Growth failure in sickle cell disease is well documented. Investigators have hypothesized that resting energy expenditure (REE) is increased during a vaso-occlusive crisis (VOC) and may play a role in growth failure. In a prospective study of 14 children (8M, 6F; 12.4±4.4 years, mean±SD) with HbSS-type sickle cell disease (SCD), clinical parameters, growth, body composition (BC), dietary intake and REE were assessed in the morning following admission for a VOC and at follow-up (FU) during the usual state of health, 31±15 days after discharge. Ht and wt were assessed using standard techniques and z-scores were calculated using NCHS reference values. BC was measured by dual energy x-ray absorptiometry scan. Dietary intake was assessed using a 24-hr recall for the day prior to hospitalization for VOC and 3 days at FU. Early morning REE was measured after a 12-hr fast and compared to WHO equations. Baseline and FU data were analyzed by repeated measures ANOVA. Significance was defined at the p<0.05 level. Children reported feeling sick 2.9±2.6 days prior to admission and missed 2.1±1.0 days of school. 50% of children noted decreased appetite and 50% of parents noted decreased food consumption by their children. Baseline ht(-0.57±0.92) and wt (-0.72±1.07) z-scores were reduced, and no change in weight from VOC to FU was noted.%Body fat, fat free mass (FFM) and fat mass did not change between VOC and FU. Hb, Hct, and reticulocyte count did not change, but the WBC count decreased significantly (15.4±5.1 vs 12.5±3.4) from VOC to FU. There was no significant difference in kcal/day consumed, but%RDA kcal (51±29 vs 73±23),%kcal as protein (11±5 vs 15±3) and%kcal as fat (25±14 vs 34±6) consumption significantly increased while%kcal as carbohydrate(64±18 vs 51±8) consumption significantly decreased from VOC to FU. REE expressed as kcal/day, kcal/kg/day, kcal/FFM/day and%WHO (96±11 vs 100±6) did not change (p=NS) from VOC to FU. In these subjects, REE was not increased during treatment of VOC relative to the usual state of health. Dietary intake during the VOC and at FU was sub-optimal and may lead to negative energy balance, hence contributing to growth failure in children with SCD.
