October 22, 2016
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Sickle Cell News For May

 Be the Match campaign highlights sickle cell disease
See all the public service announcements airing on national TV stations at http://www.marrow.org/HD/awareness2010/index.html


Research to Preserve Cognitive Abilities is Under Way

Sickle cell disease may affect brain function in adults who have few or mild complications of the inherited blood disease, according to results of the first study to examine cognitive functioning in adults with sickle cell disease. The multicenter study, funded by the National Heart, Lung, and Blood Institute (NHLBI), part of the National Institutes of Health, compared brain function scores and imaging tests in adult patients with few sickle cell complications with results in similar adults who did not have the blood disease.
Researchers report that the brain function scores in sickle cell patients were, on average, in the normal range. However, twice as many patients as healthy adults (33 percent versus 15 percent) scored below normal levels. Those who were more likely to score lower were older and had the lowest levels of hemoglobin, the protein in red blood cells that carries oxygen in the blood, compared to sickle cell participants who scored higher. Findings from brain magnetic resonance imaging scans did not explain differences in scores.
Researchers at 12 sites within the NHLBI-supported Comprehensive Sickle Cell Centers conducted the study. Their results are published in the May 12 issue of the Journal of the American Medical Association. An editorial accompanies the article.
"This study suggests that some adult patients who have sickle cell disease may develop cognitive problems, such as having difficulty organizing their thoughts, making decisions, or learning, even if they do not have severe complications such as stroke related to sickle cell disease," said NHLBI Acting Director Susan B. Shurin, M.D. "Such challenges can tremendously affect a patient's quality of life, and we need to address these concerns as part of an overall approach to effectively managing sickle cell disease."
Researchers tested cognitive functioning of 149 adult sickle cell disease patients (between the ages of 19 and 55) and compared them to 47 healthy study participants of similar age and education levels from the same communities. All of the participants were African-American.
More sickle cell disease patients scored lower on measures such as intellectual ability, short-term memory, processing speed, and attention, than participants in the healthy group. The sickle cell disease participants did not have a history of end-organ failure, stroke, high blood pressure, or other conditions that might otherwise affect brain function.
"We need to study whether existing therapies, such as blood transfusions, can help maintain brain function, or perhaps even reverse any loss of function," noted Elliott P. Vichinsky, M.D., of the Children's Hospital & Research Center Oakland, principal investigator of the study and the lead author of the paper. "These effects were found in patients who have clinically mild sickle cell disease, which raises the question of whether therapies should be given to all patients to help prevent these problems from developing."
Researchers involved in this study are recruiting patients with sickle cell disease into a clinical trial to determine whether blood transfusions may help preserve cognitive function. Participants will receive transfusions every three or four weeks for six months as part of the clinical study. Information about this study can be found at <www.clinicaltrials.gov>, search for NCT00850018.
Sickle cell disease affects about 70,000 Americans. At one time, many children died from the disease, but new therapies have enabled sickle cell disease patients to live well into middle age or beyond. As more people with sickle cell disease are living into adulthood, health care providers are uncovering previously unrecognized complications.
Studies of brain function in children who have sickle cell disease have suggested that some children with the disease, even if they have not suffered a stroke, have experienced silent brain injury. Others without obvious changes on brain scans may have some level of cognitive dysfunction that seems to worsen with age. Stroke is a common complication of sickle cell disease, and can lead to learning disabilities, lasting brain damage, long-term disability, paralysis, or death.
Sickle cell disease involves an altered gene that produces abnormal hemoglobin. Red blood cells with sickle hemoglobin that have too little oxygen become C-shaped in addition to becoming stiff and sticky. These crescent-shaped cells can clump to block blood flow, causing severe pain and potential organ damage. In the United States, the disease mainly affects those of African descent, but it is also found in other ethnic groups, including those of Hispanic and Middle Eastern descent.


Easing bone marrow transplants to widen their use By LAURAN NEERGAARD

The Associated Press  Monday, May 10, 2010; 3:08 PM  http://www.washingtonpost.com/wp-dyn/content/article/2010/05/10/AR2010051003124.html

 WASHINGTON -- Bone marrow transplants are undergoing a quiet revolution: No longer just for cancer, research is under way to ease the risks so they can target more people with diseases from sickle cell to deadly metabolic disorders

How I survived sickle cell anaemia----Wale Fanu


There is nothing in Wale Fanu's countenance to suggest that the 60 year-old is a survivor of sickle cell anaemia. At age 13, the doctor took one look at him and ruled him out of surviving beyond the age of 21. His modest office which houses Cinecraft Limited, his TV production outfit in Surulere, Lagos, teems with legacies of an accomplished personality.

Sickle Cell Disease Association of America, Inc. Call For Abstracts 

SCDAA 38th Annual Convention  September 21-24, 2010 Washington DC  -Gaylord National Hotel & Conference Center Washington D.C.  www.sicklecelldisease.org -  This conference is designed for patients, parents, health care providers, administrators, educators, advocates, researchers and community-based organizations.  The focus is on strengthening the infrastructure of the sickle cell community through education and action; fostering advocacy and collaborative networks with the best possible programs, practices and policies that result in prevention of illness, improvement of the quality of life of affected persons, and finding a cure for Sickle Cell Disease.  Click here for a PDF Abstract submission form. Click here for a Word version of the Abstract submission form

Rehospitalizations and emergency treatment for sickle cell disease.

Listen to a new podcast from AHRQ about a study showing that many people with sickle cell disease end up going back to the hospital for additional treatment http://www.healthcare411.ahrq.gov/radiocastseg.aspx?id=1097&type=seg

Advances Reported in Transitioning Pediatric Sickle Cell Disease Patients to Adult Providers


Newswise — Forty years ago, children born with sickle cell disease (SCD) were not expected to live past their teenage years. Today, medical advances are allowing children to live well into adulthood, raising concerns over who is going to care for these patients as adults. However, programs are underway to address these concerns, as reported in the March/April edition of Journal of Pediatric Oncology Nursing, published by the Association of Pediatric Hematology/Oncology Nurses (APHON).

Sickle cell disease is the most common genetic disorder among African Americans. One in 500 African-American newborns has the blood disease, but life expectancy has risen from age 14 in 1973 to around 50 today.

Donna M. Doulton, BSN, RN, is the Project Coordinator of the Regional Children's Sickle Cell Center, part of CHRISTUS Santa Rosa Children’s Hospital (CSRCH) and the University of Texas Health Science Center in San Antonio (UTHSCSA). It is a comprehensive program pro¬viding services to children and adolescents with SCD and their families. Doulton writes that the center has developed a two-part transition program to care for their pediatric patients as they reach adulthood.

“We have transitioned 20 of our 18- to 27-year-old patients to adult providers recognizing that early preparation is essential. At the newborn’s initial visit or transfer from another clinic the transition program is explained to the family. At age 13 years, all our patients are given a ‘Preparation for Transition’ binder. This binder is reviewed in detail with the patient and parent on a regular basis. At 18 years of age, coordinating with the milestone of graduating from high school and depending on developmental age, the transition is completed. The goal is a continuum of care in the transition process,” Doulton wrote.

“As a team, we are reinforcing to our patients and families that SCD is only part of them, not their entire identity. Regularly in our center we stress the positive aspects about life’s struggles, acknowledging that is it hard and it is sometimes not fair. During the development of this preparation program, one area that required tactfulness was the area of career/vocation as it involved talking about the hopes and dreams of the patient. It is truly a delicate situation to assist the patient to strive for their dreams with consideration of the realities of having a debilitating chronic illness that is unpredictable,” Doulton wrote.

“Our main goal is that children being cared for in our sickle cell center will be knowledgeable about their disease and actively participate in all aspects of their health care from cradle to commencement. It is our hope that through our collective efforts of our team that these patients will be much better prepared for a long success¬ful future, living well with sickle cell disease,” Doulton concluded.

Dr Ian Smith helps sickle cell education

Dr Ian Smith helps the Be Sickle Smart program http://www.50millionpounds.com/the_challenge/be_sickle_smart/iron_overload.aspx

Sickle Cell Empowerment tour schedule http://www.50millionpounds.com/the_challenge/be_sickle_smart/empowerment_tour.aspx


Articles in the Medical Literature

1: Vichinsky EP, Neumayr LD, Gold JI, Weiner MW, Rule RR, Truran D, Kasten J, Eggleston B, Kesler K, McMahon L, Orringer EP, Harrington T, Kalinyak K, De Castro LM, Kutlar A, Rutherford CJ, Johnson C, Bessman JD, Jordan LB, Armstrong FD; Neuropsychological Dysfunction and Neuroimaging Adult Sickle Cell Anemia Study Group. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010 May 12;303(18):1823-31. http://www.ncbi.nlm.nih.gov/pubmed/20460621

2: Okwi AL, Ocaido M, Byarugaba W, Ndugwa CM, Parkes A. Sickling and solubility tests and the peripheral blood film method for screening for sickle cell disease: A cost benefit analysis. S Afr Med J. 2009 Dec 7;99(12):887-91. http://www.ncbi.nlm.nih.gov/pubmed/20460000

 3: Barabino GA, Platt MO, Kaul DK. Sickle Cell Biomechanics. Annu Rev Biomed Eng. 2010 May 10. http://www.ncbi.nlm.nih.gov/pubmed/20455701

4: Elagouz M, Jyothi S, Gupta B, Sivaprasad S. Sickle Cell Disease and the Eye: Old and New Concepts. Surv Ophthalmol. 2010 May 7. http://www.ncbi.nlm.nih.gov/pubmed/20452638

5: Cohen RT, DeBaun MR, Blinder MA, Strunk RC, Field JJ. Smoking is associated with an increased risk of acute chest syndrome and pain among adults with sickle cell disease. Blood. 2010 May 6;115(18):3852-4. http://www.ncbi.nlm.nih.gov/pubmed/20448118

6: Jones BP, Ganesan V, Saunders DE, Chong WK. Imaging in childhood arterial ischaemic stroke. Neuroradiology. 2010 May 6. http://www.ncbi.nlm.nih.gov/pubmed/20445969

7: Wright J, Ahmedzai SH. The management of painful crisis in sickle cell disease. Curr Opin Support Palliat Care. 2010 Jun;4(2):97-106. http://www.ncbi.nlm.nih.gov/pubmed/20440206

8: Goodman BM 3rd, Artz N, Radford B, Chen IA. Prevalence of vitamin D deficiency in adults with sickle cell disease. J Natl Med Assoc. 2010 Apr;102(4):332-5. http://www.ncbi.nlm.nih.gov/pubmed/20437740

9: Enninful-Eghan H, Moore RH, Ichord R, Smith-Whitley K, Kwiatkowski JL. Transcranial Doppler Ultrasonography and Prophylactic Transfusion Program Is Effective in Preventing Overt Stroke in Children with Sickle Cell Disease. J Pediatr. 2010 Apr 29. http://www.ncbi.nlm.nih.gov/pubmed/20434165

10: Jha A, Suarez ML, Ferrans CE, Molokie R, Kim YO, Wilkie DJ. Cognitive testing of PAINReportIt in adult African Americans with sickle cell disease. Comput Inform Nurs. 2010 May-Jun;28(3):141-50. http://www.ncbi.nlm.nih.gov/pubmed/20431356

11: Prabhakar H, Haywood C Jr, Molokie R. Sickle cell disease in the United States: looking back and forward at 100 years of progress in management and survival. Am J Hematol. 2010 May;85(5):346-53. http://www.ncbi.nlm.nih.gov/pubmed/20425797

12: Ramakrishnan M, Moïsi JC, Klugman KP, Iglesias JM, Grant LR, Mpoudi-Etame M, Levine OS. Increased risk of invasive bacterial infections in African people with sickle-cell disease: a systematic review and meta-analysis. Lancet Infect Dis. 2010 May;10(5):329-37. Review. http://www.thelancet.com/journals/laninf/article/PIIS1473-3099%2810%2970055-4/fulltext

13: Aliyu ZY, Suleiman A, Attah E, Mamman AI, Babadoko A, Nouraie M, Mendelsohn L, Kato GJ, Gordeuk VR, Gladwin MT. NT-proBNP as a marker of cardiopulmonary status in sickle cell anaemia in Africa. Br J Haematol. 2010 Apr 16. http://www.ncbi.nlm.nih.gov/pubmed/20408845

14: de-Graft Aikins A, Unwin N, Agyemang C, Allotey P, Campbell C, Arhinful D. Tackling Africa's chronic disease burden: from the local to the global. Global Health. 2010 Apr 19;6(1):5. http://www.globalizationandhealth.com/content/6/1/5


Ask the Experts

Question: Should those with sickle cell disease and sickle cell trait take malaria prevention medications when traveling to an area where it is present?


Yes, See : Oniyangi O, Omari AAA. Malaria chemoprophylaxis in sickle cell disease. Cochrane Database of Systematic Reviews 2006, Issue 4. Art. No.: CD003489. DOI: 10.1002/14651858.CD003489.pub2 at http://www2.cochrane.org/reviews/en/ab003489.html full text PDF  http://www.thecochranelibrary.com/SpringboardWebApp/userfiles/ccoch/file/CD003489.pdf




and CDC guidelines at http://wwwnc.cdc.gov/travel/yellowbook/2010/chapter-2/malaria.aspx#990

Featured Web Links
Ask a geneticist - See the question and answers about sickle cell disease and trait. You can submit your own questions at

Conferences and Activities of Interest to the Sickle Cell Community

May 14, 2010  New York, NY An All Day Symposium : Sickle Cell Disease at 100   The latest Advances in Treatment  May 14, 2010 Sponsored by the   Comprehensive  Sickle Cell/Thalassemia Program  At New York Methodist Hospital  Brooklyn, New York  718-857-5643

May 23, 2010.  Glendale, CA- Sickle Cell Open Forum Time: 3PM – 5PM  Held at: Cayenne Wellness Center, 208 S. Louise Street, Glendale, CA  91205, 818 377-5120  

To RSVP or for more information Contact: Nita Thompson, African American Blood Drive and Bone Marrow Registry for Sickle Cell Disease AwarenessP O Box 1275, Inglewood, CA 90308-1275, (323) 750-1087 AA4SCDAwareness@aol.com   Http://www.aablooddriveandmarrowregistry4sicklecellawareness.webs.com

June 2nd  Raleigh, NC - North Carolina sickle cell patients are planning to go to the state capitol.  We will commemorate this 100th year of SCD and participants will share their experiences with this condition. Wednesday, June 2nd  Raleigh, NC - meet with legislators  Information: SCDAA-Eastern NC Chapter  (800) 826-1314
or Crescent Health Institute : (980) 207-3175 ext 111 or rally@crescent282.info

 June 19, 2010 Toronto, ON- World Sickle Cell Day Celebration

Saturday June 19, 2010  6:30pm - 9:30pm  Delta Toronto East  2035 Kennedy Road Toronto,  ON M1T3G2  Call the

Sickle Cell Association of Ontario 416-789-2855 email sicklecell@look.ca or email info@sicklecellontario.com visit us on the web www.sicklecellontario.com

Say It Loud! Conversations on Sickle Cell Disease Teleconference Wednesday, June 30, 2010

Choose a time best for you: 12:30 p.m. to 12:55 p.m. EST or 6:30 p.m. to 6:55 p.m. EST You’re invited to join in by dialing 1 712 432-0080

Passcode 490765# Access available as long as lines are open. Host: Mary Bentley LaMar, Executive Director

The Sickle Cell Association of New Jersey, Inc., (SCANJ)

Say It Loud! Conversations on Sickle Cell Disease is a collaborative effort initiated by The Sickle Cell Association of New Jersey, Inc., The Donna T. Darrien Memorial Foundation, The Sickle Cell Disease Association of America, Philadelphia/Delaware Valley Chapter and The Sickle Cell Disease Foundation of California.  “Say It Loud!...” is open to all who are interested in spreading the word about Sickle Cell Disease.  For more information contact Mary at sicklecellanj@gmail.com or 973 482-9070.
June 19, Edison, New Jersey -  Attendees of Sickle Cellabration can expect educational workshops for families, enjoyable activities for kids, exhibits and the Cellabration Luncheon that honors children in the New Jersey Sickle Cell Poster Child Program.   There will also be the Dr. Charles Drew Commemorative Blood Drive from 9am to 3pm.


For more information, please contact Mary Bentley LaMar at 973.482.9070 or sicklecellanj@gmail.com.

You may also log onto sicklecellabration.org
Seating is limited so please RSVP for this FREE event by  Friday, June 11.

June 17 and 18  2010  King's College London -  Sickle Cell in Focus (SCiF) is an annual, two-day summer conference held at King's College London Demark Hill campus to highlight and discuss emerging clinical complications and clinical management of sickle cell disease. The clinical and scientific research lectures are aimed at an audience of consultants, academics, trainee doctors and health professionals involved in the care of patients with the disease. It attracts local, national and international guest speakers and delegates.  Full details can be found at: http://www.kcl.ac.uk/schools/medicine/research/gcbt/scif


2nd World Sickle Cell Day, June 19, 2010  - Multi city world wide See http://aablooddriveandmarrowregistry4sicklecellawareness.webs.com/worldsicklecellday.htm

July 15 - 18 Kiawah Island Resort near Charleston, South Carolina - 10th Annual Using Transcranial Doppler, MRI/MRA and Transfusion to Prevent Stroke in Sickle Cell Disease. This activity has been approved for AMA PRA credit. For more information, contact: Office of Continuing Medical Education Medical University of South Carolina, Charleston, SC 29425 Phone: 843-876-1925 • Email: maxwells@musc.edu

July 24, 2010 Ponte Vedra Beach, Florida - Announcing sickle cell workshop at  SERGG/SERC annual meeting The SERC concurrent sickle cell workshop will be held on: Saturday, July 24, 2010 from 8:00 am – 5:00 pm   SERGG/SERC July 22-24, 2010  Sawgrass Marriott Hotel Ponte Vedra Beach, Florida 

Deadlines: Abstract submission – friday, may 21 (5:00 pm) Meeting registration – Friday, June 25 Hotel registration – Wednesday, June 30

Click here for  SERGG/SERC meeting information and registration forn  Click here for an agenda. Click here for  the abstract submission form.  Your support of the full annual meeting would be greatly appreciated; however, if you can only attend the one-day workshop, the advance registration fee is only $85.00!

July 20-23, 2010 Accra, Ghana within the First Global Congress on Sickle Cell Disease, July 20-23, 2010, co-sponsored by the Sickle Cell Center at Children’s Hospital of Philadelphia and The Sickle Cell Foundation of Ghana. For information email sicklecellsymposium@email.chop.edu

The Congress website: www.sicklecellglobalcongress.org  is now online. Please check it often for announcements and changes.

2. Abstract Submission Deadline

We have extended the abstract submission deadline to March 31, 2010.  If you want to revise and resubmit an abstract you already sent to us, you may do so. Please mark the resubmitted abstract with “RESUBMISSION” on the top.

3. Registration

Please go to our website: www.sicklecellglobalcongress.org where you can register online (in English only) or download the Registration Form (which is in English, French, and Portuguese) and send by mail or fax.  For more information, please email sicklecellsymposium@email.chop.edu or call:  Trudy Tchume-Johnson   +001-267-426-5037, Henrietta Enninful-Eghan +001-267-426-7492, or, Tannoa Jackson   +001-267-426-9338.

August 23 -27 Bethesda, MD - NIH Annual Sickle Cell Disease Clinical Research Meetings http://www.nhlbi.nih.gov/meetings/sicklecell-10/index.htm


September 21-24, 2010 Washington DC  - SCDAA 38th Annual Convention  Gaylord National Hotel & Conference Center Washington D.C. Sickle Cell Disease Association of America, Inc. www.sicklecelldisease.org -  This conference is designed for patients, parents, health care providers, administrators, educators, advocates, researchers and community-based organizations.  The focus is on strengthening the infrastructure of the sickle cell community through education and action; fostering advocacy and collaborative networks with the best possible programs, practices and policies that result in prevention of illness, improvement of the quality of life of affected persons, and finding a cure for Sickle Cell Disease.

October 4 Atlanta GA - Sickle Cell Education Day 9 - 3  - Loudermilk Center:  40 Courtland Street, Atlanta, GA 30303



22 - 27 November 2010— Raipur (Chhattisgarh) IndiaSickle Cell Disease International Organization in collaboration with Centre for Genetic Diseases & Molecular Biology Department of Biochemistry, Pt. J.N.M.MedicalCollege, Raipur (C.G.) INDIAare organizing the Fourth International Congress 2010 Sickle Cell Disease International Organization                                                           

1.Simple models of survey/ screening. 2.  Methods of counseling for :a. General population b. Youth & marriageable age group (premarital counseling) c. Post marriage counseling for carriers and sufferers including antenatal checkup, family planning, MTP, adoption of child. d. Counseling for sufferers of the disease. e. Counseling for the parents of sickle cell disease affected children. 3.       Models of treatment plan at primary, secondary and tertiary level including plan for sickle cell clinics at village level, district level and super specialty clinic at medical college level. 4.       Scope of research in developing countries. 5.       Advocacy for financial support, scope of a network and linking the various NGOs working in the field of sickle cell disease.  KEY DATES 22nd November 2010 - Preconference briefing:to be attended by NGOs, Doctors and Technicians working in the field of sickle cell anemia. 23ed -24th November 2010- Scientific Sessions. 25th November 2010- General/Executive body meeting of the congress & draft presentation of the proceedings. 26th November 2010- Sight Seeing.27th November 2010- Valedictory Function; Conclusion note, approval of proceedings. FIRST ANNOUNCEMENT & CALL FOR PAPERS  http://4sccongress.co.in

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