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RESEARCH PAGE

Sickle Cell Anemia research is constantly ongoing. As information becomes available, it will be posted at this location, it can be found at the Cleveland Clinic web site, or you can contact Ira Bragg-Grant at (216) 229-8600 for the latest available research information as it occurs.

Research Articles

For the latest news, visit this website that is updated weekly at www.scinfo.org/news.htm

In the News

Events around the world include:

NIH Halts SWiTCH Study

On behalf of the National Heart, Lung, and Blood Institute (NHLBI), we would like to share with you news about a sickle cell disease clinical trial, the Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) study. The multicenter clinical trial compared treatments to reduce the risk of additional strokes while minimizing the adverse effects of treatment such as iron overload in young participants (between the ages of 5 and 19) with sickle cell disease and a history of stroke and iron overload. The study was stopped early because a regular review of the data indicated that the experimental treatment was unlikely to prove better than the standard treatment. The NHLBI issued a press release about the early termination of the study, which can be found at: http://public.nhlbi.nih.gov/newsroom/home/GetPressRelease.aspx?id=2709.

The preliminary results of this study support the use of the standard treatment of periodic blood transfusions for reducing the risk of additional strokes in young, high-risk patients combined with deferasirox (EXJADE®), an FDA-approved drug to treat chronic iron overload. The alternative treatment that was tested -- hydroxyurea combined with phlebotomy -- does not appear to be better than this standard treatment.

Protecting study participants is the NHLBI’s priority, and when an experimental treatment fails to meet its predetermined goals, it is best to return participants to standard treatment as soon as possible. The NHLBI remains committed to continuing to support research to identify new or improved ways to reduce the burden of sickle cell anemia’s most severe symptoms on our children.

The results from SWiTCH do not affect the majority of sickle cell disease patients who are currently benefiting from hydroxyurea. NHLBI-supported research has shown that hydroxyurea helps prevent pain crises and some lung complications in adults. Patients currently taking hydroxyurea should continue taking the treatment as prescribed and should talk to their primary care provider if they have any concerns. Please let us know if you have any questions about the study. For scientific questions, please contact W. Keith Hoots, M.D., director of the NHLBI Division of Blood Diseases and Resources, at 301-435-0080.

New Book Published - Sickle Cell Disease - 100 Years Later". by Phyllis Zachery-Thomas

This book provides a transparent look into my personal struggle with sickle cell disease and how it led me to the formation of SCD Soldier Network, Inc. I co-authored the book with Atlanta Historian Dan Moore of the Apex Museum and founder of Marrow for Life. The book also includes the inspiring stories of others who battle the disease as well as it gives you the history of sickle cell and information about the blood disorder. I am very pleased with this project and am asking for your support. Please purchase your copy today, I promise you won't be disappointed. Please see attached flyer or access the website to purchase the book by clicking on the link www.createspace.com/3458126 Fifty percent of the proceeds from the sale of this book will be donated to SCD Soldier Network in order to fulfill it's mission.

Articles in the Medical Literature

Pulmonary hypertension in sickle cell disease children under 10 years of age.
Colombatti R, Maschietto N, Varotto E, Grison A, Grazzina N, Meneghello L, Teso S, Carli M, Milanesi O, Sainati L.
Br J Haematol. 2010 Jun 10. [Epub ahead of print]
Moving young people with sickle cell disease from paediatric to adult services.
Howard J, Woodhead T, Musumadi L, Martell A, Inusa BP.
Br J Hosp Med (Lond). 2010 Jun 9;71(6):310-314.
Hospital self-discharge among adults with sickle-cell disease (SCD): associations with trust and interpersonal experiences with care.
Haywood C Jr, Lanzkron S, Ratanawongsa N, Bediako SM, Lattimer-Nelson L, Beach MC.
J Hosp Med. 2010 May-Jun;5(5):289-94.
High mortality from P. falciparum malaria in children living with sickle cell anemia on the coast of Kenya.
McAuley CF, Webb C, Makani J, Macharia A, Uyoga S, Opi DH, Ndila C, Ngatia A, Scott JA, Marsh K, Williams TN.
Blood. 2010 Jun 8. [Epub ahead of print]
Management of Painful Vaso-Occlusive Crisis of Sickle Cell Anemia: Consensus Opinion.
Mousa SA, Momen AA, Al Sayegh FA, Jaouni SA, Nasrullah Z, Saeed HA, Alabdullatif A, Sayegh MA, Zahrani HA, Hegazi M, Mohamadi AA, Alsulaiman A, Omer A, Kindi SA, Tarawa A, Othman FA, Qari M.
Clin Appl Thromb Hemost. 2010 Jun 7. [Epub ahead of print]
Depression and loneliness in Jamaicans with Sickle Cell Disease.
Asnani MR, Fraser RA, Lewis NA, Reid ME.
BMC Psychiatry. 2010 Jun 7;10(1):40. [Epub ahead of print]
Lactate dehydrogenase as a predictor of kidney involvement in patients with sickle cell anemia.
Gurkan S, Scarponi KJ, Hotchkiss H, Savage B, Drachtman R.
Pediatr Nephrol. 2010 Jun 2. [Epub ahead of print]
The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up.
Steinberg MH, McCarthy WF, Castro O, Ballas SK, Armstrong FD, Smith W, Ataga K, Swerdlow P, Kutlar A, DeCastro L, Waclawiw MA; Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia and MSH Patients' Follow-Up.
Am J Hematol. 2010 Jun;85(6):403-8.
Mortality of children with sickle cell disease: a population study.
Fernandes AP, Januário JN, Cangussu CB, de Macedo DL, Viana MB.
J Pediatr (Rio J). 2010 May 27;86(4). [Epub ahead of print]
GMI-1070, a novel pan-selectin antagonist, reverses acute vascular occlusions in sickle cell mice.
Chang J, Patton JT, Sarkar A, Ernst B, Magnani JL, Frenette PS.
Blood. 2010 May 27. [Epub ahead of print]
Association of G6PD with lower haemoglobin concentration but not increased haemolysis in patients with sickle cell anaemia.
Nouraie M, Reading NS, Campbell A, Minniti CP, Rana SR, Luchtman-Jones L, Kato GJ, Gladwin MT, Castro OL, Prchal JT, Gordeuk VR.
Br J Haematol. 2010 May 9. [Epub ahead of print]
Prevalence and clinical correlates of microalbuminuria in children with sickle cell disease.
Becton LJ, Kalpatthi RV, Rackoff E, Disco D, Orak JK, Jackson SM, Shatat IF.
Pediatr Nephrol. 2010 Aug;25(8):1505-11. Epub 2010 May 27.
Dietary Water and Sodium Intake of Children and Adolescents With Sickle Cell Anemia.
Fowler KT, Williams R, Mitchell CO, Levy MC, Pope LF, Smeltzer MP, Wang WC.
J Pediatr Hematol Oncol. 2010 May 24. [Epub ahead of print]
A controlled, longitudinal study of the social functioning of youth with sickle cell disease.
Noll RB, Kiska R, Reiter-Purtill J, Gerhardt CA, Vannatta K.
Pediatrics. 2010 Jun;125(6):e1453-9. Epub 2010 May 24.
Role of Arginase in Sickle Cell Lung Disease and Hemolytic Anemias Claudia R. Morris The Open Nitric Oxide Journal, 2010, 2, 41-54

Conferences and Activities of Interest to the Sickle Cell Community

Articles in the Medical Literature

Raphael JL, Shetty PB, Liu H, Mahoney DH, Mueller BU.  A critical assessment of transcranial doppler screening rates in a large  pediatric sickle cell center: opportunities to improve healthcare quality. Pediatr Blood Cancer. 2008 Nov;51(5):647-51. http://www.ncbi.nlm.nih.gov/pubmed/18623200?ordinalpos=2&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Wang WC, Pavlakis SG, Helton KJ, McKinstry RC, Casella JF, Adams RJ, Rees RC; BABY HUG Investigators.   MRI abnormalities of the brain in one-year-old children with sickle cell anemia. Pediatr Blood Cancer. 2008 Nov;51(5):643-6. http://www.ncbi.nlm.nih.gov/pubmed/18478575?ordinalpos=3&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Moreira-Almeida A, Koenig HG.  Religiousness and spirituality in fibromyalgia and chronic pain patients.  Curr Pain Headache Rep. 2008 Oct;12(5):327-32.  http://www.ncbi.nlm.nih.gov/pubmed/18765136?ordinalpos=5&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Okumura MJ, Heisler M, Davis MM, Cabana MD, Demonner S, Kerr EA.  Comfort of general internists and general pediatricians in providing care for  young adults with chronic illnesses of childhood. J Gen Intern Med. 2008 Oct;23(10):1621-7. Epub 2008 Jul 26. http://www.ncbi.nlm.nih.gov/pubmed/18661191?ordinalpos=6&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Sonati MD, Costa FF.  The genetics of blood disorders: the hereditary hemoglobinopathies.  J Pediatr (Rio J). 2008 Sep 12;84(4 (Suppl)). Full Text PDF http://www.jped.com.br/conteudo/AA_12908_A01/ing.pdf
Gold JI, Mahrer NE, Treadwell M, Weissman L, Vichinsky E.  Psychosocial and behavioral outcomes in children with sickle cell disease and their healthy siblings. J Behav Med. 2008 Sep 11.   http://www.ncbi.nlm.nih.gov/pubmed/18784995?ordinalpos=11&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Mittal H, Roberts L, Fuller GW, O'Driscoll S, Dick MC, Height SE, Thein SL, Rees DC.  The effects of air quality on haematological and clinical parameters in children   with sickle cell anaemia. Ann Hematol. 2008 Sep 4.   http://www.ncbi.nlm.nih.gov/pubmed/18769920?ordinalpos=19&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Ulug P, Vasavda N, Kumar R, Keir L, Awogbade M, Cunningham J, Rees DC, Menzel  S, Thein SL.   Hydroxyurea therapy lowers circulating DNA levels in sickle cell anemia. Am J Hematol. 2008 Sep;83(9):714-6.  http://www.ncbi.nlm.nih.gov/pubmed/18615556?ordinalpos=21&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Koch J, Manworren R, Clark L, Quinn CT, Buchanan GR, Rogers ZR.  Pilot study of continuous co-infusion of morphine and naloxone in children with  sickle cell pain crisis. Am J Hematol. 2008 Sep;83(9):728-31.  http://www.ncbi.nlm.nih.gov/pubmed/18543345?ordinalpos=22&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Rovner AJ, Stallings VA, Kawchak DA, Schall JI, Ohene-Frempong K, Zemel BS.  High risk of vitamin d deficiency in children with sickle cell disease.  J Am Diet Assoc. 2008 Sep;108(9):1512-6.  http://www.ncbi.nlm.nih.gov/pubmed/18543345?ordinalpos=22&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum
Palermo TM, Riley CA, Mitchell BA.  Daily functioning and quality of life in children with sickle cell disease pain:   relationship with family and neighborhood socioeconomic distress. J Pain. 2008 Sep;9(9):833-40. Epub 2008 Jun 12. http://www.ncbi.nlm.nih.gov/pubmed/18550443?ordinalpos=35&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Ask the Experts

Question:
Is delayed puberty an issue for sickle cell patients?

Answer:
Delayed growth & delayed puberty are very common features of sickle cell disease. Poor nutrition may make the delay worse, and a nutrition consultation may be helpful. A few centers use supplements of growth hormone or testosterone to help with growth, but decisions to use those hormones need a detailed assessment by a pediatric endocrinologist. Anti-sickling therapy with hydroxyurea or chronic transfusion are likely to help with growth. Bone marrow transplantation has complex effects on growth and puberty. I encourage you to discuss these inter-related issues with your son's hematologist, endocrinologist, and nutritionist
Here is the abstract of one recent journal article on this topic.
Pediatr Res. 2007 May;61(5 Pt 1):607-13.

Effects of delayed pubertal development, nutritional status, and disease severity on longitudinal patterns of growth failure in children with sickle cell disease.

Zemel BS, Kawchak DA, Ohene-Frempong K, Schall JI, Stallings VA.
Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA. zemel@email.chop.edu
Previous studies of children with sickle cell disease (SCD) reported poor growth and delayed maturation. However, the prevalence, magnitude, and correlates of suboptimal growth remain poorly understood. A prospective longitudinal study was undertaken to determine the effects of disease severity and nutritional status on growth, an indicator of childhood well-being. Children, birth to 18 y of age, with SCD-SS were evaluated annually for 4 y. Growth, nutritional status, skeletal and sexual maturation, disease severity, dietary intake, and maternal education were assessed. In this sample of 148 children (78 females), growth in height, weight, or body mass index declined in 84% of subjects; 38% fell below the 5th percentile in one or more measures. Puberty was delayed 1 to 2 y, and median age at menarche was 13.2 y. Skeletal age was delayed by 0.7 +/- 1.4 y overall and by 1.3 +/- 1.5 y in children 10 to 15 y old. Height status declined over time and was positively associated with advancing puberty and hematological measures in girls, and nutritional status in girls and boys. Growth failure and maturational delay remain significant chronic problems in children with SCD-SS and are related to potentially modifiable factors such as nutritional status.
Sincerely,
Lewis Hsu, MD, PhD
Pediatric Hematologist
For more frequently asked questions please see: http://www.scinfo.org/faq.htm
American Society of Hematology Launches New Campaign to Educate Consumers on Vital Connection Between Blood and Personal Health http://www.bloodthevitalconnection.org/
Program developed in response to results of national survey indicating most Americans have low awareness of common blood conditions

Dr. Charles Whitten, M.D., associate dean emeritus of the Wayne State University School of Medicine, died Aug. 13. He was 86.

“Dr. Whitten was a pioneer in the field of medical education,” said Robert Frank, M.D., executive vice dean for the School of Medicine. “He founded the post baccalaureate program at Wayne State University School of Medicine, which was a national model for the inclusion of under-represented minority students in schools of medicine. Dr. Whitten revolutionized the curriculum at our School of Medicine, and was a personal mentor to many of our current medical educators.”

The post baccalaureate program led to Wayne State University leading the nation’s 125 medical schools (exclusive of Howard and Meharry) in the total number of African-American graduates from 1981 to 1997. One-third of them had entered through his program.

In addition to developing the post baccalaureate program in 1969, Dr. Whitten formed the Sickle Cell Detection and Information Center, the most comprehensive community program in the country, and facilitated the creation of the National Association for Sickle Cell Disease.

As chief of Pediatrics at Detroit Receiving Hospital, he was the first African-American physician to head a department in a Detroit hospital.

Dr. Whitten, who served more than 40 years as a member of the School of Medicine faculty, served 16 years as associate dean for Curriculum before entering semi-retirement in 1993 as professor and dean emeritus.

“Dr. Whitten was best known for his pioneering work in sickle cell anemia screening and development of novel educational tools for teaching children and families with sickle cell anemia,” said Yaddanapudi Ravindranath, M.B.B.S., professor of Pediatrics and the Georgie Ginopolis Chair for Pediatric Cancer and Hematology at the School of Medicine, and co-director of the Division of Hematology/Oncology for Children's Hospital of Michigan. “His forceful advocacy paved the way for the routine newborn screening for sickle cell anemia in Michigan and later in the United States.”
http://prognosis.med.wayne.edu/article/dr-whitten-pioneer-of-sickle-cell-screening-and-champion-of-africanamerican-medical-students-dies
Bio at http://www.med.umich.edu/haahc/Oralbios/whitten.htm

Ask the Experts

Question:
I can not afford the medications prescribed for me, Are there ant programs that can help?

Answer:
There are some very good programs that help such as
http://www.needymeds.com/ http://www.rxassist.org/ https://www.pparx.org/Intro.php
Work with your clinician to see if these programs can help

Question:
Which is better erythrocytopheresis or simple blood transfusion for the prevention of childhood stroke?

Answer:
Nobody has hard data on the number of sickle cell patients in the USA, but the estimate from the Sickle Cell Disease Association of America is 100,000. The estimate is that 10% of the children with sickle cell disease SS are at risk for stroke and will benefit from chronic transfusion as primary or secondary stroke prevention.

Some studies comparing costs of chronic simple transfusion plus iron chelation vs bone marrow transplant also include erythrocytapheresis. The costs depend fairly strongly on whether the target HbS is 30percent or 50 percent.

As you know, the use of RBC exchange is far better than simple transfusion in terms of iron overload but requires technical expertise and a good cost structure. When I was practicing in Atlanta, we wrote numerous business plans about setting up sickle cell erythrocytapheresis centers for the many pediatric sickle cell patients. The limiting factors were:
1) nursing expertise for venous access. Some children requiring erythrocytapheresis may be as young as 2yrs. Not every patient can maintain an indwelling catheter for pheresis, often they get infected or clotted. The pheresis service needs superb nurses who can place a large-bore peripheral IV in any patient.
2) most insurance companies paid the same amount for erythrocytapheresis as for a simple blood transfusion - which covered costs for neither but was much less favorable from a financial perspective for erythrocytapheresis.
3) greater demand for specially matched PRBC (negative for C, E, Kell antigens and negative for sickle hemoglobin) would strain the Blood Bank supply. Many pheresis units require partnering with a special donor recruitment programs to increase blood donations by African-Americans who are more likely than Caucasians to have RBC that are negative for C, E, Kell. The PRBC demand depends fairly strongly on whether the target HbS is 30percent or 50 percent.

My impression is that medical centers offering erythrocytapheresis combine it somehow with hemodialysis units or some other service to help break even. Or the medical centers just eat the cost because it is better for the patients and perhaps feature this service as a marketing tool.

Dr. Hae-won Kim at Children's Hospital of Philadelphia has the largest erythrocytapheresis center for sickle cell patients that I have seen. She taught many centers how to set up sickle cell erythrocytapheresis.

Sincerely,
Lewis Hsu, MD, PhD
Pediatric Hematologist
For more frequently asked questions please see:  http://www.scinfo.org/faq.htm

Gaining Ground On Sickle Cell Disease. - Children's Hospital Boston (2008, July 16). ScienceDaily. Retrieved July 17, 2008, from http://www.sciencedaily.com/releases/2008/07/080715132723.htm

Although sickle cell disease is a single-gene disorder, its symptoms are highly variable. In a study published online July 14 by the Proceedings of the National Academy of Sciences, scientists at Children's Hospital Boston and the Dana Farber Cancer Institute (DFCI), in collaboration with the Broad Institute of MIT and Harvard, report five gene variants that could potentially be helpful in predicting sickle cell disease severity, perhaps even leading to better treatment approaches in the future. The gene variants influence blood levels of fetal hemoglobin (HbF), which are known to affect symptom severity in sickle cell disease--with some patients experiencing frequent, severe pain crises and organ damage, while others are scarcely aware of their disease.

"Our study is a first step towards a better understanding of fetal hemoglobin regulation in patients with sickle cell disease," says Guillaume Lettre, PhD, of the Broad Institute and Children's Hospital Boston, and co-first author on the paper. "But further validation experiments are needed before these findings can become useful in the clinic." "Eventually, understanding the factors giving rise to heterogeneity in HbF levels might allow us to take severely affected patients and make them more like those with more benign symptoms," adds Vijay Sankaran, co-first author on the paper with Lettre and an MD-PhD student in the laboratory of Stuart Orkin, MD. (Orkin is chair of pediatric oncology at DFCI and a Howard Hughes Medical Institute investigator at Children's.)

In sickle cell disease, a single genetic mutation results in the production of an abnormal type of hemoglobin, the main component of red blood cells. The abnormal hemoglobin molecules tend to form long chains, causing red blood cells to become stiff and sickle-shaped. The distorted cells have difficulty passing through blood vessels and can block the smaller vessels, resulting in severe pain and eventual organ damage as tissues are robbed of their blood supply. The sickle-shaped red blood cells also have a very short lifespan, causing patients to be chronically anemic.

Previous research had established that retaining high levels of another type of hemoglobin--HbF, found at high levels in the fetus--can ameliorate sickle cell disease symptoms. At birth, HbF comprises between 50 to 95 percent of a child's hemoglobin, gradually declining as the switch is made to adult hemoglobin production -- consistent with clinicians' observations that newborns diagnosed with sickle cell disease usually do not become symptomatic until they are about a year old. Population studies in Saudi Arabia and parts of India had identified groups of sickle cell patients with very high levels of HbF and relatively benign forms of the disease, and additional epidemiologic studies led by Orah Platt, MD, chief of laboratory medicine at Children's, showed that HbF is an ameliorating factor. "The more you have, the better off you are," says Sankaran.

Studying 1600 patients with sickle cell disease, the researchers found that previously identified DNA sequence variants in three chromosome locations (small regions on chromosome 2, 6, and 11) were associated with high or low HbF levels. When they added these five variants to a model previously designed by Platt to predict disease severity, which also factors in age, sex, degree of anemia and HbF levels, the model's predictive ability was enhanced.

The findings need to be validated in large, prospective clinical studies, but the researchers are hopeful about the possible future clinical implications of their work. "As we find gene variants that regulate HbF levels or predict severity, we might eventually want to genotype patients for these variants, to get more predictive information on their disease," Sankaran says.

Finally, once this study is validated, understanding how these variants actually affect HbF levels might someday lead to new drugs that do the same thing. "If we can gain better insight into what these variants are doing, we may eventually have better, more targeted therapies for sickle cell disease," adds Sankaran.

Lettre and Sankaran shared first authorship of the paper. Orkin and Joel Hirschhorn, MD, PhD, of Children's and the Broad Institute, were senior authors.This study was funded by grants from the National Heart, Lung and Blood Institute, the National Institute of Diabetes and Digestive and Kidney Diseases, and the Howard Hughes Medical Institute.
Medicinal use of video games growing, may help pain
By DAVID TWIDDY The Associated Press http://www.kansascity.com/382/story/705991.html

This fall, researcher Carmen Russoniello will hand sickle cell anemia patients video game controllers and see whether playing the games helps them control stress and reduce pain caused by their disease.

Scientists, intrigued by video games' intrinsic ability to distract and focus the mind, have for decades looked for ways to use them to improve health and patient outcomes. Much of the work, however, has dwelt in obscurity as researchers struggled with small study sizes and lingering biases against what many considered a juvenile or even anti-social pastime. Even the video game industry has been unsure of what to make of the research, instead focusing primarily on the enjoyment aspect of game design.

But Russoniello, who has studied the physical and mental effects of recreation for 20 years, believes those perceptions are changing. For example, his sickle cell anemia study will be held in a clinical center operated by the highly selective and influential National Institutes of Health. In addition, insurance companies and a leading philanthropic organization have begun throwing big money behind video game research."Ten years ago, they would have laughed me out of that place," the East Carolina University researcher said. "But there's an acceptance of things. (Video games) aren't panaceas but they have their place and we need to find where that place is."

The Robert Wood Johnson Foundation announced in May that it would provide more than $2 million in grants for a dozen studies on the use of computer games for health. The projects range from using active video games, such as the Nintendo Wii, to encourage weight loss in children to seeing whether playing a driving-type video game improves cognitive and visual functions in senior citizens.This year's Games for Health conference, an annual get-together for medical scientists to review the latest in games-related research, drew more than 320 attendees, up from 120 when the meetings began four years ago, said co-founder Ben Sawyer.Among those attending this year's conference were representatives of such major health care organizations as Humana Inc., Cigna Corp. and Kaiser Permanente, which have all launched gaming projects in the past year.

Cigna, for instance, is distributing copies of the HopeLab-developed game ReMission to oncologists to give to their teenage cancer patients. The sci-fi shooter lets players blast cancer cells while learning how the disease progresses and what they can do to improve their health and well-being."Frankly, teens are a difficult group to reach," said Joe Mondy, assistant vice president of IT communications. "We think a video game approach reaches those kids where they are."

As happens in other areas of health, the research is actually trailing behind public behavior.A recent customer survey by Seattle-based PopCap Games Inc., maker of such casual games as Bejeweled, found that more than 20 percent of respondents identified themselves as having some level of physical or mental disability, and most of those said they used games as part of their therapy.One of those is Gail Nichols, 48, who said she has used video games to combat severe depression since the mid-1990s.The northeast Kansas resident, who said she suffers from post-traumatic stress disorder and other ailments, said she carries a Nintendo DS or Gameboy with her when she travels in case she begins feeling anxious or confused in public.

"If I get stressed out, my service dog is there with me. I'll pull (the game) out of her pack and between her being there with me and sitting there playing the game I won't be so nervous about people around me," Nichols said. "I would hope the medical community will add this to their bag of tricks."
Stem cell transplant for sickle cell disease subject of clinical trial

By Beth Miller http://mednews.wustl.edu/news/page/normal/12009.html

July 11, 2008 -- Children with sickle cell disease often face severe pain, organ damage, recurrent strokes and repeated, prolonged hospital stays. Although there are medical interventions that can lessen the symptoms, there is no cure.

In an effort to change that, researchers at Washington University School of Medicine in St. Louis are leading a nationwide, multicenter clinical trial to determine the effectiveness of transplanting blood stem cells from unrelated donors into children with severe sickle cell disease.

The Phase II clinical trial is led by Shalini Shenoy, M.D., associate professor of pediatrics at Washington University School of Medicine in St. Louis and medical director of the pediatric bone marrow transplant program at St. Louis Children's Hospital, and co-principal investigator Naynesh Kamani, M.D., of Children's National Medical Center in Washington, D.C. Children with severe sickle cell disease who qualify will receive a blood stem cell transplant from either bone marrow or umbilical cord blood to replace their own red blood cells. The trial seeks to enroll 45 patients ages 3-16 with symptoms of severe sickle cell disease, such as strokes, frequent pain or repeated episodes of acute chest syndrome, a painful obstruction of the blood vessels in the lungs.

To prepare to receive blood stem cells from a donor, patients must go through an intense treatment called conditioning to reduce production of their own blood cells and prevent their immune system from rejecting donor cells. For this trial, patients will receive reduced-intensity conditioning that could help them better tolerate the transplant with fewer toxic side effects of conditioning, such as infertility. As part of the trial, researchers will determine if the reduced-intensity conditioning treatment will allow the healthy donor blood stem cells to grow successfully in the patient.

Sickle cell disease is an inherited blood disorder affecting red blood cells, which contain hemoglobin, the substance that carries oxygen from the lungs to all parts of the body. In patients with this disease, red blood cells contain an abnormal type of hemoglobin that causes the normally round, flexible red blood cells to become stiff and sickle- or crescent-shaped. The sickle cells can't pass through tiny blood vessels, which can prevent blood from reaching some tissues and can result in tissue and organ damage, pain and stroke. In addition, sickle cells are short lived and lead to a shortage of red blood cells and anemia.

Sickle cell disease affects about 70,000 people in the United States and occurs in about 1 of every 500 African-American births and 1 of every 1,000 to 1,400 Hispanic-American births. Blood transfusions and bone marrow transplants have been shown to be effective treatments by replacing sickle cells with healthy red blood cells. However, blood transfusions have to be repeated regularly and can cause iron overload. Bone marrow transplants have a 10 percent mortality rate because of the possibility of infections, organ damage or graft-versus-host disease as donor cells work against the patient.

A healthy sibling who has the same tissue type as the patient is the best donor for a blood stem cell transplant, but few patients have such a suitable donor in their family. "So using unrelated donors who are close matches is the next best option if transplant is to be considered for this disease," Shenoy says. "Though, the probability of finding a good match is lower for minorities due to fewer minority donors registered in the National Marrow Donor Program, we think we can certainly find a good match for several of our patients," Shenoy says.

Physicians should only consider stem cell transplantation in children with severe cases of sickle cell disease and who have a closely matched donor, according to Shenoy."You only take a patient to transplant if the risk/benefit ratio is better by offering them the transplant," she says. "Right now, blood stem cell transplant is the only potential curative therapy for severe sickle cell disease."

In the preliminary trial, about 10 patients with sickle cell disease nationwide had successful blood stem cell transplants. The promising results generated interest in expanding the trial to a larger group of patients."For a successful outcome we need the red blood cells from the patient to be replaced by donor red cells without complications such as major graft-versus-host disease or organ damage," Shenoy says. "We think we have a good chance. If the donor red cells are rejected, we expect that the patient will recover his or her own red cells and remain at baseline with the disease."

The trial is supported by the Bone Marrow Transplant Clinical Trials Network of the National Heart, Lung, and Blood Institute, the National Marrow Donor Program, the Sickle Cell Disease Clinical Research Network and the Pediatric Blood and Marrow Transplant Consortium.

For more information about participating in the trial or about becoming a donor, contact Shenoy or Yvonne Barnes at (314) 454-6018 or visit the Bone Marrow Transplant Clinical Trials Network Web site at https://web.emmes.com/study/bmt
Sickle Cell Drug Underused by Physicians- Review of data on hydroxyurea shows it works, but long-term effects are unknown
Posted June 20, 2008 http://health.usnews.com/articles/health/healthday/2008/06/20/sickle-cell-drug-underused-by-physicians.html

FRIDAY, June 20 (HealthDay News) -- A drug that has shown some effectiveness in treating sickle cell anemia is not being used often enough by doctors who are uncertain about its proper use and possible long-term effects, according to a new report.

Researchers at Johns Hopkins University say their extensive review of studies on hydroxyurea clearly shows its helps people with sickle cell anemia, an inherited disorder that affects mostly people of African and Hispanic heritage. Their report, published in the June 17 Annals of Internal Medicine, concluded that hydroxyurea is a viable treatment option for now, but more quality research is needed.

About 70,000 people in the United States have sickle cell anemia, in which sickle-shaped blood cells periodically clump inside blood vessels, blocking circulation. This results in severe anemia, an increased risk of infections or strokes, and extreme episodes of pain that last for days.

The U.S. Department of Health and Human Services asked Johns Hopkins researchers to examine all previously published studies on hydroxyurea in an effort to increase awareness about the drug's potential.

"We know that many people with sickle cell disease aren't being offered this drug, which is the only one we have to treat this disease," Dr. Sophie Lanzkron, director of the Sickle Cell Center for Adults at Johns Hopkins, said in a prepared statement.

The team analyzed 246 quality articles on the drug and concluded it clearly works. The data suggests that once patients started taking hydroxyurea:

* Their episodes of painful sickle cell "crises" fell by 68 percent to 84 percent.
* Their hospital admissions declined by 18 percent to 32 percent.
* Their levels of fetal hemoglobin, a blood component that appears to eases sickle cell symptoms, increased by 4 percent to 20 percent.

The review also found hydroxyurea impairs sperm development in mice and may do the same in humans. The team, though, could not conclusively back theories that hydroxyurea increased or decreased the risk of leukemia or other tumors, leg ulcers and pregnancy complications from its review.

"It's clear from our literature review that hydroxyurea works, but we need to do much more work to understand how it works and the best ways to use it," Lanzkron says
Natural conception yields perfect match for transplant

Andrea W. Dilworth • Special to The Clarion-Ledger • July 15, 2008 http://www.clarionledger.com/apps/pbcs.dll/article?AID=/20080715/HEALTH/807150354/1242

Tammy and Anthony Witherspoon of McComb, both carriers of the sickle cell trait, were hesitant to bring another child into the world because of the 25 percent chance their offspring would be born with the genetic blood disease.

But when they learned son Anthony II's best chance for a cure was the umbilical cord blood of a healthy sibling, they decided to try in vitro fertilization, which they hoped would give them the opportunity to selectively implant an egg that was both free of sickle cell and a match.

"I had to cure my son," said Tammy, director of the Adolescent Offender's Program for the McComb School District.She mentioned it to Dr. Gail Megason, professor of pediatrics and director of the Division of Pediatric Hematology Oncology at the University of Mississippi Medical Center."Her words to me were, 'Bring me a healthy baby, a match.' So we did," Tammy said.

But it wasn't that simple. The in vitro fertilization, which cost the Witherspoons $12,000, was unsuccessful. The day of the scheduled implantation, Tammy and Anthony were told none of the 10 eggs doctors had retrieved from her had survived. Devastated but fertile from the medication she been taking to boost her fertility for implantation, Tammy and Anthony decided to "go home and do it the godly way. We put our faith in God and conceived the same day."

Nine months later, Amani (Swahili for faith in God) was born. Not only was he free of sickle cell, but he was a perfect match for Anthony II.Cord blood transplants are often the only option for patients with sickle cell, which primarily strikes African Americans, Megason said.

"We can find cord blood sometimes easier. It's harder finding a match for African-American patients because they are not on the national registry. It's just a cultural fear."The Witherspoons arranged to have Amani's umbilical cord blood collected by Viacord, a private cord blood bank. Months before her due date, Viacord sent the cord collection kit to the Witherspoons, who in turn took it with them to the hospital when Tammy was ready to deliver.

After Amani's delivery, doctors collected the cord blood, and a representative from Viacord picked it up for processing and storage, which takes six to eight weeks.Private banks usually charge $1,500 to $2,000 to collect, process and store units, but because of the Witherspoons' pre-existing need, their fees were waived.Anthony II wasn't able to undergo the transplant right away, though. Doctors needed to extract bone marrow from Amani to serve as a boost during the cord blood transplant, Tammy Witherspoon said. Amani had to be at least a 1-year-old for the procedure. It was a long wait, but well worth it.

Three years later, at 13, there are no signs of sickle cell anemia in Anthony II's blood, Tammy said."He is the healthiest one in the house. Every mother's dream is to have a healthy kid."

Megason does not recommend that all expectant parents make plans to bank their newborn's cord blood for private use.The Witherspoons, however, are a special case."For patients who have a child with sickle cell, leukemia or other genetic diseases that can be cured by transplantation, if you already have a child with such a disease, you would want to collect cord blood," Megason said.
National guidance issued on treating sickle cell in England

* Published: 09 July 2008 12:25 Author: Alice Coubrough http://www.nursingtimes.net/clinicalnews/2008/07/national_guidance_issued_on_treating_sickle_cell.html

The standards, published by the Sickle Cell Society, http://www.sicklecellsociety.org include guidelines for nurses in primary care as well as specialist networks for hospitals and clinics. The guidance deals with topics such as the management of chronic and acute conditions, blood transfusions, and screening.

Dr Allison Streetly, programme director for the NHS Sickle Cell and Thalassaemia Screening Programme, said: 'The introduction of these much needed national guidelines on caring for adults with the disease is greatly welcome.'Together with the care standards for thalassaemia they lay the foundation for a care framework where patient needs are properly understood both close to where people live and in specialist centres,' she added.

With sickle cell being one of the most commonly inherited genetic diseases in England, the charity said its next challenge would be to improve understanding of sickle cell and thalassaemia among both the public and healthcare professionals. The standards coincide with Sickle Cell Awareness Month, which has activities running across the country throughout July to improve awareness and management of the disease.

Dr Lorna Bennett, Chairperson, Sickle Cell Society said: ' The reality is that provision of care for adults with sickle cell disease can vary significantly between individual professionals as well as health care provision organisations. These standards are the tool needed to address the inequalities in provision and access to good quality care.' The full text PDF guide book can be downloaded athttp://www.sicklecellsociety.org/CareBook.pdf
New Center for Sickle Cell Disease Research, treatment, care to be brought together

By Katerina Pesheva Johns Hopkins Medicine http://www.jhu.edu/~gazette/2008/07jul08/07sickle.html

Johns Hopkins Children's Center has received a nearly $5 million grant from the National Heart, Lung and Blood Institute to establish a basic and translational research center for sickle cell disease that will consolidate research, treatment and care of adult and pediatric patients under one roof and speed up the translation of scientific discovery from bench to bedside. In addition, the center will offer counseling and education services to patients and their families.

Sickle cell disease is an inherited blood disorder marked by a mutation in the hemoglobin genes. The defect causes oxygen-starved, abnormal crescent-shaped red blood cells that give the disorder its name. The sickle-shaped cells get stuck in blood vessels, leading to excruciatingly painful strokes and organ damage. Sickle cell anemia affects nearly 72,000 Americans, primarily African-Americans.

"This center will be a marriage of all aspects of science and treatment, from basic science and clinical research to patient care and public health research, all part of the quest to treat and ultimately cure sickle cell disease," said lead investigator James F. Casella, Rainey Professor and chief of Pediatric Hematology at Johns Hopkins.

By drawing on the expertise of researchers from diverse areas, the center's faculty and staff expect to advance promising therapies more rapidly. For example, a basic-science project led by Allen Everett, a pediatric cardiologist, will use the science of proteomics, the study of proteins, to look for biomarkers involved in silent strokes, which are a leading cause of neurologic complications in sickle cell patients. Discoveries in this area will ultimately lead to better understanding, earlier diagnosis, treatment and prevention of neurovascular problems in sickle cell patients.

On the public health end of the spectrum, researchers from the Johns Hopkins Bloomberg School of Public Health, led by Cynthia Minkovitz, will examine how local public health services affect disease course and survival, and then pinpoint public health measures that will help eliminate state-to- state disparities in patient outcomes.

The translational arm of the center, led by urologist Arthur Burnett, will help take lab discoveries to the patient's bedside. For example, scientists will study the role of nitric oxide in sickle cell priapism, long-lasting painful erections that are a common complication of the disease. Researchers will then examine whether certain medications that affect nitric oxide levels might reduce and prevent this dreaded complication, which often requires treatment at the emergency room or hospitalization.

The center will also support a faculty scholar in sickle cell disease and a summer program in sickle cell disease research for high school students.Other partners on the grant include the University of Alabama, which, like Johns Hopkins, has a long history of sickle cell disease research and care. In February, The Johns Hopkins Hospital opened an urgent care center that specializes in treating sickle cell patients experiencing acute pain. In 2000, Hopkins opened an adult sickle cell disease center that focuses on chronic care.

New hope for bone marrow patients - An advance in stem cell research could one day give hope to patients in need of bone marrow transplants or blood transfusions, scientists have said.

Edinburgh experts used blood stem cells from mice to mimic how humans produce the stem cells and found they were able to multiply them by 150 times. They hope their findings will lead to efficient production of blood stem cells in the laboratory. They could then multiply in the body to renew a patient's blood supply.

The body generates billions of blood cells every day, which are produced by blood stem cells in the bone marrow tissue. These include red blood cells, which deliver oxygen to different organs, and white blood cells, such as lymphocytes and macrophages, which play an important role in the body's immune system.

http://news.bbc.co.uk/2/hi/uk_news/scotland/edinburgh_and_east/7507752.stm

The University of Wisconsin Pain & Policy Studies Group (PPSG) Releases new Documents for Pain Management

· Achieving Balance in Federal and State Pain Policy: A Guide to Evaluation (Fifth edition), http://www.painpolicy.wisc.edu/Achieving_Balance/EG2008.pdf

· Achieving Balance in State Pain Policy: A Progress Report Card (Fourth edition)http://www.painpolicy.wisc.edu/Achieving_Balance/PRC2008.pdf

These resources are part of the PPSG’s continuing pain and public policy research program.

The Evaluation Guide is the fifth in a series of evaluations of federal and state pain policies. The Progress Report Card quantifies state pain policies, and tracks progress to promote pain management and reduce policy barriers by comparing 2008 state policy grades with those from 2000, 2003, 2006, and 2007. These two reports are important tools that can be used by government and non-government organizations, as well as policy-makers, healthcare professionals, and advocates to understand the policies in their state that reinforce the right to pain management, or that can hinder patient access to effective treatment.

Visit the PPSG website at www.painpolicy.wisc.edu to view or download these reports, as well as a national press release, Frequently Asked Questions, and a summary of grade changes
Texas Program targets sickle cell disease
by JAN JARVIS jjarvis@star-telegram.com  http://www.star-telegram.com/health/story/746443.html

Desmond Woods tried to tough it out the morning he woke up with pain pulsating through his body. But by that night, he could no longer stand what is a common and debilitating effect of sickle cell disease.

"It’s like being stabbed over and over," said Woods, 27, of Fort Worth. "All my joints were just aching, and I was in tears crying because of the pain."

Like other adults with the disease, Woods has been in and out of hospitals searching for relief. Getting care can be difficult. Some medical workers "don’t even know what sickle cell is all about," he said. To address the concern, Harris Methodist Southwest Hospital is spearheading a program to close the gap in care for adults with sickle cell disease. Tarrant County Public Health, the Sickle Cell Disease Association and several area hospitals are also working on the project.

Goals include:

* Reducing quick-fix emergency-room visits by helping patients better manage pain.
* Putting protocols in place so patients get aggressive care as soon as they arrive in the emergency room.
* Encouraging all Tarrant County hospitals to adopt the protocols.
* Developing a day clinic and finding physicians to manage the patients.

In Texas, infants are screened for the disease and children receive care through pediatric specialists, said Mary Robinson, vice president of patient care services at Harris Methodist Southwest. But access to care changes when patients turn 18. Tarrant County has several comprehensive pediatric programs for children with sickle cell disease, said Linda Humphries, clinical nurse specialist at Harris Methodist Southwest. But once those children grow up, they tend to fall through the cracks.

"They’re not finding consistent care, so they start using the ER as their source of treatment for sickle cell," she said. Along with acute pain in their joints and backs, patients often are dehydrated when they arrive at the hospital. The kidneys, spleen, liver and other organs can be damaged. Pneumonia, urinary tract infections, gallstones and joint destruction can lead to lengthy hospitalizations. "This year I’ve been hospitalized at least four or five times," Woods said.

By teaching adults to identify triggers — including stress, infection and weather changes — the hope is they will be better able to manage their disease and avoid hospitalizations, Robinson said. When patients do end up at the hospital, knowing how to treat them aggressively will also make a difference.

Decades ago, many sickle cell patients did not reach adulthood; today, many live to their 40s. "They are surviving longer and they are still leading productive lives," Humphries said.

New Pain Advocacy Resource at www.inthefaceofpain.com

Sickle Cell Disease is featured in In the Face of Pain®, a new online advocacy toolkit designed to help healthcare professionals and patient advocates achieve greater awareness and understanding of undertreated pain as a serious national health problem.

In the Face of Pain is an interactive toolkit that enables advocates to create individualized action plans, educational materials, and presentations tailored to a specific pain-related topic. The toolkit – which can be accessed at www.inthefaceofpain.com – serves as a one-stop location for pain-related statistics and provides guidance on implementing advocacy outreach through various channels, including:

* Legislative
* Media
* Community groups
* Professional organizations

“We hope this toolkit will be a valuable resource for patients, caregivers and healthcare professionals as they work to alleviate unnecessary suffering and improve pain care practice and policy through education and advocacy,” said Pamela Bennett, RN, BSN, Executive Director, Healthcare Alliance Development at Purdue Pharma L.P., which developed the In the Face of Pain Online Advocacy Toolkit.
Caution a must for athletes with sickle-cell trait

http://www.orlandosentinel.com/sports/orl-ucfside1908jul19,0,564522.story

Athletes who possess the sickle-cell trait can play competitive sports but should be monitored by trainers if they participate in intense workouts, according to guidelines published by both the NCAA and the National Athletic Trainers Association.

The Orange County medical examiner found that symptoms associated with sickle-cell trait caused UCF football player Ereck Plancher to collapse and die after an off-season workout on March 18.

The 2008-09 NCAA Sports Medicine Handbook urges athletic trainers to exercise caution when supervising athletes diagnosed with the blood disorder, as UCF officials confirm Plancher was in 2007. Sickle-cell trait can hamper the ability of cells to carry oxygen when triggered by physical stress, a condition called sickling.

"The harder and faster athletes go, the earlier and greater the sickling," the handbook states. "Sickling can begin in only two to three minutes of sprinting, or in any other all-out exertion of sustained effort, thus quickly increasing the risk of collapse. Athletes with sickle-cell trait cannot be conditioned out of the trait and coaches pushing these athletes beyond their normal physiological response to stop and recover place these athletes at an increased risk of collapse."
Even the most fit athletes with the trait can experience a collapse, the NCAA warns.

Plancher is the 10th athlete between the ages of 12 and 19 to die after intense physical exertion from complications related to sickle-cell trait, according to NATA, which in 2007 released a "consensus statement" in which it warned trainers across the country that athletes with the sickle-cell trait were at risk during "intense exertion." Scott Anderson, head athletic trainer at the University of Oklahoma and co-chair on the task force that produced the NATA statement, said there was a lack of knowledge about the trait within the athletic population so the goal was to educate across the board. NATA suggests screening athletes for the trait and says 64 percent of Division I-A schools who responded to a survey do test for it. UCF is among those schools.

Also see http://www.orlandosentinel.com/sports/orl-ucfbox1908jul19,0,1811971.story

http://www.orlandosentinel.com/sports/college/orl-ucftrait1908jul19,0,7380501.story

Ereck Plancher's death has been linked to a single genetic flaw that millions of Americans share. It's called sickle-cell trait, and many of those who have it never know they do. That's because it rarely causes symptoms and is not even considered a medical condition or disease.

Yet an expert said Friday that some people with sickle-cell trait are vulnerable to sudden death under severe stress -- such as heat, physical exertion and dehydration. The reasons are not clear, however, and doctors say most with the trait have nothing to fear.

"It only very rarely leads to problems -- it's almost always benign," said Dr. James R. Eckman, an expert in sickle-cell trait and a professor of hematology and oncology at Emory University in Atlanta. "But under the extremes of human endurance, there can be problems. People need to understand this is a very unusual situation." There is one important caveat: Those with the trait can pass the errant gene on to their children. And if both parents have it, they have a one-in-four chance of having a child with full-scale sickle-cell disease. That's why African Americans and people in other risk groups are encouraged to find out whether they carry the gene. Beyond that, Eckman said, those with the trait do not have medical concerns in most circumstances.

One exception may be under extreme physical duress. Eckman said research suggests that exertion, dehydration and heat may trigger the distortion of red blood cells that can impede flow and starve organs of needed oxygen. But he said that risk is very small and can be reduced even further if athletes take basic precautions by drinking plenty of fluids and not overdoing it. And that's good advice for everyone -- even those without sickle-cell trait.

"Again, this is very rare," Eckman said. "In most cases, a person with sickle-cell trait does not have any symptoms, and they should be able to participate in all sports."
Sickle: A sickle crisis? (2008)

A Health outcomes study group in England, NCEPOD, was pleased to undertake a review of current haemoglobinopathy mortality, to obtain broad baseline data and make recommendations to alter practice. In this way, we hope to contribute to improving the quality of life of patients – whose numbers and attendances at health care centres are inevitably going to increase. A Full text PDF of the report and summary slides are available at http://www.ncepod.org.uk/2008sc.htm

New Grant RFP

Title: Meetings, Conferences, and Networks for Research Partnerships to Improve Functional Outcomes (R13)

Details at http://grants.nih.gov/grants/guide/pa-files/PAR-08-207.html

Background

In 2004, the NIH Rehabilitation Coordinating Committee developed a program announcement (PAR-04-077) entitled “Research Partnerships to Improve Functional Outcomes” in order to stimulate multi-disciplinary research into the difficult problems of chronic disease and rehabilitation The goal of this initiative was to encourage applicants with clinical expertise in rehabilitation or management of chronic disease to partner with scientists outside their fields to develop innovative approaches to problems. Although the announcement did generate a number of responsive applications across a wide scope of research problems, most applicants had significant difficulties in articulating how the expertise of their teams would actually go about addressing their chosen problems. In other words, applicants were successful in recruiting appropriate multi-disciplinary teams, but not in working with them to forge coherent research plans.

Goals

The present FOA attempts to address this problem by supporting meetings and workshops to bring together investigative teams to facilitate the process of developing appropriate research plans. We seek to help investigators who have the same interests in solving particular problems of rehabilitation and chronic disease, including mental disorders, and who have complementary research or clinical expertise and/or resources team up and form partnerships to coordinate, exchange, and disseminate information or to explore or clarify a defined subject, problem or area of knowledge. Proposed workshops and conferences should especially address issues in research methodology, including (but not limited to) selection of appropriate subjects, sample size, subject recruitment, measurement, trial design, and analytic strategies. It is anticipated that these workshops and conferences will help investigators become successful in submitting competitive applications for investigator-initiated research projects in the future.

Articles in the Medical Literature

Adams-Graves P, Lamar K, Johnson C, Corley P. Development and validation of SIMS: an instrument for measuring quality of life
of adults with sickle cell disease. Am J Hematol. 2008 Jul;83(7):558-62.http://www3.interscience.wiley.com/journal/117873602/abstract?CRETRY=1&SRETRY=0

Streetly A, Clarke M, Downing M, Farrar L, Foo Y, Hall K, Kemp H, Newbold J, Walsh P, Yates J, Henthorn J. Implementation of the newborn screening programme for sickle cell disease in England: results for 2003-2005. J Med Screen. 2008;15(1):9-13.http://www.ncbi.nlm.nih.gov/pubmed/18416948?ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Zempsky WT, Loiselle KA, McKay K, Blake GL, Hagstrom JN, Schechter NL, Kain ZN. Retrospective evaluation of pain assessment and treatment for acute vasoocclusive episodes in children with sickle cell disease. Pediatr Blood Cancer. 2008 Aug;51(2):265-8.http://www.ncbi.nlm.nih.gov/pubmed/18386784?ordinalpos=2&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Taori KB, Chaudhary RS, Attarde V, Dhakate S, Sheorain V, Nimbalkar P, Wasnik PN. Renal Doppler indices in sickle cell disease: early radiologic predictors of renovascular changes. AJR Am J Roentgenol. 2008 Jul;191(1):239-42.http://www.ncbi.nlm.nih.gov/pubmed/18562752?ordinalpos=5&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Klings ES, Anton Bland D, Rosenman D, Princeton S, Odhiambo A, Li G, Bernard SA, Steinberg MH, Farber HW. Pulmonary arterial hypertension and left-sided heart disease in sickle celldisease: clinical characteristics and association with soluble adhesion molecule expression.cAm J Hematol. 2008 Jul;83(7):547-53. http://www.ncbi.nlm.nih.gov/pubmed/18383329?ordinalpos=6&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Field JJ, Glassberg J, Gilmore A, Howard J, Patankar S, Yan Y, Davies SC, Debaun MR, Strunk RC. Longitudinal analysis of pulmonary function in adults with sickle cell disease. Am J Hematol. 2008 Jul;83(7):574-6.http://www.ncbi.nlm.nih.gov/pubmed/18383325?ordinalpos=7&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Lanzkron S, Strouse JJ, Wilson R, Beach MC, Haywood C, Park H, Witkop C, Bass EB, Segal JB. Systematic review: Hydroxyurea for the treatment of adults with sickle cell disease. Ann Intern Med. 2008 Jun 17;148(12):939-55. Epub 2008 May 5. Full Texthttp://www.annals.org/cgi/content/full/148/12/939

Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, James AH, Laraque D, Mendez M, Montoya CJ, Pollock BH, Robinson L, Scholnik AP, Schori M. National Institutes of Health Consensus Development Conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med. 2008 Jun 17;148(12):932-8. http://www.annals.org/cgi/content/full/148/12/932

Harban FM, Connor P, Crook R, Bingham R. Cardiopulmonary bypass for surgical correction of congenital heart disease in children with sickle cell disease: a case series. Anaesthesia. 2008 Jun;63(6):648-51. Epub 2008 Feb 29.http://www.ncbi.nlm.nih.gov/pubmed/18312603?ordinalpos=12&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Alvarez O, Lopez-Mitnik G, Zilleruelo G. Short-term follow-up of patients with sickle cell disease and albuminuria. Pediatr Blood Cancer. 2008 Jun;50(6):1236-9. http://www.ncbi.nlm.nih.gov/pubmed/18293385?ordinalpos=17&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Singh SA, Koumbourlis AC, Aygun B. Resolution of chronic hypoxemia in pediatric sickle cell patients after treatment with hydroxyurea. Pediatr Blood Cancer. 2008 Jun;50(6):1258-60. http://www.ncbi.nlm.nih.gov/pubmed/18293380?ordinalpos=18&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Dyson, SM; Abuateya, H; Atkin, K; Culley, LA; Dyson, SE; and Rowley, DT (2008) Local authorities and the education

of young people with sickle cell disorders (SCD) in England International Studies in Sociology of Education 18 (1) 47-60. [

ISSN 0962-1214] doi:10.1080/09620210802196168 http://dx.doi.org/10.1080/09620210802196168

Abuateya, H; Atkin, K; Culley, LA; Dyson, SE and Dyson, SM (2008) Young People with Sickle Cell Disorder and Education:

A Knowledge Review Diversity in Health and Social Care 5 (2): 123-135. [ISSN 1743-1913]

http://www.ingentaconnect.com/content/rmp/dhsc/2008/00000005/00000002/art00007

Ask the Experts

Question:
Are there some famous people with sickle cell disease

Answer:
All patients with sickle cell disease are heroes, but here are some who are in the public eye

* Miles Davis, jazz musician.
* Paul Williams, singer (The Temptations)
* Georgeanna Tillman, singer (The Marvelettes)
* Tionne "T-Boz" Watkins, singer (TLC)
* Prodigy, rapper (Mobb Deep).
* Hertz Nazaire - Artist

Let us know if there are others.

For more frequently asked questions please see:  http://www.scinfo.org/faq.htm
Featured Web Links
A recommitment to sickle cell disease research - The NIH agenda full text athttp://bloodjournal.hematologylibrary.org/cgi/content/full/111/10/4852

Patient Advocate Foundation at http://www.patientadvocate.org/

A New and Improved HealthFinder.gov web site at http://beta.healthfinder.gov

New Pain Advocacy Resource at www.inthefaceofpain.com

Conferences and Activities of Interest to the Sickle Cell Community

www.sicklecelldisease.org Call for abstractshttp://www.sicklecelldisease.org/docs/2008%20Call%20for%20Abstracts%2004.09.08.doc

September 18, 2008 Chicago - Management of Sickle Cell Disease Conference 2008 “ Multidisciplinary Approach” Hilton Oak Lawn- Astoria Ballroom 9333 S Cicero Ave. Oak Lawn IL. 60453 Contact: Jo Ann Allen 312-345-1100.

Friday September 5, 2008 Annual Sickle Cell Golf Classic Ramblewood Golf & Country Club, 200 Country Club Parkway, Mt. Laurel, N.J. Tee Time: 11:00 am
Saturday September 20, 2008 Annual Sickle Cell Seminar Philadelphia International House 3701 Chestnut Street 10:00 am- 4:00 pm.
Saturday September 27, 2008 11th Annual Walter E. Brandon Sickle Walkathon & " Lighten the Load " CD Launch Party Philadelphia Carousel House
Belmont Avenue & North Concourse Drive 8:00 am- 2:00 pm
Saturday September 27, 2008 " Dancing with the Philadelphia Stars " 7:00 - 12:00 pm
For specific information about each event or sponsorship opportunities contact: Sickle Cell Disease Association of America, Philadelphia/ Delaware Valley Chapter215-471-8686 or visit our website at www.sicklecelldisorder.com

The Sickle Cell Thalassemia Patients Network , NY, The Sickle Cell Disease Association of America, of Southern, CT The Sickle Cell Disease Association of America, Philadelphia/Delaware Valley The Donna T. Darrien Memorial Foundation For Sickle Cell Inc. Newark, NJ

Celebrities Isaac Hayes and WBLS DJ Dr. Bob Lee For more information E-mail: scdaasouthernct@sbcglobal.net

http://www.scdaaofsouthernct.org/

Special Guest Speaker: Dr Michael R DeBaun Venue: Governor’s Hall, St Thomas Hospital , London . United Kingdom

· New Diagnosis Frontiers, including Mass spectrometry (MSMS) · Cutting Edge Research and Iron Chelation · Stem Cell Transplantation, Pre-implantation Genetic Diagnosis. · Asthma, Obstructive Sleep, Avascular Necrosis , Endocrine · NECPOD: Confidential Enquiry into Sickle Cell Disease
Contact: Mary.Abiri@gstt.nhs.uk, 44(0)2071887774, or Helen.Appleby@gstt.nhs.uk

Web Site: www.mysite.verizon.net/bizst88q/ Email: stpetesicklcell@verizon.net phone 727-896-2355

Can Flying High Make you Sick?
By Kate McHugh, Ivanhoe Health Correspondent http://www.ivanhoe.com/channels/p_channelstory.cfm?storyid=16579
ORLANDO (Ivanhoe Newswire) -- Could that turning feeling in your stomach during an airplane flight be something other than nerves?
A new study in The New England Journal of Medicine tested the effects of exposure to high altitudes to see if acute mountain sickness -- a sensation felt by visitors to mountainous areas -- could be experienced by airplane passengers as well.
Researchers found oxygen in the blood decreased by 4.4 percent at 7,000 to 8,000 feet -- the altitude at which acute mountain sickness is experienced. However, only 7.4 percent of the participants reported complaints of mountain sickness. The symptoms were first felt after 3 to 9 hours of exposure to high altitudes.

So why are symptoms different when the altitudes are the same? Michael, Zimring, M.D., and director of the Center for Wilderness and Travel Medicine at the Mercy Medical Center in Baltimore, Maryland, has a theory -- and it's all about exertion.

"When you go up in a plane to 8,000 feet, you're sitting, you're doing nothing, you're not exerting energy, you don't need as much oxygen, so therefore you're not going to have a problem with a couple hour flight with altitude sickness," Dr. Zimring told Ivanhoe. "Whereas when you go up skiing, if you're young and active and healthy, you're going to be exerting yourself right away. That's where you get acute mountain sickness."
Dr. Zimring recommends all travelers drink lots of water to stay hydrated. He also says unhealthy patients with lung cancer, heart disease or sickle cell disease may experience altitude sickness in airplanes as well.

Sickle cell testing for athletes increases MU athletes do not have to be tested, but they can opt for test.

By ALEX LANGE http://www.columbiamissourian.com/stories/2007/07/13/sickle-cell-testing-athletes-increases/
It’s been two years since MU football player Aaron O’Neal died during a voluntary practice at Faurot Field. O’Neal’s death was initially attributed to lymphocytic meningitis by then-Boone County Medical Examiner Valerie Rao. A University Hospital neuropathologist who examined O’Neal’s brain later said sickle cell trait could have played a role. As a result, the sickle cell trait might factor into the wrongful death lawsuit brought by O’Neal’s parents against 14 current and former members of the athletic department. In the two years since O’Neal’s death, the number of MU student athletes who have asked to be tested for sickle trait has increased tenfold — from five to almost 50. But MU’s athletic department has not instituted mandatory testing for the trait, nor does the NCAA require it. “Testing is still not mandatory, and I don’t think that it is ever going to be mandatory,” said Rex Sharp, MU’s director of sports medicine. “I just don’t see that happening right now.” Sickle cell trait is a condition that can cause red blood cells to logjam in veins and arteries and block the vital flow of oxygen to muscle tissue during strenuous exercise.

Athletic trainers step up efforts to combat sickle cell trait

http://www.latimes.com/sports/football/nfl/la-spw-trainers28jun28,1,2323976.story?coll=la-headlines-sports-nfl&ctrack=1&cset=true
The National Athletic Trainers Assn., with more than 6,000 in attendance in Anaheim this week, on Wednesday took a first step toward making the athletic field safer for black athletes, as well as others who carry the potentially lethal sickle cell trait.

Having spearheaded a task force that included more than 20 organizations, the NATA recommended that colleges and high schools show greater awareness of the typically benign condition, which poses a grave risk during intense exertion of physical activity.

"You can take a body of athletic trainers, a body of team physicians, a body of family practice physicians, a body of coaches and athletes, if you ask them what are the non-traumatic causes of deaths for athletes, No. 1 they would say cardiac, No. 2 they would say heat stroke, but after that you'd probably get a lot of head scratching," said Scott Anderson, head athletic trainer at Oklahoma and co-chair of the task force. "Sickle cell trait is the third-leading cause of non-traumatic death at the secondary and collegiate level."

The inherited blood disorder ranks just ahead of asthma, the No. 4 killer.
"We have a lot of kids with asthma," said Lou Randall, who coached Riverside North to the Southern Section's Eastern Division football title. His program is comprised of about 60% black athletes and, statistically, would have about seven or eight players with the trait. "As far as I see, there's nothing in the medical history that shows" sickle cell trait. "I could be wrong. I look for what they're allergic to and if they've had any major injuries."
Randall isn't the only one in the dark.

Anderson cited the similarity in deaths of the first college football player to die of sickle cell trait, Colorado sophomore Polie Portier in 1974, and the most recent, Rice freshman defensive back Dale Lloyd in 2006.

"Thirty-two years later, the athlete, the coaches, the athletic trainer didn't know he had it. Nothing's changed," Anderson said. "There was no association with exertion rate and sickle cell trait. There's a lack of awareness of the syndrome or the risk."

Sickle cell trait is the inheritance of one gene for sickle hemoglobin and one for normal hemoglobin. Under extensive exertion, the sickle hemoglobin can change the shape of red blood cells to that of a quarter moon, or the shape of a farmer's sickle.

Although newborn children are screened for the trait, by the time they enter high school they are often unaware of their condition, which is why the NATA recommended secondary institutions and universities consider testing an athlete, Anderson said, essentially "for the cost of a pizza" from certain laboratories.

Over the past seven years, nine athletes — including a 14-year-old female basketball player and two 12-year-olds training for football — have died when their red blood cells began "sickling." The sickle-shaped cells create a logjam in the blood vessels which can lead to collapse from the rapid breakdown of muscles starved for blood.

Deadly sickling can begin in two to three minutes of any all-out exertion, such as running sprints or stadium steps, and poses a grave risk to athletes if they aren't allowed a longer recovery period between repetitions. Lloyd, the Rice football player, died after running 16 sprints of 100 yards.
One of the critical points of the consensus statement was that having the trait should not prevent an athlete from participation, however training should build up slowly with paced progressions. Athletes should ideally work at their own pace, engage in year-round strength and conditioning programs that meet sport-specific demands, and cease activity with the onset of symptoms such as muscle cramping, pain, swelling, weakness, tenderness, the inability to catch one's breath or fatigue.

About 8% of the general African-American population in the U.S. has the trait, and a study showed that 7% of African-American players in the NFL have it. Among those are Devard Darling, wide receiver for the Baltimore Ravens, whose twin brother, Devaughn, a freshman linebacker at Florida State, died of complications from sickle cell trait in 2001. Devard, one of the featured speakers Wednesday, also carries the trait.
"A lot of people who are in position to help, coaches at the secondary level, they aren't aware of the trait, aren't up to date with the protocols, the trauma," Darling said. "You can't bury your head in the sand and think nothing is happening. Kids are dying, and it's time for people to do something about it. Athletic trainers, they have to know. More important, the athletes have to know their bodies and have to know they carry the trait.
"It needs to be on every high school application."

More information is available at http://www.nata.org

Network Model Predicts Risk of Death in Sickle Cell (Boston) – Researchers from Boston University School of Medicine (BUSM) and Boston University School of Public Health (BUSPH) have developed a method to estimate sickle cell disease severity and predict the risk of death in people with this disease. The study appears online in the June issue of the journal Blood http://www.bu.edu/phpbin/news/releases/display.php?id=1367

Recovery Difficult, but New Treatment Gives Teen New Hope
Experimental bone marrow transplant rids girl's body of sickle cell
http://www.ajc.com/metro/content/metro/northfulton/stories/2007/07/11/sicklens_0712.html

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