If caregivers were better educated about NS and the advantages/disadvantages of the different forms of NS, their preferences may change. There have been policy changes at St. Jude have been implemented since this study, and an outpatient dietitian now provides education to caregivers about NS during the pre-evaluation for HSCT.

Sickle cell anemia (SCA) is an inherited condition of chronic hemolytic anemia that includes several different genotypes. People with SCA are encouraged to drink extra fluids in order to prevent dehydration that may lead to red cell sickling and consequently more serious problems. Proper hydration is an important factor in maintaining homeostasis in the body; it is necessary to regulate body temperature, maintain energy levels, aid digestion and eliminate toxins.

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Methods ParticipantsInclusion criteria for this energy expenditure study were: active patients, who were 5-18 years old, had a diagnosis of Hb SS and weighed at least 15 kg. Subjects were excluded if they were receiving hydroxyurea or another anti-sickling agent, chronic transfusion, AbstractPurpose: Pediatric health quality of life (HQOL) refers to a child's physical, emotional, and social well being. HQOL is often compromised in persons with sickle cell anemia (SCA). The purpose of this study was to compare HQOL with hemoglobin in children with SCA. Methods:In a pilot study looking at energy expenditure in children with SCA, we measured subjects' QOL using the PedsQL survey and compared it to their hemoglobin (Hb) levels at baseline. Results:Twenty-five subjects, all with HbSS, completed the instrument. Their mean age (standard deviation) was 11.4 (3.25) years and 52% were male. Their mean Hb level was 8.4 (1.2) g/dL. Higher scores in social functioning (R=0.63, p=0.0001) and school functioning (R=0.40, p=0.05) were significantly associated with higher Hb levels. Conclusions: We conclude that Hb level is related to HQOL and that treatments aimed at improving Hb may help to improve overall HQOL.