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Sickle Cell News Round-Up


WASHINGTON -  Girl, 12, reflects progress made in sickle cell disease

 Sickle cell cure 

 Health watch Atlanta

 NIH Directors award to  Ga Tech  sickle cell research   

Vanderbilt And Meharry Establish Center For Excellence In Sickle Cell Disease

The NHLBI has just published a new Program Announcement (PA) entitled:  "Sickle Cell Disease: Inflammation, Thrombosis and Vascular Dysfunction (R01)".

The contact for this PA is Andrei Kindzelski, MD, PhD,

NIH funds Center of Excellence for Molecular Hematology at Cincinnati Children's - Cincinnati Children's Hospital Medical Center has been named one of five national Centers of Excellence for Molecular Hematology to find new gene and cell therapies for inherited diseases affecting blood cells


Articles in the Medical Journals


Guidelines for the Prevention of Stroke in Patients With Stroke or Transient Ischemic Attack. A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association.

Furie KL, Kasner SE, Adams RJ, Albers GW, Bush RL, Fagan SC, Halperin JL, Johnston SC, Katzan I, Kernan WN, Mitchell PH, Ovbiagele B, Palesch YY, Sacco RL, Schwamm LH, Wassertheil-Smoller S, Turan TN, Wentworth D; on behalf of the American Heart Association Stroke Council, Council on Cardiovascular Nursing, Council on Clinical Cardiology, and Interdisciplinary Council on Quality of Care and Outcomes Research.

Stroke. 2010 Oct 21. [Epub ahead of print]



Combined effects of arginine and hydroxyurea on BFU-E derived colony growth and hbf synthesis in erythroid progenitors isolated from sickle cell blood.

Baliga BS, Haynes J Jr, Obiako B, Mishra N.

Cell Mol Biol (Noisy-le-grand). 2010 Jun 1;56 Suppl:OL1290-8.



One hundred years of sickle cell disease.

Serjeant GR.

Br J Haematol. 2010 Oct 19. doi: 10.1111/j.1365-2141.2010.08419.x. [Epub ahead of print]



A randomized phase II trial of Arginine Butyrate with standard local therapy in refractory sickle cell leg ulcers.

McMahon L, Tamary H, Askin M, Adams-Graves P, Eberhardt RT, Sutton M, Wright EC, Castaneda SA, Faller DV, Perrine SP.

Br J Haematol. 2010 Oct 19. doi: 10.1111/j.1365-2141.2010.08395.x. [Epub ahead of print]



The lives of adults over 30 living with sickle cell disorder.

Caird H, Camic PM, Thomas V.

Br J Health Psychol. 2010 Oct 14. [Epub ahead of print]



High reticulocyte count is an independent risk factor for cerebrovascular disease in children with sickle cell anemia.

Silva CM, Giovani P, Viana MB.

Pediatr Blood Cancer. 2010 Oct 14. [Epub ahead of print]



Pain management in children and adolescents with sickle cell disease.

Jerrell JM, Tripathi A, Stallworth JR.

Am J Hematol. 2010 Sep 16. [Epub ahead of print] No abstract available.



Pulmonary hypertension in hemolytic anemias.

Wahl S, Vichinsky E.

F1000 Med Rep. 2010 Feb 11;2. pii: 10.



Maternal and perinatal outcomes amongst haemoglobinopathy carriers: a systematic review.

Jans SM, de Jonge A, Lagro-Janssen AL.

Int J Clin Pract. 2010 Nov;64(12):1688-98. doi: 10.1111/j.1742-1241.2010.02451.x.



Silent cerebral infarcts occur despite regular blood transfusion therapy after first stroke in children with sickle cell disease.

Hulbert ML, McKinstry RC, Lacey JL, Moran CJ, Panepinto JA, Thompson AA, Sarnaik SA, Woods GM, Casella JF, Inusa B, Howard J, Kirkham FJ, Anie KA, Mullin JE, Ichord R, Noetzel M, Yan Y, Rodeghier M, Debaun MR.

Blood. 2010 Oct 12. [Epub ahead of print]



β-Globin Gene Cluster Haplotypes in a Cohort of 221 Children with Sickle Cell Anemia or Sβ-Thalassemia and Their Association with Clinical and Hematological Features.

Belisário AR, Martins ML, Brito AM, Rodrigues CV, Silva CM, Viana MB.

Acta Haematol. 2010 Oct 12;124(3):162-170. [Epub ahead of print]



Walter Clement Noel—first patient described with sickle cell disease.

Steensma DP, Kyle RA, Shampo MA.

Mayo Clin Proc. 2010 Oct;85(10):e74-5. No abstract available.



Phytomedicines (medicines derived from plants) for sickle cell disease.

Oniyangi O, Cohall DH.

Cochrane Database Syst Rev. 2010 Oct 6;10:CD004448.



Inhibition of cell adhesion by anti-P-selectin aptamer: a new potential therapeutic agent for sickle cell disease.

Gutsaeva DR, Parkerson JB, Yerigenahally SD, Kurz JC, Schaub RG, Ikuta T, Head CA.

Blood. 2010 Oct 6. [Epub ahead of print]



Coagulation responses after a submaximal exercise in sickle cell trait carriers.

Beltan E, Chalabi T, Tripette J, Chout R, Connes P.

Thromb Res. 2010 Oct 4. [Epub ahead of print] No abstract available.



Pulmonary complications of hemoglobinopathies.

Vij R, Machado RF.

Chest. 2010 Oct;138(4):973-83.



Clinical complications in severe pediatric sickle cell disease and the impact of hydroxyurea.

Tripathi A, Jerrell JM, Stallworth JR.

Pediatr Blood Cancer. 2010 Oct 4. [Epub ahead of print]



The Safety, Tolerability, and Efficacy of a Liquid Formulation of Deferiprone in Young Children With Transfusional Iron Overload.

El Alfy M, Sari TT, Lee CL, Tricta F, El-Beshlawy A.

J Pediatr Hematol Oncol. 2010 Oct 1. [Epub ahead of print]



Tourniquet Use and Sickle Cell Hemoglobinopathy: How Should We Proceed?

Fisher B, Roberts CS.

South Med J. 2010 Sep 30. [Epub ahead of print]



A survey of perioperative management of sickle cell disease in North America.

Firth PG, McMillan KN, Haberkern CM, Yaster M, Bender MA, Goodwin SR.

Paediatr Anaesth. 2010 Sep 29. doi: 10.1111/j.1460-9592.2010.03415.x. [Epub ahead of print]



Leg ulcers in sickle cell disease.

Minniti CP, Eckman J, Sebastiani P, Steinberg MH, Ballas SK.

Am J Hematol. 2010 Oct;85(10):831-3.



Hydroxyurea and Acute Painful Crises in Sickle Cell Anemia: Effects on Hospital Length of Stay and Opioid Utilization During Hospitalization, Outpatient Acute Care Contacts, and at Home.

Ballas SK, Bauserman RL, McCarthy WF, Castro OL, Smith WR, Waclawiw MA; the Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia.

J Pain Symptom Manage. 2010 Sep 21. [Epub ahead of print]


Beneficial effects of nitric oxide breathing in adult patients with sickle cell crisis.
Head CA, Swerdlow P, McDade WA, Joshi RM, Ikuta T, Cooper ML, Eckman JR.
Am J Hematol. 2010 Oct;85(10):800-2.


Ask the Experts

Please I need some information regarding being absent from school and school work and how not to fall behind. my son is 5 yrs old  with SS. I need some information about 504 plans, home bound and all. Thanks.

Thank you for your question. Most children with sickle cell do not miss a lot of school, but it is good to meet with the school early in the year to make plans just in case.  

1. A little bit of information on sickle cell for the principal, teachers, and other staff can go a long way toward making the school supportive for the child with sickle cell disease. One of the best guides I have seen is from Children's Mercy Hosp in Kansas City Pages 16-18 are very relevant to your question, especially the sample 504 plan on page 17.   The New Jersey Dept of Health website is quite good also


Atlanta CHOA guides at

St Jude’s Guide

2. Most sickle cell centers feel that keeping the child in school whenever possible is better for overall development.  I recommend prolonged homebound instruction only for exceptional situations for most children with sickle cell disease.  Most children would do better, I think, with a 504 plan or IEP. 

 3. Not falling behind in school is obviously easier for a 5-yr old level than for high school or beyond.  Preventing pain & hospitalization with good hydration, avoiding extremes of heat or cold, and avoiding exhaustion are all important. If pain or acute chest syndrome become very frequent, you can talk to your hematologist about possible hydroxyurea therapy to reduce the frequent events. 


-Lewis Hsu, MD, PhD

Pediatric Hematologist


 Website Review:




Say It Loud! Conversations on Sickle Cell Disease.Teleconference Thursday, December 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) Topic: Sickle Cell Trait?  Get it Straight!

            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.
November 13 Boston MA, Greater Boston Sickle Disease Association Scholarship & Community Awards  Gala on Saturday, November 13, 2010 time: 7 pm -  10 pm @   Lombardo's Banquet Center , 6 Billings Street,  Randolph , MA. 
22 - 27 November 2010— Raipur (Chhattisgarh) India- Sickle Cell Disease International Organization in collaboration with Centre for
Genetic Diseases & Molecular Biology Department of Biochemistry, Pt. J.N.M. Medical College, Raipur (C.G.) INDIA are organizing the Fourth
International Congress 2010 Sickle Cell Disease International Organization

Nov. 16 and 17 Bethesda, Maryland* The Herrick Symposium will be held on Nov. 16 and 17 (Tuesday and Wednesday) at the NIH campus in Bethesda, Maryland. Registration is now open at

Feb 23-25 , 2011 Hollywood FL* - 5th Annual Sickle Cell Disease Research and Educational Symposium and Annual National Scientific Meeting - Westin Diplomat Resort and Spa, Holly wood FL. Feb 21 - 22 Grant Writing Institute



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