NUTRITION IN SICKLE CELL DISEASE

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Current nutrition studies of children with sickle cell disease at CHOP reveal a pattern of poor growth and nutritional status. Chronic energy deficiency is a likely cause of the observed growth failure. Studies of resting energy expenditure indicate that energy requirements are increased 15-20% in children with SCD compared to unaffected controls. In addition, low energy intake during periods of acute illness, documented in children 0 to 5 years of age, contribute to energy deficiency. Intakes of other nutrients, particularly zinc and calcium, may not be adequate to meet the special of children of SCD. The proposed study consists of three projects to determine whether nutritional supplementation and educational intervention improves growth, bone density and dietary intake in children with SCD. The first project is a double blind randomized trial of zinc supplementation to determine whether improvements in growth and body composition (amount of muscle, fat and bone) occur in a twelve month period in pre-pubertal children with SCD given oral zinc supplements compared to a group of children with SCD receiving a placebo. The second project involves calcium supplementation in a double blind randomized trial to determine whether increased calcium intake involves bone density in children with SCD compared to a control group of children with SCD receiving a placebo. The third study is a program to develop and test a nutrition educational intervention program. Building on our quantitative data on dietary intake and eating problems in children with SCD, and educational and behavioral modification program will be developed using written and videotaped materials and teaching sessions with a research dietician. The multi-cultural (African-American, Caribbean, west African) aspects of the population will be considered in developing and presenting the material. The goal of the program will be to increase caloric intake and weight gain in the group participating in the intervention program. A control group receiving usual care will be assessed for comparative purposes. These three projects will be first of their kind in determining whether nutrition intervention improves growth, nutritional status and dietary intake in children with sickle cell disease.