Sickle Cell Disease (SCD) is old, more than a century old, yet we don’t have a widely available cure for this genetically inherent disease. SCD refers to a group of hemoglobinopathies in which at least one sickle (S) beta-globin gene is inherited together with another type of abnormal hemoglobin. SCD is an umbrella term that includes diseases such as sickle cell anemia (HbSS), hemoglobin SC disease (HbSC) and hemoglobin Sβ thalassemia (HbSβthal) major and minor. Sickle cell anemia is the most dangerous type primarily affecting people of African descent though its geographically spread all around the world. It has also been estimated most children with SCD are born in developing countries-almost 2,00,000 HbSS cases occur annually in sub-Saharan Africa, 2% newborns in Ghana are born with SCD while almost 25% of the Ghanaian population are carriers of the sickle cell gene. Despite such high prevalence in the African continent we don’t have much information about SCD here and surprisingly there is a significant amount of information available in countries such as Jamaica, India and US. Despite this, SCD remains as one of the most underappreciated and least referred sources of childhood mortality and morbidity.
SCD normally paves way to vaso-occlusive events that might lead to tissue, bone and organ damage and increased hemolysis that might result in anemia. Other side effects include pain, stroke and anorexia that have a huge impact on dietary intake of kids leading to impaired growth, poor nutritional status and delayed skeletal and sexual maturation. In order to provide such kids with a better growing experience there have been constant efforts made to minimize severity of clinical problems foremost of which includes frequent hospital admission for pain but undernutrition was not included in the list of serious side effects until 1980s. SCD causes a huge blow on physical growth during childhood and adolescent years and these kids are often leaner and shorter than their healthy counterparts. Researchers blame hematologic and cardiovascular status, endocrine and metabolic functions and nutritional status as the critical causes for growth failure while these patients also suffer from several micronutrient deficiencies such as vitamin A, B2, B6, B12, C, D and E, folic acid, iron, calcium, magnesium and zinc. Improving the nutrient intake of these patients might improve their body composition and decrease morbidity and mortality rates due to SCD. Despite numerous cases on growth retardation and under-nutrition the exact part played by intrinsic and extrinsic factors remains unknown which might include insufficient consumption of food or increase in food demands due to higher energy expenditure. The study below clearly helps us understand whether impaired growth and nutritional intake has a take on SCD severity in kids.
Observational Study on Kids with SCD
The study included individuals who were between 6 months and 18 years of age with SCD problem but devoid of any comorbidities. 14 males (48.3%) and 15 females whose mean age was 9.95 years, mean weight was 32.93 kg, height 135 cm and BMI 17.11 were involved in the study. Their nutritional intake was assessed with a 24-hr food recall which also included details about the weekly consumption of major food groups such as meats, fish, eggs, fruits, vegetables, cheese, grains, legumes and sugary beverages. The total calorie, macronutrient, micronutrient and daily intakes were calculated for each child and this was matched against the required nutrient intakes for the child.
Disease severity was assessed through total hemoglobin (Hb) and fetal hemoglobin (HbF) and lactic dehydrogenase (LDH) levels, through total number of days of hospitalization and through lifetime episodes of acute chest syndrome (ACS). Hospital admission rate was inversely related to protein, lipid, phosphorus, vitamin B1 and PP intakes while HbF levels were inversely correlated to carbohydrate, lipid, iron, phosphorus, vitamin B1 and B2 levels. Protein, vitamin PP and A intakes were well above daily requirements while total calorie intake, carbohydrate, lipid, mineral (except phosphorus) and other vitamin intakes were well below daily requirements. Total calorie intake was not significantly correlated to any of the severity indices including vitamin A and C intake. Lifetime episodes of ACS, total Hb and LDH levels were not related to calorie-, macro- and micronutrient intakes. The study clearly shows that various macro- and micronutrient intakes were inadequate the result of which was increased number and days of hospitalization.
Kids with SCD have a basal metabolic rate and resting rate expenditure higher than healthy peers and this causes an increase in their metabolic, energy and protein demands. Calcium and phosphorus intake were inversely related to the number of hospitalizations initially and then to both, number and days of hospitalizations. Iron and phosphorus intake showed a negative correlation to HbF levels seeming to be protective in SCD. A research team evaluating nutrient intakes in Children in America found that low intake of vitamin D, E, folic acid, calcium, phosphorus, magnesium and zinc contributed to a poorer diet with increase in age. Iron deficiency was also commonly seen in these kids but this could be beneficial for patients with SCD as it reduces red blood cells sickling by decreasing mean corpuscular hemoglobin concentration with reduction of hemolysis. But lack of iron impairs physical and intellectual growth and these kids are at a risk of growth and neurocognitive delay. Hence, inadequate nutrient intake, body weight and BMI clearly affect SCD severity but it was also seen that low intake of micro- and macronutrients has a beneficial effect on HbF levels.
Are We Giving Enough Attention to Macronutrient Levels in Patients with SCD?
There has been quite a lot of research happening on micronutrients and what we need now are dietary recommendations for SCD involving more macronutrients. There has been a study in 1985 done on macronutrients intervention that shows measurable improvements in clinical conditions and reduced hospitalizations. There have been various studies done showing the importance of various macronutrients needed by patients with SCD:
Arginine: This is an amino acid whose metabolism is impaired in SCD contributing towards endothelial dysfunction and hypertension. Research shows that low dose supplementation of L-arginine improves liver function, increases plasma arginine concentration and nitric acid levels in patients with SCD. Eating beets, spinach, radish, celery and mustard greens improves nitrate and nitrite contents and also including foods rich in vitamin E, C and A is helpful.
Glutamine: This is a non-essential amino acid whose synthesis is ATP-dependent and lack of it can lead to stress, increased resting energy expenditure (REE), muscle wasting and decreased immune function. Supplementing patients with L-glutamine is useful in decreasing the incidence of SCD-related vaso-occlusive (VOC) pain events.
Vitamin D: Vital for calcium homeostasis and bone mineralization SCD patients often lack in this nutrient due to dark skin pigmentation, limited sun exposure, increased catabolism and decreased nutrient and energy intake. Vitamin D deficiency leads to osteopenia and osteoporosis in 80% of SCD patients. Studies show that supplementing SCD kids with Vitamin D protects them against respiratory infections that commonly attack such kids.
Hydration: Hydration is essential in patients with SCD in the absence of which cells become sickled and poorly hydrated erythrocytes lead to increased viscosity and vaso-occlusive crisis in SCD. SCD patients must drink enough water and fluids, avoid physical activity and extreme weather that can cause sweating and limit sodium intake to keep themselves hydrated.
Maintaining a healthy gut microbiota by consuming enough fruits, vegetables, whole grains, legumes and fermented foods might help SCD patients to optimize immune system functioning.
Calculating dietary needs of patients with SCD can fulfill their macronutrient requirements, fulfilling elevated metabolism of individuals with SCD improves growth and development, promotes weight maintenance, conserves muscle mass and reduces inflammation of these patients. Increasing intake of foods rich in polyphenolic phytochemicals, flavanols, gut microbial required prebiotics, vitamins, minerals and omega-3 fatty acids helps in improving quality of life, sustainable health and longevity of the SCD-affected patient.
Nutrition Impact on SCD Patients in Ghana
The cross-sectional study conducted in Ghana included 120 children aged 3-12 years diagnosed with SCD but free from chronic medical conditions such as HIV, tuberculosis, cleft palate and sores in mouth and throat. Dietary intake was noted by means of a 24-hour recall and a food frequency questionnaire and the kids’ weight and height measurements were also taken. The child was categorized as malnourished or well-nourished depending on the presence or absence of nutritional deficiencies.
Mean energy and protein intake of kids were around 1350 Kcal and when the children’s nutrient consumption was compared to RDA it was found that almost all kids met protein requirements but none of them met vitamin E and calcium requirements. Less than 50% of the participants met requirements of vitamin B12 (46.7%), vitamins A and C (45%), zinc (30.8%), energy (28.3%), folate (9.2%) and magnesium (1.7%). As the kids grew their ability to meet nutrient requirements decreased. While kids aged three met almost five nutrients assessed older children met less than 4. Almost 53.3% met energy recommendations and at least 4 of the 10 nutrients investigated. But, in the 4-8 years and 9-12 years age group not even 25% of kids met recommendations for at least five nutrients. Stunting, underweight, wasting and thinness was present in 25.8, 20.0, 6.8 and 15.8% kids respectively. 38% kids were classified as malnourished, 98% children were anemic, mean hemoglobin was 7.8 and most of them (94%) were suffering from moderate to severe forms of anemia. Thus, this study clearly shows that SCD-affected kids have lower intake of energy and micronutrients (specifically calcium and antioxidant nutrients, vitamin C and E) whose levels further decreased with increasing age.
Effects of Nutritional Intake on Disease Severity in Children with Sickle Cell Disease: https://nutritionj.biomedcentral.com/articles/10.1186/s12937-016-0159-8
Nutrition in Sickle Cell Disease: https://www.dovepress.com/nutrition-in-sickle-cell-disease-recent-insights-peer-reviewed-fulltext-article-NDS
The Role of Nutrition in Sickle Cell Disease: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3085005/
Dietary Intakes & Nutritional Status of Children with Sickle Cell Disease: https://bmcnutr.biomedcentral.com/articles/10.1186/s40795-018-0241-z
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