pathways that cause vascular injury. This review explores the pathophysiology of hemoglobinopathies and novel drugs that have reached phase 1 and 2 clinical trials. Therapies that alter cellular adhesion to endothelium, inflammation, nitric oxide dysregulation, oxidative injury, altered iron metabolism, and hematopoiesis will be highlighted. To evaluate these therapies optimally, recommendations for improving clinical trial design in hemoglobinopathies are discussed.
Free Article
PMID: 23233591 [PubMed - in process]
14. Hematology Am Soc Hematol Educ Program. 2012;2012:208-14. doi: 10.1182/asheducation-2012.1.208.
Baby on board: what you need to know about pregnancy in the hemoglobinopathies.
Naik RP, Lanzkron S.
1Department of Medicine, Division of Hematology, Johns Hopkins University, Baltimore, MD.
Abstract
Pregnancy poses a unique challenge to patients with sickle cell disease and β-thalassemia, who often have exacerbations of hemolysis or anemia during the gestational period, experience higher rates of obstetric and fetal complications, and may have distinct underlying comorbidities related to vasculopathy and iron overload that can endanger maternal health. Optimal management of pregnant women with hemoglobinopathies requires both an understanding of the physiologic demands of pregnancy and the pathophysiology of disease-specific complications of inherited blood disorders. A multidisciplinary team of expert hematologists and high-risk obstetricians is therefore essential to ensuring appropriate antenatal maternal screening, adequate fetal surveillance, and early recognition of complications. Fortunately, with integrated and targeted care, most women with sickle cell disease and β-thalassemia can achieve successful pregnancy outcomes.
Free Article
PMID: 23233583 [PubMed - in process]
15. Hemoglobin. 2012 Dec 7. [Epub ahead of print]
Safety And Efficacy Of 4 Years Of Deferasirox Treatment For Sickle Cell Disease Patients.
Vlachaki E, Mainou M, Bekiari E, Vetsiou E, Tsapas A.
Thalassemia Unit, Second Medical Department, Hippokratio General Hospital, Aristotle University Thessaloniki , Greece.
Abstract
Deferasirox (DFRA) is a novel oral chelator agent for treatment of iron overload. Although well established in the treatment of β-thalassemia major (β-TM), it has not yet been fully investigated in patients with sickle cell disease. The aim of this report is to present the preliminary results of a pilot study assessing the effect of 4 years of DFRA treatment in six patients with sickle cell disease who are in need of recurrent transfusions. Our results show a significant reduction of ferritin levels and improvement of liver hemosiderosis, assessed by means of magnetic resonance imaging T2* (MRI T2*). None of the patients presented any serious adverse effects and the treatment was well tolerated. These results are in accordance with previous studies about the use of DFRA in sickle cell disease.
PMID: 23215738 [PubMed - as supplied by publisher]
16. J Pediatr Hematol Oncol. 2012 Nov 30. [Epub ahead of print]
The Other Side of Abnormal: A Case Series of Low Transcranial Doppler Velocities Associated With Stroke in Children With Sickle Cell Disease.
Buchanan ID, James-Herry A, Osunkwo I.
*Morehouse School of Medicine, Department of Pediatrics ‡Division of Hematology/Oncology, Department of Pediatrics, Emory University §Aflac Cancer Center and Blood Disorder Services of Children Healthcare of Atlanta †Sickle Cell Consortium, Atlanta, GA.
Abstract
The prevalence of cerebrovascular events in sickle cell disease (SCD) can be as low as 10% by the age of 18 for overt cerebral infarction or strokes, up to 35% for silent cerebral infarction, and as high as 43/100 patient years for acute silent cerebral ischemic events. These events typically occur during childhood with a peak incidence between the age of 4 and 7 years. The cumulative risk of central nervous system events in SCD increases with age. Transcranial Doppler (TCD) ultrasonography is an established screening tool for detecting children with SCD at highest risk for stroke by measuring the flow velocity in the large intracranial vessels. Velocities are considered abnormal with readings >200 cm/s and chronic red cell transfusions are recommended to reduce further risk or progression. Red cell transfusions have reduced the rate of cerebrovascular accidents by 90%. We describe the case of 5 children with sickle cell anemia, whose antecedent screening TCD velocities were measured to be ≤70 cm/s in the study. All patients developed some form of cerebral insults, an overt cerebral infarctions, silent stroke or transient ischemic attack, and are now receiving chronic transfusion to prevent further progression. On the basis of these cases, low TCD velocities may identify another group of children at risk for cerebrovascular disease. We suggest TCD velocities <70 cm/s in major vessels (MCA, ACA, and ICA) be considered another type of "abnormal," prompting more sensitive evaluations (such as a brain MRI and MRA) for the presence of central nervous system disease, and, if negative, decrease intervals between subsequent TCD assessments.
PMID: 23211694 [PubMed - as supplied by publisher]
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17. Pediatr Blood Cancer. 2012 Nov 28. doi: 10.1002/pbc.24395. [Epub ahead of print]
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Sickle Cell News for November 2012
Sickle cell anemia: maps and newborn estimates In 2010 around 300,000 babies were born with sickle cell anaemia, a serious blood disorder which can be fatal if untreated, and 5.5 million newborns inherited the sickle cell gene, a new study suggests. The 5.5 million who only inherit the gene will usually not present any clinical complications, these individuals could still pass this gene on to their offspring and give birth to babies suffering from sickle cell anaemia. Accurate estimates of the numbers and geographical distribution of those affected is vital for effective prevention and treatment policies to be put in place.
The research by the Malaria Atlas Project (MAP; http://www.map.ox.ac.uk), a multinational team of researchers funded mainly by the Wellcome Trust, maps the geographical contemporary distribution of sickle haemoglobin - a genetic disorder causing sickle cell anaemia. It also estimates the number of newborns affected by this condition.Historically, the sickle cell gene (haemoglobin S or HbS) was common in people of African, Mediterranean and Indian origin but, following human migrations, it is now much more widespread. The MAP team's estimates suggest that about half of the affected newborns are born in Nigeria, the Democratic Republic of the Congo, and India, but important uncertainties remain in large parts of these countries due to a lack of data.
An image showing areas with high predicted frequencies of sickle haemoglobin [credit: MAP]
Dr Fred Piel from Oxford University's Department of Zoology, who led the research, said: "Sickle cell disease has now been studied intensively for more than a hundred years but our knowledge about its current distribution and burden is really poor. Our aim was to use available evidence-based epidemiological data from the literature combined with modern mapping and modelling methods to come up with the best maps and estimates. In the future, we hope that accessing additional data, including from national screening programmes, would help further refine these results."
This study provides the first rigorous assessment of the contemporary distribution of this disorder and uses state-of-the-art methodology to estimate the number of newborns affected globally, regionally and nationally. The team was inspired by work conducted by Frank B Livingstone, in the 1970s and 80s. Although ageing, his global database on inherited blood disorder frequencies still represents a unique resource. There is growing awareness about the burden of genetic blood disorders, sickle cell disease in particular, and it is crucial for public health policy makers to access evidence-based quantitative epidemiological data allowing the assessment of the current situation and to measure changes in the future. The data will be released in open access on the MAP website (www.map.ox.ac.uk).Professor Sir David Weatherall, who has shared his unique expertise in the field and provided exceptional support to this project, said: "The inherited haemoglobin disorders, notably sickle cell disease and the different forms of thalassaemia, are by far the commonest monogenic diseases and the vast majority of births affected occur in low or middle income countries. Previous work suggested that their distribution varied considerably even within short geographical distances and data regarding their true frequency is extremely difficult to obtain. Hitherto, they have been largely ignored by the international health community and it is absolutely vital that better information is obtained regarding their true frequency so that their control and better management can be achieved, particularly in the developing countries where they are so common. The impressive work described in this paper provides an invaluable base for future work of this kind."
For further information visit http://www.map.ox.ac.uk or contact Dr Fred Piel of Oxford University on +44 (0)1865 271 132 or email fred.piel@zoo.ox.ac.uk or Professor Simon Hay of Oxford University on +44 (0)1865 271 243 or email simon.hay@zoo.ox.ac.uk. Sickle Cell Transplants Could See Wider UseNY Times article - CDC committee recommends HibMenCY for infantsThe Centers for Disease Control and Prevention’s Advisory Committee for Immunization Practices voted on October 24 to recommend a meningococcal vaccination for infants at increased risk of contracting the disease.The committee recommended that infants with anatomic or functional asplenia, including sickle cell disease, or with recognized persistent complement pathway deficiencies receive the HibMenCY meningococcal vaccine. The recommended vaccination practice includes four doses of the vaccine at two, four, six and 12 through 15 months.The vaccine can be used in infants ages two through 18 months who live in communities with serogroup Y and C meningococcal disease outbreaks.“The ACIP meningococcal vaccine working group concluded that the recently licensed HibMenCY infant vaccine should be routinely given to those infants at high risk for meningococcal disease due to certain immunocompromising conditions,” Alison Patti, a representative with the CDC, said. “Now that this vaccine is available, it made clinical and public health sense to routinely administer it to high risk infants. Before HibMenCY, no infant meningococcal vaccine was available.”ACIP’s recommendations were forwarded to the CDC’s director for approval. If approved, the recommendations will represent the official CDC recommendations for U.S. immunizations. Until that time, the recommendations are considered provisional.Meningococcal disease is a vaccine-preventable and serious bacterial infection caused by Neisseria meningitis bacteria. The two most common illnesses caused by the bacteria include bloodstream infections and meningitis. Infants with medical conditions like a complement component deficiency or sickle cell disease are at increased meningococcal disease risk.“It’s estimated that 5,000 kids per year will get HibMenCY vaccine through this high risk recommendation,” Patti said. “Most of these children are at lifelong risk for meningococcal disease, so they can now be protected younger than ever before.”Video ResourcesNew Webinar Posted for November10/25/12 Webinar - Strategies from the Field – Data Collection and Harmonization CDC’s Division of Blood Disorders and RuSH Project StatesThe video link is mms://realaudio.service.emory.edu/SOM/PA/PLATT/SICKLE/CDCdatacollection.wmvSee all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-resources/cdc-webinars-hemoglobinopathies-and-public-healthWe would appreciate your feedback on this year’s “Public Health Webinar Series on Hemoglobinopathies” hosted by the Division of Blood Disorders, CDCby completing a brief online survey: http://www.surveymonkey.com/s/M986NWM Please forward the survey link to colleagues who participated on this webinar series as a group.Your feedback is valuable and will help us improve the series for 2013.Thank you for your participation! CDC Web based Sickle Cell ResourcesCDC’s YouTube Link: http://www.youtube.com/watch?v=gnrvYsZWR1c&list=UUiMg06DjcUk5FRiM3g5sqoQ&index=2&feature=plpp_video CDC Video Archive (you can get the video code from this site and embed the video on another webpage, or download it): http://www.cdc.gov/NCBDDD/video/SickleCell/index.html CDC Sickle Cell Disease Webpage: http://wwwdev.cdc.gov/NCBDDD/sicklecell/video.html Articles in the Medical Literature for November1. Mediterr J Hematol Infect Dis. 2012;4(1):e2012065. doi: 10.4084/MJHID.2012.065. Epub 2012 Oct 3.Sickle cell anaemia and malaria.Luzzatto L.Honorary Professor of Haematology, University of Florence, Scientific Director, Istituto Toscano Tumori. Firenze. Italy.AbstractSickle cell anaemia is a major chapter within haemolytic anaemias; at the same time, its epidemiology is a remarkable signature of the past and present world distribution of Plasmodium falciparum malaria. In this brief review, in keeping with the theme of this journal, we focus on the close and complex relationship betweeen this blood disease and this infectious disease. On one hand, heterozygotes for the sickle gene (AS) are relatively protected against the danger of dying of malaria, as now firmly established through a number of clinical field studies from different parts of Africa. In addition, experimental work is consistent with a plausibile mechanism: namely, that in AS heterozygotes P falciparum-infected red cells sickle preferentially and are then removed by macrophages. On the other hand, patients who are homozygous for the sickle gene and therefore suffer from sickle cell anaemia (SCA) are highly susceptible to the lethal effects of malaria. The simplest explanation of this fact is that malaria makes the anaemia of SCA more severe; in addition, in SCA there is often hyposplenism, which reduces clearance of parasites. From the point of view of public health it is important that in malaria-endemic countries patients with SCA, and particularly children, be protected from malaria by appropriate prophylaxis.PMCID: PMC3499995 Free PMC Article
PMID: 23170194 [PubMed - in process]
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2. J Hosp Med. 2012 Nov 20. doi: 10.1002/jhm.1987. [Epub ahead of print]"I'm Talking About Pain": Sickle cell disease patients with extremely high hospital use.Weisberg D, Balf-Soran G, Becker W, Brown SE, Sledge W.Department of Psychiatry, Yale University School of Medicine, New Haven, Connecticut.AbstractBACKGROUND: A small minority of sickle cell disease patients accounts for the majority of inpatient hospital days. Admitted as often as several times a month, over successive years, this cohort of patients has not been studied in depth despite their disproportionate contribution to inpatient hospital costs in sickle cell disease.OBJECTIVE: To characterize the subjective experience of extremely high hospital use in patients with sickle cell disease, and generate hypotheses about the antecedents and consequences of this phenomenon.DESIGN: Qualitative study involving in-depth, open-ended interviews using a standardized interview guide.SETTING: A single urban academic medical center.PARTICIPANTS: Eight individuals, of varying age and gender, identified as the sickle cell disease patients who are among the highest hospital use patients over a 3-year period.RESULTS: A common narrative emerged from the interview transcripts. Participants were exposed to the hospital environment and intravenous (IV) opioids at a young age, and this exposure was associated with extremely high hospital use in adulthood, evident in descriptions of multiple dimensions of their lives: pain and opioid medication use, interpersonal relationships, and personal development.CONCLUSIONS: Our results suggest a systematic, self-reinforcing process of isolation from mainstream society, support structures, and caregivers, based on increasing hospitalization, growing dependency on opioid medications, as well as missed developmental milestones. Further study and interventions should be geared towards breaking this spiraling cycle with long-term strategies in disease management and social integration. Journal of Hospital Medicine 2012; © 2012 Society of Hospital Medicine.Copyright © 2012 Society of Hospital Medicine.
PMID: 23169484 [PubMed - as supplied by publisher]
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3. Cochrane Database Syst Rev. 2012 Nov 14;11:CD008394. doi: 10.1002/14651858.CD008394.pub2.Interventions for treating leg ulcers in people with sickle cell disease.Martí-Carvajal AJ, Knight-Madden JM, Martinez-Zapata MJ.Facultad de Ciencias de la Salud Eugenio Espejo, Universidad Tecnológica Equinoccial, Quito, Ecuador.AbstractBACKGROUND: The frequency of skin ulceration makes it an important contributor to the morbidity burden in people with sickle cell disease. Many treatment options are available to the healthcare professional, although it is uncertain which treatments have been assessed for effectiveness in people with sickle cell disease.OBJECTIVES: To assess the clinical effectiveness and safety of interventions for treating leg ulcers in people with sickle cell disease.SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register.We searched LILACS (1982 to August 2012), the African Index Medicus (up to August 2012), ISI Web of Knowledge (1985 to August 2012), and the Clinical Trials Search Portal of the World Health Organization (August 2012). We checked the reference lists of all the trials identified. We also contacted those groups or individuals who may have completed relevant randomised trials in this area.Date of the last search of the Group's Haemoglobinopathies Trials Register: 25 May 2012.SELECTION CRITERIA: Randomised controlled trials of interventions for treating leg ulcers in people with sickle cell disease compared to placebo or an alternative treatment.DATA COLLECTION AND ANALYSIS: Two authors independently selected studies for inclusion. All three authors independently assessed the risk of bias of the included studies and extracted data.MAIN RESULTS: Six studies met the inclusion criteria (198 participants with 250 ulcers). Each trial investigated a different intervention and within this review we have grouped these as systemic pharmaceutical interventions (L-cartinine, arginine butyrate, isoxsuprine) and topical pharmaceutical interventions (Solcoseryl(®) cream, RGD peptide dressing, topical antibiotics). Three interventions reported on the change in ulcer size (arginine butyrate, RGD peptide, L-cartinine). Of these, RGD peptide matrix significantly reduced ulcer size compared with a control group, mean reduction 6.60cm(2) (95% CI 5.51 to 7.69). Three trials reported on the incidence of complete closure (isoxsuprine, arginine butyrate, RGD peptide matrix). None reported a significant effect. No trial reported on: the time to complete ulcer healing; ulcer-free survival following treatment for sickle cell leg ulcers; quality of life measures; or incidence of amputation. There was no reported information on the safety of these interventions.AUTHORS' CONCLUSIONS: There is evidence that a topical intervention (RGD peptide matrix) reduced ulcer size in treated participants compared to controls. This evidence of efficacy is limited by the generally high risk of bias associated with these reports.We planned to analyse results according to general groups: pharmaceutical interventions (systemic and topical); and non-pharmaceutical interventions (surgical and non-surgical). However, we were unable to pool findings due to the heterogeneity in outcome definitions, and inconsistency between the unit of randomisation and the unit of analysis. This heterogeneity, along with a paucity of identified trials, prevented us performing any meta-analyses.This Cochrane review provides some evidence for the effectiveness of one topical intervention - RGD peptide matrix. However, this intervention was assessed as having a high risk of bias due to inadequacies in the single trial report. Other included studies were also assessed as having a high risk of bias. We recommend that readers interpret the trial results with caution. The safety profile of the all interventions was inconclusive.
PMID: 23152256 [PubMed - in process]
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4. Cochrane Database Syst Rev. 2012 Nov 14;11:CD007652. doi: 10.1002/14651858.CD007652.pub3.Gene therapy for sickle cell disease.Olowoyeye A, Okwundu CI.Lagos University Teaching Hospital, P.O.Box 8893 Marina, Lagos, Nigeria.AbstractBACKGROUND: Sickle cell disease encompasses a group of genetic disorders characterized by the presence of at least one hemoglobin S (Hb S) allele, and a second abnormal allele that could allow abnormal hemoglobin polymerisation leading to a symptomatic disorder.Autosomal recessive disorders (such as sickle cell disease) are good candidates for gene therapy because a normal phenotype can be restored in diseased cells with only a single normal copy of the mutant gene.OBJECTIVES: The objectives of this review are:- to determine whether gene therapy can improve survival and prevent symptoms and complications associated with sickle cell disease;- to examine the risks of gene therapy against the potential long-term gain for people with sickle cell disease.SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises of references identified from comprehensive electronic database searches and searching relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 21 June 2012.SELECTION CRITERIA: All randomised or quasi-randomised clinical trials (including any relevant phase 1, 2 or 3 trials) of gene therapy for all individuals with sickle cell disease, regardless of age or setting.DATA COLLECTION AND ANALYSIS: No trials of gene therapy for sickle cell disease were found.MAIN RESULTS: No trials of gene therapy for sickle cell disease were reported.AUTHORS' CONCLUSIONS: No randomised or quasi-randomised clinical trials of gene therapy for sickle cell disease were reported. Thus, no objective conclusions or recommendations in practice can be made on gene therapy for sickle cell disease. This systematic review has identified the need for well-designed, randomised controlled trials to assess the benefits and risks of gene therapy for sickle cell disease.
PMID: 23152248 [PubMed - in process]
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5. Pediatr Blood Cancer. 2012 Nov 14. doi: 10.1002/pbc.24394. [Epub ahead of print]Mental health disorders influence admission rates for pain in children with sickle cell disease.Myrvik MP, Burks LM, Hoffman RG, Dasgupta M, Panepinto JA.Department of Pediatrics, Hematology/Oncology/Bone Marrow Transplantation, Children's Research Institute/Medical College of Wisconsin, Milwaukee, Wisconsin. mmyrvik@mcw.edu.AbstractBACKGROUND: Patients with sickle cell disease (SCD) experience a broad range of mental health disorders placing them at risk for more complicated hospitalizations for pain. The current study examined the impact of mental health disorders on admission rates and hospital length of stay (LOS) for vaso-occlusive pain events (VOE) in pediatric patients with SCD.PROCEDURE: Patients (5-18 years old) with a primary discharge diagnosis of SCD with crisis were acquired through the Pediatric Health Information System and categorized by history of mental health disorders (mood disorder, anxiety disorder, disruptive behavior disorder, and substance use disorder). Using a retrospective cohort design, hospital admission rates for VOE were examined as the primary outcome and LOS as a secondary outcome.RESULTS: A total of 5,825 patients accounted for 23,561 admissions for SCD with crisis with approximately 8% of the patients having a mental health diagnosis. Longer LOS was found among patients with a history of any mental health diagnosis (P < 0.0001) and within the mood disorder (P < 0.0001), anxiety disorder (P < 0.0001), and substance use disorder (P = 0.01) subtypes. Hospital admissions rates for VOE were higher among patients with a history of any mental health diagnosis (P < 0.0001) and within the mood disorder (P < 0.0001), anxiety disorder (P < 0.0001), disruptive behavior disorder (P = 0.002), and substance use disorder (P < 0.0001) subtypes.CONCLUSIONS: Pediatric patients with SCD and a history of a mental health diagnosis have longer LOS and higher admission rates for management of VOE. Ultimately, these findings suggest that mental health pose a challenge to the management of sickle cell pain. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.Copyright © 2012 Wiley Periodicals, Inc.
PMID: 23151972 [PubMed - as supplied by publisher]
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6. Pediatr Blood Cancer. 2012 Nov 14. doi: 10.1002/pbc.24392. [Epub ahead of print]National trends in incidence rates of hospitalization for stroke in children with sickle cell disease.McCavit TL, Xuan L, Zhang S, Flores G, Quinn CT.Division of Pediatric Hematology-Oncology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas; Children's Medical Center Dallas, Dallas, Texas. tim.mccavit@childrens.com.AbstractBACKGROUND: The success of primary stroke prevention for children with sickle cell disease (SCD) throughout the United States is unknown. Therefore, we aimed to generate national incidence rates of hospitalization for stroke in children with sickle cell disease (SCD) before and after publication of the Stroke Prevention Trial in Sickle Cell Anemia (STOP trial) in 1998.PROCEDURE: We performed a retrospective trend analysis of the 1993-2009 Nationwide Inpatient Sample and Kids' Inpatient Databases. Hospitalizations for SCD patients 0-18 years old with stroke were identified by ICD-9CM code. The primary outcome, the trend in annual incidence rate of hospitalization for stroke in children with SCD, was analyzed by linear regression. Incidence rates of hospitalization for stroke before and after 1998 were compared by the Wilcoxon rank-sum test.RESULTS: From 1993 to 2009, 2,024 hospitalizations were identified for stroke. Using the mean annual incidence rate of hospitalization for stroke from 1993 to 1998 as the baseline, the rate decreased from 1993 to 2009 (point estimate = -0.022/100 patient years [95% CI, -0.039, -0.005], P = 0.027). The mean annual incidence rate of hospitalization stroke decreased by 45% from 0.51 per 100 patient years in 1993-1998 to 0.28 per 100 patient years in 1999-2009 (P = 0.008). Total hospital days and charges attributed to stroke also decreased by 45% and 24%, respectively.CONCLUSIONS: After publication of the STOP trial and hydroxyurea licensure in 1998, the incidence of hospitalization for stroke in children with SCD decreased across the United States, suggesting that primary stroke prevention has been effective nationwide, but opportunity for improvement remains. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.Copyright © 2012 Wiley Periodicals, Inc.
PMID: 23151905 [PubMed - as supplied by publisher]
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Sickle Cell News for October 2012
Researchers identify painful symptoms of sickle cell disease
Researchers at Drexel University have identified the physical forces in red blood cells and blood vessels underlying the painful symptoms of sickle cell disease. Their experiment, the first to answer a scientific question about sickle cell disease using microfluidics engineering methods, may help future researchers better determine who is at greatest risk of harm from the disease. They report their findings in Cell Press'sBiophysical Journal today.
Like many scientific questions, this discovery began with a mystery. Normal, healthy red blood cells are extremely flexible, squeezing and slipping through blood vessels with ease, even passing through the smallest capillaries that are narrower than the red blood cells themselves. But in sickle cell disease, red blood cells are prone to deforming and turning rigid while flowing through the body. A seemingly logical explanation for sickle cell disease was that its symptoms - painful episodes and organ damage caused by oxygen deprivation - resulted from the rigid sickle cells forming inside narrow capillaries and then getting stuck there.
In fact, sickle cells do not get stuck inside capillaries. The symptoms of sickle cell disease come from partial obstructions in slightly wider blood vessels farther downstream-vessels wide enough that sickle cells should be wide enough to flow through. The mystery, then, was why? How do wide, rigid cells regularly pass through the narrowest channels without getting stuck?http://www.news-medical.net/
1% Of Kuwait Population Has Sickle Cell Disease
DESPITE its being one of the most commonly inherited genetic disorders on earth, there is a far-reaching lack of awareness of Sickle Cell Disease and its effects, in a case where awareness may be all it takes to for the prevention of potentially tragic results. Moreover, being that SCD is common around tropics where malaria is prevalent, the Arabian Peninsula is one of the areas commonly afflicted with SCD, with a rate of approximately 1% of the Kuwait population suffering -- in many cases unknowingly -- from the disease.
After years of planning, Thursday 18 October sees the opening of a day care centre at Homerton Hospital for sufferers of both Sickle Cell Anemia and Thassalmania.
It’s the most serious of inherited conditions in the UK, yet it is the least known about and Homerton Hospital already manages over 300 local patients with the condition, while providing advice and support for those who aren’t themselves affected but ‘carry’ the condition, and could pass it on to their children. In England alone, there are approximately 12,500 people who suffer from this disease and an estimated 240,000 carriers of sickle cell in England http://www.hackneyhive.co.uk/
New Sickle Cell Clinic in Missouri
University of Missouri Health Care is opening a new clinic to treat patients with sickle cell disease. The clinic will be open the second and fourth Thursdays of every month.
Sickle cell is a genetic disease where red blood cells are in short supply. According to Children’s Mercy Hospital, most of the treatment centers are aimed toward children. Elizabeth Gunier, a sickle cell coordinator at Women's and Children's Hospital's blood disorder and cancer unit, says the new clinic is specifically for adults.
“This is the first time since I’ve been here that we are able to set up an adult clinic, just specifically for them," she says. "It’s something that’s been in the works, something that everybody’s wanted to do.”
The clinic is set to open at Ellis Fischel Cancer Center on Oct. 25, 2012. It is the first such clinic in Columbia. Others are located in St. Louis and Kansas City
Tionne "T-Boz" Watkins, a member of the 1990s R&B trio TLC, is getting her own reality show, TMZ reports.
The show, called Totally T-Boz, will showcase Watkins juggling being a single mom with attempting to re-launch her music career, sources tell TMZ . The show will air on (what else?) TLC, which has reportedly ordered four episodes.
Watkins was diagnosed with sickle-cell anemia as a child and is a spokesperson for the Sickle Cell Disease Association of America. In 2006, she discovered that she had a potentially fatal brain tumor, which was removed in 2009. After the surgery, Watkins underwent therapy to re-learn how to walk and talk, according to TMZ.
Watkins filed for bankruptcy protection twice in 2011, but TLC recently announced plans for a reunion tour that will feature a hologram of Lisa "Left-Eye" Lopes, who died in a car accident in 2002.
http://www.tvguide.com/News/
Video Resources
New Webinar Posted for October
:Improving Quality of Care for Sickle Cell Pediatrics in the Emergency Department Dr. James Moses, Boston Medical Center 9/27/12
The video link is mms://realaudio.service.emory.
Schedule of Free CDC 2012 Webinars
“Public Health Webinar Series on Hemoglobinopathies”
Hosted by: The Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities (NCBDDD), Centers for Disease Control and Prevention (CDC) 4th Thursday of every month from 2:00PM – 3:00PM ET The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
Hemoglobinopathy Webinars are archived at http://scinfo.org
To Join The Webinar
Copy this address and paste it into your web browser:https://www.livemeeting.com/
Copy and paste the required meeting ID: 84QK2D and click “join”.
First Time Users: To save time before the meeting, check your system to make sure it is ready to use Microsoft Office Live Meeting. To hear the presentation you must call in to the number below.
For Audio
Dial 1-877-953-6706 and enter participant code: 9706616
If you are unable to join us on the internet for viewing, you are welcome to call in for audio access only. Participants outside the United States must be able to access 800 numbers to the US. Otherwise, please RSVP the location and number of participants for alternative international conference line access.
10/25: Strategies from the Field – Data Collection and HarmonizationCDC’s Division of Blood Disorders and RuSH Project States
November/December: --- No Webinars---
See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-
New Web Resource
NHS Sickle Cell and Thalassaemia Screening Programme Resources
Adult Carrier Leaflets:
Adults who attend antenatal screening will receive an Adult Carrier Leaflet with information about being a carrier of a significant haemoglobin variant to assist them in their understanding of their haemoglobin carrier status. These leaflets can be used in an antenatal setting as well as in other settings such as in a GP surgery or other healthcare centres.
The leaflets cover the carrier states Hb AS, Hb AC, Hb AD, Hb AE, Beta Thalassaemia, Delta beta thalassaemia, O Arab, and Hb Lepore and can be downloaded at sct.screening.nhs.uk/
NICE Guidelines:
The Sickle Cell Acute Painful Episode are published and are available on the National Institute for Health and Clinical Excellence (NICE) websitehttp://www.nice.org.uk/CG143
You can find the consultation comments and responses on this page:http://guidance.nice.org.uk/
See all the publications at http://sct.screening.nhs.uk/
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CDC Web based Sickle Cell Resources CDC’s YouTube Link: http://www.youtube.com/watch? CDC Video Archive (you can get the video code from this site and embed the video on another webpage, or download it):http://www.cdc.gov/NCBDDD/ CDC Sickle Cell Disease Webpage: http://wwwdev.cdc.gov/NCBDDD/ Articles in the Medical Literature for October
Sickle Cell Conferences and Events |
West Coast Sickle Cell Nurses Conference Friday, October 26, 2012Presented by
Children's Hospital Los Angeles -Improving Outcomes for Children and Adults
with Sickle Cell Disease Location: John Stauffer Conference Room Children's Hospital Los Angeles Registration fee: $45.00 5 CEU's Lunch provided
Please note, this is a NURSES ONLY conference.
For adults and parents, please provide this information to your nurse or health care provider. For more information, contact Trish Peterson attpeterson@chla.usc.edu
or Debbie Harris at dharris@chla.usc.edu
The Caribbean Organization of Sickle Cell Associations (COSCA), with the Saint Lucia Sickle Cell Association, the Saint Lucia Medical and Dental Association, and the University of the West Indies Medical School (Cave Hill), will be hosting the eighth Caribbean Sickle Cell Conference in Saint Lucia from October 26 to 28, 2012, at the Bay Gardens Hotel.
In attendance will be medical practitioners, government representatives, sickle cell patients and support personnel from the region and internationally. Speakers will come from Canada, the United States, Jamaica, Martinique, Guadeloupe and Saint Lucia.
Several topics will be discussed, including the prevention of sickle cell disease, research on bone marrow transplant, stroke prevention and management, the socio- economic and policy impact of sickle cell disease (SCD), the Sick Kids Caribbean haematology-oncology outreach programme, Caribbean sickle cell experience, pain management, ethical dilemmas and social issues.
the Cuban Society of Hematology VII National Congress for the Spring of 2013 (May 20-24) at the Havana International Conference Center, under the name of HEMATOLOGIA 2013. Toghether with this event, we will be celebrating the III Caribbean Conference on Sickle Cell Disease (from CAREST), as well as the IX Latinamerican Meeting on Hematology, Immunology and Transfusion Medicine, the IV International Workshop on Hemophilia, and the III Internatioal Symposium of Regenerative Medicine.
emails: hematologia2013@infomed.sld.cu
7th Annual Sickle Cell Disease Research and Educational Symposium & Annual National Sickle Cell Disease Scientific meeting April 12 – 17, 2013 Miami, FL http://www.florida-sickle.org/
Sickle Cell News for September 2012
September is Sickle Cell Month
WISCH (Working to Improve Sickle Cell Healthcare)
WISCH (Working to Improve Sickle Cell Healthcare) is NICHQ’s portfolio of projects focused on improving the quality of care for individuals with sickle cell disease across the lifespan. These projects include the Sickle Cell Disease Newborn Screening Program and the Sickle Cell Disease Treatment Demonstration Program, both of which are funded through the Health Resources and Services Administration (HRSA).
NICHQ is working with 15 grantee sites through two HRSA-funded projects to improve systems of detection and care for people living with SCD. Teams made up of healthcare providers, patients, hematologists, nurses and others are using quality improvement techniques to implement:
- Timely acute care management
- Better coordination of care
- Better transition from pediatric to adult care
- Improved screening, counseling, and education for individuals with SCD and trait
- Enhanced education for providers on treating, assessing and monitoring SCD
WISCH Overview Video at https://vimeo.com/49602847
Q&A with Lynnie Reid, NICHQ’s Senior Manager of Patient and Family Partnerships
Lynnie Reid went from being a young mother who knew nothing about sickle cell disease to becoming a passionate champion for people affected by the blood disorder.
"Hemoglobinopathy Learning Collaborative: Using Quality Improvement (QI) to Achieve Equity in Health Care Quality, Coordination, and Outcomes for Sickle Cell Disease" [Journal Article]
This journal article centers on NICHQ's Working to Improve Sickle Cell Healthcare (WISCH) project, which seeks to improve outcomes and care across the life course through improvement science for individuals living with sickle cell disease (SCD).
The authors would like to acknowledge the contribution of improvement teams who participated in the expert panel meeting to review and rate indicators and to provide feedback on the feasibility of collecting data for project indicators.
- Note: The full text of this article is being shared with the permission of Meharry Medical College and the Journal of Health Care for the Poor and Underserved (JHCPU).
- Citation: Oyeku SO, Wang JC, Scoville R, Vanderkruik R, Clermont E, McPherson ME, Adams WG, and Homer CJ. "Hemoglobinopathy Learning Collaborative: Using Quality Improvement (QI) to Achieve Equity in Health Care Quality, Coordination, and Outcomes for Sickle Cell Disease." Journal of Health Care for the Poor and Underserved. Volume 23 (2012): 34–48.
- Access the full article from the Journal of Health Care for the Poor and Underserved.
Treating Pain More Quickly for Children with Sickle Cell Disease at Boston Medical Center Hospital uses quality improvement techniques to cut the wait to pain medications in half.
New Tennessee Center Offers Affordable Care to Sickle Cell Patients A team of healthcare specialists is working to coordinate care, reduce ER visits, and spread awareness
Partially-matched bone marrow transplants can eliminate sickle cell disease in some patients
In a preliminary clinical trial, investigators at Johns Hopkins have shown that even partially-matched bone marrow transplantscan eliminate sickle cell disease in some patients, ridding them of painful and debilitating symptoms, and the need for a lifetime of pain medications and blood transfusions. The researchers say the use of such marrow could potentially help makebone marrow transplants accessible to a majority of sickle cell patients who need them.
After a median follow-up of two years, the transplants successfully eliminated sickle cell disease in 11 of 17 patients. Three were fully matched to their donors and eight received half-matched donor marrow. All 11 patients are free of painful sickle cell crises and 10 no longer have anemia. There were no deaths and no unexpected toxicities.
Six of the 11 patients (all half-matched) have stopped taking immunosuppressive drugs, although some still require narcotics for chronic pain because of sickle cell-related organ damage. Blood tests on the six patients show that their red cells are now completely derived from their donor’s marrow.
Patients with severe sickle cell disease (SCD) face shortened life spans, intractable pain and eventual organ damage as a result of their disease, an inherited disorder caused by a mistake in the oxygen-carrying hemoglobin molecules in red blood cells. The flawed genetic code stiffens red cells, and shapes them into a pronged “sickle” that clump and stick into blood vessel walls, cutting off blood and oxygen to tissues and organs throughout the body.
SCD occurs in approximately one in 400 African Americans, and rarely in Caucasians. An estimated 100,000 people are currently living with sickle cell disease in the U.S.
Most patients die before age 50, and many suffer poor quality of life with frequent episodes of “off-the-charts” pain, and an increased risk for kidney failure, stroke, deep-vein thrombosis, and lung disease.
Treatments include blood transfusions and a drug, hydroxyurea. Many patients use narcotics to control severe pain and have repeat hospitalizations. Bone marrow transplants have been successful in curing some cases, but matching donors are rare and the procedure itself poses risk.
In the current study, 17 patients at the Johns Hopkins Hospital were offered bone marrow transplant options, including the use of half-matched donor marrow to try and replace their “sickled” blood cells with new, healthy ones. The transplants were successful in 11 of the patients, of whom eight were only half-matches. Results of the trial were published in the Sept. 6 early online edition of Blood.
“We’re trying to reformat the blood system and give patients new blood cells to replace the diseased ones, much like you would replace a computer’s circuitry with an entirely new hard drive,” says Robert Brodsky, M.D., director of the Division ofHematology at Johns Hopkins and The Johns Hopkins Family Professor of Medicine and Oncology. “While bone marrow transplants have long been known to cure sickle cell disease, only a small percentage of patients have fully matched, eligible donors.”
National registries often are of little help in finding donors for sickle cell patients, because most of those in need are African American and other minorities who are vastly underrepresented in registries, say the Johns Hopkins researchers.
To overcome the shortage of donors, investigators at Johns Hopkins developed techniques, recently tested in leukemia andlymphoma patients, to transplant with bone marrow that is half-identical or “haploidentical” to the patient’s tissue type. Half-matched bone marrow can be obtained from parents, children and most siblings, and is extracted by needle from the hip bone.
For the study, the Johns Hopkins team screened 19 patients to find bone marrow donors with either half-identical or fully matched tissue. Each transplant candidate had experienced many severe pain crises, significant organ problems, or had failed hydroxyruea, the only drug known to curtail sickle cell symptoms. The team found donors for 17 of the 19 patients: 14 were half-identical and three were fully matched siblings. The youngest patient was 15; the oldest 46.
Oklahoma-based Selexys Pharmaceuticals has just completed a $23m equity financing to help fund the next stage of development of the drug, which is an anti-P-selectin antibody called SelG1.
And Novartis has taken an exclusive option to acquire Selexys and SelG1 after the completion of a phase II study in patients with sickle cell disease, a condition in which red blood cells form an abnormal sickle or crescent shape.
The deformation of the red blood cells causes them to stiffen so they cannot pass through small blood vessels so easily, which in turn prevents oxygen from being delivered to tissues. In severe episodes - known as vaso-occlusive crises - the blood vessels become obstructed leading to ischaemia, pain and organ damage.
"Patients with sickle cell disease endure great suffering and frequent hospitalisation due to painful vaso-occlusive crises," said Selexys' president and chief executive Dr Scott Rollins.
The company developed SelG1 based on the idea that blocking P-selectin - an adhesion molecule that causes cells to clump together - may help avert vaso-occlusive crises in sickle cell patients.
A recently-completed phase I trial supported the antibody's safety in human volunteers and the company is now pressing ahead with a phase II evaluation in patients with the disease.
Rollins said the financing will help Selexys fund not only the SelG1 trial but also a phase I study of a second antibody candidate which targets PSGL-1 and could have activity in the treatment of inflammatory bowel diseases such as Crohn's.
Sickle cell disease is currently treated using a handful of pharmacological therapies. The only drug specifically approved to treat the underlying pathology in the disease is hydroxyurea, with other drugs used to treat symptoms such as pain.
Another drug in trials for sickle cell is Emmaus Medical's L-glutamine, which is currently in phase III testing and has been designated an orphan drug by the US FDA.
Biotechnology company honored for work in development of clinical drug candidate for the potential treatment of vaso-occlusive crisis of sickle cell disease
GlycoMimetics, Inc., a clinical-stage biotechnology company developing a new class of glycobiology-based therapies for a broad range of indications, announced today that the William E. Proudford Sickle Cell Fund presented the company with its 2012 Unsung Hero Award last night. The award was presented during the organization's 7th Annual "Rockin' the Red" Fundraiser which was held at the Legg Mason World Headquarters, in Baltimore, Maryland.
The Unsung Hero Award is bestowed to individuals and organizations that have helped to raise awareness about sickle cell disease and sickle cell trait. Each year, a different segment of the community is chosen in an effort to emphasize that a community-wide effort is needed to help combat sickle cell disease. Previous Unsung Hero awardees have included United States Senator Thomas Carper, Maryland State Delegate Shirley Nathan-Pulliam, Dr. Sophie Lanzkron with Johns Hopkins, and Ms. Jean R. Wadman with Nemours duPont Hospital for Children.
"We are extremely honored to be recognized for our research of treatment options for people living with sickle cell disease by such a remarkable organization," said Chief Executive Officer Rachel K. King. "Receiving this award bolsters our company's efforts to advance our lead drug candidate, GMI-1070, which is currently in a Phase 2 clinical trial of patients experiencing sickle cell crisis. We hope this drug can make a difference for people living with sickle cell disease."
GlycoMimetics is studying vaso-occlusive crisis (VOC) of sickle cell disease, one of the most debilitating effects of sickle cell disease, where changes in blood protein cause red blood cells to become rigid and stick inside small blood vessels, causing blockages in blood flow and pain. Researchers are studying the potential of GlycoMimetics' lead drug candidate, GMI-1070, to treat VOC by reducing the cell adhesion and inflammation that is believed to contribute to blood flow blockages.
Enrollment complete in Gamida Cell’s NiCord Phase I/II trial for hematological problems
NiCord is in development as an experimental treatment for a series of indications that potentially could be cured with a bone marrow transplantation including hematological malignancies (blood cancer), sickle cell disease, thalassemia, severeautoimmune diseases and metabolic diseases. The clinical trial announced today (clinicaltrials.gov identifier NCT01221857) is studying NiCord as an alternative investigational treatment for hematological malignancies (HM). A combined total of 11 patients were transplanted at Duke University Medical Center and at Loyola University Medical Center. Dr. Mitchell E. Horwitz of Duke University Medical Center is the principal investigator. Final results of the Phase I/II study are expected within 6 months. The company is also actively enrolling for a Phase I/II study of NiCord as an experimental treatment for sickle cell, a genetic blood disease (clinicaltrials.gov identifier NCT01590628).
NiCord is an expanded cell graft derived from an entire unit of umbilical cord blood enriched with stem cells. NiCord was developed based on Gamida Cell's proprietary NAM technology. As the Phase I/II trial for HM is a first in man safety andefficacy study, for this stage, NiCord was transplanted with a second un-manipulated cord blood unit in a double cord blood configuration.
Steelers' Clark launches sickle cell campaign
safety Ryan Clark is teaming up with a Pittsburgh hospital to help raise awareness for those who suffer from sickle cell blood disorder.
Clark and UPMC launched the ''Cure League'' on Tuesday, hoping to bring more attention to sickle cell disease. Clark is one an estimated 2 million Americans who suffer from the disorder. He had to have his spleen removed after the disease was aggravated playing at high elevation in Denver in 2007. He eventually lost 35 pounds and was forced to miss the rest of the season. He will travel but not play for the Steelers on Sunday night when they hit the road to face the Broncos to open the 2012 campaign.
This initiative focuses on education, donations and support that will enable researchers to learn how to provide better care and hopefully help lead to a cure.
On the Web: www.CureLeague.org.
Video Resources
Schedule of Free CDC 2012 Webinars
“Public Health Webinar Series on Hemoglobinopathies”
Hosted by: The Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities (NCBDDD), Centers for Disease Control and Prevention (CDC) 4th Thursday of every month from 2:00PM – 3:00PM ET The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
Hemoglobinopathy Webinars are archived at http://scinfo.org
To Join The Webinar
Copy this address and paste it into your web browser: https://www.livemeeting.com/
Copy and paste the required meeting ID: 84QK2D and click “join”.
First Time Users: To save time before the meeting, check your system to make sure it is ready to use Microsoft Office Live Meeting. To hear the presentation you must call in to the number below.
For Audio
Dial 1-877-953-6706 and enter participant code: 9706616
If you are unable to join us on the internet for viewing, you are welcome to call in for audio access only. Participants outside the United States must be able to access 800 numbers to the US. Otherwise, please RSVP the location and number of participants for alternative international conference line access.
9/27: Improving Quality of Care for Sickle Cell Pediatrics in the Emergency Department Dr. James Moses, Boston Medical Center
10/25: Strategies from the Field – Data Collection and Harmonization CDC’s Division of Blood Disorders and RuSH Project States
November/December: --- No Webinars---
See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-
New Web Resource
NHS Sickle Cell and Thalassaemia Screening Programme Training Modules
Standards are key in the delivery of an expert antenatal and newborn screening programme. The NHS Sickle Cell and Thalassaemia Screening Programme has invested in a laboratory module where laboratory personnel can participate in online training which tests them on the basic heterozygous conditions which must be detected by the antenatal screening programme.
The module has been designed to incorporate the standards of the Screening Programme’s Laboratory Handbook
See all the publications at http://sct.screening.nhs.uk/
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Articles in the Medical Literature for September
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Sickle Cell News for August 2012
September is Sickle Cell Month – see all the events at the end of the newsletter Living Well with Sickle CellForty-eight-year-old Ayoola Olajide is living well with sickle cell anaemia (SS). Olajide, the Editor of African Sickle Cell News and World Report, and author of Menace In My Blood- my affliction with sickle cell anaemia is married to a woman with the AA genotype (without the SS trait) and they have three boys (AS, without anaemia but are carriers). Olajide, a journalist, is on a crusade to address the genetic disorder through aggressive public enlightenment.. He said Nigerians with sickle cell could live well into old age if they have the right information and others could avoid having children with the condition by making the right choices. http://www.ngrguardiannews.com/index.php?option=com_content&view=article&id=96379:living-well-with-sickle-cell-anaemia-for-48-years&catid=44:natural-health&Itemid=599 Affordable Care Act (ACA) by the United States Supreme Court - SCDAA Summary at http://www.sicklecelldisease.org/index.cfm?page=chief-medical-officer-newsThe Sickle Cell Disease Association of America, Inc. (SCDAA) applauds the upholding of many of the provisions of the Affordable Care Act (ACA) by the United States Supreme Court. The ACA represents a significant victory for individuals with chronic diseases such as sickle cell disease. Fear of moving from your pediatric doctor to an adult doctor because you may not have insurance may be unnecessary under the ACA. How does the ACA protect you as an individual living with sickle cell disease? SCDAA wants to point out how ACA can improve the healthcare of people with sickle cell disease. 1. Insurers can no longer deny coverage to anyone with a chronic or pre-existing health condition such as sickle cell disease. Children and adults are now able to get insurance that covers treatment of their illnesses.2. Lifetime caps on coverage have been removed and insurers have to set annual limits on essential health services at a minimum of $750,000. What does this mean? This means that health insurance companies cannot deny coverage to anyone with a chronic health condition such as sickle cell disease. Furthermore, your health insurance company can no longer set a limit on how much of your health care costs they will pay forever, this is known as "lifetime cap". Currently, the limit on how much they will pay for your heath care in each year has been set at $750,000. The long-term goal of ACA is to eliminate annual limits that are currently practiced by insurance companies from imposing any annual limits at all. SCDAA will closely monitor and report to you and our member organizations as to the progress the ACA law makes in removing these annual limits completely. Video ResourcesNew CDC Videos PostedTranslating Research to Policy Dr. Shawn Bediako, University of Maryland, Baltimore County at mms://realaudio.service.emory.edu/SOM/PA/PLATT/SICKLE/CDCBediako.wmvSchedule of Free CDC 2012 Webinars “Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities (NCBDDD), Centers for Disease Control and Prevention (CDC) 4th Thursday of every month from 2:00PM – 3:00PM ET The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists. Hemoglobinopathy Webinars are archived at http://scinfo.org To Join The Webinar Copy this address and paste it into your web browser: https://www.livemeeting.com/cc/cdc/join Copy and paste the required meeting ID: 84QK2D and click “join”. First Time Users: To save time before the meeting, check your system to make sure it is ready to use Microsoft Office Live Meeting. To hear the presentation you must call in to the number below. For Audio Dial 1-877-953-6706 and enter participant code: 9706616 If you are unable to join us on the internet for viewing, you are welcome to call in for audio access only. Participants outside the United States must be able to access 800 numbers to the US. Otherwise, please RSVP the location and number of participants for alternative international conference line access. 9/27: Improving Quality of Care for Sickle Cell Pediatrics in the Emergency Department Dr. James Moses, Boston Medical Center 10/25: Strategies from the Field – Data Collection and Harmonization CDC’s Division of Blood Disorders and RuSH Project States November/December: --- No Webinars---See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-resources/cdc-webinars-hemoglobinopathies-and-public-healthNew Web ResourceNHS Sickle Cell and Thalassaemia Screening Programme Training ModulesStandards are key in the delivery of an expert antenatal and newborn screening programme. The NHS Sickle Cell and Thalassaemia Screening Programme has invested in a laboratory module where laboratory personnel can participate in online training which tests them on the basic heterozygous conditions which must be detected by the antenatal screening programme.The module has been designed to incorporate the standards of the Screening Programme’s Laboratory Handbook See all the publications at http://sct.screening.nhs.uk/ Articles in the Medical Literature for August1. Br J Haematol. 2012 Aug 28. doi: 10.1111/bjh.12019. [Epub ahead of print]High dose vitamin D therapy for chronic pain in children and adolescents with sickle cell disease: results of a randomized double blind pilot study.Osunkwo I, Ziegler TR, Alvarez J, McCracken C, Cherry K, Osunkwo CE, Ofori-Acquah SF, Ghosh S, Ogunbobode A, Rhodes J, Eckman JR, Dampier C, Tangpricha V.SourceAflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta, Atlanta, GA, USA; Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.AbstractWe report results of a pilot study of high-dose vitamin D in sickle cell disease (SCD). Subjects were given a 6-week course of oral high-dose cholecalciferol (4000-100 000 IU per week) or placebo and monitored prospectively for a period of six months. Vitamin D insufficiency and deficiency was present at baseline in 82·5% and 52·5% of subjects, respectively. Subjects who received high-dose vitamin D achieved higher serum 25-hydroxyvitamin D, experienced fewer pain days per week, and had higher physical activity quality-of-life scores. These findings suggest a potential benefit of vitamin D in reducing the number of pain days in SCD. Larger prospective studies with longer duration are needed to confirm these effects.© 2012 Blackwell Publishing Ltd.
PMID: 22924607 [PubMed - as supplied by publisher]
Related citations
2. ScientificWorldJournal. 2012;2012:949535. Epub 2012 Aug 1.Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management.Ballas SK, Kesen MR, Goldberg MF, Lutty GA, Dampier C, Osunkwo I, Wang WC, Hoppe C, Hagar W, Darbari DS, Malik P.SourceCardeza Foundation and Department of Medicine, Thomas Jefferson University, 1015 Walnut Street, Philadelphia, PA 19107, USA.AbstractThe sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.PMCID: PMC3415156 Free Article
PMID: 22924029 [PubMed - in process]
Related citations
3. Blood. 2012 Aug 24. [Epub ahead of print]Sickle cell pain: a critical reappraisal.Ballas SK, Gupta K, Adams-Graves P.SourceCardeza Foundation, Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadephia, PA, United States;AbstractSickle cell pain includes three types: acute recurrent painful crises, chronic pain syndromes and neuropathic pain. The acute painful crisis is the hallmark of the disease and the most common cause of hospitalization and treatment in the Emergency Department. It evolves through four phases: prodromal, initial, established and resolving. Each acute painful episode is associated with inflammation that worsens with recurrent episodes, often culminating in serious complications and organ damage such as acute chest syndrome, multi-organ failure and sudden death. Three pathophysiologic events operate in unison during the prodromal phase of the crisis: vaso-occlusion, inflammation and nociception. Aborting the acute painful episode at the prodromal phase could potentially prevent or minimize tissue damage. Our hypothesis is that managing these events with hydration, anti-inflammatory drugs, aggressive analgesia and possibly vasodilators could abort the crisis and prevent or minimize further damage. Chronic pain syndromes are associated with or accompany avascular necrosis and leg ulcers. Neuropathic pain is not well studied in patients with sickle cell disease but has been modeled in the transgenic sickle mouse. Management of sickle cell pain should be based on its own pathophysiologic mechanisms rather than borrowing guidelines from other non-sickle pain syndromes.
PMID: 22923496 [PubMed - as supplied by publisher]
Related citations
4. Chest. 2012 Aug 20. doi: 10.1378/chest.12-0611. [Epub ahead of print]The impact of Sickle Cell Disease on exercise capacity in children.Chaudry12 RA, Bush1 A, Rosenthal1 M, Crowley2 S.AbstractABSTRACTBACKGROUND: Little is known about pulmonary vascular complications in children with Sickle Cell Disease (SCD). We hypothesised that transfer factor (DLco) may be used as a surrogate for the size of the pulmonary vascular bed, and that pulmonary vascular abnormalities in SCD children may limit exercise capacity.METHODS: 50 stable SCD patients aged10 to-18 years and 50 healthy controls matched for race and age were recruited. Incremental ergometer cardiopulmonary exercise testing (CPET) was performed using respiratory mass spectrometry (RMS) for exhaled gas analysis. A rebreathing manoeuvre was used to measure functional residual capacity (FRC), effective pulmonary blood flow (Qpeff) and DLCO, and helium dilution was used to calculate minute ventilation (VE), oxygen consumption (VO2) and carbon dioxide production (CO2).RESULTS: In the 89 evaluable subjects, there were no ventilatory differences between SCD and controls. Qpeff was consistently 15-20% greater in SCD than controls at all stages but Dlco corrected for both surface area and haemoglobin was only about 7-10% greater in SCD at all stages As a result the Dlco/Qpeff ratio was considerably lower in SCD at all stages. Arteriovenous oxygen content difference was about one third less in SCD at all stages.CONCLUSIONS: Contrary to our hypothesis, failure to maintain a sufficient Qpeff to compensate for anaemia led to exercise limitation. The ratio of Pulmonary capillary blood volume to flow is reduced throughout, implying subtle pulmonary vascular disease; however this was not a factor limiting exercise.1Royal Brompton Hospital, London, United Kingdom.2St Georges' Hospital, London, United Kingdom.Corresponding author's details (also author for reprint requests): Dr Mark Rosenthal, Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London SW3 6NP Email: M. Rosenthal@rbht.nhs.uk.
PMID: 22922408 [PubMed - as supplied by publisher]
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5. Blood. 2012 Aug 22. [Epub ahead of print]Impact of hydroxyurea on clinical events in the BABY HUG trial.Thornburg CD, Files BA, Luo Z, Miller ST, Kalpatthi R, Iyer R, Seaman P, Lebensburger J, Alvarez O, Thompson B, Ware RE, Wang WC.SourceDuke University Medical Center, Durham, NC, United States;AbstractBABY HUG was a Phase III multicenter, randomized, double-blind placebo-controlled clinical trial of hydroxyurea in infants (beginning at 9-18 months of age) with sickle cell anemia (SCA). An important secondary objective of this study was to compare clinical events between the hydroxyurea and placebo groups. One hundred and ninety-three subjects were randomized to hydroxyurea (20 mg/kg/day) or placebo; there were 374 patient-years of on-study observation. Hydroxyurea was associated with statistically significantly lower rates of initial and recurrent episodes of pain, dactylitis, ACS and hospitalization; even infants who were asymptomatic at enrollment had less dactylitis as well as fewer hospitalizations and transfusions if treated with hydroxyurea. Despite expected mild myelosuppression, hydroxyurea was not associated with an increased risk of bacteremia or serious infection. These data provide important safety and efficacy information for clinicians considering hydroxyurea therapy for very young children with SCA. This clinical trial is registered with the NIH (NCT00006400, www.clinicaltrials.gov).
PMID: 22915643 [PubMed - as supplied by publisher]
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6. Adv Skin Wound Care. 2012 Sep;25(9):420-428.Sickle Cell Disease and Leg Ulcers.Ladizinski B, Bazakas A, Mistry N, Alavi A, Sibbald RG, Salcido R.SourceBarry Ladizinski, MD, BS • Clinical Research Fellow • Duke University Medical Center • Durham, North Carolina Andrea Bazakas, BS • Clinical Trials Assistant II • Duke University Medical Center • Durham, North Carolina Nisha Mistry, MD, BSc, FRCPC(Derm), DABD • Community Dermatologist • Mississauga, Ontario, Canada Afsaneh Alavi, MD, FRCPC(Derm) • Dermatologist and Wound Care Consultant • Women's College Hospital • Toronto, Ontario, Canada R. Gary Sibbald, BSc, MD, Med, FRCPC(Med Derm), MACP, FAAD, MAPWCA • Professor of Public Health and Medicine • University of Toronto • Toronto, Ontario, Canada • Director • International Interprofessional Wound Care Course & Masters of Science in Community Health (Prevention & Wound Care) • Dalla Lana School of Public Health • University of Toronto • President World Union of Wound Healing Societies • Clinical Editor • Advances in Skin & Wound Care • Ambler, Pennsylvania Richard Salcido, MD • Editor-in-Chief • Advances in Skin & Wound Care • Ambler, Pennsylvania • Course Director • Annual Clinical Symposium on Advances in Skin & Wound Care • William Erdman Professor • Department of Rehabilitation Medicine • Senior Fellow • Institute on Aging • Associate • Institute of Medicine and Bioengineering • University of Pennsylvania Health System • Philadelphia, Pennsylvania.AbstractPURPOSE:: To enhance the learner's competence with knowledge of sickle cell disease (SCD) and its relationship to leg ulcers. TARGET AUDIENCE:: This continuing education activity is intended for physicians and nurses with an interest in skin and wound care. OBJECTIVES:: After participating in this educational activity, the participant should be better able to:1. Demonstrate knowledge of SCD-associated leg ulcer pathophysiology, symptomatology, diagnostic testing, and risk factors.2. Apply knowledge of pain management and treatment options for SCD-associated leg ulcers to patient care scenarios. ABSTRACT: Sickle cell disease is a genetic disorder of hemoglobin synthesis leading to a deformation of the red blood cell. This disorder is associated with painful, slow-to-heal leg ulcers. This article discusses the wound bed preparation paradigm as a guide to the treatment of sickle cell-associated leg ulcers.
PMID: 22914039 [PubMed - as supplied by publisher]
Sickle Cell News for July 2012.
Sickle cell trait can cause sudden cardiac death in black athletes: Why is this controversial? http://www.eurekalert.org/pub_releases/2012-07/mhif-sct072312.phpWhile some published research has hinted at the connection between the sickle cell trait and sudden cardiac death among young, athletic African-American males, which was initially observed in black military recruits 25 years ago, a new study with the first sizeable patient series definitively confirms this risk for these individuals during competitive sports.The sickle cell trait, for which all U.S. African Americans are tested at birth, affects approximately 8 percent of the population. The Minneapolis Heart Institute Foundation maintains a 32-year-old forensic database, the U.S. Sudden Death in Athletes Registry, which researchers interrogated to determine the frequency, epidemiology and clinical profile of sickle cell trait-related deaths in a large population of competitive athletes for the purposes of this study. The findings from this registry show there is "convincing evidence of a causal relationship between the sickle cell trait and the deaths of young, black competitive athletes, especially football players," says the study's senior author Barry J. Maron, MD, director of the Hypertrophic Cardiomyopathy Center at the Minneapolis Heart Institute Foundation. The study will be published in the October edition of the American Journal of Cardiology, but currently is available online.Prior to this registry study, a lawsuit and previous research prompted the National Collegiate Athletic Association (NCAA) to conduct mandatory screening for the sickle cell trait in all division I athletes prior to their participation in college athletics. As of yet, the NCAA has not expanded to the screening program to division II or III athletes, nor has the association shared its data with the medical community. Of the 2,462 athlete deaths in the U.S. Sudden Death in Athletes Registry, which provides the first and largest published record of athletes who died of sudden cardiac death on an athletic field, 23 occurred in association with the sickle cell trait (ages 12 to 22 years): 21 were male and all were African Americans. The deaths most often occurred in college-aged athletes (19-23 years) during football conditioning drills early in the season, and with those exposed to high environmental temperatures."The registry was initially started by Dr. Maron to help the medical community understand why any athlete would collapse on a field," explains the study's lead author Kevin M. Harris, MD, co-director of the Acute Aortic Dissection Program and director of the echocardiography laboratory at the Minneapolis Heart Institute® at Abbott Northwestern Hospital in Minneapolis. "We decided to assess the connection between the sickle cell trait and sudden death within our large registry," Harris continues. "As a result, we have developed the first sizable series of competitive athletes in whom sickle cell trait was associated with otherwise unexplained sudden, unexpected collapse and death."Maron, who has been assessing cardiovascular-related deaths of young athletes for approximately 35 years, is surprised at the level of skepticism he's witnessed regarding the sickle cell trait as a cause of sudden in young, black athletes, even in the scientific medical community. In the study, the researchers concluded that the sickle cell trait "can be associated with largely unpredictable sudden collapse and death and apparent predilection for African American college football players during conditioning. Understanding the risks, mechanisms, and event triggers of the sickle cell trait may allow lifesaving alterations in training methods to be implemented."In order to implement such lifesaving alterations in training methods, particularly due to the unpredictable nature of sickle cell trait events, there needs to be a greater understanding and acceptance of this lethal connection. "To not acknowledge this link between sickle cell trait and sudden death creates the possibility of a failure to fully protect the athlete community," Maron said. http://www.ajconline.org/article/S0002-9149(12)01531-7/abstract
Texas Children's Center for Global Health celebrates 1-year anniversary of successful sickle cell disease screening program in Angola http://www.marketwatch.com/story/texas-childrens-center-for-global-health-celebrates-1-year-anniversary-of-successful-sickle-cell-disease-screening-program-in-angola-2012-07-19Exactly one year after launching the Angolan Sickle Cell Initiative, Texas Children's Center for Global Health is proud to announce that more than 16,000 babies have been screened for this once-overlooked killer of children in Africa. Encouraged by the First Lady of Angola, and aided by the vision and support of Mr. Ali Moshiri, president, Chevron Africa and Latin America Exploration and Production Company and Dr. Mark Kline, physician-in-chief at Texas Children's Hospital and chairman of pediatrics at Baylor College of Medicine (BCM), the Angolan sickle cell initiative was launched in March 2011 by the Texas Children's Center for Global Health. With financial support from Chevron Corporation, and in collaboration with the Ministry of Health of Angola, this bold initiative quickly reached fruition on July 19, 2011, when babies born at Lucrecia Paim Maternity Hospital in the Luanda province became the first Angolan infants to ever receive newborn screening for sickle cell disease. This landmark program in Angola serves as a bold step forward in their efforts to improve healthcare in the country. "Until last year, no newborn infant was tested for sickle cell disease in Angola," explains Dr. Russell E. Ware, Director of the Texas Children's Center for Global Health and a professor of pediatrics at BCM. "Sadly, many die in the first two years of life from preventable infections related to the disease, because they are never properly diagnosed. But after witnessing the success of our first year screening babies for sickle cell in Luanda, we know we can tip the scales and make a sea change of difference for these babies." Angola has one of the highest child mortality rates in the world, and each year, approximately 2 percent of babies (close to 10,000) are born with sickle cell disease. Of these babies, the majority die before they reach 5 years-old. With proper intervention, almost all babies with sickle cell disease will reach adulthood, as has been proven in the United States, where newborn infants are tested soon after birth and given access to proper treatment. Thanks to the Angolan Sickle Cell Initiative, more than 16,000 babies have already been tested, and several hundred babies with sickle cell disease have been identified. "The goal of the Sickle Cell program is to not only provide screening, diagnosis and care for this neglected population of children, but to also develop a training and education program that will be sustainable from within Angola and easily replicated in countries that need it the most," says Meg Ferris, Ph.D., MPH, administrative director Texas Children's Center for Global Health. Angolan obstetrical nurses have been trained to collect the blood samples, Angolan laboratory technicians have become expert in diagnostic testing, and Angolan pediatric nurses and doctors have learned to provide life-saving medical interventions, all important facets to making the program sustainable from within the country. Over the next 12 months, the program will expand to a second Angolan province to screen even more babies and save more lives. In addition, plans are in the works to expand the program into more countries in Africa and South America. For more information http://globalhealth.texaschildrens.org/ . Further information on the Sickle Cell Disease Screening Program can be accessed here: http://www.youtube.com/watch?v=06bmu2HcCGo&list=PL46A8079C258A67D0&index=3 Conference in Bahrain http://www.gulf-daily-news.com/NewsDetails.aspx?storyid=334648
HUNDREDS of patients and doctors are expected to attend a major conference on sickle cell disease next month at the Gulf Air Club, Salmabad. The Bahrain Society for Sickle Cell Anaemia Patients Care is organising the annual event to spread awareness about the disease.This year's meeting follows a spate of deaths among sickle cell sufferers, with nine patients dying in the space of less than three weeks from June 28 to July 19.The society has so far registered 17 sickle cell deaths this year and hopes to promote its ID card initiative during the meeting on August 1, which starts at 9pm and is expected to be attended by Health Minister Sadiq Al Shehabi and other officials.It is pushing for a scheme in which sickle cell patients are issued ID cards listing details such as blood type, CPR number and other health-related information so they receive proper treatment. The gathering will feature speeches by society members and a special film showcasing the work of sickle cell activist Jehad Rabia, who died last month from the disease.She was among several Bahraini patients who died at Salmaniya Medical Complex (SMC), forcing health authorities to respond to a spate of deaths. "A short film of Jehad's work with the society will be showcased along with another Bahraini film titled Wabel, that highlights the stages of life of a sickle cell patient," said society member Samah Hussain. She said she hoped the fact that it fell in Ramadan would encourage more people to take part in the event."We want more people to know about the disease and those living with it," she added. After several deaths of sickle cell patients in a short period, Mr Al Shehabi this month admitted errors had been made in the treatment of sufferers. He ordered officials to come up with a comprehensive plan to tackle the problem and treat patients.Campaigners have blamed inadequate facilities at SMC and shortage of Intensive Care Unit beds for sickle cell deaths, although officials maintain they have tried their best to provide patients with the best care. The GDN reported on July 20 that as a temporary measure it was planning to treat male sickle cell patients at the Ebrahim Khalil Kanoo Community Medical Centre in Salmaniya.According to ministry figures, 18,000 sickle cell patients receive treatment at SMC. However, society members say the total number of carriers of the disease, excluding those being treated, is about 65,000. A BD2.5 million, four-storey facility that will treat all patients with blood diseases including sickle cell is now being built.The 90-bed facility expected to open early next year will treat all patients with blood diseases, but should be of particular help to those suffering from sickle cell disease. Also see http://www.gulf-daily-news.com/NewsDetails.aspx?storyid=334370Video
ResourcesNew CDC Videos PostedNew Posting : Dr. Lanetta Jordan’s presentation on “Sickle Cell Trait – What Every CBO Needs To Know” at mms://realaudio.service.emory.edu/SOM/PA/PLATT/SICKLE/CDCJordan.wmv2012-05-24 - Dr. Paula Tanabe, Duke UniversityStreaming Video: mms://realaudio.service.emory.edu/SOM/PA/PLATT/SICKLE/CDCertreat.wmvPDF of Presentation Handout: Sickle Cell Disease and Emergency Department Use2012-04-26 - Dr. Kathryn Hassell, University of Colorado Denver Streaming Video: View RecordingPDF of Presentation Handout: Sickle Cell – Adult Providers NetworkSchedule of Free CDC 2012 Webinars “Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities (NCBDDD), Centers for Disease Control and Prevention (CDC) 4th Thursday of every month from 2:00PM – 3:00PM ET The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists. Hemoglobinopathy Webinars are archived at http://scinfo.org To Join The Webinar Copy this address and paste it into your web browser: https://www.livemeeting.com/cc/cdc/join Copy and paste the required meeting ID: 84QK2D and click “join”. First Time Users: To save time before the meeting, check your system to make sure it is ready to use Microsoft Office Live Meeting. To hear the presentation you must call in to the number below. For Audio Dial 1-877-953-6706 and enter participant code: 9706616 If you are unable to join us on the internet for viewing, you are welcome to call in for audio access only. Participants outside the United States must be able to access 800 numbers to the US. Otherwise, please RSVP the location and number of participants for alternative international conference line access. 7/26: -Canciled 8/23:
Translating Research to Policy Dr. Shawn Bediako, University of Maryland, Baltimore County 9/27: Improving Quality of Care for Sickle Cell Pediatrics in the Emergency Department Dr. James Moses, Boston Medical Center 10/25: Strategies from the Field – Data Collection and Harmonization CDC’s Division of Blood Disorders and RuSH Project States November/December: --- No Webinars---See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-resources/cdc-webinars-hemoglobinopathies-and-public-healthNew Web ResourceThe Sickle Cell Society - http://www.sicklecellsociety.org Newsletter for July. See the news about the Atlanta Blood Disorders conference on page 9 by Iyamide Thomas.http://issuu.com/communityeventsuk/docs/sickle_cell_society_newsletter_july_2012_pages_1-2?mode=window&backgroundColor=%23222222 NHS- UK Sickle Cell Acute Painful Episode Guideline PublicationYou may be interested to know that the clinical guidelines for Sickle Cell Acute Painful Episode were published on Wednesday 27th June and are now available on the NICE website at http://www.nice.org.uk/CG143 You can find the consultation comments and responses on this page: http://guidance.nice.org.uk/CG/Wave24/6 If you have any queries on the Institute’s guideline development process or the progress of a specific guideline, please do not hesitate to contact me.Thank you for your contributions to the development of these guidelines.Many thanks and kind regards, Laura DoneganiClinical Guidelines Coordinator National Institute for Health and Clinical ExcellenceLevel 1A | City Tower | Piccadilly Plaza | Manchester M1 4BT | United Kingdom
Tel: 44 (0)161 870 3147 | Fax: 44 (0)207 061 9755Web: http://nice.org.uk NHS Sickle Cell and Thalassaemia Screening Programme Statement on HbA2 analysis The work that the NHS Sickle Cell and Thalassaemia Screening Programme has been doing with UK NEQAS has shown biases between different analytical platforms for HbA2 [1]. These findings are obviously of concern when there is a national cut off for the diagnosis of beta thalassaemia carriers. The NHS Sickle Cell and Thalassaemia Screening programme is working with the international bodies of the International Federation of Clinical Chemistry and Laboratory Medicine (IFCC ), the International Council for Standardization in Haematology (ICSH) and the World Health Organisation (WHO), who have also recognised the problem and are working with manufacturers. An international standard is in the early stage of preparation and a statement on the use of the current WHO standard is awaited. Haematology laboratories in the UK should be aware of these problems and ensure they have optimised their methods as much as possible. When considering replacement purchases, haematology laboratories should review all available evidence including the UK NEQAS report. In the meantime laboratories should consider including a statement on this issue in their local risk assessment. If you have any queries about this issue, please contact the NHS Sickle Cell and Thalassaemia Screening Programme Centre by telephone on 020 7848 6634, or email at the following address: haemscreening@kcl.ac.uk 1. Batterbee, H., et al., 2010. Evaluation of UK NEQAS (H) Hb A2 and related performance data, Watford: UK NEQAS (H). The full report is available at http://sct.screening.nhs.uk/cms.php?folder=2419#fileid11145Working to Improve Sickle Cell Healthcare (WISCH) Website at http://www.nichq.org/our_projects/sickle_cell_landing_page.htmlWorking to Improve Sickle Cell Healthcare (WISCH) is NICHQ’s portfolio of projects focused on improving the quality of care for individuals with sickle cell disease across the lifespan. These projects include the Sickle Cell Disease Newborn Screening Program and the Sickle Cell Disease Treatment Demonstration Program, both of which are funded through the Health Resources and Services Administration (HRSA).Free Sickle Cell Pain Fact sheet at http://www.inthefaceofpain.com/content/uploads/2011/09/factsheet_Sickle_Cell.pdf Articles in the Medical Literature for July1. J Pediatr Health Care. 2012 Jul 18. [Epub ahead of print]A Transition Pilot Program for Adolescents With Sickle Cell Disease.Hankins JS, Osarogiagbon R, Adams-Graves P, McHugh L, Steele V, Smeltzer MP, Anderson SM.AbstractINTRODUCTION: Transition from pediatric to adult care is challenging for adolescents with chronic illnesses, including those with sickle cell disease (SCD). We describe a pilot program created to facilitate transition from pediatric to adult care by helping adolescents with SCD identify an adult medical home.METHODS: We investigated the feasibility of this program by evaluation of overall participation, satisfaction, and acceptance. A secondary objective was to compare the proportion of adolescents who fulfilled a first appointment with an adult hematologist among participants and nonparticipants.RESULTS: During the first 18 months of the program, 83 adolescents were invited and 34 (41%) agreed to participate; 25 (74%) completed their first visit within 3 months after leaving the pediatric program, compared with 16 of 49 (33%) of nonparticipants (p = .0002). Overall, 41 of 83 adolescents (49%) completed an appointment with an adult SCD program, regardless of program participation, in contrast with 11 of 75 adolescents (15%) who did so during the 18 months before the program was created (p < .0001).DISCUSSION: This transition pilot program was feasible, and most adolescent participants with SCD established an adult medical home.Copyright © 2012 National Association of Pediatric Nurse Practitioners. Published by Mosby, Inc. All rights reserved.
PMID: 22819193 [PubMed - as supplied by publisher]
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2. Transfus Clin Biol. 2012 Jul 18. [Epub ahead of print]Relevance of alloimmunization in haemolytic transfusion reaction in sickle cell disease.Noizat-Pirenne F.SourceÉtablissement français du sang Île-de-France, hôpital Henri-Mondor, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil, France; Inserm U955, hôpital Henri-Mondor, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil, France.AbstractTransfusion remains a key treatment in sickle cell disease. The occurrence of a delayed haemolytic transfusion reaction is not rare and is a life-threatening event. The main cause of delayed haemolytic transfusion reaction is production of alloantibodies against red blood cell antigens. The high rate of alloimmunization in sickle cell disease patients is mainly due to the differences of red blood groups between patients of African descent, and the frequently Caucasian donors. From an immuno-haematological point of view, delayed haemolytic transfusion reaction in sickle cell disease patients has specific features: classical antibodies known to be haemolytic can be encountered, but otherwise non significant antibodies, autoantibodies and antibodies related to partial and rare blood groups are also frequently found in individuals of African descent. In some cases, there are no detectable antibodies. As alloimmunization remains the main cause of delayed haemolytic transfusion reaction, it is extremely important to promote blood donation by individuals of African ancestry to make appropriate blood available.Copyright © 2012 Elsevier Masson SAS. All rights reserved.
PMID: 22818360 [PubMed - as supplied by publisher]
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3. J Thromb Haemost. 2012 Jul 20. doi: 10.1111/j.1538-7836.2012.04861.x. [Epub ahead of print]Hydroxyurea is associated with reduction of hypercoagulability markers in sickle cell anemia.Colella MP, de Paula EV, Conran N, Machado-Neto JA, Annicchino-Bizzacchi JM, Costa FF, Saad ST, Traina F.SourceHematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas, São Paulo, Brazil.AbstractSeveral lines of evidence indicate that sickle cell anemia (SCA) is a state of increased hemostatic activation [1-4]. SCA patients present an elevated rate of thrombotic complications including pulmonary embolism, stroke and pregnancy-related venous thromboembolism [5]. Hydroxyurea (hydroxycarbamide) is currently one of the main pillars of SCA management, improving both clinical complications and mortality in SCA [6] and has several well-defined beneficial effects that could contribute to an inhibition of the hypercoagulability state in SCA. These include a reduction of phosphatidylserine exposure by erythrocytes, reduction of adhesive properties of erythrocytes and leukocytes, endothelial activation, nitric oxide depletion, platelet activation and adhesive properties [7-9]. © 2012 International Society on Thrombosis and Haemostasis.© 2012 International Society on Thrombosis and Haemostasis.
PMID: 22817333 [PubMed - as supplied by publisher]
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4. Med Sci Sports Exerc. 2012 Jul 17. [Epub ahead of print]ACSM and CHAMP Summit on SCT: Mitigating Risks for Warfighters and Athletes.O'Connor FG, Bergeron MF, Cantrell J, Connes P, Harmon KG, Ivy E, Kark J, Klossner D, Lisman P, Meyers BK, O'Brien K, Ohene-Frempong K, Thompson AA, Whitehead J, Deuster PA.Source1Department of Military and Emergency Medicine, Uniformed Services University of the Health Sciences, Bethesda, MD; 2National Institute for Athletic Health and Performance, Sanford USD Medical Center, Sioux Falls, SD; 3Department of Preventive Medicine and Biometrics, Uniformed Services University of the Health Sciences, Bethesda, MD; 4UMR Inserm 665, Universite Antilles-Guyane, Pointe-a-Pitre, Guadeloupe; 5Departments of Family Medicine and Orthopaedics and Sports Medicine, University of Washington, Seattle, WA; 6National Heart, Lung, and Blood Institute, Bethesda, MD; 7Hematology-Oncology Division, Howard University Hospital, Washington, DC; 8National Collegiate Athletic Association, Indianapolis, IN; 9Division of Preventive Medicine, Walter Reed Army Institute of Research, Silver Spring, MD; 10Madigan Army
Sickle Cell News for June 2012
June 19 World Sickle Cell Day celebrated around the world
Read the stories:
http://www.bet.com/news/health/2012/06/19/world-sickle-cell-day-is-today.html
http://www.insidetoronto.com/community/health/article/1379275--malvern-s-taibu-spreads-awareness-on-sickle-cell-disease
http://www.businessdayonline.com/NG/index.php/component/content/article/126-health/39934-sickle-cell-disease-how-well-are-people-informed
http://www.plenglish.com/index.php?option=com_content&task=view&id=518051&Itemid=1
Chicago Woman Cured Of Sickle Cell Disease at 33 Chicagoan Ieshea Thomas is the first Midwest patient to receive a successful stem cell transplant to cure her sickle cell disease without chemotherapy in preparation for the transplant. University of Illinois Hospital & Health Sciences System physicians performed the procedure using medication to suppress her immune system and one small dose of total body radiation right before the transplant. The transplant technique is relatively uncommon and is a much more tolerable treatment for patients with aggressive sickle cell disease who often have underlying organ disease and other complications, says Dr. Damiano Rondelli, professor of medicine at UIC, who performed Thomas's transplant. The procedure initially allows a patient's own bone marrow to coexist with that of the donor. Since the patient's bone marrow is not completely destroyed by chemotherapy or radiation prior to transplant, part of the immune defense survives, lessening the risk of infection. The goal is for the transplanted stem cells to gradually take over the bone marrow’s role to produce red blood cells -- normal, healthy ones. Thomas, 33, had her first sickle cell crisis when she was just 8 months old. Her disease became progressively worse as an adult, particularly after the birth of her daughter. She has spent most of her adult life in and out of hospitals with severe pain and has relied on repeated red blood cell transfusions. Her sickle cell disease also caused bone damage requiring two hip replacements. "I just want to be at home with my daughter every day and every night,"said Thomas, who depends on family to help care for her daughter during her frequent hospitalizations. This type of stem cell transplant is only possible for patients who have a healthy sibling who is a compatible donor. Thomas' sister was a match and agreed to donate blood stem cells through a process called leukapheresis. Several days prior to leukapheresis, Thomas' sister was given drugs to increase the number of stem cells released into the bloodstream. Her blood was then processed through a machine that collects white cells, including stem cells. The stem cells were frozen until the transplant. Last Nov. 23, four bags of frozen stem cells were delivered to the hospital's blood and marrow transplant unit. One by one, the bags were thawed and hung on an IV pole for infusion into Thomas. The procedure took approximately one hour. Her 13-year-old daughter, Miayatha, was at her bedside. Six months after the transplant, Thomas is cured of sickle cell disease and no longer requires blood transfusions. "The donor cells have taken over completely, and blood tests show no sickle cell disease," said Rondelli, director of the blood and marrow transplant program at UI Hospital. Thomas continues to take medication to prevent rejection of the donor stem cells. About 25 adults have received a similar chemotherapy-free stem cell transplant for sickle cell disease in recent years at the National Institutes of Health in Bethesda, Md. Approximately 85 percent have been cured. "Sickle cell disease is devastating -- both emotionally and physically," said Dr. Dennis Levinson, a private rheumatologist in Chicago and clinical associate professor of medicine at UIC, who has taken care of Thomas for the past 16 years. "I've been terribly frustrated with Ieshea's disease over the years, and I've cared for many other sickle cell patients who have died." Levinson says the stem cell transplant provides new hope for patients who often live day-to-day on painkillers and who are often misunderstood by clinicians. As the former chief of medicine at the now closed Michael Reese Hospital, he said he has cared for many patients with sickle cell anemia and was determined to seek out the best treatment option for Thomas. Sickle cell disease primarily affects people of African descent. It is an inherited defect of the red blood cells that causes them to be shaped like a crescent, or sickle. These abnormal cells deliver less oxygen to the body's tissues and can result in severe pain, stroke and organ damage. Approximately one in every 500 African Americans born in the U.S. has sickle cell disease. The disease affects 80,000 Americans of different ethnic backgrounds. The University of Illinois Hospital & Health Sciences System provides comprehensive, life-long care for pediatric and adult sickle cell patients. For more information, visit http://www.hospital.uillinois.edu. [Editor's note: Video report available at http://youtu.be/E89xXeeby-A and photos available at http://newsphoto.lib.uic.edu/v/bone_marrow_transplant/.] Children Stories needed from around the worldThe authors of Hope & Destiny: The Patient and Parent's Guide to Sickle Cell Disease and Sickle Cell Trait are planning a children’s version and would like parents to send a short 1 page story written by their school aged child with sickle cell disease that would encourage a child with sickle cell in another country.. Any selected story will be published anonymously with the parent’s permission. There is no compensation, only the joy of being published: Send stories with your child’s age and initials to aplatt@emory.edu New Web resourceNational Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention2011 Annual Report on Sickle Cell Disease and Thalassemia at http://www.cdc.gov/ncbddd/AboutUs/blood-disorders-sicklecell.html
Video Resources
New CDC Videos Posted
2012-05-24 - Dr. Paula Tanabe, Duke University
Streaming Video: mms://realaudio.service.emory.edu/SOM/PA/PLATT/SICKLE/CDCertreat.wmv
PDF of Presentation Handout: Sickle Cell Disease and Emergency Department Use
2012-04-26 - Dr. Kathryn Hassell, University of Colorado Denver
Streaming Video: View Recording
PDF of Presentation Handout: Sickle Cell – Adult Providers NetworkSchedule of Free CDC 2012 Webinars
“Public Health Webinar Series on Hemoglobinopathies” Hosted by: The Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities (NCBDDD), Centers for Disease Control and Prevention (CDC) 4th Thursday of every month from 2:00PM – 3:00PM ET The purpose of this webinar series is to offer a hemoglobinopathies learning collaborative platform for providers, consumers, educators, and scientists.
This month’s webinar will take place on Thursday, June 28th from 2:00pm – 3:00pm ET, featuring
Dr. Lanetta Jordan’s presentation on “Sickle Cell Trait – What Every CBO Needs To Know” Hemoglobinopathy Webinars are archived at http://scinfo.org
To Join The Webinar Copy this address and paste it into your web browser: https://www.livemeeting.com/cc/cdc/join
Copy and paste the required meeting ID: 84QK2D and click “join”.
First Time Users: To save time before the meeting, check your system to make sure it is ready to use Microsoft Office Live Meeting. To hear the presentation you must call in to the number below.
For Audio Dial 1-877-953-6706 and enter participant code: 9706616
If you are unable to join us on the internet for viewing, you are welcome to call in for audio access only. Participants outside the United States must be able to access 800 numbers to the US. Otherwise, please RSVP the location and number of participants for alternative international conference line access.
7/26: Improved Survival of Children and Adolescents with Sickle Cell Disease Dr. Charles Quinn, Cincinnati Children's Hospital Medical Center
8/23: Translating Research to Policy Dr. Shawn Bediako, University of Maryland, Baltimore County
9/27: Improving Quality of Care for Sickle Cell Pediatrics in the Emergency Department Dr. James Moses, Boston Medical Center
10/25: Strategies from the Field – Data Collection and Harmonization CDC’s Division of Blood Disorders and RuSH Project States
November/December: --- No Webinars---
See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-resources/cdc-webinars-hemoglobinopathies-and-public-healthFree ASH webinars http://hematology.org/Meetings/Webinars/6832.aspx
Pain in Sickle cell disease and Stroke, Renal Disease, and Treatment with Hydroxyurea in Adults with Sickle Cell Disease
Articles in the Medical Literature for June
1. Patient Educ Couns. 2012 May 31. [Epub ahead of print]
Use of social support during communication about sickle cell carrier status.
Bradford L, Roedl SJ, Christopher SA, Farrell MH.
Source
Center for Patient Care and Outcomes Research, Medical College of Wisconsin, Milwaukee, WI, USA.
Abstract
OBJECTIVE:
To examine the use of social support behaviors by primary care providers during delivery of positive newborn screening results for Sickle Cell Anemia carrier status.
METHODS:
Transcripts from 125 primary care providers who conveyed Sickle Cell Anemia carrier status to standardized parents were content analyzed using categories derived from Cutrona and Suhr's social support taxonomy. Frequencies and cross-tabulation matrices were calculated to study providers' social support utilization.
RESULTS:
Results showed most primary care providers (80%) incorporate social support behaviors into delivery of Sickle Cell Anemia carrier results and most frequently employed social network (61.6%) and informational support (38.4%) behaviors. Providers used tangible aid (8%), esteem (1.6%), and emotional support (9.6%) behaviors less frequently.
CONCLUSION:
Cutrona and Suhr's taxonomy may be a useful tool for assessing supportive communication during the delivery of Sickle Cell Anemia carrier status and could be incorporated into population scale assessments of communication quality assurance.
PRACTICE IMPLICATIONS:
Primary care providers may need training in how to adapt supportive behaviors to parents' needs during communication of Sickle Cell Anemia carrier status. They also may benefit from specific training about how to use esteem and emotional support.
Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.
PMID: 22658247 [PubMed - as supplied by publisher]
Related citations
2. Anemia. 2012;2012:428137. Epub 2012 May 14.
FK228 Analogues Induce Fetal Hemoglobin in Human Erythroid Progenitors.
Makala L, Di Maro S, Lou TF, Sivanand S, Ahn JM, Pace BS.
Source Department of Pediatrics, Georgia Health Sciences University, Augusta, GA 30912, USA.
Abstract
Fetal hemoglobin (HbF) improves the clinical severity of sickle cell disease (SCD), therefore, research to identify HbF-inducing agents for treatment purposes is desirable. The focus of our study is to investigate the ability of FK228 analogues to induce HbF using a novel KU812 dual-luciferase reporter system. Molecular modeling studies showed that the structure of twenty FK228 analogues with isosteric substitutions did not disturb the global structure of the molecule. Using the dual-luciferase system, a subgroup of FK228 analogues was shown to be inducers of HbF at nanomolar concentrations. To determine the physiological relevance of these compounds, studies in primary erythroid progenitors confirmed that JMA26 and JMA33 activated HbF synthesis at levels comparable to FK228 with low cellular toxicity. These data support our lead compounds as potential therapeutic agents for further development in the treatment of SCD.
PMCID: PMC3359661 Free PMC Article
PMID: 22655179 [PubMed - in process]
Related citations
3. Am J Respir Crit Care Med. 2012 Jun 7. [Epub ahead of print]
A Novel Molecular Signature for Elevated Tricuspid Regurgitation Velocity in Sickle Cell Disease.
Desai AA, Zhou T, Ahmad H, Zhang W, Mu W, Trevino S, Wade MS, Raghavachari N, Kato GJ, Peters-Lawrence MH, Thiruvoipati T, Turner K, Artz N, Huang Y, Patel AR, Yuan JX, Gordeuk VR, Lang RM, Garcia JG, Machado RF.
Source
Medicine, University of Illinois at Chicago, Chicago, Illinois, United States.
Abstract
RATIONALE:
Pulmonary hypertension defined by right heart catheterization and an increased tricuspid regurgitation jet velocity (TRV≥2.5m/s) on transthoracic echocardiography both independently confer increased mortality in sickle cell disease.
OBJECTIVE:
We explored the utility of peripheral blood mononuclear cell-derived gene signatures as biomarkers for an elevated tricuspid regurgitation jet velocity in sickle cell disease. Methods &
MEASUREMENTS:
Twenty-seven sickle patients underwent echocardiography and of peripheral blood mononuclear cell isolation for expression profiling and 112 sickle patients were genotyped for SNPs.
MAIN RESULTS:
Genome-wide gene and miRNA expression profiles were correlated against tricuspid regurgitation velocity, yielding 631 transcripts and 12 miRNAs. Support vector machine analysis identified a 10-gene signature including GALNT13 (encoding polypeptide N-acetylgalactosaminyltransferase 13) that discriminates patients with and without increased TRV with 100% accuracy. This finding was then validated in a cohort of sickle patients without (n=10) and with pulmonary hypertension (n=10, 90% accuracy). Increased tricuspid regurgitation velocity-related miRNAs revealed strong in silico binding predictions of miR-301a to GALNT13 corroborated by microarray analyses demonstrating an inverse correlation between their expression. A genetic association study comparing patients with an elevated (n=49) versus normal (n=63) tricuspid regurgitation velocity revealed 5 significant SNPs within GALNT13 (p<0.005), four trans-acting (p<2.1e-07) and one cis-acting (p=0.6e-04) expression quantitative trait loci (eQTLs) upstream of the adenosine-A2B receptor gene (ADORA2B).
CONCLUSION:
These studies validate the clinical utility of genomic signatures as potential biomarkers and highlight ADORA2B and GALNT13 as potential candidate genes in sickle-associated elevated tricuspid regurgitation velocity.
PMID: 22679008 [PubMed - as supplied by publisher]
Related citations
4. Hemoglobin. 2012 Jun 19. [Epub ahead of print]
Newborn Screening Shows a High Incidence of Sickle Cell Anemia in Central India.
Jain DL, Sarathi V, Upadhye D, Gulhane R, Nadkarni AH, Ghosh K, Colah RB.
Source Department of Paediatrics, Government Medical College , Nagpur-440009 , India.
Abstract
There is limited data on the incidence of sickle cell anemia in Central India; we therefore conducted a study to estimate the incidence of this disease in Central India. Mothers who delivered a live baby at the Government Medical College, Nagpur, India were screened for the presence of the sickle cell hemoglobin {Hb S: [β6 (A3) Glu →Val, GAG >GTG]} using the solubility test within 48 hours of delivery. Infants of mothers who showed the presence of Hb S then underwent Hb analysis by high performance liquid chromatography (HPLC). A total of 8243 mothers was screened, 1178 of whom were positive. One thousand, one hundred and sixty-two infants of mothers with a positive solubility test underwent Hb analysis by HPLC; 530 infants were normal, while 536 were heterozygous for Hb S (sickle cell trait), 88 babies were homozygous for Hb S (sickle cell anemia), while another eight babies had other Hb abnormalities. The incidence of sickle cell anemia was highest in the Scheduled caste group (1:50). We concluded that the incidence of sickle cell anemia is high in central India.
PMID: 22712682 [PubMed - as supplied by publisher]
Related citations
5. Infect Genet Evol. 2012 Jun 12. [Epub ahead of print]
The emergence and maintenance of sickle cell hotspots in the Mediterranean.
Penman BS, Gupta S, Buckee CO.
Source
Department of Zoology, University of Oxford, UK.
Abstract
Genetic disorders of haemoglobin (haemoglobinopathies), including the thalassaemias and sickle cell anemia, abound in historically malarious regions, due to the protection they provide against death from severe malaria. Despite the overall spatial correlation between malaria and these disorders, inter-population differences exist in the precise combinations of haemoglobinopathies observed. Greece and Italy present a particularly interesting case study: their high frequencies of beta thalassaemia speak to a history of intense malaria selection, yet they possess very little of the strongly malaria protective mutation responsible for sickle cell anemia, despite historical migrational links with Africa where high frequencies of sickle cell occur. Twentieth century surveys of beta thalassaemia and sickle cell in Greece, Sicily and Sardinia have revealed striking sickle cell 'hotspots' - places where the frequency of sickle cell approaches that seen in Africa while neighboring populations remain relatively sickle cell free. It remains unclear how these hotspots have been maintained over time without sickle cell spreading throughout the region. Here we use a metapopulation model to show that (i) epistasis between the alpha and beta forms of thalassaemia can restrict the spread of sickle cell through a network of linked subpopulations, and (ii) the emergence of sickle cell hotspots requires relatively low levels of gene flow, but the aforementioned epistasis increases the chances of hotspots forming.
Copyright © 2012. Published by Elsevier B.V.
PMID: 22704979 [PubMed - as supplied by publisher]
Related citations
6. Cytokine. 2012 Jun 14. [Epub ahead of print]
Plasma BDNF and PDGF-AA levels are associated with high TCD velocity and stroke in children with sickle cell anemia.
Hyacinth HI, Gee BE, Adamkiewicz TV, Adams RJ, Kutlar A, Stiles JK, Hibbert JM.
Source Genomics and Hemoglobinopathies Training Program, Department of Microbiology, Biochemistry and Immunology, Morehouse School of Medicine, 720 Westview Drive SW, Atlanta, GA, USA.
Abstract
Sickle cell anemia (SCA) associated cerebrovascular disease includes vascular remodeling, abnormal cerebral blood flow (CBF) and infarction. We studied the relationships between plasma brain derived neurotropic factor (BDNF), platelet derived growth factors (PDGF-AA and -AB/BB) and high trans-cranial Doppler (TCD) velocity as an indication of CBF velocity. Baseline plasma samples from 39 children (19 SCA with abnormal/high TCD [SATCD], 13 SCA with normal TCD [SNTCD] and 7 healthy non-SCA), were assayed for BDNF, PDGF-AA and -AB/BB plus 11 other cytokines. The sensitivity, specificity and usefulness of these biomarkers for stroke prediction was investigated. All subject groups were of similar age and gender distribution. Mean BDNF was significantly higher among SATCD than SNTCD (p=0.004) as was mean PDGF-AA (p=0.001). Similarly, mean PDGF-AA was higher among SCA subjects who developed stroke than those who did not (p=0.012). Elevated BDNF and PDGF-AA were good predictors of the presence of abnormally high CBF velocity and were both associated with severity of anemia. Elevated PDGF-AA predicted risk for stroke development. Stroke incidence and high TCD velocity were associated with elevated BDNF and PDGF-AA. These findings suggest a role for BDNF and PDGF-AA in the patho-physiological mechanism of cerebrovascular disease in SCA.
Copyright © 2012 Elsevier Ltd. All rights reserved.
PMID: 22704695 [PubMed - as supplied by publisher]
Related citations
7. Anemia. 2012;2012:492428. Epub 2012 Jun 4.
Integrating interactive web-based technology to assess adherence and clinical outcomes in pediatric sickle cell disease.
Crosby LE, Barach I, McGrady ME, Kalinyak KA, Eastin AR, Mitchell MJ.
Source
College of Medicine, University of Cincinnati, Cincinnati, OH 45221, USA.
Abstract
Research indicates that the quality of the adherence assessment is one of the best predictors for improving clinical outcomes. Newer technologies represent an opportunity for developing high quality standardized assessments to assess clinical outcomes such as patient experience of care but have not been tested systematically in pediatric sickle cell disease (SCD). The goal of the current study was to pilot an interactive web-based tool, the Take-Charge Program, to assess adherence to clinic visits and hydroxyurea (HU), barriers to adherence, solutions to overcome these barriers, and clinical outcomes in 43 patients with SCD age 6-21 years. Results indicate that the web-based tool was successfully integrated into the clinical setting while maintaining high patient satisfaction (>90%). The tool provided data consistent with the medical record, staff report, and/or clinical lab data. Participants reported that forgetting and transportation were major barriers for adherence to both clinic attendance and HU. A greater number of self-reported barriers (P < .01) and older age (P < .05) were associated with poorer clinic attendance and HU adherence. In summary, the tool represents an innovative approach to integrate newer technology to assess adherence and clinical outcomes for pediatric patients with SCD.
PMCID: PMC3372407 Free PMC Article
PMID: 22701785 [PubMed - in process]
Related citations
8. Ultrasound Med Biol. 2012 Jun 12. [Epub ahead of print]
Stenosis or Hyperperfusion in Sickle Cell Disease - Ultrasound Assessment of Cerebral Blood Flow Volume.
Doepp F, Kebelmann-Betzing C, Kivi A, Schreiber SJ.
Source
Department of Neurology, University Hospital Charité, Berlin, Germany.
Abstract
Increased blood flow velocity (BFV) in basal cerebral arteries measured by transcranial color-coded sonography (TCCS) is a stroke risk factor in sickle cell disease (SCD). Raised BFV may be caused by vessel narrowing or by hyperperfusion. In 44 SCD patients and 14 controls, intracranial arterial BFVs and global cerebral blood flow (CBF) were analyzed by TCCS and extracranial duplex ultrasound, respectively. Magnetic resonance imaging and magnetic resonance angiography were performed in all patients with pathologic intracranial BFV rise. Intracranial BFVs and CBF in SCD were significantly higher than in controls. CBF in SCD correlated with BFV in all intracranial arteries and correlated inversely with age and hemoglobin values. Magnetic resonance angiography failed to demonstrate any stenosis in our SCD patients, thus raised intracranial BFVs must be interpreted as an anemia-dependent cerebral hyperperfusion. These findings suggest that the pathomechanism of stenosis-derived arterio-arterial embolism might be less relevant in SCD-related ischemic stroke, and other factors like small vessel disease or sickle cell-induced microvascular blood clotting have to be considered.
Copyright © 2012 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.
PMID: 22698503 [PubMed - as supplied by publisher]
Related citations
9. Cochrane Database Syst Rev. 2012 Jun 13;6:CD005406.
Fluid replacement therapy for acute episodes of pain in people with sickle cell disease.
Okomo U, Meremikwu MM.
Source
Viral Diseases Programme, Medical Research Council (UK), Atlantic Boulevard, Fajara, Gambia, P.O. Box 273.
Abstract
BACKGROUND:
Treating vaso-occlusive painful crises in people with sickle cell disease is complex and requires multiple interventions. Extra fluids are routinely given as adjunct treatment, regardless of the individual's state of hydration with the aim of slowing or stopping the sickling process and thereby alleviating pain.
OBJECTIVES:
To determine the optimal route, quantity and type of fluid replacement for people with sickle cell disease with acute painful crises.
SEARCH METHODS:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.We also conducted searches of EMBASE (November 2007), LILACS and www.ClinicalTrials.gov (05 January 2010).Date of most recent search of the Group's Haemoglobinopathies Trials Register: 09 February 2012.
SELECTION CRITERIA:
Randomised and quasi-randomised controlled trials that compared the administration of supplemental fluids adjunctive to analgesics by any route in people with any type of sickle cell disease during an acute painful episode, under medical supervision (inpatient, day care or community).
DATA COLLECTION AND ANALYSIS:
No relevant trials have yet been identified.
MAIN RESULTS:
Sixteen trials were identified by the initial search. Of these, 15 were not suitable for inclusion in this review and one study is awaiting further assessment.
AUTHORS' CONCLUSIONS:
Treating vaso-occlusive crises is complex and requires multiple interventions. Extra fluids, generally oral or intravenous, are routinely administered during acute painful episodes to people with sickle cell disease regardless of the individual's state of hydration. Reports of their use during these acute painful episodes do not state the efficacy of any single route, type or quantity of fluid compared to another. However, there are no randomised controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid. This systematic review identifies the need for a multicentre randomised controlled trial assessing the efficacy and possible adverse effects of different routes, types and quantities of fluid administered to people with sickle cell disease during acute painful episodes.
PMID: 22696351 [PubMed - in process]
Related citations
10. Acad Emerg Med. 2012 Jun;19(6):664-72. doi: 10.1111/j.1553-2712.2012.01364.x.
Risk Factors for Increased ED Utilization in a Multinational Cohort of Children With Sickle Cell Disease.
Glassberg JA, Wang J, Cohen R, Richardson LD, Debaun MR.
Source
From the Department of Emergency Medicine (JG, LDR) and the Department of Health Evidence and Policy (JW), Mount Sinai School of Medicine, New York, NY; Pediatrics, Drexel University College of Medicine, Division of Pediatrics, and St. Christopher's Hospital for Children (RC), Philadelphia, PA; and the Departments of Pediatrics and Medicine, Vanderbilt Children's Hospital (MRD), Nashville, TN.
Abstract
ACADEMIC EMERGENCY MEDICINE 2012; 19:664-672 © 2012 by the Society for Academic Emergency Medicine ABSTRACT: Objectives: The objective was to identify clinical, social, and environmental risk factors for increased emergency department (ED) use in children with sickle cell disease (SCD). Methods: This study was a secondary analysis of ED utilization data from the international multicenter Silent Cerebral Infarct Transfusion (SIT) trial. Between December 2004 and June 2010, baseline demographic, clinical, and laboratory data were collected from children with SCD participating in the trial. The primary outcome was the frequency of ED visits for pain. A secondary outcome was the frequency of ED visits for acute chest syndrome. Results: The sample included 985 children from the United States, Canada, England, and France, for a total of 2,955 patient-years of data. There were 0.74 ED visits for pain per patient-year. A past medical history of asthma was associated with an increased risk of ED utilization for both pain (rate ratio [RR] = 1.28, 95% confidence interval [CI] = 1.04 to 1.58) and acute chest syndrome (RR = 1.60, 95% CI = 1.03 to 2.49). Exposure to environmental tobacco smoke in the home was associated with 73% more ED visits for acute chest syndrome (RR = 1.73, 95% CI = 1.09 to 2.74). Each $10,000 increase in household income was associated with 5% fewer ED visits for pain (RR = 0.95, 95% CI = 0.91 to 1.00, p = 0.05). The association between low income and ED utilization was not significantly different in the United States versus countries with universal health care (p = 0.51). Conclusions: Asthma and exposure to environmental tobacco smoke are potentially modifiable risk factors for greater ED use in children with SCD. Low income is associated with greater ED use for SCD pain in countries with and without universal health care.
© 2012 by the Society for Academic Emergency Medicine.
PMCID: PMC3375948 [Available on 2013/6/1]
PMID: 22687181 [PubMed - in process]
Related citations
Sickle Cell Conferences and Events
July 5-7, 2012 Venetian / Palazzo Hotel Las Vegas - Association of Nigerian Physicians in the Americas “The 18th Annual ANPA Convention & Scientific Assembly will be held July 5-7, 2012 at the Venetian / Palazzo Hotel in Las Vegas, Nevada. The theme for this year’s assembly will be “Righting the Wrong in Sickle Cell Disease”, and “Information Technology in Medical Practice”. The ANPA Annual Meeting features three full-day meetings, providing participants’ knowledge related to the challenges facing minority health care providers in the provision of clinical and therapeutic services for diseases and conditions related to a variety of medical specialties. The meeting will feature member lecturers providing state of the art technological advances that will impact the practice of medicine. The annual convention is of great value to our membership and other health care professionals by providing them with opportunities to keep abreast of developments in various areas of health care delivery and also offering continuing medical education (CME) credits. For more information please visit www.anpa.org or call 913.402.7102. “
September 25 – 29, 2012 The Sickle Cell Disease Association of America 40th Annual Conference Baltimore MD
The conference will be held September 25 – 29, 2012 at the Baltimore Marriott Waterfront Hotel in Baltimore, MD. We promise that this will be one of the most educational and empowering events to take place within the sickle cell community! http://www.sicklecelldisease.org/index.cfm?page=annual-convention
May Sickle Cell News
June 19 World Sickle Cell Day
In the year 2008, the General Assembly of the United Nations adopted a resolution which determines sickle cell disease as a public health problem and one of the world’s foremost genetic disease, requiring heightened awareness and activism, diagnosis and management. The result of the resolution was that June 19th was declared as World Sickle Cell to increase awareness of the condition all over the world.
The World Health Organization (WHO) has started work to promote a world wide agenda to address hemoglobin dysfunctions.
WHO has made a commitment to:
Recognize that sickle cell disease is a major health issue.
Increase awareness of the world community regarding sickle cell disease.
Eliminate harmful and wrong prejudices associated with sickle cell disease.
Urges member countries where sickle cell disease is a public health problem to establish health programs at the national level and operate specialized centers for sickle cell disease and facilitate access to treatment.
Promote satisfactory access to medical services to people affected with sickle cell disease.
Provide technical support to all countries to prevent and manage sickle cell disease.
Promote and help research to improve the lives of people affected with sickle cell disease.
The World Sickle Cell day is celebrated across the globe with special emphasis in African Nations and Asia. The celebrations include a press, media campaigns, music shows, cultural activities, and talk shows.
The main emphasis is hence on educating medical professionals, care givers, and associated personnel about prevention, research, and resources to minimize the complications due to sickle cell disease. Hence June 19th is devoted mainly to spread awareness, through talks, seminars, pamphlets, literature and consultations.
In honor of World Sickle Cell day, Scell Media is offering afree edition of SICKLE CELL news at http://www.scdjournal.com/free.html
NIH SELECTS 11 CENTERS OF EXCELLENCE IN PAIN EDUCATIONNIH Pain Consortium partners with selected health professional schools The National Institutes of Health Pain Consortium has selected 11 health professional schools as designated Centers of Excellence in Pain Education (CoEPEs). The CoEPEs will act as hubs for the development, evaluation, and distribution of pain management curriculum resources for medical, dental, nursing and pharmacy schools to enhance and improve how health care professionals are taught about pain and its treatment. Twenty institutes, centers and offices at NIH are involved in the consortium. "Virtually all health professionals are called upon to help patients suffering from pain," said NIH Director Francis S. Collins, M.D., Ph.D. "These new centers will translate current research findings about pain management to fill what have been recognized as gaps in curricula so clinicians in all fields can work with their patients to make better and safer choices about pain treatment." The new Centers of Excellence in Pain Education were selected by the NIH Pain Consortium <http://painconsortium.nih.gov/index.html> after a contract solicitation process and review. The awardees are the University of Washington, Seattle; the University of Pennsylvania Perelman School of Medicine, Philadelphia; Southern Illinois University, Edwardsville; the University of Rochester, N.Y.; the University of New Mexico, Albuquerque; the Harvard School of Dental Medicine, Boston; the University of Alabama at Birmingham; the Thomas Jefferson University School of Medicine, Philadelphia; the University of California, San Francisco; the University of Maryland, Baltimore; and the University of Pittsburgh. Many of the new CoEPEs will build curricula across several of their health professional schools. Chronic pain affects approximately 100 million Americans, costing up to $635 billion in medical treatment and lost productivity, and producing immeasurable suffering for people of every age. Yet, pain treatment is not taught extensively in many health professional schools, and clinical approaches can be inconsistent. The curricula developed by the CoEPEs will advance the assessment, diagnosis, and safe treatment of a wide variety of pain conditions while minimizing the abuse of opioid pain relievers. They will include multiple case-based scenarios, many taught in video or electronic formats popularly used in contemporary academic settings. Types of pain of particular interest to the NIH Pain Consortium are rehabilitation pain, arthritis and musculoskeletal pain, neuropathic pain, and headache pain. In addition, the curricula will teach about the pathophysiology and pharmacology of pain and its treatment, the latest research in complementary and integrative pain management, factors that contribute to both under- and over-prescribing of pain medications, and how pain manifests itself differently by gender, in children, in older adults and in diverse populations. "While opioid pain medications have improved the quality of life for millions who suffer from pain, they can also produce harmful consequences, including addiction," said NIDA Director Nora D. Volkow, M.D., a member of the consortium's executive committee. "These new CoEPEs can help prevent negative outcomes by designing curricula that promote appropriate screening and management of chronic pain patients, along with education about the risks of prescription drug abuse." NIH supports the full spectrum of pain research from basic understanding of pain mechanisms through translation of discoveries into treatments and prevention strategies. In FY 2011, NIH supported $386 million in research focused on chronic pain, not including the related diseases that often cause chronic pain, such as cancer, arthritis, diabetes, and stroke. The details of individual pain-focused grants are publicly available on the NIH RePORTER website <http://projectreporter.nih.gov/reporter.cfm>. Enhancing education of pain care professionals was highlighted in the June 2011 Institute of Medicine report, "Relieving Pain in America: A Blueprint for Transforming Prevention, Care, Education, and Research." About the NIH Pain Consortium: The NIH Pain Consortium was established to enhance pain research and promote collaboration among researchers across the many NIH Institutes and Centers that have programs and activities addressing pain. Its goals include the development of a comprehensive and forward-thinking pain research agenda for the NIH; to identify key opportunities in pain research within NIH and the scientific community; to increase visibility for pain research; and to pursue the pain research agenda through Public-Private partnerships. For more information on the Pain Consortium, visit <http://painconsortium.nih.gov/index.html>.
New Book Resource
"Living With Sickle Cell Disease: The Struggle to Survive" A Memoir by Author Judy Gray Johnson with Leroy Williams Jr.
This is a well written self- published life account of growing up with sickle cell disease in the 1960s and the problems with treatment into 2012. There are many accounts of unfavorable Emergency department encounters commonly shared by those with sickle cell disease. The book is factual and medically accurate.
http://www.judygrayjohnson.com/home.html
http://www.blacknews.com/news/living_with_sickle_cell_disease_judy_gray_johnson101.shtml
Video Resources
Schedule of Free CDC 2012 Webinars
6/28: Sickle Cell Trait – What Every CBO Needs to KnowDr. Lanetta Jordan, Memorial Regional Hospital
7/26: Improved Survival of Children and Adolescents with Sickle Cell DiseaseDr. Charles Quinn, Cincinnati Children's Hospital Medical Center
8/23: Translating Research to PolicyDr. Shawn Bediako, University of Maryland, Baltimore County
9/27: Improving Quality of Care for Sickle Cell Pediatrics in the Emergency DepartmentDr. James Moses, Boston Medical Center
10/25: Strategies from the Field – Data Collection and HarmonizationCDC’s Division of Blood Disorders and RuSH Project States
November/December:--- No Webinars---
See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-resources/cdc-webinars-hemoglobinopathies-and-public-health
Free ASH webinars http://hematology.org/Meetings/Webinars/6832.aspx
Pain in Sickle cell disease and Stroke, Renal Disease, and Treatment with Hydroxyurea in Adults with Sickle Cell Disease
Articles in the Medical Literature for May
1. Cochrane Database Syst Rev. 2012 May 16;5:CD004344.
Treatment for avascular necrosis of bone in people with sickle cell disease.
Martí-Carvajal AJ,Solà I,Agreda-Pérez LH.
Source
Universidad de Carabobo and Iberoamerican Cochrane Network, Valencia, Edo. Carabobo, Venezuela.
Abstract
BACKGROUND:
Avascular necrosis of bone is a frequent and severe complication of sickle cell disease and its treatment is not standardised.
OBJECTIVES:
To determine the impact of any surgical procedure compared with other surgical interventions or non-surgical procedures, on avascular necrosis of bone in people with sickle cell disease in terms of efficacy and safety.
SEARCH METHODS:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Additional trials were sought from the reference lists of papers identified by the search strategy.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 21 February 2012.
SELECTION CRITERIA:
Randomised clinical trials comparing specific therapies for avascular necrosis of bone in people with sickle cell disease.
DATA COLLECTION AND ANALYSIS:
Each author independently extracted data and assessed trial quality. Since only one trial was identified, meta-analysis was not possible.
MAIN RESULTS:
One trial (46 participants) was eligible for inclusion. After randomisation eight participants were withdrawn, mainly because they declined to participate in the trial. Data were analysed for 38 participants at the end of the trial. After a mean follow up of three years, hip core decompression and physical therapy did not show clinical improvement when compared with physical therapy alone using the score from the original trial (an improvement of 18.1 points for those treated with intervention therapy versus an improvement of 15.7 points with control therapy). There was no significant statistical difference between groups regarding major complications (hip pain, relative risk (RR) 0.95 (95% confidence interval (CI) 0.56 to 1.60; vaso-occlusive crises, RR 1.14 (95% CI 0.72 to 1.80); and acute chest syndrome, RR 1.06 (95% CI 0.44 to 2.56)). This trial did not report results on mortality or quality of life.
AUTHORS' CONCLUSIONS:
We found no evidence that adding hip core decompression to physical therapy achieves clinical improvement in people with sickle cell disease with avascular necrosis of bone compared to physical therapy alone. However, we highlight that our conclusion is based on one trial with high attrition rates. Further randomised controlled trials are necessary to evaluate the role of hip-core depression for this clinical condition. Endpoints should focus on participants' subjective experience (e.g. quality of life and pain) as well as more objective 'time-to-event' measures (e.g. mortality, survival, hip longevity). The availability of participants to allow adequate trial power will be a key consideration for endpoint choice.
PMID: 22592696 [PubMed - in process]
Related citations
2. J Pediatr Hematol Oncol. 2012 May 10. [Epub ahead of print]
Single-session Biofeedback-assisted Relaxation Training in Children With Sickle Cell Disease.
Myrvik MP,Campbell AD,Butcher JL.
Source
*Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI †Department of Pediatrics, University of Michigan, Ann Arbor, MI.
Abstract
Sickle cell disease (SCD) pain remains difficult to manage. This pilot study evaluated single-session biofeedback-assisted relaxation training (BART) for SCD pain in children. Ten participants (mean=12.1 y) completed a 1-hour BART session using thermal biofeedback and home practice. Participants demonstrated changes in peripheral body temperature after the training session (d=1.08) and at 6-week follow-up (d=0.97) relative to their baseline visit. Reductions in patient-reported pain frequency were found after completing BART. Health-related quality of life and pain-related disability improvements were observed; however, effect sizes were small to minimal. Single-session BART may be a promising, complementary approach to medical management of pediatric SCD pain.
PMID: 22584783 [PubMed - as supplied by publisher]
Related citations
3. BMC Blood Disord. 2012 May 14;12(1):5. [Epub ahead of print]
Zinc finger nucleases for targeted mutagenesis and repair of the sickle-cell disease mutation: An insilico study.
Wayengera M.
Abstract
ABSTRACT: BACKGROUND: Sickle cell disease (or simply, SCD) is an inherited hemoglobinopathy which is mostly prevalent among persons of African descent. SCD results from a monogenic (Hemoglobin, beta) point-mutation (substitution of the base Adenine with Thymine at position six) that leads to replacement of the amino acid glutamic acid (E) with valine (V). Management of SCD within resource-poor settings is largely syndromic, since the option of cure offered by bone-marrow transplantation (BMT) is risky and unaffordable by most affected individuals. Despite previous reports of repair and inhibition of the sickle beta-globin gene and messenger ribonucleic acids (mRNAs), respectively in erythrocyte precursor cells via gene-targeting using an oligomer-restriction enzyme construct and either ribozyme- or RNA-DNA chimeric oligonucleotides (or simply third strand binding), gene-therapy to treat SCD still remains largely preclinical. In the wake of the advances in target- gene- mutagenesis and repair wrought by zinc finger nuclease (ZFN) technology, it was hypothesized that SCD may be cured by the same. The goal of this study thus, was constructing a database of zinc finger arrays (ZFAs) and engineering ZFNs, that respectively bind and cleave within or around specific sequences in the sickle hemoglobin, beta (betaS) gene. Methods and results First, using the complete 1606 genomic DNA base pair (bp) sequences of the normal hemoglobin-beta (betaA) chain gene, and the ZiFiT-CoDA-ZFA software preset at default, 57 three-finger arrays (ZFAs) that specifically bind 9 base-pair sequences within the normal hemoglobin-beta chain, were computationally assembled. Second, by serial linkage of these ZFAs to the Flavobacterium okeanokoites endonuclease Fok Ifour ZFNs with unique specificity to >24 bp target-sequences at the genomic contextual positions 82, 1333, 1334, and 1413 of the betaA chain-gene were constructed in-silico. Third, localizing the pointmutation of SCD at genomic contextual position 69-70-71- bp (a position corresponding to the 6th codon) of the betaA chain-gene, inspired the final design of five more ZFNs specific to >24 bp target-sequences within the 8,954 bp that are genomically adjacent to the 5' end of the betaA chain-gene. CONCLUSIONS: This set of 57 ZFAs and 9 ZFNs offers us gene-therapeutic precursors for the targeted mutagenesis and repair of the SCD mutation or genotype.
Free Article
PMID: 22583379 [PubMed - as supplied by publisher]
Related citations
4. J Pain Symptom Manage. 2012 May 11. [Epub ahead of print]
Detecting the Emergence of Chronic Pain in Sickle-Cell Disease.
Hollins M,Stonerock GL,Kisaalita NR,Jones S,Orringer E,Gil KM.
Source
Department of Psychology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
Abstract
CONTEXT:
Sickle-cell disease (SCD) is an inherited hematological disease marked by intense pain. Early in life the pain is episodic, but it becomes increasingly chronic in many cases. Little is known about this emergence of a chronic pain state.
OBJECTIVES:
The goal of this study was to determine whether adult SCD patients whose pain is still largely episodic show early signs of the disturbed pain processing (hyperalgesia and increased temporal summation) and cognition (hypervigilance and catastrophizing) that are characteristic of a chronic pain state.
METHODS:
SCD patients (n=22) and healthy controls (n=52) received noxious pressure stimulation for up to three minutes and periodically reported pain intensity and unpleasantness on 0-10 scales, allowing the rate of pain increase (temporal summation) to be determined. Pain intensity discrimination also was measured, and attitudes toward pain were assessed.
RESULTS:
There were no overall differences in pain ratings or temporal summation between patient and control groups. However, patients' experimental pain ratings tended to increase with age and those reporting a history of very painful episodes showed particularly rapid temporal summation of pain unpleasantness. Patients were significantly impaired at discriminating intensities of noxious stimulation. Patients were more hypervigilant than controls, but catastrophizing was elevated only during pain episodes.
CONCLUSION:
Most SCD patients whose pain remits entirely between episodes are not in a chronic pain state, but some-those who are older and have a history of highly painful episodes-appear to be transitioning into it. These early signs of disturbed processing may aid clinicians seeking to forestall disease progression.
Copyright © 2012 U.S. Cancer Pain Relief Committee. Published by Elsevier Inc. All rights reserved.
PMID: 22579409 [PubMed - as supplied by publisher]
Related citations
5. J Pediatr Psychol. 2012 May 7. [Epub ahead of print]
Evaluating the Protective Role of Racial Identity in Children with Sickle Cell Disease.
Lim CS,Welkom JS,Cohen LL,Osunkwo I.
Source
Department of Clinical & Health Psychology, University of Florida, Department of Psychology, Georgia State University and Children's Healthcare of Atlanta, Emory University School of Medicine.
Abstract
OBJECTIVE:
This study examined whether racial identity moderates the relation between pain and quality of life (QOL) in children with sickle cell disease (SCD).
METHODS:
100 children 8-18 years of age with SCD participated during a regularly scheduled medical visit. Children completed questionnaires assessing pain, QOL, and regard racial identity, which evaluates racial judgments.
RESULTS:
Analyses revealed that regard racial identity trended toward significance in moderating the pain and physical QOL relation, (β = -0.159, t(93) = -1.821, p = 0.07), where children with low pain and high regard reported greater physical QOL than children with low pain and low regard. Regard racial identity did not moderate the relation between pain and other QOL dimensions. Pain significantly predicted all dimensions of QOL and regard racial identity significantly predicted social QOL.
CONCLUSIONS:
Racial identity may be important to consider in future research examining QOL in children with SCD.
PMID: 22566667 [PubMed - as supplied by publisher]
Related citations
6. Pediatr Blood Cancer. 2012 May 4. doi: 10.1002/pbc.24179. [Epub ahead of print]
Indications and complications of transfusions in sickle cell disease.
Smith-Whitley K,Thompson AA.
Source
Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. whitleyk@email.chop.edu.
Abstract
Red cell transfusion remains an important part of the management of acute and chronic complications in SCD. The ongoing and emerging uses of transfusions in SCD may have significant benefits; however, the potential complications of transfusions also deserve careful consideration. In this report we review current indications for transfusions, transfusion complications, modifications of transfusion practices to mitigate risk, and potential considerations to improve transfusion outcomes. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
PMID: 22566388 [PubMed - as supplied by publisher]
Related citations
7. Blood. 2012 May 4. [Epub ahead of print]
Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management.
Yazdanbakhsh K,Ware RE,Noizat-Pirenne F.
Source
Laboratory of Complement Biology, New York Blood Center, New York, New York, United States;
Abstract
Red blood cell transfusions have reduced morbidity and mortality for patients with sickle cell disease (SCD). Transfusions can lead to erythrocyte alloimmunization, however, with serious complications for the patient including life-threatening delayed hemolytic transfusion reactions (DHTRs) and difficulty in finding compatible units, which can cause transfusion delays. In this review, we will discuss the risk factors associated with alloimmunization with emphasis on possible mechanisms that can trigger DHTR in SCD, and describe the challenges in transfusion management of these patients, including opportunities and emerging approaches for minimizing this life-threatening complication.
PMID: 22563085 [PubMed - as supplied by publisher]
Related citations
8. Cold Spring Harb Perspect Med. 2012 May;2(5):a011825.
Hematopoietic stem cell transplantation in thalassemia and sickle cell anemia.
Lucarelli G,Isgrò A,Sodani P,Gaziev J.
Source
International Center for Transplantation in Thalassemia and Sickle Cell Anemia-Mediterranean Institute of Hematology, Policlinic of the University of Rome Tor Vergata, Tor Vergata, Italy.
Abstract
The globally widespread single-gene disorders β-thalassemia and sickle cell anemia (SCA) can only be cured by allogeneic hematopoietic stem cell transplantation (HSCT). HSCT treatment of thalassemia has substantially improved over the last two decades, with advancements in preventive strategies, control of transplant-related complications, and preparative regimens. A risk class-based transplantation approach results in disease-free survival probabilities of 90%, 84%, and 78% for class 1, 2, and 3 thalassemia patients, respectively. Because of disease advancement, adult thalassemia patients have a higher risk for transplant-related toxicity and a 65% cure rate. Patients without matched donors could benefit from haploidentical mother-to-child transplantation. There is a high cure rate for children with SCA who receive HSCT following myeloablative conditioning protocols. Novel non-myeloablative transplantation protocols could make HSCT available to adult SCA patients who were previously excluded from allogeneic stem cell transplantation.
PMCID: PMC3331690Free PMC Article
PMID: 22553502 [PubMed - in process]
Related citations
9. Sultan Qaboos Univ Med J. 2012 May;12(2):177-83. Epub 2012 Apr 9.
Adult Sickle Cell Disease: A Five-year Experience of Intensive Care Management in a University Hospital in Oman.
Tawfic QA,Kausalya R,Al-Sajee D,Burad J,Mohammed AK,Narayanan A.
Source
Departments of Anaesthesia & Intensive Care and.
Abstract
OBJECTIVES:
Sickle cell disease (SCD) is an inherited disease caused by an abnormal type of haemoglobin. It is one of the most common genetic blood disorders in the Gulf area, including Oman. It may be associated with complications requiring intensive care unit (ICU) admission. This study investigated the causes of ICU admission for SCD patients.
METHODS:
This was a retrospective analysis of all adult patients ≥12 years old with SCD admitted to Sultan Qaboos University Hospital (SQUH) ICU between 1st January 2005 and 31st December 2009.
RESULTS:
A total number of 49 sickle cell patients were admitted 56 times to ICU. The reasons for admission were acute chest syndrome (69.6%), painful crises (16.1%), multi-organ failure (7.1%) and others (7.2%). The mortality for SCD patients in our ICU was 16.1%. The haemoglobin (Hb) and Hb S levels at time of ICU admission were studied as predictors of mortality and neither showed statistical significance by Student's t-test. The odds ratio, with 95% confidence intervals, was used to study other six organ supportive measures as predictors of mortality. The need for inotropic support and mechanical ventilation was a good predictor of mortality. While the need for non-invasive ventilation, haemofiltration, blood transfusions and exchange transfusions were not significant predictors of mortality.
CONCLUSION:
Acute chest syndrome is the main cause of ICU admission in SCD patient. Unlike other supportive measures, the use of inotropic support and/or mechanical ventilation is an indicator of high mortality rate SCD patient.
PMCID: PMC3327564Free PMC Article
Sickle Cell Conferences and Events
Friday, June 8,2012. Albany NY - The NYS Perinatal Association holds an annual education conference This year Ed Kloza MS CGC will be a plenary speaker on Friday, June 8,2012. This is an opportunity to learn more about NIPD and have an opportunity to have questions answered.Conference registration offers the option of a one-day registration. The Role of Non-Invasive Prenatal Genetic Testing Using Maternal Plasma— Ed Kloza, MS, CGC, Senior Re-search Coordinator, Institute for Preventive Medicine & Medical Screening, Women & Infants Hospital, Providence, RI
On Line Conference brochure and registration: www.nysperinatal.org
SO CAL CELL-A-BRATES THE 4TH WORLD SICKLE CELL DAY
WHAT: A United Nations resolution made June 19 World Sickle Cell Day, to promote awareness because “Sickle Cell is a Global Health Problem!” The African American Blood Drive and Bone Marrow Registry for Sickle Cell Disease Awareness and Friends will cell-a-brate while bring awareness to our So Cal Community.
WHEN: Saturday,June 16, 2012 from 11A.M. – 3 P.M.
WHERE:LRH Community Center Auditorium, [8039 S. Vermont, Los Angeles CA 90044] Contact Nita Thompson atAA4SCDAwareness@aol.com or (323) 750-1087 to register for this event and to sign up to donate blood. We look forward to your presence and participation in World Sickle Cell Day 2012.
July 5-7, 2012 Venetian / Palazzo Hotel Las Vegas - Association of Nigerian Physicians in the Americas“The 18th Annual ANPA Convention & Scientific Assembly will be held July 5-7, 2012 at the Venetian / Palazzo Hotel in Las Vegas, Nevada. The theme for this year’s assembly will be “Righting the Wrong in Sickle Cell Disease”, and “Information Technology in Medical Practice”. The ANPA Annual Meeting features three full-day meetings, providing participants’ knowledge related to the challenges facing minority health care providers in the provision of clinical and therapeutic services for diseases and conditions related to a variety of medical specialties. The meeting will feature member lecturers providing state of the art technological advances that will impact the practice of medicine. The annual convention is of great value to our membership and other health care professionals by providing them with opportunities to keep abreast of developments in various areas of health care delivery and also offering continuing medical education (CME) credits. For more information please visit www.anpa.org or call 913.402.7102. “
September 25 – 29, 2012 The Sickle Cell Disease Association of America 40th Annual Conference Baltimore MD
The conference will be held September 25 – 29, 2012 at the Baltimore Marriott Waterfront Hotel in Baltimore, MD. We promise that this will be one of the most educational and empowering events to take place within the sickle cell community! http://www.sicklecelldisease.org/index.cfm?page=annual-convention
Related citations
Sickle Cell News for March 2012
NIH LAUNCHES CONSUMER-FRIENDLY TIPS SERIES ON COMPLEMENTARY HEALTH PRACTICES A new series of monthly health tips, Time to Talk Tips, will provide consumers with easy-to-read information on complementary health practices. The effort is managed by the National Center for Complementary and Alternative Medicine (NCCAM) at the National Institutes of Health. A resource in NCCAM's Time to Talk campaign, the series highlights specific health topics, such as the safe use of dietary supplements, natural products used for the flu and colds, and mind and body approaches used to manage symptoms of a variety of conditions. The series will include simple tips, such as: Taking vitamin C regularly does not reduce the likelihood of getting a cold but may improve some cold symptoms, and some dietary supplements may interact with medications (prescription or over-the-counter) or other dietary supplements. "An increasing number of consumers and patients use the Internet to answer health questions, yet the information they find can be overwhelming and is not always relevant or credible," said Josephine P. Briggs, M.D., director of NCCAM. "This series will give people evidence-based facts to help them make more informed health care decisions. Health care providers can also provide these tips to their patients who are interested in learning more about complementary health practices." The tips accompany topics found in the NCCAM Clinical Digest, a monthly e-newsletter for health care providers that addresses the state of science on complementary health practices for a variety of health conditions. These same topics will also be discussed in monthly Twitter chats (@NCCAM) < https://twitter.com/#!/nccam>, allowing the public to interact with the center, ask questions, and receive answers in real time. Nearly 40 percent of Americans use some form of complementary health practice, according to the 2007 National Health Interview Survey. Through its Time to Talk campaign, NCCAM encourages patients and providers to talk about the use of complementary health practices by offering tools and resources --such as wallet cards and tip sheets --that are available for free at http://nccam.nih.gov/timetotalk The mission of the National Center for Complementary and Alternative Medicine (NCCAM) is to define, through rigorous scientific investigation, the usefulness and safety of complementary and alternative medicine interventions and their roles in improving health and health care. For additional information, call NCCAM's Clearinghouse toll free (in the U.S.) at 1-888-644-6226, or visit the NCCAM Web site at http://nccam.nih.gov.
SCInfo.org Poster Presentation
Allan Platt1, James Eckman1, Adrya Stembridge1,Talat Khan 2, Lewis L. Hsu 2
1Emory University, Atlanta, GA,2 University of Illinois , Chicago, IL
HYPOTHESIS: The most visits are on webpages for the primary target audience: patients and families with sickle cell.
METHODS: Website usage tracking was added in February 2011, with Google Analytics standard automated technology for off-site web analytics. The measures, which do not track individually identifiable information, are commonly used for commercial websites (Busby,Brown, WAA 2008): Landing Page, Visits, Pages/Visit,Average Time on Site, % New Visits, and Bounce Rate. Website usage from February 1 through December 17, 2011 are reported.
RESULTS: The Sickle Cell Information Center homepage had 21,946 visits, with an average of 3.59 pages per visit and 2 minutes 54 seconds per visit. The 10 most-visited webpages are shown in Table 1.
CONCLUSION: The webpages most frequently visited in the Sickle Cell Information Center website are for the target audiences of patients and families. Continued monitoring of usage statistics will provide a priority list for the Advisory Board to regularly review and ensure that health information is up to date. We will develop mechanisms to keep a current list of sickle cell clinical centers on the website.
REFERENCE: Burby J, Brown A, and WAA Standards Committee. Web Analytics Definitions Vol 1. 2008 www.webanalyticsassociation.org/resource/resmgr/pdf_standards/webanalyticsdefinitionsvol1.pdf
SUPPORT: Website is supported by the Southeast Genetics Collaborative.
Please verify your clinic information athttp://scinfo.org/sickle-cell-clinics-contacts-and-resources and let us know if there are any updates atlewhsu@uic.edu
Online Newsletters
Sickle Cell Society based in London, England latest e-news letter is:
http://issuu.com/communityeventsuk/docs/sickle_cell_societywinter_final?mode=window&backgroundColor=%23222222
AFRICAN SC NEWS & WORLD REPORT at www.scdjournal.com/news-special
1. ObtainFree Special Edition ofSICKLE CELL NEWSatwww.scdjournal.com/free.html
2. Obtain our digital editions, self-help books, memoirs, etc athttp://www.scdjournal.com/scdreader.html
2. Let's give a Talk on sickle cell at your educational/religious institution.register here
2012 INTERNATIONAL PAIN POLICY FELLOWSHIP ANNOUNCED
The Pain & Policy Studies Group is pleased to announce the2012 International Pain Policy Fellowship. Now entering its 6th year, the goal of the Fellowship Program is to improve the availability of opioid analgesics for pain management in developing countries for patients with pain from cancer, HIV/AIDS, or other chronic diseases.
Applications will be accepted from health professionals (e.g. oncologists, AIDS clinicians, pharmacists, pain and palliative care physicians), health care administrators, policy experts, social workers or lawyers from low or middle income countries who have an interest in drug policy advocacy to improve availability of opioid analgesics for pain relief and palliative care. [Please note that Fellowships have previously been awarded to the following countries: Argentina, Armenia, Colombia, Georgia, Guatemala, Jamaica, Kenya, Moldova, Nepal, Nigeria, Panama, Serbia, Sierra Leone, Uganda, and Vietnam. As such, except in special cases, it is unlikely that additional Fellowships will be awarded to applicants from these countries.]
The two-year Fellowship program will include training, mentoring, and an in-country pain policy project. A salary stipend will be given to cover a portion of each fellow’s professional salary as the Fellow must commit to spending approximately 8 hours per week of his/her professional time on the project. Applicants must have a sincere interest in improving access to opioid analgesics for pain management through drug policy advocacy.
Fellows are required to participate in a five-day learner-centered training program at the University of Wisconsin in Madison, Wisconsin from 6-10 August 2012. The training program will cover the relationships between disease, pain, palliative care, and inadequate opioid availability, and will use WHO Guidelines to examine regulatory barriers and resources for evaluating national policy, as well as examples of their use. Each Fellow will be responsible for outlining their drug availability project plan and timeline during the training program in Madison, and then incorporating it into their in-country pain policy project.
The deadline for receipt of completed applications is Friday, 20 April 2012.
This program is supported byLIVESTRONG and directed by the Pain & Policy Studies Group of the University of Wisconsin Comprehensive Cancer Center.
More information, including the application, are attached to this message. These materials are also available atwww.painpolicy.wisc.edu.
Video Resources
New Posting: CDC Webinar 2/23/12:Medical Home Model for Sickle Cell Disease
Dr. Michael DeBaun, Vanderbilt University
mms://realaudio.service.emory.edu/SOM/PA/PLATT/SICKLE/CDCHome.wmv
Schedule of Free CDC 2012 Webinars
4/26: Sickle Cell – Adult Providers NetworkDr. Kathryn Hassell, University of Colorado School of Medicine
5/24: Sickle Cell Disease and Emergency Department UseDr. Paula Tanabe, Duke University School of Nursing
6/28: Sickle Cell Trait – What Every CBO Needs to KnowDr. Lanetta Jordan, Memorial Regional Hospital
7/26: Improved Survival of Children and Adolescents with Sickle Cell DiseaseDr. Charles Quinn, Cincinnati Children's Hospital Medical Center
8/23: Translating Research to PolicyDr. Shawn Bediako, University of Maryland, Baltimore County
9/27: Improving Quality of Care for Sickle Cell Pediatrics in the Emergency DepartmentDr. James Moses, Boston Medical Center
10/25: Strategies from the Field – Data Collection and HarmonizationCDC’s Division of Blood Disorders and RuSH Project States
November/December:--- No Webinars---
See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-resources/cdc-webinars-hemoglobinopathies-and-public-health
John Hopkins - Urban Health Radio Program (UHRP)
"Breaking It Down: Our Health Our Way" Features Sickle Cell talks in February
WOLB 1010 AM Thursdays from 11 a.m. - 12 p.m. Listen live on the web at http://www.hopkinsmedicine.org/diversity_cultural_competence/urban_health_radio_program/
Free ASH webinars http://hematology.org/Meetings/Webinars/6832.aspx
Pain in Sickle cell disease and Stroke, Renal Disease, and Treatment with Hydroxyurea in Adults with Sickle Cell Disease
Articles in the Medical Literature for March
1.
Am J Respir Crit Care Med. 2012 Mar 23. [Epub ahead of print]
Pulmonary Complications of Sickle Cell Disease.
Miller AC,Gladwin MT.
Source
Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States.
Abstract
Sickle cell disease (SCD) is a common monogenetic disorder with high associated morbidity and mortality. The pulmonary complications of SCD are of particular importance, as acute chest syndrome and pulmonary hypertension have the highest associated mortality rates within this population. This manuscript will review the pathophysiology, diagnosis, and treatment of clinically significant pulmonary manifestations of SCD including acute chest syndrome, asthma, and pulmonary hypertension in adult and pediatric patients. Clinicians should be vigilant in screening and treating such co-morbidities in order to improve patient outcomes.
PMID: 22447965 [PubMed - as supplied by publisher]
2.
J Pediatr Health Care. 2012 Mar 23. [Epub ahead of print]
Facilitating Pediatric Patient-Provider Communications Using Wireless Technology in Children and Adolescents With Sickle Cell Disease.
Jacob E,Pavlish C,Duran J,Stinson J,Lewis MA,Zeltzer L.
Abstract
INTRODUCTION:
Use of wireless devices has the potential to transform delivery of primary care services for persons with sickle cell disease (SCD). The study examined text message communications between patients and an advanced practice registered nurse (APRN) and the different primary care activities that emerged with use of wireless technology.
METHODS:
Patients (N = 37; mean age 13.9 ± 1.8 years; 45.9% male and 54.1% female) engaged in intermittent text conversations with the APRN as part of the Wireless Pain Intervention Program. Content analyses were used to analyze the content of text message exchanges between patients and the APRN.
RESULTS:
The primary care needs that emerged were related to pain and symptom management and sickle cell crisis prevention. Two primary care categories (collaborating and coaching), four primary care subcategories (screening, referring, informing, and supporting), and 16 primary care activities were evident in text conversations.
DISCUSSION:
The use of wireless technology may facilitate screening, prompt management of pain and symptoms, prevention or reduction of SCD-related complications, more efficient referral for treatments, timely patient education, and psychosocial support in children and adolescents with SCD.
Copyright © 2012 National Association of Pediatric Nurse Practitioners. Published by Mosby, Inc. All rights reserved.
PMID: 22446036 [PubMed - as supplied by publisher]
3.
Br J Sports Med. 2012 Apr;46(5):325-30.
Sickle cell trait associated with a RR of death of 37 times in national collegiate athletic association football athletes: a database with 2 million athlete-years as the denominator.
Harmon KG,Drezner JA, Klossner D, Asif IM.
Source
Departments of Family Medicine and Sports Medicine and Orthopaedics, 4060 East Stevens Circle, Hall Health Sports Medicine Clinic, University of Washington, Seattle, WA 98195, USA. kharmon@u.washington.edu.
Abstract
Background This study examines sickle cell trait (SCT) as a cause of sudden death in National Collegiate Athletic Association (NCAA) athletes and explores the cost-effectiveness of different screening models. Methods The authors reviewed the cause of all cases of sudden death in NCAA student-athletes from January 2004 through December 2008. The authors also explored the cost-effectiveness of screening for this condition in selected populations assuming that identifying athletes with SCT would prevent death. Results There were 273 deaths and a total of 1 969 663 athlete-participant-years. Five (2%) deaths were associated with SCT. In football athletes, there were 72 (26%) deaths. Of these, 52 (72%) were due to trauma unrelated to sports activity and 20 (28%) were due to medical causes; nine deaths were cardiac (45%), five were associated with SCT (25%). Thirteen of the 20 deaths due to medical causes occurred during exertion; cardiac (6, 46%) SCT associated (5, 39%), and heat stroke unrelated to SCT (2, 15%). All deaths associated with SCT occurred in black Division I football athletes. The risk of exertional death in Division I football players with SCT was 1:827 which was 37 times higher than in athletes without SCT. The cost per case identified varied widely depending on the population screened and the price of the screening test. Conclusions Exertional death in athletes with SCT occurs at a higher rate than previously appreciated. More research is needed to (1) understand the pathophysiology of death in SCT-positive athletes and (2) determine whether screening high-risk populations reduces mortality.
PMID: 22442191 [PubMed - in process]
Related citations
4.
Pediatr Blood Cancer. 2012 Apr;58(4):611-5.
Bone marrow transplant options and preferences in a sickle cell anemia cohort on chronic transfusions.
Hansbury EN,Schultz WH, Ware RE, Aygun B.
Source
Baylor College of Medicine, Houston, Texas, USA.
Comment in
Pediatr Blood Cancer. 2012 Apr;58(4):485-6.
Abstract
BACKGROUND:
Bone marrow transplantation (BMT) using human leukocyte antigen (HLA)-matched sibling donors can be curative for children with sickle cell anemia (SCA). However, minimal data exist regarding availability of HLA-identical matched siblings for transplant-eligible children, and family interest in pursuing transplantation.
METHODS:
We retrospectively analyzed a pediatric SCA cohort receiving chronic transfusions between July 2004 and January 2011. Data were analyzed regarding the number of full siblings and half-siblings, availability, and family interest in HLA testing the full siblings, and interest in proceeding with HLAmatched transplantation.
RESULTS:
Among 113 patients, 46 (41%) had at least 1 full sibling and 40 (35%) had an unaffected full sibling who could serve as a BMT donor. The families of 23 of these patients (58%) agreed to HLA-type sibling, 8 of whom (35%) were matched. Transfusion indications for families agreeing to HLA typing included stroke (46%) abnormal TCD (29%), acute chest syndrome (21%), and other CNS reasons (4%). Common reasons to decline HLA typing or transplantation included fear of the process, toxicities of the procedure, and comfort with current quality of life on transfusions. Only 8 of 113 (7%) were eligible for matched BMT, and only 3 (3%) underwent HLA-matched transplantation. Two unmatched children received haploidentical transplantation.
CONCLUSIONS:
Most families of children with SCA on chronic transfusions choose to proceed with HLA typing. However, when a matched sibling was identified, most families declined to proceed with matched-sibling transplantation. Discussing BMT as a treatment option, offering HLA typing and identifying barriers may improve acceptance of this treatment modality.
PMID: 22435112 [PubMed - in process]
Related citations
5.
Am J Hematol. 2012 Jan 31. doi: 10.1002/ajh.23137. [Epub ahead of print]
Genotypic screening of the main opiate-related polymorphisms in a cohort of 139 sickle cell disease patients.
Joly P,Gagnieu MC, Bardel C, Francina A, Pondarre C, Martin C.
Source
Unité de Pathologie Moléculaire du Globule Rouge (Centre de Référence des Thalassémies), Laboratoire de Biochimie et de Biologie Moléculaire, Hôpital Edouard Herriot, Hospices Civils and Université Claude Bernard-Lyon 1, Lyon, France; Centre de Recherche et d'Innovation sur le Sport (EA 647), Université de Lyon, Villeurbanne, France. philippe.joly@chu-lyon.fr.
PMID: 22430884 [PubMed - as supplied by publisher
Related citations
6.
Pediatr Blood Cancer. 2012 Mar 15. doi: 10.1002/pbc.24129. [Epub ahead of print]
Ambulatory Care Connections of Medicaid-Insured Children With Sickle Cell Disease.
Bundy DG,Muschelli J, Clemens GD, Strouse JJ, Thompson RE, Casella JF, Miller MR.
Source
Division of Quality and Safety, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland; Department of Health Policy & Management, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland. dbundy@jhmi.edu.
Abstract
BACKGROUND:
Sickle cell disease (SCD) requires coordinated ambulatory care from generalists and hematologists. We examined when children with SCD establish ambulatory care connections, whether these connections are maintained, and how these connections are used before and after hospitalizations.
PROCEDURE:
We conducted a retrospective cohort study of Medicaid-insured Maryland children with SCD from 2002 to 2008. For children enrolled from birth, time to first, second, and third generalist and first hematologist visits was plotted. For all children, we analyzed ambulatory visits by age group, by emergency department (ED) and hospital use, and before and after hospitalizations.
RESULTS:
The overall study cohort comprised 851 children; 178 provided data from birth. Ambulatory care connections to generalists were made rapidly; connections to hematologists occurred more slowly, if at all (38% of children had not seen a hematologist by age 2 years). Visits with generalists decreased as patients aged, as did visits with hematologists (54% of children in the 12-17 year age group had no hematology visits in 2 years). Children with higher numbers of ED visits or hospitalizations also had higher numbers of ambulatory visits (generalist and hematologist). Most children had visits with neither generalists nor hematologists in the 30 days before and after hospitalizations.
CONCLUSIONS:
Medicaid-insured children with SCD rapidly connect with generalists after birth; connections to hematologists occur more slowly. The observation that connections to generalists and hematologists diminish with time and are infrequently used around hospitalizations suggests that the ambulatory care of many Medicaid-insured children with SCD may be inadequate. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
PMID: 22422739 [PubMed - as supplied by publisher
Related citations
7.
J Thromb Haemost. 2012 Mar 15. doi: 10.1111/j.1538-7836.2012.04697.x. [Epub ahead of print]
Pulmonary Embolism in Sickle Cell Disease: A Case-Control Study.
Novelli EM,Huynh C,Gladwin MT, Moore CG, Ragni MV.
Source
Department of Medicine, Division Hematology/Oncology, University of Pittsburgh Hemophilia Center of Western PA, Pittsburgh, PA, USA Vascular Medicine Institute, University of Pittsburgh Division of Pulmonary Medicine, University of Pittsburgh Department of Medicine, Center for Research on Health Care Data Center, Pittsburgh, PA.
Abstract
Introduction: Pulmonary embolism (PE) is a leading cause of mortality in hospitalized patients, yet the prevalence of PE in sickle cell disease (SCD) and its relation to disease severity or intrinsic hypercoagulability are not established. Methods: We estimated inpatient PE incidence and prevalence among SCD and non-SCD populations in Pennsylvania, and compared severity of illness and mortality, using Pennsylvania Health Care Cost Containment Council (PHC4) discharge data, 2001-2006. Risk factors for PE were assessed in a case-control study of discharges from the University of Pittsburgh Medical Archival Records System (MARS). Results: The incidence of inpatient PE was higher in the SCD PA population than in the non-SCD Pennsylvania population, 2001-2006. The PE prevalence among SCD discharges <50 years of age, 0.57%, was similar to that in non-SCD Pennsylvania discharges, 0.60%, and unchanged after adjustment for race. Among SCD discharges, those developing PE were significantly older, with longer length of stay, greater severity of illness, and higher mortality, p<0.001, than SCD without PE. Among PE discharges, SCD had similar severity of illness, p=0.77, and mortality, p=0.39, but underwent fewer computerized tomographic scans, p=0.006, than non-SCD with PE. In the local case-control study, no clinical or laboratory feature was associated with PE. Conclusions: The incidence of PE is higher and chest CT utilization is lower in SCD than non-SCD inpatients, suggesting that PE may be under-diagnosed. © 2012 International Society on Thrombosis and Haemostasis.
© 2012 International Society on Thrombosis and Haemostasis.
PMID: 22417249 [PubMed - as supplied by publisher]
Related citations
8.
Spec Care Dentist. 2012 Mar;32(2):55-60. doi: 10.1111/j.1754-4505.2012.00235.x.
Sickle cell disease does not predispose to caries or periodontal disease.
Passos CP,Santos PR,Aguiar MC,Cangussu MC, Toralles MB, da Silva MC, Nascimento RJ, Campos MI.
Source
Graduate Program of Interactive Processes of Organs and Systems, Institute of Health Science Scholarship Program of Tutorial Education, School of Dentistry Department of Biomorphology, Institute of Health Science Department of Social Dentistry and Pediatrics, School of Dentistry Department of Pediatrics, School of Medicine Professor Edgar Santos University Hospital Department of Biointeraction, Institute of Health Science-Federal University of Bahia, Salvador, Brazil.
Abstract
This study investigated the prevalence of dental caries and periodontal condition in a population with sickle cell disease (SCD), analyzing some associations with disease severity. The Decayed, Missing and Filled Teeth index (DMFT) and Community Periodontal Index (CPI) were recorded for 99 individuals with SCD and 91 matched controls. Socio-demographic status, oral health behaviors, and history of clinical severity of SCD were assessed. Statistical comparisons were performed between the group with SCD and the control group, as well as multivariate logistic regression analyses with DMFT index and CPI as the dependent variables. The mean number of decayed teeth was significantly higher in individuals with HbSS. Older age, female gender, and daily smoking were identified as risk factors for higher DMFT, while older age and absence of daily use of dental floss were risk factors for the development of periodontal disease. In conclusion, risk factors known to cause caries and periodontal disease had more influence on oral health than the direct impact of SCD.
© 2012 Special Care Dentistry Association and Wiley Periodicals, Inc.
PMID: 22416987 [PubMed - as supplied by publisher]
9.
Curr Treat Options Cardiovasc Med. 2012 Mar 6. [Epub ahead of print]
Hemoglobinopathies and Stroke: Strategies for Prevention and Treatment.
Ali N,Srey R,Pavlakis S.
Source
Pediatric Neurology, Maimonides Infants and Children's Hospital, 977 48th Street, Brooklyn, NY, 11219, USA.
Abstract
OPINION STATEMENT: Current treatment options for stroke in sickle cell disease (SCD) and thalassemia are limited. Hypercoagulation occurs in both diseases partly due to activated platelets and red blood cell dysmorphology and dysfunction, resulting in chronic anemia. This overlapping pathophysiology of the nervous system promotes the role of some common treatment modalities for these similar diseases. The current evidence suggests that chronic exchange transfusion and stem cell transplantation/bone marrow transplant (BMT) can be used in both diseases. Exchange transfusion is the mainstay of therapy of acute stroke in SCD whereas blood transfusions and hydroxyurea appear to be the most effective current treatments. However, evidence suggests that exchange transfusion should be initiated in acute ischemic stroke (AIS) and chronic transfusion continued in both diseases after AIS. Exchange transfusion can also be used acutely in AIS with thalassemia as this disorder is also associated with hypervolemia at baseline, occurring secondary to chronic anemia. The ideal length of chronic transfusions for both primary and secondary stroke prevention still needs to be better defined. Stem cell transplant or BMT is the only curative treatment for both diseases. However, timing needs to be further investigated. If transplantation is effective, it may need to be done before the child with SCD expresses disease, such as in infancy. However, in infancy, we cannot predict the severity of the phenotype in SCD with certainty, so an individual decision about transplantation is difficult to make. In thalassemia, transplantation may be effective later because vasculopathy is not the problem as in SCD. Furthermore, cerebrovascular disease occurs later in thalassemia than in SCD. Finally, aspirin is a treatment modality that also warrants further investigation. There are limited studies on the effectiveness of aspirin in SCD and thalassemias. Few studies have demonstrated clinical improvement of stroke in patients with hemoglobinopathies. Given the successful use of aspirin in the treatment and prevention of recurrent cardioembolic events in patients without hemoglobinopathies, diseases with hypercoagulability, such as SCD and thalassemia, may also benefit from the use of aspirin for treatment and prevention. However, the evidence available is based on case and retrospective studies, necessitating future larger and more valid studies to evaluate safety and effectiveness.
PMID: 22392612 [PubMed - as supplied by publisher]
Related citations
Sci Transl Med. 2012 Feb 29;4(123):123ra26.
A Biophysical Indicator of Vaso-occlusive Risk inSickleCell Disease.
Wood DK,Soriano A,Mahadevan L, Higgins JM, Bhatia SN.
Source
Division of Health Sciences and Technology, Massachusetts Institute of Technology, Cambridge, MA 02139, USA.
Abstract
The search for predictive indicators of disease has largely focused on molecular markers. However, biophysical markers, which can integrate multiple pathways, may provide a more global picture of pathophysiology. Sicklecell disease affects millions of people worldwide and has been studied intensely at the molecular, cellular, tissue, and organismal level for a century, but there are still few, if any, markers quantifying the severity of this disease. Because the complications ofsicklecell disease are largely due to vaso-occlusive events, we hypothesized that a physical metric characterizing the vaso-occlusive process could serve as an indicator of disease severity. Here, we use a microfluidic device to characterize the dynamics of "jamming," or vaso-occlusion, in physiologically relevant conditions, by measuring a biophysical parameter that quantifies the rate of change of the resistance to flow after a sudden deoxygenation event. Our studies show that this single biophysical parameter could be used to distinguish patients with poor outcomes from those with good outcomes, unlike existing laboratory tests. This biophysical indicator could therefore be used to guide the timing of clinical interventions, to monitor the progression of the disease, and to measure the efficacy of drugs, transfusion, and novel small molecules in an ex vivo setting.
Am J Hematol. 2012 Mar;87(3):340-6. doi: 10.1002/ajh.22271. Epub 2012 Feb 3.
Framing the research agenda forsickle cell trait: building on the current understanding of clinical events and their potential implications.
Goldsmith JC,Bonham VL,Joiner CH,Kato GJ,Noonan AS,Steinberg MH.
Source
Blood Diseases Branch, Division of Blood Diseases and Resources, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892-7950, USA. goldsmithjc@nhlbi.nih.gov
Abstract
Sickle Cell Trait (HbAS), the heterozygous state for thesickle hemoglobin beta globin gene is carried by as many as 100 million individuals including up to 25% of the population in some regions of the world (World Health Organization, Provisional agenda item 4.8, EB117/34 (22 December 2005) or World Health Organization, Provisional agenda item 11.4 (24 April 2006)). Persons with HbAS have some resistance to falciparum malaria infection in early childhood (Piel FB, Patil AP, Howes RE, et al., Nat Commun 2010;1104:1-7 and Aidoo M, Terlouw DJ, Kolczak M, et al., Lancet 2002;359:1311-1312) and as a result individuals with HbAS living in malarial endemic regions of Africa have a survival advantage over individuals with HbAA. Reports from the US emphasize possible health risks for individuals with HbAS including increased incidence of renal failure and malignancy, thromboembolic disorders, splenic infarction as a high altitude complication, and exercise-related sudden death. The National Heart, Lung, and Blood Institute, National Institutes of Health convened a workshop in Bethesda, Maryland on June 3-4, 2010, Framing the Research Agenda forSickle Cell Trait, to review the clinical manifestations of HbAS, discuss the exercise-related sudden death reports in HbAS, and examine the public health, societal, and ethical implications of policies regarding HbAS. The goal of the workshop was to identify potential research questions to address knowledge gaps.
Am J Hematol. 2012 Mar;87(3):333-6. doi: 10.1002/ajh.22265. Epub 2012 Jan 9.
Inpatient management of sickle cell pain: a 'snapshot' of current practice.
Miller ST,Kim HY,Weiner D,Wager CG,Gallagher D, Styles L, Dampier CD; Investigators of the Sickle Cell Disease Clinical Research Network (SCDCRN).
Collaborators (95)
Source
Division of Hematology/Oncology, State University of New York-Downstate Medical Center/Kings County Hospital Center, Brooklyn, New York 11203, USA. scott.miller@downstate.edu
Abstract
The Sickle Cell Disease Clinical Research Network (SCDCRN) designed the PROACTIVE Feasibility Study (ClinicalTrials.gov NCT00951808) to determine whether elevated serum levels of secretory phospholipase A2 (sPLA2) during hospitalization for pain would permit preemptive therapy of sickle cell acute chest syndrome (ACS) by blood transfusion. While PROACTIVE was not designed to assess pain management and was terminated early due to inadequate patient accrual, collection of clinical data allowed a "snapshot" of current care by expert providers. Nearly half the patients admitted for pain were taking hydroxyurea; hydroxyurea did not affect length of stay. Providers commonly administered parenteral opioid analgesia, usually morphine or hydromorphone, to adults and children, generally by patient-controlled analgesia (PCA). Adult providers were more likely to prescribe hydromorphone and did so at substantially higher morphine equivalent doses than were given to adults receiving morphine; the latter received doses similar to children who received either medication. All subjects treated with PCA received higher daily doses of opioids than those treated by time-contingent dosing. Physicians often restricted intravenous fluids to less than a maintenance rate and underutilized incentive spirometry, which reduces ACS in patients hospitalized for pain.
Sickle Cell Conferences
April 12 – Atlanta -Sickle Cell Disease: Equity and Ethics. Emory University
The symposium will include a keynote address and panel discussion on bioethics in sickle cell disease. The talks will discuss the racialization of the disease, social determinants, public policy, treatment and care. Confirmed participants includeDr. Carlton Haywood, Jr. from the Johns Hopkins University and Dr. Camara P. Jones from the Centers for Disease Control and Prevention, and Dr. Bruce Mitchell from the School of Medicine.
Date: Thursday, April 12th
Location: Psychology Building, Room 290
Time: 4:00-6:00 PM. A reception will follow.
The symposium is co-sponsored by the Program for Science and Society, the Center for Ethics, the Race and Difference Initiative, and the Graduate Institute of Liberal Arts.
For more information contact Melissa Creary atmcreary@emory.edu
April 20, 2012 , Indianapolis, Indiana.Martin Center Announces 2012 Indiana Statewide Sickle Cell Conference Free Community Event -The free one day conference will be held on April 20, 2012 at the Indiana Landmarks Center at 1201 N. Central Avenue, Indianapolis, Indiana. The conference will begin with registration and a continental breakfast at 8:00 A.M. and will conclude at 4:30 P.M.
The conference has been planned with the assistance of multiple health organizations, including the Indiana Hemophilia and Thrombosis Center, Riley Hospital for Children, the Indiana State Department of Health, Indiana University Health Hospital, the North Central Sickle Cell Program at South Bend Memorial Hospital and the Indiana Minority Health Coalition. Martin Center and its partners have designed the 2012 Indiana Statewide Sickle Cell Conference to alert our communities about the continued presence of Sickle Cell Disease, as well as informing the public about the varieties of pain (psychological, emotional, social, economic and spiritual) in addition to the physical pain associated with it. The conference is also intended to inform the community about current treatment protocols for Sickle Cell and to inspire hope for those who have the disease by demonstrating the shared commitment that others have to make life better for them. http://www.themartincenter.org
May 4, 2012 Sickle Cell Disease: Overview and Update New York NY 7:30 AM- 5 PM Sponsored by the Sickle Cell/Thalassemia Program at New York Methodist Hospital for information please call 718-857-5643 or email torib9001@nyp.org
24th May - 25th May 2012Event name: Sickle Cell in Focus 2(SCiF)Venue: King’s College London Guy’s campus, London Programme Director: Professor Swee Lay Thein, King’s College London / King’s College Hospital, UK
Description: Sickle Cell in Focus is an annual, two-day intensive educational conference held to highlight and discuss emerging clinical complications and management of sickle cell disease. The clinical and scientific lectures are aimed at consultants, trainee doctors and other health professionals involved in the care of patients with the disease and academic researchers in this field. It attracts local, national and international guest speakers and delegates.
For more information: SCiF is now being organized via the South Thames Sickle Cell and Thalassaemia Network (STSTN). Please visit the websitefor more information – http://www.ststn.co.uk Contact:info@ststn.co.uk orbelle.kelly@kcl.ac.uk
July 5-7, 2012 Venetian / Palazzo Hotel Las Vegas - Association of Nigerian Physicians in the Americas“The 18th Annual ANPA Convention & Scientific Assembly will be held July 5-7, 2012 at the Venetian / Palazzo Hotel in Las Vegas, Nevada. The theme for this year’s assembly will be “Righting the Wrong in Sickle Cell Disease”, and “Information Technology in Medical Practice”. The ANPA Annual Meeting features three full-day meetings, providing participants’ knowledge related to the challenges facing minority health care providers in the provision of clinical and therapeutic services for diseases and conditions related to a variety of medical specialties. The meeting will feature member lecturers providing state of the art technological advances that will impact the practice of medicine. The annual convention is of great value to our membership and other health care professionals by providing them with opportunities to keep abreast of developments in various areas of health care delivery and also offering continuing medical education (CME) credits. For more information please visit www.anpa.org or call 913.402.7102. “
September 25 – 29, 2012 The Sickle Cell Disease Association of America 40th Annual Conference Baltimore MD
The conference will be held September 25 – 29, 2012 at the Baltimore Marriott Waterfront Hotel in Baltimore, MD. We promise that this will be one of the most educational and empowering events to take place within the sickle cell community! http://www.sicklecelldisease.org/index.cfm?page=annual-convention
Stroke Deaths Down in African-American Children
A new study shows that the gap in ischemic stroke mortality rates between African-American and white children has narrowed by about two thirds in recent years, researchers report.
They speculate that this reduction may relate to publication of evidence from the Stroke Prevention Trial in Sickle Cell Anemia (STOP) that ultrasonographic screening and blood transfusions in children with sickle cell anemia can prevent strokes.
"The research is implying that by doing ultrasounds and transfusion therapy, we are preventing strokes and decreasing mortality, which is very important; we are making a difference in pediatric stroke," said lead researcher Laura Lehman, MD, clinical fellow in the Cerebrovascular Disorders and Stroke Program, Neurology Department, Children's Hospital, Boston, Massachusetts.
Their findings were presented at the American Stroke Association's International Stroke Conference 2012. http://www.medscape.com/viewarticle/759149
Charles F. Whitten, Pioneering Sickle Cell Doctor, Has Papers Acquired By National Institutes Of Health Library
The National Institutes of Health's National Library of Medicine has acquired the papers of the late Charles F. Whitten, a respected Detroit pediatrician known for his expertise and work related to Sickle Cell Disease.
Whitten, who passed away in 2008 at the age of 86, served as chief of pediatrics at Detroit Receiving Hospital and was the first African-American to head a hospital department in the city. He was also a distinguished professor of pediatrics at Wayne State University.
The doctor played an instrumental role in the formation of the Sickle Cell Disease Association of America and Detroit's Sickle Cell Detection and Information Center. http://www.huffingtonpost.com/2012/02/13/charles-f-whitten-national-institutes-of-health-library_n_1273365.html
MEMBERS OF NEW INTERAGENCY PAIN RESEARCH COORDINATING COMMITTEE ANNOUNCED
More than 100 million Americans suffer from migraines, arthritis and other chronic pain conditions with an annual economic toll of nearly $600 billion in medical bills and lost productivity. To help address this problem, Congress directed the U.S. Department of Health and Human Services, through the Affordable Care Act, to create a new Interagency Pain Research Coordinating Committee. Its members, announced today by the National Institutes of Health, include biomedical researchers, representatives from nonprofit public advocacy organizations, and representatives of seven federal government organizations that deal with pain research and patient care.
The committee will work to identify critical gaps in basic and clinical research on the symptoms, causes, and treatment of pain and will recommend federal research programs in these areas. The focus will be to coordinate pain research activities across the federal government with the goals of stimulating pain research collaboration, fully leveraging the government resources dedicated to supporting pain research, and providing an important avenue for public involvement. The committee will explore public-private partnerships to broaden collaborative, cross-cutting research and consider best practices in disseminating information about pain to public and professional audiences.
"Pain is a universal condition, a serious and costly public health issue, and a challenge for family, friends, and health care providers," said Story Landis, Ph.D., director of NIH's National Institute of Neurological Disorders and Stroke (NINDS) and the committee chair. "This committee will play an important role working with federal agencies spearheading pain research. I am pleased that its membership reflects a great depth and wide range of both scientific expertise and effective public advocacy."
The committee appointees include leading federal officials together with six non-federal scientists, physicians, and other health professionals, as well as six members of the general public who are representatives of leading research, advocacy and service organizations. After tracking the work of several government agencies that conduct and support pain research, the committee will develop a report on scientific advances in the diagnosis, prevention, and treatment of chronic and acute pain.
Nigeria’s first stem cell transplant
Some months ago, the University of Benin Teaching Hospital (UBTH) successfully pioneered stem cell transplantation surgery in Nigeria. On the continent, this medical feat has previously been carried out only in Egypt and South Africa. The successful, cutting-edge procedure by an 18-man team led by Dr. Nosakhare Bazuaye was conducted in the hospital on a seven-year-old sickle cell anaemia patient, Mathew Ndik.
The institution commenced the project three years ago when it sent the experts to Basel, Switzerland to acquire requisite knowledge for stem cell transplantation procedure. Nigerians can now access the novel medical procedure for N2.5 million as against the N40 million needed for same outside the country.
This development represents great news for sickle cell patients and others that require regenerative medicine for a cure. This new procedure can also offer relief or even cures for many serious diseases, including Alzheimer’s, heart disease, diabetes, leukemia, thalassemia, cancer and multiple myeloma. It could also help patients recover from spinal cord injuries and strokes. UBTH’s strict adherence to procedure in this connection is worth acknowledging. The institution officially unveiled the successful breakthrough a few weeks ago, only after the patient crossed the 100-day mark traditionally observed before a hospital could be certified by WHO as having successfully carried out a stem cell transplant. http://allafrica.com/stories/201202071230.html
Say It Loud! The Sickle Cell Association of New Jersey (SCANJ) Public Service Announcement Contest 2012: Goal: to increase understanding of Sickle Cell Disease (SCD) and programs and services for individuals with SCD, primarily among youth ages 13-25 years via a competitive and non-competitive PSA video awareness campaign.
For all the information see http://sayitloudscanjcontest.eventbrite.com/
New Online Reference Book Resource
A classic educational resource on sickle cell, which has been out of print for several years, has recently been made available again as an open education resource. The resource is the late Frank B Livingstone’s 1985 book Frequencies of Hemoglobin Variants. With the kind permission of Frank’s daughter Amy Livingstone, the book has been scanned and made available on-line as an open education resource as part of an open education resources project led by Dr Vivien Rolfe at De Montfort University in the UK. Availableas a PDF at: http://www.sicklecellanaemia.org/teaching-resources/resources/scooter82/scooter82.html
The resource is free to use and/or to create educational derivatives from it, subject to the conditions described below Please do let the project lead, Dr Vivien Rolfe vrolfe@dmu.ac.uk , know if you make use of it and if so how. .
Online Newsletters
Sickle Cell Society based in London, England latest e-news letter is:
http://issuu.com/communityeventsuk/docs/sickle_cell_societywinter_final?mode=window&backgroundColor=%23222222
AFRICAN SC NEWS & WORLD REPORT at www.scdjournal.com/news-special
1. Obtain Free Special Edition of SICKLE CELL NEWS at www.scdjournal.com/free.html
2. Obtain our digital editions, self-help books, memoirs, etc at http://www.scdjournal.com/scdreader.html
2. Let's give a Talk on sickle cell at your educational/religious institution. register here
New Hemoglobinopathy Degree Program at University College London (UCL)
The Haemoglobinopathy MSc programme at UCL is the first and only online master’s degree providing a comprehensive holistic training in thalassaemia and sickle cell disease.
Who should do the programme:This is for all doctors in the field of general medicine, pediatrics, genetics, primary care who wish to specialise in areas related to Haemoglobinopathy. For further information, please visit our website: http://www.mschaemoglobinopathy.co.uk/ or Email: MSc_Haemoglobinopathy@ucl.ac.uk
Web Resource
DNA Learning Center with excellent video animations http://www.dnalc.org/resources/3d/17-sickle-cell.html
Video Resources
CDC Webinar 10/27: The Use of Hydroxyurea for Sickle Cell Disease by Dr. Nancy Green, Columbia University Medical Center in Windows Media Player at mms://realaudio.service.emory.edu/SOM/PA/PLATT/SICKLE/CDCHydrea.wmv
Schedule of Free CDC 2012 Webinars
4/26: Sickle Cell – Adult Providers Network Dr. Kathryn Hassell, University of Colorado School of Medicine
5/24: Sickle Cell Disease and Emergency Department Use Dr. Paula Tanabe, Duke University School of Nursing
6/28: Sickle Cell Trait – What Every CBO Needs to Know Dr. Lanetta Jordan, Memorial Regional Hospital
7/26: Improved Survival of Children and Adolescents with Sickle Cell Disease Dr. Charles Quinn, Cincinnati Children's Hospital Medical Center
8/23: Translating Research to Policy Dr. Shawn Bediako, University of Maryland, Baltimore County
9/27: Improving Quality of Care for Sickle Cell Pediatrics in the Emergency Department Dr. James Moses, Boston Medical Center
10/25: Strategies from the Field – Data Collection and Harmonization CDC’s Division of Blood Disorders and RuSH Project States
November/December: --- No Webinars---
See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-resources/cdc-webinars-hemoglobinopathies-and-public-health
John Hopkins - Urban Health Radio Program (UHRP)
"Breaking It Down: Our Health Our Way" Features Sickle Cell talks in February
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WOLB 1010 AM Thursdays from 11 a.m. - 12 p.m. Listen live on the web at http://www.hopkinsmedicine.org/diversity_cultural_competence/urban_health_radio_program/ |
Free ASH webinars http://hematology.org/Meetings/Webinars/6832.aspx
Pain in Sickle cell disease and Stroke, Renal Disease, and Treatment with Hydroxyurea in Adults with Sickle Cell Disease
Articles for February
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Modi AC, Crosby LE, Hines J, Drotar D, Mitchell MJ. |
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J Pediatr Hematol Oncol. 2012 Jan 23. [Epub ahead of print] |
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PMID: 22278205 [PubMed - as supplied by publisher] |
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Cheung AT, Miller JW, Miguelino MG, To WJ, Li J, Lin X, Chen PC, Samarron SL, Wun T, Zwerdling T, Green R. |
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J Pediatr Hematol Oncol. 2012 Jan 23. [Epub ahead of print] |
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PMID: 22278200 [PubMed - as supplied by publisher] |
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Adeniyi A, Saminu K. |
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Afr Health Sci. 2011 Sep;11(3):303-8. |
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PMID: 22275916 [PubMed - in process] |
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Molecular Link between Intravascular Hemolysis and Vascular Occlusion in Sickle Cell Disease. |
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Zhou Z, Yee DL, Guchhait P. |
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Curr Vasc Pharmacol. 2012 Jan 20. [Epub ahead of print] |
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PMID: 22272904 [PubMed - as supplied by publisher] |
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Portocarrero ML, Portocarrero ML, Sobral MM, Lyra I, Lordêlo P, Barroso U Jr. |
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J Urol. 2012 Jan 18. [Epub ahead of print] |
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PMID: 22264459 [PubMed - as supplied by publisher] |
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Hirst C, Williamson L. |
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Cochrane Database Syst Rev. 2012 Jan 18;1:CD003149. |
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PMID: 22258951 [PubMed - in process] |
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Association of coagulation activation with clinical complications in sickle cell disease. |
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Ataga KI, Brittain JE, Desai P, May R, Jones S, Delaney J, Strayhorn D, Hinderliter A, Key NS. |
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PLoS One. 2012;7(1):e29786. Epub 2012 Jan 11. |
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PMID: 22253781 [PubMed - in process] Free PMC Article |
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Risk factors for alloimmunization in patients with sickle cell anemia. |
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Pinto PC, Braga JA, Santos AM. |
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Rev Assoc Med Bras. 2011 Dec;57(6):668-673. English, Portuguese. |
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PMID: 22249547 [PubMed - as supplied by publisher] Free Article |
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Upper Airway Lymphoid Tissue Size in Children with Sickle Cell Disease. |
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Strauss T, Sin S, Marcus CL, Mason TB, McDonough JM, Allen JL, Caboot JB, Bowdre CY, Jawad AF, Smith-Whitley K, Ohene-Frempong K, Pack AI, Arens R. |
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Chest. 2012 Jan 12. [Epub ahead of print] |
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PMID: 22241762 [PubMed - as supplied by publisher] |
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Messonnier L, Samb A, Tripette J, Gogh BD, Loko G, Sall ND, Féasson L, Hue O, Lamothe S, Bogui P, Connes P. |
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Clin Hemorheol Microcirc. 2012 Jan 3. [Epub ahead of print] |
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PMID: 22240384 [PubMed - as supplied by publisher] |
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Aygun B, Wruck LM, Schultz WH, Mueller BU, Brown C, Luchtman-Jones L, Jackson S, Iyer R, Rogers ZR, Sarnaik S, Thompson AA, Gauger C, Helms RW, Ware RE; for the TCD With Transfusions Changing to Hydroxyurea (TWiTCH) Trial Investigators. |
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Am J Hematol. 2011 Dec 29. doi: 10.1002/ajh.23105. [Epub ahead of print] No abstract available. |
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PMID: 22231377 [PubMed - as supplied by publisher] |
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Inpatient management of sickle cell pain: A 'snapshot' of current practice. |
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Miller ST, Kim HY, Weiner D, Wager CG, Gallagher D, Styles L, Dampier CD; for the Investigators of the Sickle Cell Disease Clinical Research Network (SCDCRN). |
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Am J Hematol. 2011 Dec 17. doi: 10.1002/ajh.22265. [Epub ahead of print] No abstract available. |
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PMID: 22231150 [PubMed - as supplied by publisher] |
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Pneumococcal Bacteremia in a Vaccinated Pediatric Sickle Cell Disease Population. |
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Ellison AM, Ota KV, McGowan KL, Smith-Whitley K. |
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Pediatr Infect Dis J. 2012 Jan 5. [Epub ahead of print] |
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PMID: 22228232 [PubMed - as supplied by publisher] |
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Prospects for primary stroke prevention in children with sickle cell anaemia. |
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Jordan LC, Casella JF, Debaun MR. |
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Br J Haematol. 2012 Jan 9. doi: 10.1111/j.1365-2141.2011.09005.x. [Epub ahead of print] |
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PMID: 22224940 [PubMed - as supplied by publisher] |
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Sickle Cell News for January 2012
American Society of Hematology Statement on Screening for Sickle Cell Trait and Athletic Participation January26,2012
http://www.hematology.org/advocacy/policy-statements/7704.aspx
Background
In April 2010, the National Collegiate Athletic Association (NCAA) adopted a policy requiring Division I institutions to perform sickle cell trait testing for all incoming student athletes. This requirement arose from a lawsuit following the death during preseason training of a college football player, who was subsequently found to have sickle cell trait. The policy includes an opt-out provision for students who can provide results from a prior test and for those who are willing to sign a waiver of liability against the university and the NCAA. There have been no well-controlled, hypothesis-driven prospective studies on sickle cell trait and exertional collapse. Most cases reported to date are autopsy descriptions or case reports in which other potential contributors are not fully investigated. The extremely small number of deaths in a highly prevalent carrier state implies that other genetic or environmental factors play a role. Rhabdomyolysis and exertional collapse also occur in individuals without sickle cell trait.
ASH Policy
Additional Material
Sickle Cell Patient in New Movie- Joyful Noise
Dexter Darden knows all about perseverance. Darden was diagnosed with Sickle Cell Anemia as a child, but even at the age of 8, his talent shined through at the Paul Newman’s Hole in the Wall Gang Camp for children with blood diseases and cancer. Newman recognized his talents so Darden found himself performing at star-studded galas (with the likes of Julia Roberts, Matt Damon and Morgan Freeman) to raise money for the camp and the cause, and dancing, modeling and acting roles followed. Darden graduated from Victory Christian High School in Williamstown.
High blood pressure, anemia put sickle cell kids at risk for strokes
1. Abnormalities in Cardiac Structure and Function in Adults with Sickle Cell Disease are not Associated with Pulmonary Hypertension. Knight-Perry JE, de Las Fuentes L, Waggoner AD, Hoffmann RG, Blinder MA, D VG, Field JJ.
J Am Soc Echocardiogram
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PMID: 21873028 [PubMed - as supplied by publisher]
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2. Increased concentrations of IL-18 and uric acid in sickle cell anemia: Contribution of hemolysis, endothelial activation and the inflammasome. Cerqueira BA, Boas WV, Zanette AD, Reis MG, Goncalves MS.
Cytokine
.. 2011 Aug 24. [Epub ahead of print]
PMID: 21871815 [PubMed - as supplied by publisher]
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3. Cardiovascular autonomic dysfunction in sickle cell anemia.
Martins WD, Lopes HF, Consolim-Colombo FM, Gualandro SD, Arteaga-Fern??ndez E, Mady C.
Auton Neurosci
.. 2011 Aug 24. [Epub ahead of print]
PMID: 21868290 [PubMed - as supplied by publisher]
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4. Lower Airway Nitric Oxide is Increased in Children with Sickle Cell Disease.
Radhakrishnan DK, Bendiak GN, Mateos-Corral D, Al-Saleh S, Bhattacharjee R, Kirby-Allen M, Grasemann H.
J Pediatr
.. 2011 Aug 23. [Epub ahead of print]
PMID: 21868036 [PubMed - as supplied by publisher]
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5. Transition of chronically ill youth to adult health care: experience of youth with hemoglobinopathy.
Bryant R, Young A, Cesario S, Binder B.
J Pediatr Health Care
.. 2011 Sep-Oct;25(5):275-83. Epub 2010 May 1.
PMID: 21867855 [PubMed - in process]
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6. Clinical spectrum of serious bacterial infections among splenectomized patients with hemoglobinopathies in Israel: a 37-year follow-up study.
Sakran W, Levin C, Kenes Y, Colodner R, Koren A.
Infection
.. 2011 Aug 25. [Epub ahead of print]
PMID: 21866338 [PubMed - as supplied by publisher]
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7. Prevalence of Hemoglobinopathy Disorders in Adult Patients Sent for Diagnosis of Anemia in Saudi Arabia.
Alsaeed AH.
Genet Test Mol Biomarkers
.. 2011 Aug 23. [Epub ahead of print]
PMID: 21861712 [PubMed - as supplied by publisher]
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8. Increased prevalence of osteonecrosis of the femoral head in children and adolescents with sickle-cell disease.
Mahadeo KM, Oyeku S, Taragin B, Rajpathak SN, Moody K, Santizo R, Catherine Driscoll M.
Am J Hematol
.. 2011 Sep;86(9):806-8. doi: 10.1002/ajh.22103. No abstract available.
PMID: 21850660 [PubMed - in process]
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9. Sickle cell trait is not independently associated with susceptibility to end-stage renal disease in African Americans.
Hicks PJ, Langefeld CD, Lu L, Bleyer AJ, Divers J, Nachman PH, Derebail VK, Bowden DW, Freedman BI.
Kidney Int
.. 2011 Aug 17. doi: 10.1038/ki.2011.286. [Epub ahead of print]
PMID: 21849968 [PubMed - as supplied by publisher]
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10. Tapered oral dexamethasone for the acute chest syndrome of sickle cell disease.
Quinn CT, Stuart MJ, Kesler K, Ataga KI, Wang WC, Styles L, Smith-Whitley K, Wun T, Raj A, Hsu LL, Krishnan S, Kuypers FA, Setty Y, Rhee S, Key NS, Buchanan GR; on Behalf of the Investigators of the Comprehensive Sickle Cell Centers.
Br J Haematol
.. 2011 Aug 16. doi: 10.1111/j.1365-2141.2011.08827.x. [Epub ahead of print]
PMID: 21848879 [PubMed - as supplied by publisher]
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11. Quality-of-Care Indicators for Children With Sickle Cell Disease.
Wang CJ, Kavanagh PL, Little AA, Holliman JB, Sprinz PG.
Pediatrics
.. 2011 Aug 15. [Epub ahead of print]
PMID: 21844055 [PubMed - as supplied by publisher]
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12. Molecular blood typing augments serologic testing and allows for enhanced matching of red blood cells for transfusion in patients with sickle cell disease.
Wilkinson K, Harris S, Gaur P, Haile A, Armour R, Teramura G, Delaney M.
Transfusion
.. 2011 Aug 9. doi: 10.1111/j.1537-2995.2011.03288.x. [Epub ahead of print]
PMID: 21827505 [PubMed - as supplied by publisher]
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13. When children with sickle-cell disease become adults: Lack of outpatient care leads to increased use of the emergency department.
Hemker BG, Brousseau DC, Yan K, Hoffmann RG, Panepinto JA.
Am J Hematol
.. 2011 Jun 14. doi: 10.1002/ajh.22106. [Epub ahead of print] No abstract available.
PMID: 21815184 [PubMed - as supplied by publisher]
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14. Monitoring toxicity, impact, and adherence of hydroxyurea in children with sickle cell disease.
Brandow AM, Panepinto JA.
Am J Hematol
.. 2011 Sep;86(9):804-6. doi: 10.1002/ajh.22101. Epub 2011 Aug 3. No abstract available.
PMID: 21815183 [PubMed - in process]
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15. Recurrent, severe wheezing is associated with morbidity and mortality in adults with sickle cell disease.
Cohen RT, Madadi A, Blinder MA, Debaun MR, Strunk RC, Field JJ.
Am J Hematol
.. 2011 Sep;86(9):756-61. doi: 10.1002/ajh.22098. Epub 2011 Aug 2.
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