Sickle Cell Disease: Nutritional Considerations
Patients with sickle cell anemia have greater than average requirements for both calories and micronutrients. During sickle cell crises, energy intake can be especially poor. Children frequently hospitalized for sickle cell disease (SCD) commonly show poor linear growth, lean body mass, and reduced fat-free mass. For reasons that are poorly understood, many patients are deficient in essential micronutrients. A diet emphasizing fruits, vegetables, whole grains, and legumes will provide a greater proportion of essential nutrients than a typical Western diet, and appropriate supplementation (1-3 times the recommended intakes for most essential nutrients) can prevent deficiency and may decrease the likelihood of disease exacerbation.
High-calorie, nutrient-dense diet. The average energy intake of sickle cell patients is typically below the suggested allowance for calories during the quiescent phase of the disease, and it drops to roughly half the recommended levels during times of illness requiring hospitalization.6 As a result, children with SCD have impaired growth and significantly lower fat and fat-free mass, compared with unaffected individuals.7 Standard nutritional assessment methods used to calculate energy needs typically underestimate resting energy expenditure in persons with SCD.8,9 A careful nutritional assessment and the addition of energy supplements are indicated.
Adequate fluid consumption to maintain hydration. Sickling of erythrocytes increases in patients with SCD who exercise in the heat without consuming fluids, compared with those who maintain well-hydrated status.10
Micronutrient status may need correction. Blood levels of several vitamins and minerals are often low in individuals with SCD, including vitamin A and carotenoids,11,12 vitamin B6,13 vitamin C,14 vitamin E,15 magnesium,16 and zinc.17,18 These deficiencies cause a significant depreciation in blood-antioxidant status in these patients,19, and the resulting oxidative stress may precipitate vaso-occlusion-related acute chest syndrome.20 Studies indicate that vitamin-mineral supplements of certain nutrients (vitamins C and E, zinc, magnesium) or treatment with a combination of high-dose antioxidants can reduce the percentage of irreversibly sickled cells.15,21-24
Omega-3 fatty acid supplements. The serum phospholipids of children with SCD contain reduced proportions of both the parent (alpha-linolenic acid) and the long-chain omega-3 polyunsaturated fatty acids (eicosapentanoic acid, EPA, and docosahexanoic acid, DHA), compared with healthy controls.25,26 Long-chain omega-3 fatty acids (EPA/DHA) increase the fluidity of red blood cell membranes,27 whose lack characterizes sickle cell crisis.28 A small preliminary study indicated that the antithrombotic effect of fish oil (0.1 g/kg/day) reduced the number of painful episodes requiring hospitalization, compared with olive oil treatment.27 However this finding has not yet been confirmed in controlled trials.
High-potency multiple vitamin with minerals, 1 tablet by mouth daily.
Protein-calorie supplements per nutrition consultant.
What to Tell the Family
Good nutrition can help safeguard healthy growth in children with sickle cell disease and may reduce the risk of complications. A registered dietitian can advise the patient and family on how to meet macronutrient and micronutrient needs. Supplemental nutrients may be required and ordered by the physician.
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