Josiah Brown Poster Abstract


Laura Hernandez Santiago
Moran Gotesman, MD
Guy Elgar
Analysis of Nutritional Status in Patients with Sickle Cell Disease

Patients with sickle cell disease (SCD) suffer from many chronic complications including significant growth failure and poor nutrition (Henderson, 1994). Higher metabolic needs and increased energy expenditure has been thought to contribute to patients’ growth and nutritional complications (Singhal, 1993). Recently L-Glutamine has been approved for patients with SCD for reduction in the number of pain crisis (Endari [package insert] Torrance, CA: Emmaus Medical, Inc; 2017). Not only may L-Glutamine decrease the number of pain crisis but, it may also improve their overall nutritional status. This hypothesis prompted us to reevaluate our sickle cell patients’ baseline nutritional status. The goal of this study was to gather growth and nutritional data from our SCD patients at Harbor-UCLA Medical Center (HUMC). We assessed our sickle cell cohort of 48 patients and analyzed their height and weight z-scores. Using a nutritional questionnaire, we were able to collect data for 32 patients. We quantified their average caloric intake and compared it to their estimated caloric need based on height and weight parameters. Our data demonstrated that approximately 50% of our patients meet the caloric requirements, but the other 50% do not. On average, our patients’ liquid intake is approximately eight-8 oz. cups per day, and they have 3 meals/day. In addition, less than 25% of our patients take multivitamins daily. Moving forward, this information will be used to assess how the patients’ caloric intake compares to their height and weight Z-scores as well as their BMI percentage. This baseline analysis of the nutritional status and growth will give us a better understanding of the role that nutrition plays in our SCD patients at HUMC. We will continue to assess their growth and nutrition after the initiation of L-Glutamine (Endari) and see if this nutritional supplement provides any augmentation to their growth and overall nutrition.