Pediatric Patients With Sickle Cell Disease at a Public Hospital: Nutrition, Compliance and Early Experience With L-Glutamine Therapy

Background/Aim: Hydration and hydroxyurea (HU) can modify sickle cell disease (SCD) severity. Optimal nutrition and L-glutamine (Gln) may provide further amelioration. Patients and Methods: Reviews of medical records and nutrition surveys were used to investigate severity of pediatric patients with SCD in relation to nutrition, growth, hematologic parameters, and disease-modifying agents. Results: Among 25 females and 25 males (9.1 +/- 7 years), beta-globin genotypes were: HbSS/S beta(0), 60%; HbSC, 32%; HbS beta(+), 8%. The mean number of annual pain crises (APC) was 0.97 +/- 1.1. APCs increased >= 2-fold as HbF dropped to <10% with age. Proper hydration and nutrition correlated with younger ages and fewer APCs. Height and weight Z-scores were <=-1SD in 20% of 35 surveyed patients (12 +/- 7.8 years), who had more APCs (2.5 +/- 2.5 vs. 1 +/- 1.3, p=0.03). Prealbumin levels were overall low. Twenty-two of 28 patients on HU reported >= 90% adherence - with higher mean corpuscular volume (92 +/- 9.6 vs. 74 +/- 10 f/l, p<0.01). Seventy percent of Gln prescriptions were filled. Compliance over 23 months was >= 70% in 12 patients, including 2 on chronic transfusion. Of 10 evaluable patients, 6 (8.8 +/- 2.2 years) had fewer APCs with Gln (mean 0.2 vs. 0.9, p=0.016), with increasing prealbumin levels (14.1 to 15.8 mg/dl, p=0.1). Conclusion: Younger, and well-nourished, well-hydrated patients have a milder clinic course. Disease severity was the worse in undernourished teenagers with suboptimal compliance. L-Glutamine with prealbumin monitoring should be considered for further evaluation in SCD.