Malnutrition Screening in Sickle Cell Disease: A Pediatric, Single Center Experience

Objective

Patients living with Sickle Cell Disease (SCD) are at risk for nutritional deficiencies due to the increased metabolic demand from chronic anemia, hemolysis and inflammation. Low weight and height for age and delayed pubertal maturation may reflect a state of undernutrition in SCD patients. Though dietary intake in high-resource countries may seem adequate when referencing the recommended daily dietary allowance for age and sex, this may be insufficient for patients living with SCD. In addition, no disease-specific growth curve exists for SCD, as has been established in other chronic illnesses.

The lack of SCD specific growth reference ranges and dietary recommendations pose a challenge in screening for malnutrition in SCD patients. We sought to study the use of the Pediatric Yorkhill Malnutrition Score (PYMS), a validated pediatric malnutrition screening tool, in identifying SCD patients at risk for malnutrition at our institution. In addition, we describe patient growth parameters and disease variables associated with nutritional status in SCD patients seen at our clinic between 2023-2024.

Methods

The PYMS is used at our institution in both the hospital and clinic setting to screen patients at risk for malnutrition. The PYMS is administered and scored by bedside nursing at each clinic visit. Scores equal to or higher than 2 prompt a dietary consultation and close dietary follow up. PYMS scores were collected retrospectively on patients with SCD (any genotype) who had one or more in-person maintenance clinic visits to the Children's Blood and Cancer Center (CBCC) between January 2023 to January 2024. SCD patients receiving chronic transfusions, only seen for sick visits, or only had a virtual visit within the year were excluded. In addition, patient genotype, age, sex, body mass index (BMI), height, weight, hemoglobin, absolute reticulocyte count, and Hydroxyurea use were also collected.

Results

During the study period, there were 144 eligible patients seen in the hematology clinic. Mean age was 9.3 years, 52.8% male, and 62.5% had HbSS disease. Mean hemoglobin (Hgb) values by genotype were HbSS and HbS-beta null 9.1g/dL, HbSC 11.4 g/dL, and HbS-Beta plus thalassemia 10.5 g/dL. Despite Hgb differences, there was no statistically significant difference in BMI by genotype (17.9 vs 19.4 vs 18.7, p=0.2665). 23 (16%) patients met criteria for low BMI for age and gender, and 19 (82.6%) of these patients had HbSS or HbS-beta null genotype. Mean Hgb and BMI increased and ARC decreased for HbSS patients prescribed HU, though this only met statistical significance for ARC (p=0.074, p=0.0918, p=0.011, respectively).

When evaluating the use of the PYMS to screen for malnutrition in SCD patients, 20 (13.8%) of patients did not have a PYMS score completed. Of the patients who met criteria for low BMI for age and gender, 95% did not have an accurate BMI score documented in PYMS. 144 (100%) of patients did not have an accurate score for the question regarding “will the child's nutrition be affected by the medical condition” in the PYMS tool. 28 (19.4%) patients were incorrectly assigned a PYMS score of less than 2.

Conclusions:

We found missed opportunities to identify SCD patients at risk of malnutrition using the PYMS screening tool in an outpatient Hematology clinic. A quality improvement project to improve malnutrition screening for SCD patients is now ongoing at our center. Approximately a fifth of patients with SCD met criteria for low BMI based on age and gender, and the majority of these patients had HbSS genotype. There was a trend towards improved BMI and anemia in HbSS patients prescribed Hydroxyurea.

No relevant conflicts of interest to declare.