Influence of Nutrition on Disease Severity and Health-Related Quality of Life in Adults With Sickle Cell Disease

Abstract

Background & Aims: Sickle cell disease (SCD has a worldwide distribution and causes significant morbidity and mortality in children and adults. Few studies addressed the determinants of SCD severity in adults; therefore, we investigated the impact of nutrition on the outcome of SCD and health-related quality of life (HRQoL) in adult patients,
 Methods: In this longitudinal study, we recruited and prospectively followed 62 adults with SCD (aged ?18 years) for a median of 93 months. At entry and follow-up, patients provided medical and dietary history, had a physical examination and anthropometric measurements, assessment of protein-energy intake, measurement of micronutrient levels, estimation of SCD severity score, and determination of the HRQoL (SF-26v2). The study outcome was a composite of hospitalization due to SCD crises or death.
 Results: At baseline, 42 (67.74%) patients had macro and, or micro-undernutrition (Group A), and 20 (32.26%) were well nourished. (Group B). The BMI and most anthropometric measurements were significantly lower in SCD patients compared to control subjects. In several SCD patients, undernutrition resulted from reduced food intake and reduced absorption due to concomitant gastrointestinal disorders. Seventy percent of SCD patients had vitamin D, vitamin B12, and zinc deficiencies. Under-nourished patients had significantly higher SCD severity scores, frequent SCD related hospitalizations, higher mortality rates, and reduced HRQoL compared to well-nourished patients. Protein-energy and micronutrient deficiencies were independent predictors of severe SCD and mortality. Correction of undernutrition and hydroxyurea therapy improved SCD severity scores and HRQoL.
 Conclusion: Patients with sickle cell disease have various degrees of macro and micro deficiencies, which increase SCD severity and hospitalizations and reduce the health-related quality of life. Early diagnosis and prompt correction of macro and micronutrient deficiencies need to be incorporated in the standard of care of SCD patients to improve the disease outcomes.

Low vitamin B12 blood levels in sickle cell disease: Data from a large cohort study in Tanzania

“…A study conducted among hospitalized adult SCD patients in Saudi Arabia revealed lower vitamin B 12 concentrations compared to healthy controls, the degree of deficiency being proportional to severity of disease in the patients. 27 This supports the findings of another study in a Saudi Arabian hospital that reported vitamin B 12 deficiency in nearly half of its SCD patients. 28 In contrast, Kennedy et al found that vitamin B 12 deficiency was rare, and its dietary intake was adequate among hospitalized SCD children in the United States.…”

The relationship between nutrition, gut dysbiosis, and pediatric sickle cell pain outcomes: A pilot study

“…Poor nutritional status has been shown to be prevalent in individuals with SCD 35–38 . Limited studies also show that malnutrition and undernutrition affect QoL and are associated with frequent hospitalizations 35,39 . Similarly, our study showed significant associations between nutritional status and worse QoL scores in the pain domains and overall mean QoL score in the SCD cohort.…”

Micronutrient levels and haemato-biochemical status of patients with sickle cell anaemia at a tertiary hospital in Abakaliki, south-eastern Nigeria: a cross-sectional study

“…Body Mass Index is a measure for indicating nutritional status in adults. In the current study, the low BMI of the patients with SCA could be attributable to the increased resting energy expenditure, hypermetabolism, nutrient diversion, and micronutrient deficiencies [ 11 ]. The patients with SCA in our study had lower Mg 2+ and calcium levels, similar to reports by Yousif et al .…”