Nutrition assessment in children with sickle cell disease.

Children with sickle cell disease have decreased height and weight when compared with their peers. Although exact reasons for poor growth have not been established, increased calorie and protein needs and deficiencies in zinc, folic acid, and vitamins A, C, and E may be factors. To determine whether inadequate nutrient intake contributes to this poor growth, we conducted a survey of the nutrition knowledge and practices of families affected by sickle cell disease. Sixty-one patients with a median age of 8 years (range, 13 months to 17 years) participated in the study. Patients with homozygous S hemoglobin (sickle cell) disease (Hb SS, n = 34) and sickle beta zero thalassemia (Hb S beta zero-thalassemia, n = 2) were combined; 19% were below the fifth percentile for height. The other patients, with sickle hemoglobin C disease (Hb SC, n = 21) and sickle beta plus thalassemia (Hb beta(+)-thalassemia, n = 4), were grouped, and 4% were below the fifth percentile for height (P = .043). Ninety percent of the study patients or their parents were familiar with the food groups indicated on the US Department of Agriculture's Food Guide Pyramid, but most patients failed to consume appropriate amounts from those groups. Although two thirds of the patients ate the recommended number of servings daily from the meat group, only 20% to 31% of the recommended servings from each of the other food groups was consumed. This was possibly related to low socioeconomic status. The patients in the Hb SS group ate significantly less from the bread (P < .037) and milk (P < .022) categories than the patients in the Hb SC group. Fifty-nine percent of families had incomes below the poverty level, and 79% participated in a food assistance program. We conclude that the nutrient intake of patients with sickle cell disease is often inadequate. Education for patients with sickle cell disease should focus on (1) specific nutrient needs, with proper distribution of dietary intake among the food groups, (2) ways to provide nutritious meals on a limited income, and (3) methods for increasing calorie and protein intake.