NUTRITIONAL STUDIES IN CHILDREN W/ SICKLE CELL DISEASE

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Second five-year funding cycle: This study consists of three projects designed to examine and improve aspects of growth and nutritional status of children with Sickle Cell Disease (SCD). Project 1: The effect of zinc supplementation on growth & body composition in children with SCD Project 2: The effect of calcium supplementation on bone mineral density in children with SCD Project 3: Nutrition education program for families of children with SCD The first of these projects is the only active study, the only project thus far submitted to the GCRC and the only project that will be further described. The calcium supplementation project will be submitted to the GCRC for approval in spring 1999. In our longitudinal study we reported (J Pediatr 1998;132;467-71) that 44% of those sampled had low levels of plasma zinc and that low zinc levels are a common problem in children with SCD. Low plasma zinc was associated with poor growth in height, weight, muscle mass, skeletal size and sexual maturation. There was no significant difference in age, gender, skinfold thickness or age-adjusted skeletal maturation according to zinc status. Based on these preliminary data we designed a project to study the effect of zinc supplementation on the growth of children with SCD. Enrollment for the zinc supplementation project began in August 1998 and 32 subjects have been enrolled to-date with a target enrollment of 42 children. This study is set up as a double-blinded, placebo controlled study in children between the ages of four and ten years. Half the subjects receive cherry syrup with zinc and the other half receive cherry syrup without zinc. The families and investigators are blinded to the randomization. The CHOP research pharmacists are randomizing the subjects based on age group (4.0-6.9 or 7.0 to 10.9 yrs), gender and height z-score(<-0.15, > -0.15 ). Subjects are scheduled to be measured at baseline, 3, 6 and 12 months. To date, 19 subjects have also completed their three-month evaluation and one has completed a six-month evaluation. Compliance with the cherry syrup is being assessed by: having the children and families keep monthly calendars which they mail back to us, by periodic phone calls, and the families are asked to return their bottle of cherry syrup. Dietary information is being collected using three 24-hour recalls at each growth evaluation.