October 21, 2016
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Sickle Cell News Round-Up

WASHINGTON - NIH recognizes sickle cell disease awareness month

2010 marks 100th anniversary of first scientific paper on inherited condition; November symposium

September is National Sickle Cell Disease Awareness Month. This year's commemoration takes on added significance: 2010 is the 100th anniversary of the first research paper on sickle cell disease in Western medical literature.

Chicago physician James B. Herrick, M.D., published a paper in the November 1910 issue of the Archives of Internal Medicine that described the irregular shape of a patient's red blood cells " a hallmark of the condition now known as sickle cell disease. To honor Herrick's legacy and celebrate the research progress that has been made during the past 100 years, the National Institutes of Health will host the James B. Herrick Symposium " Sickle Cell Disease Care and Research Past, Present, and Future on Nov. 16 and 17 in Bethesda, Md.

"While we have made significant strides in the last century to improve the lives of those who have sickle cell disease, our work is far from over," said Susan B. Shurin, M.D., acting director of the National Heart, Lung, and Blood Institute (NHLBI), part of the NIH. "Researchers around the globe are continuing to search for better treatments to ease the significant burden of people who live with the disease and its cardinal manifestations of anemia and pain."

Treatment advances made by researchers working with volunteer participants in clinical trials, have prevented most of the complications that were life threatening in childhood even a few decades ago. In that era, children were at risk of overwhelming infection and damage to the spleen and lungs. More recent advances such as the drug hydroxyurea and the use of blood transfusions for the prevention of stroke in children now allow many people with sickle cell disease to live into their 40s, 50s, and beyond. Bone marrow transplants can cure sickle cell disease in some patients, but this treatment is limited by a scarcity of bone marrow matches.

As part of the NHLBI's continuing efforts to move important discoveries into practice, the Institute has asked a panel of the nation’s leading sickle cell experts to review the scientific evidence and create a set of clinical practice guidelines. These guidelines will target primary care providers to help them give the best possible care to those who live with sickle cell disease. The guidelines are expected to be released in 2011, supported by an NHLBI-led public awareness and education campaign.

In mid-November 2010, in conjunction with the Herrick Symposium, the public can comment on the expert panel’s draft recommendations on the use of hydroxyurea for adults with sickle cell disease. Members of the public will have the opportunity to comment on the complete draft guidelines in 2011.

While the primary goal of sickle cell disease research has been to help people who live with the condition, the scientific advances have offered broader benefits. Research on sickle cell disease has helped spur advances in other areas of medicine. Much of our understanding of genomics and the control of gene function have derived from work done in sickle cell disease and a related disorder, thalassemia.

"Any researcher now delving into the mysteries of genetics or molecular biology owes a debt of gratitude to the pioneering scientists who researched sickle cell disease," Shurin said. "Basic discoveries in sickle cell disease laid the groundwork for advancing the field of genetics."

Sickle cell disease affects between 70,000 and 100,000 Americans. Those who inherit this disease carry two copies of the sickle globin gene. The gene produces an abnormal hemoglobin, the protein in red blood cells that carries oxygen throughout the body. This genetic defect leads to the production of misshapen red blood cells, many of which have the sickle shape that gives the disease its name. Those who live with sickle cell disease have life-long anemia and can experience severe pain episodes known as crises. Variants of sickle cell disease occur when a person inherits a sickle globin gene from one parent and another abnormal gene, such as a thalassemia gene or hemoglobin C gene, from the other parent.

Red blood cells are normally round and smooth, which allows them to pass easily through blood vessels. Sickled cells tend to be sticky and can clump in blood vessels, which can lead in turn to blockages that trigger pain crises. If left untreated, sickle cell disease can lead to organ failure and other serious consequences.

Symptoms and complications of sickle cell disease vary widely depending on the severity of the condition. Some people have mild symptoms, while others experience severe symptoms that can require hospitalization. The disease is most prevalent in the African-American community, though individuals of Middle Eastern, Mediterranean, Central and South American, and Asian Indian heritage can develop the disease.

Part of the National Institutes of Health, the National Heart, Lung, and Blood Institute (NHLBI) plans, conducts, and supports research related to the causes, prevention, diagnosis, and treatment of heart, blood vessel, lung, and blood diseases; and sleep disorders. The Institute also administers national health education campaigns on women and heart disease, healthy weight for children, and other topics. NHLBI press releases and other materials are available online at www.nhlbi.nih.gov.

The National Institutes of Health (NIH) — The Nation's Medical Research Agency — includes 27 Institutes and Centers and is a component of the U.S. Department of Health and Human Services. It is the primary federal agency for conducting and supporting basic, clinical and translational medical research, and it investigates the causes, treatments, and cures for both common and rare diseases. For more information about NIH and its programs, visit www.nih.gov.

Diseases and Conditions Index: Sickle Cell Disease: http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_Treatments.html Herrick Symposium: http://www.nhlbi.nih.gov/meetings/James-Herrick-Sicklecell/index.htm Sickle Cell Disease Information Center: http://www.nhlbi.nih.gov/new/sicklecell.htm
Sickle Cell is no barometer for fullness of life http://www.stlamerican.com/articles/2010/09/16/your_health_matters/health_news/health01.txt

Colleges mandate sickle cell testing http://www.washingtonpost.com/wp-dyn/content/article/2010/09/19/AR2010091904763.html
College athletics' bid to identify sickle-cell carriers: Not ready for prime time? http://www.latimes.com/health/boostershots/la-heb-sickle-cell-20100908,0,7157293.story?track=rss

A decision by the National Collegiate Athletic Association (NCAA) to require screening for sickle cell carrier status among Division I athletes ultimately may do more harm than good, researchers predict. http://www.medpagetoday.com/Genetics/GeneticTesting/22082
New Book
New Book on sickle cell disease entitled "The Stranger Within Me" by
Shirley Renee. We all know her as Shirley Miller formerly at the
Southwestern Comprehensive Sickle Cell Center in Dallas Texas. Shirley
is now pursuing a Masters degree in Health Communication at Boston
University online. Please contact Shirley for more information at shirleyrenee3@gmail.com.

Articles in the Medical Journals

1: Ballas SK, McCarthy WF, Guo N, Brugnara C, Kling G, Bauserman RL, Waclawiw MA.
Early detection of response to hydroxyurea therapy in patients with sickle cell
anemia. Hemoglobin. 2010;34(5):424-9.http://www.ncbi.nlm.nih.gov/pubmed/20854115

2: Ngô C, Kayem G, Habibi A, Benachi A, Goffinet F, Galactéros F, Haddad B.
Pregnancy in sickle cell disease: maternal and fetal outcomes in a population
receiving prophylactic partial exchange transfusions. Eur J Obstet Gynecol Reprod
Biol. 2010 Sep 14. [Epub ahead of print] PubMed PMID: 20846778.

3: Mont MA, Zywiel MG, Marker DR, McGrath MS, Delanois RE. The natural history of
untreated asymptomatic osteonecrosis of the femoral head: a systematic literature
review. J Bone Joint Surg Am. 2010 Sep 15;92(12):2165-70. http://www.ncbi.nlm.nih.gov/pubmed/20846778

4: Bollinger LM, Nire KG, Rhodes MM, Chisolm DJ, O'Brien SH. Caregivers'
perspectives on barriers to transcranial doppler screening in children with
sickle-cell disease. Pediatr Blood Cancer. 2010 Sep 14. [Epub ahead of print]

5: Maier-Redelsperger M, Lévy P, Lionnet F, Stankovic K, Haymann JP, Lefèvre G,
Avellino V, Perol JP, Girot R, Elion J. Strong association between a new marker
of hemolysis and glomerulopathy in sickle cell anemia. Blood Cells Mol Dis. 2010
Sep 9. http://www.ncbi.nlm.nih.gov/pubmed/20833087

6: Richards JR, Richards IN, Ozery G, Derlet RW. Droperidol Analgesia for
Opioid-Tolerant Patients. J Emerg Med. 2010 Sep 10. [Epub ahead of print] PubMed

7: Green NS, Barral S. Genetic modifiers of HbF and response to hydroxyurea in
sickle cell disease. Pediatr Blood Cancer. 2010 Sep 9. [Epub ahead of print]

8: Bonham VL, Dover GJ, Brody LC. Screening student athletes for sickle cell
trait--a social and clinical experiment. N Engl J Med. 2010 Sep 9;363(11):997-9.

9: Mohammed FA, Mahdi N, Sater MA, Al-Ola K, Almawi WY. The relation of
C-reactive protein to vasoocclusive crisis in children with sickle cell disease.
Blood Cells Mol Dis. 2010 Aug 31. http://www.ncbi.nlm.nih.gov/pubmed/20813565

10: Ali SB, Reid M, Fraser R, Moosang M, Ali A. Seizures in the Jamaica cohort
study of sickle cell disease. Br J Haematol. 2010 Aug 31. http://www.ncbi.nlm.nih.gov/pubmed/20813003

11: Bediako SM, Lattimer L, Haywood C Jr, Ratanawongsa N, Lanzkron S, Beach MC.
Religious coping and hospital admissions among adults with sickle cell disease. J
Behav Med. 2010 Sep 2. http://www.ncbi.nlm.nih.gov/pubmed/20812027

12: Oyeku SO, Feldman HA, Ryan K, Muret-Wagstaff S, Neufeld EJ. Primary care
clinicians' knowledge and confidence about newborn screening for sickle cell
disease: randomized assessment of educational strategies. J Natl Med Assoc. 2010
Aug;102(8):676-82. http://www.ncbi.nlm.nih.gov/pubmed/20806678

13: van den Tweel XW, van der Lee JH, Heijboer H, Peters M, Fijnvandraat K.
Development and validation of a pediatric severity index for sickle cell
patients. Am J Hematol. 2010 Jul 28. http://www.ncbi.nlm.nih.gov/pubmed/20806231

14: Wilkie DJ, Johnson B, Mack AK, Labotka R, Molokie RE. Sickle cell disease: an
opportunity for palliative care across the life span. Nurs Clin North Am. 2010
Sep;45(3):375-97. PubMed PMID: 20804884; http://www.ncbi.nlm.nih.gov/pubmed/20804884
Upcoming Events
Sickle Cell Disease Association of America, Philadelphia/ Delaware Valley Chapter and AFSCME District Council 33
WHAT: 13th Annual Walter E. Brandon Sickle Cell Walkathon WHEN: Saturday September 25, 2010
WHERE: Fairmount Park- Belmont Avenue & North Concourse Drive, across from the Please Touch Museum in Philadelphia
TIME: 6:30 am: Registration7:30 am: Morning Ceremony 8:30 am: Walkathon Kick- off call 215-471-8686 or go to www.sicklecelldisorder.com

October 4 Atlanta GA Sickle Cell Education Day 9 - 3 - Loudermilk Center: 40 Courtland Street, Atlanta, GA 30303 http://www.choa.org/default.aspx?id=2365
4th Annual Sickle Cell and Thalassaemia Advanced Update Course 4-8 October 2010 St Thomas Venue: Governor’s Hall, St Thomas Hospital, London.
Guest Lecturer Janet Kwiatkowski, MD, MSCE Director of Thalassaemia Program
Director of Stroke Studies The Children’s Hospital Philadelphia Contacts: Mary.Abiri@gstt.nhs.uk 02071887774
Giselle.Padmore-Penniston@gstt.nhs.uk 02071884486
Hemoglobinopathy Counselor Professional, Community & Peer Educator Training Course October 6 7, & 8 2010
8 AM – 4 PM (W & TH); 8 – 2 (F) Hospital for Special Care, Research and Education Center370 Osgood Ave., New Britain, CT (entrance on Corbin Ave.)
$ 200 Registration fee for out of state participants ($100 with student ID) $25 Registration fee for in-state participants (refundable upon attendance)
Nurses, Social Workers, Health Educators, Community-Based Counselors and others are invited to participate.
This program certifies participants in one of 4 levels:
· Hemoglobinopathy Counselor (attend all 3 days)
Professional Educator,
Community Educator, or
Peer Educators (attend first 2 days)
Counselors will be expected to provide up-to 4 sessions per year.
Please contact: Martine Saint-Cyr (860) 827-1958 ext. 5834 saintcym@hfsc.org
Robin R. Leger RN, PhD 860-679-4932 rleger@uchc.edu

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 http://4sccongress.co.in <http://4sccongress.co.in/>

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 http://www.nhlbi.nih.gov/meetings/James-Herrick-Sicklecell/index.htm

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 http://floridasickle.org/




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