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Research Identifies Need for Improved Understanding of Sickle Cell Disease in Adulthood
Date:12/8/2007

ty and toxicity profile (100 percent), patient compliance (100 percent), effective follow-up (100 percent), drug availability (100 percent), affordability (100 percent), and concern for reactivation of latent tuberculosis (50 percent), carcinogenesis (100 percent), and teratogenicity (associated with birth defects) (100 percent).

No patient was currently on hydroxyurea treatment and only 5 percent of the patients interviewed had been informed of or were aware of hydroxyurea. Patient-related barriers to hydroxyurea included lack of awareness (95 percent), cost (100 percent), availability (100 percent), need for frequent follow-up (90 percent), risk of infections (98 percent), and pregnancy restrictions and need for concurrent contraceptive use (98 percent).

The authors concluded that the absolute lack of hydroxyurea utilization in a major health-care center in Nigeria, the country with the highest incidence of sickle cell disease in the world with about 150,000 children born with the disease annually, underscores the need for education of local health-care providers as well as patient counseling and education. In addition, clinical studies designed to assess the safety and efficacy of hydroxyurea in unique African settings is needed to facilitate the introduction and utilization of hydroxyurea in Nigeria and other parts of Africa.

-- Cognitive function impaired among sickle cell patients; transfusion trial needed to assess how to mitigate brain damage, especially as patients age [Abstract #428]

Elliott Vichinsky, MD, Children's Hospital and Research Center, Oakland, CA

Many consider brain dysfunction to be the most important and least-studied problem afflicting the aging sickle cell population. About 25 percent of neurologically intact sickle cell pediatric patients have neuropsychological dysfunction -- a deficit in cognitive ability -- and silent central nervous system (CNS) infarction -- death of tissue within the CNS sys
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SOURCE American Society of Hematology
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