Critical Reviews in Food Science and Nutrition
ISSN: 1040-8398 (Print) 1549-7852 (Online) Journal homepage: https://www.tandfonline.com/loi/bfsn20
The role of nutrition in the pathophysiology and
management of sickle cell disease among children:
A review of literature
Agartha Ohemeng & Isaac Boadu
To cite this article: Agartha Ohemeng & Isaac Boadu (2018) The role of nutrition in
the pathophysiology and management of sickle cell disease among children: A review
of literature, Critical Reviews in Food Science and Nutrition, 58:14, 2299-2305, DOI:
10.1080/10408398.2017.1319794
To link to this article:  https://doi.org/10.1080/10408398.2017.1319794
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Published online: 14 Aug 2017.
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CRITICAL REVIEWS IN FOOD SCIENCE AND NUTRITION, 2018 
VOL. 58, NO. 14, 2299–2305
https://doi.org/10.1080/10408398.2017.1319794
The role of nutrition in the pathophysiology and management of sickle cell disease
among children: A review of literature
Agartha Ohemeng and Isaac Boadu
Department of Nutrition and Food Science, School of Biological Sciences, University of Ghana, Legon, Accra, Ghana
ABSTRACT KEYWORDS
Sickle cell disease (SCD) is one of the common inherited blood disorders in humans and has been Sickle cell disease; nutritional
associated with decreased dietary intake which results in poor nutritional status and impaired growth. management; painful
Nutrition is one of the most important but often forgotten aspect of care of patients with chronic episodes; children
disorders and there have been emerging concern in literature on increased nutritional needs of SCD
patients. This paper sought to review the available literature on the roles of individual nutrients in the
pathophysiology and management of SCD among children. Children with SCD have been shown to
exhibit suboptimal status with respect to both macronutrients and micronutrients. Thus, nutrition could
play an important role in the management of SCD. However, there is paucity of evidence coming from
trials with large sample sizes to support the suggestion that supplementation with various nutrients that
have been considered in this review will be helpful.
Introduction studies have shown that nutrient supplementation of these chil-
Sickle cell disease (SCD) is one of the common inherited blood dren led to improvement in growth indices (Zemel et al., 2002).
disorders in humans and has a widespread distribution in dif- Although there are some studies that have reported on
ferent parts of the world with variable clinical manifestations. nutritional status of children with SCD (Cox et al., 2011;
SCD is a group of genetic disorders that affects hemoglobin Osei-Yeboah et al., 2011), there is limited information regard-
synthesis, the molecule in red blood cells that delivers oxygen ing the specific ways in which various nutrients in our diets
to cells throughout the body. It is characterized by the predom- relate to this health condition among children. There is there-
inance of sickle hemoglobin (HbS) produced as a result of a fore the need to ascertain the roles of different nutrients and
point mutation in the substitution of valine for glutamic acid whether nutritional interventions can be incorporated into the
in the b-globin polypeptide chain (Moheeb et al., 2007). The care process so as to provide a holistic management tool for
main consequences resulting from this abnormality are vaso- SCD. This paper therefore seeks to review the existing literature
occlusive events and increased hemolysis. Vaso-occlusive events within the period 1985–2016 on the role of nutrition in the
may lead to bone, tissue, and organ damage while chronic pathophysiology and management of SCD among children.
hemolysis may lead to anemia with base line hemoglobin level
as low as 6.0 g/dL (Stuart and Nagel, 2004; Makani et al., Sickle cell epidemiology
2007). SCD patients also have increased susceptibility to infec-
tions (Di Nuzzo and Fonseca, 2004; Booth et al., 2010). SCD affects almost all populations across the globe. Approxi-
Since its discovery, the understanding of the metabolic path- mately 300,000 children are born every year with SCD in the
ways and pathophysiology of SCD have improved substantially, world with majority (70%) of the disease occurring in Africa
resulting in the better management and treatment of the disease (Ansong et al., 2013; Justus et al., 2015). The sickle cell gene
manifestations among patients. Advances in the clinical care of also has a wide spread distribution in some parts of Europe, the
children with SCD, such as earlier diagnosis, penicillin Americas, and the Caribbean (Center for Disease Control and
prophylaxis, folate supplementation, blood transfusion and Prevention, 2016). This is mainly due to the Atlantic slave trade
hydroxyurea therapy have reduced morbidity and mortality as well as the economic migration of Africans to these parts of
(Zemel et al., 2007). However, comorbidities such as pain, the world. In Germany and UK for example, there are currently
stroke, and anorexia which are associated with SCD are likely an estimated 1000 and 12,500 patients with SCD respectively
to lead to decreased dietary intake which in turn results in poor (Jaeckel et al., 2010). In the United States, SCD affects approxi-
nutritional status, impaired growth, and delayed skeletal and mately 100,000 people and each year, 2000 new diagnoses are
sexual maturation. Impaired growth may among children with detected via newborn screening (Arnold et al., 2015). In India,
SCD may partly be due to nutrient inadequacies, and some the beta sickle (bS) gene is prevalent especially in the tribal
CONTACT Dr. Agartha Ohemeng, PhD anohemeng@ug.edu.gh, agartha.cofie@mail.mcgill.ca Department of Nutrition and Food Science, University of Ghana,
Legon, P. O. Box LG 134, Accra, Ghana.
Color versions of one or more of the figures in the article can be found online at www.tandfonline.com/bfsn.
© 2017 Taylor & Francis Group, LLC
2300 A. OHEMENG AND I. BOADU
populations and the prevalence rate varies from 0 to 40% in dif- in hemoglobin synthesis and production of red blood cells
ferent population groups (Mukherjee and Gangakhedkar, (Koury and Ponka, 2004; Stover and Field, 2015), adequate status
2004). In sub-Saharan African countries such as Cameroon, of vitamin B6 and folate will aid the process of compensating for
Republic of Congo, Gabon, Ghana, and Nigeria, the prevalence the rapid loss of red blood cells that occurs in SCD patients.
of sickle cell trait and SCD has been estimated to be 25 and 2%
respectively with Nigeria recording the highest prevalence of Protein and energy
SCD in the world (Adegoke and Kuteyi, 2012). In countries
where the trait prevalence is above 20% the disease affects Protein is an essential macronutrient needed to promote
about 2% of the population (WHO, 2010). For example in growth in children and synthesize hemoglobin. Children with
Ghana where the sickle cell trait is 25%, it has been docu- SCD may require more than the recommended dietary allow-
mented that about 2% of neonates are affected by SCD leading ance to maintain normal metabolism and physiological pro-
to 14,000 new cases annually (Wilson et al., 2012). The geo- cesses. This is because higher protein turnover have been
graphic distribution of the sickle-cell trait is very similar to that reported among SCD patients and this adds an additional
of malaria. The sickle cell trait has a partial protective effect nutritional burden (Borel et al., 1998). Although similar energy
against malaria, and this may explain why it has been main- and protein intakes for both SCD and matched control children
tained at such high prevalence levels in Africa (Bartolucci, have been observed (Singhal et al., 2002), there is consistent
2014). evidence of poor growth among SCD children compared to
non-SCD (Cox et al., 2011; Mukherjee and Gangakhedka,
2004; Al-Saqladi et al., 2008; Kazadi et al., 2017). Additionally,
Interaction between nutrition and SCD
patients with sickle cell anemia displayed greater than average
Sickle cell patients often report decreased appetite, possibly requirements for both protein and energy when compared with
because of the chronic inflammatory state and inadequate edu- their sex- and age-matched peers (Salman et al., 1996). This
cation concerning nutritional requirements which results in increased requirement is poorly understood but it has been sug-
several nutritional deficiencies and poor clinical outcomes gested that hypermetabolism due to shortened life-span of
(Figure 1). These nutrient deficiencies are associated with erythrocytes places an increased demand on protein stores,
immunologic, growth, and maturation abnormalities (Hyacinth accelerates whole body protein turnover and consequently
et al., 2013). Although nutrient deficiencies that occur among increases energy expenditure (Reid, 2013). The most common
SCD patients are poorly understood, several factors have been indicator of poor growth among SCD children is wasting,
proposed for the limited energy and nutrients observed among which is associated with increased hospitalization and poor
these patients. These include; reduced intake potentially from clinical outcomes. Heyman et al. (1985) studied five growth-
the anorexic effects of comorbidities such as pain (Malinauskas retarded children with HbSS, who were below the fifth percen-
et al., 2000; Jacob et al., 2006), decreased absorption of tile for both weight and height. After naso-gastric supplements
nutrients, increased degradation and losses of nutrients, of protein and calories, in addition to their regular diets, two of
increased requirements as a result of elevated basal metabolic the growth retarded children showed improvement and accel-
rate (Kopp-Hoolihan et al., 1999), and alterations in metabolic erated growth. Although a firm inference of their report is lim-
pathways. ited due to the small sample size, the results demonstrates that
Good nutrition is essential to promote healthy growth in malnutrition is one of the complications of HbSS and that
children with SCD and may reduce the risk of complications. A energy and protein supplements could be beneficial for growth
balance between minerals and antioxidants is important in in HbSS children. Additionally, there is some information sug-
maintaining red cell membrane integrity and function (Khan gesting that increased dietary requirements as much as needed
et al., 2013). Minerals such as copper, zinc, iron, chromium, in pregnancy and growth is necessary to improve clinical out-
magnesium, selenium, and antioxidant vitamins like vitamin C comes (Hyacinth et al., 2010), although currently, there are no
and E as well as vitamin A may be needed to perform these special dietary recommendations for protein and/or energy for
protective roles (Lukaski, 2004). Additionally, due to their roles patients with SCD.
Polyunsaturated fatty acids
Fatty acids are important components of cell membranes and
may play important role in improving and maintaining the
overall health and clinical outcomes in patients with SCD. The
essential polyunsaturated fatty acids (PUFA), particularly
omega-3 and omega-6) are needed to synthesize and repair cell
membranes, promote growth of children particularly for neural
development and maturation of sensory systems. Blood cell
aggregation and adherence to vascular endothelium and
inflammation play a central role in vaso-occlusive crisis in
SCD. Omega-3 (n¡3) fatty acids (DHA and EPA) have been
Figure 1. Relationship between infection, malnutrition, and pathophysiology of shown to have anti-aggregatory, anti-adhesive, anti-inflamma-
SCD. tory, and vasodilatory properties (Mori and Beilin, 2004). It has
CRITICAL REVIEWS IN FOOD SCIENCE AND NUTRITION  2301 
therefore been suggested that fatty acids can improve the clini- showed a decrease in the length of hospital stay from approxi-
cal outcomes of sickle cell patients particularly in the reduction mately five days to an average of three days (P < 0.01;
of painful episodes. A double-blinded, olive controlled trial Brousseau et al., 2004). However, the Magnesium for Children
(n D 10, 5 in each study arm) reported a significant decrease in in Crises (MAGiC) study conducted among 4- to 21-year olds
pain episodes among those who received 12% eicosapentaenoic with SCD or Sb0_Thallasemia (Brousseau et al., 2015) showed
acid (EPA) for one year (Tomer et al., 2001). Similarly, a ran- that intravenous magnesium did not shorten length of hospital
domized double-blinded placebo controlled trial among stay, nor did it improve the quality of life for the patients. In
patients with sickle cell anemia in Sudan reported that treat- addition, Goldman et al. (2013) reported that although intrave-
ment reduced the rate of clinical vaso-occlusive events from at nous magnesium was well tolerated in SCD children, there was
least 1 to 0.21 (95% confidence interval (CI), 0.09–0.47; P < no difference in the length of stay in the hospital among
0.001), severe anemia (3.2% compared with 16.4%; P < 0.05) Canadian children with SCD. The authors suggested that pro-
and blood transfusion (4.5% compared with 16.4%; P < 0.05; viding magnesium when crisis has already set in may be too
Daak et al., 2013). There is however, paucity of studies that late, but it is possible that oral therapy may prevent the epi-
look at the effects of PUFA on the pathophysiology of children sodes. There is however, lack of information on studies that
with SCD and the available studies are mostly limited by small have looked at the effect of oral magnesium therapy on clinical
sample sizes. The available results however, point to the poten- outcomes. In conclusion, the available evidence intravenous
tial therapeutic benefits of dietary omega-3 fatty acids in SCD. interventions do not support the inclusion of magnesium in the
Das (2013) suggests that the positive effect of PUFA supple- management of SCD.
mentation in these studies could be ascribe to the formation of
potent anti-inflammatory and anti-aggregatory bioactive lipids Iron
such as lipoxins, resolvins, and protectins. There is the need for
larger, multicenter studies that will provide adequate evidence Iron is an important trace mineral required for hemoglobin syn-
before policies can be formulated regarding PUFA and SCD. thesis and proper immune function. Although iron is an impor-
tant component of red blood cells, excess of it has been shown to
contribute to the generation of free radicals, which lead to lipid
Micronutrients
peroxidation, severe membrane damage, and increased hemolysis
Micronutrients are essential vitamins and minerals required for in sickle cell patients (Pack-Mabien et al., 2015). It has been sug-
good health. Some micronutrient deficiencies have been associ- gested that diet for sickle cell patients should be low in absorbable
ated with SCD. These include iron, zinc, copper, folic acid, iron but high in vegetable proteins. Thus, iron-rich foods, such as
pyridoxine, and vitamin E (Hyacinth et al., 2010). Possible liver, iron-fortified formula, iron-fortified cereals, and iron-
mechanisms by which micronutrient deficiency may develop in fortified energy bars should be excluded (Mahan et al., 2012).
SCD include decreased intake, intestinal malabsorption, and There are discrepancies in studies from developing and devel-
increased catabolism of specific nutrients. oped countries regarding amount of iron stores in sickle cell
patients. Although studies from most developing countries have
Magnesium found low iron stores in their sickle cell participants (Okeahialam
and Obi, 1982; Vichinsky et al., 1981), the vice versa has been
Magnesium is the second most abundant intracellular cation in reported in the developed world. This could be explained partly
the body. Magnesium homeostasis is governed by intestinal by the lower socioeconomic status among the developing world
absorption and renal excretion. Aside its role in stabilizing the which leads to a general inadequate intake of dietary intake of
structure of ATP in ATP-dependent enzyme catalyzed reac- iron and subsequent general low iron status in the population.
tions, the macromineral is also vital in neuromuscular trans- Although iron deficiency anemia is a problem in many
mission and activity. High magnesium intake is associated with developing countries, it is often uncommon among patients
greater bone density (Rude and Gruber, 2004) and the mineral with SCD because of increased gastrointestinal absorption asso-
is thought to decrease sickle erythrocyte dehydration by dimin- ciated with hemolysis and the iron provided by red cell from
ishing the activity of the K-Cl cotransport pathway, a major blood transfusions (Vichinsky et al., 1981). However, it is esti-
factor in the dehydration of erythrocytes in SCD (Brugnara, mated that about a third of hemolysis that occur in SCA
1999). In vitro experiments showed that magnesium could patients is intravascular, resulting in urinary losses of iron and
decrease sickle cell hemoglobin polymerization by 48% iron deficiency (Koduri, 2003). Iron deficiency may also occur
(Nwaoguikpe and Braide, 2012b). Available studies on the level due to repeated phlebotomies and hematuria secondary to renal
of magnesium (Mg) in SCD patients have reported conflicting papillary necrosis. Conversely, SCA patients are at an increased
results. Oladipo et al. (2005) did not observe any difference in risk of experiencing iron overload. Blood transfusion improves
the serum magnesium levels between pediatric SCD cases and the blood flow by reducing the proportion of red blood cells
controls in Nigeria. Others (De Franceschi et al., 1997; Hankins capable of forming HbS polymers (Raghupathy et. al., 2010). In
et al., 2008) have reported reduction in the number of dense addition, transfusions increase the oxygen-carrying capacity of
erythrocytes and improvement in erythrocyte membrane trans- SCA patients. As such, it is recommended as both prophylaxis
port abnormalities as well as increased erythrocyte hydration of and therapy, particularly for stroke (Adams and Brambilla,
patients with SCD on oral magnesium supplements. In a study 2005; Adams et al., 1998). However, transfusion can lead to
to determine the effect of magnesium on length of stay for pedi- iron overload (Porter and Garbowski, 2013). Thus iron status
atric sickle cell pain crisis, those who received intravenous Mg needs to be effectively monitored among SCA patients.
2302 A. OHEMENG AND I. BOADU
Zinc anemia (Van der Dijs et al., 2002). The reported positive effects
of supplementation of the vitamin include reversal of develop-
Zinc is involved in all major aspects of cellular functions
mental delay, reduced dactylitis, and reduction of homocysteine
including metabolism, detoxification, antioxidant defenses,
levels (Al-Yassin et al., 2012). This subsequently leads to
signal transduction, and gene regulation (Bao et al., 2008). Its
reduced cardiovascular disease, stroke, and venous thrombosis
deficiency can cause physiological defects and cell injury.
risk. Conversely, others have reported adequate folate status
Manifestations of zinc deficiency include anorexia, skin lesions,
among pediatric patients, as well as subsequent lack of
growth retardation, neurosensory defects, and immune dys-
improvement in hemoglobin status, growth characteristics, and
function in humans (Prasad, 2008). In the context of SCD and
infections among patients with SCD after supplementation
nutrition, more attention has been focused on zinc than any
(Rabb et al., 1983). It has been suggested that the observed dif-
other mineral. The deficiency of zinc has been reported more
ferences in the findings is due to genetic polymorphisms in folic
among people with “HbSS” genotype, the most severe form of
acid/homocysteine metabolism and inconsistent supplementa-
SCD. A study in Nigeria reported that SCD children experienc-
tion doses (Al-Yassin et al., 2012).The limited literature indi-
ing painful crisis had lower serum zinc levels compared their
cates that although folic acid supplementation may increase
counterparts who were in steady condition at time of data col-
serum folate level, its effect on anemia and other symptoms of
lection (Temiye et al., 2011). Also, zinc deficiency was most
SCD among children remains unclear (Dixit et al., 2016). An
common among Canadian children with SCD, and was associ-
important limitation of routine folate supplementation is that it
ated with home pain crises and increased incidence of hospital-
could mask vitamin B12 deficiency in SCD patients, which
ization (Martyres et al., 2016). Benefits of zinc supplementation
could lead to the development of neurologic dysfunction (Dhar
on nutritional indices and anthropometric parameters are well
et al., 2003). This has led some researchers to suggest that rou-
established in literature. In a 12-month, randomized, placebo-
tine folate supplementation among SCD should be discontin-
controlled trial of zinc supplementation in children aged
ued. However, this can be addressed by including a consistent
4–10 years with SCD-SS, Zemel and colleagues (2002) reported
monitoring of vitamin B12 status. A review of literature shows
significantly greater increases in height and arm circumference
that there is the need for more longitudinal studies with large
z scores in the zinc group compared to the control. Other
sample size and longer follow-up periods in this subject area.
anthropometric indices such as height-for-age and weight-
for-age z scores also decreased significantly in the control group
but did not change significantly in the zinc group. The role of Antioxidant vitamins
zinc in decreasing oxidative stress and inflammatory cytokines,
Vitamin E is the most important lipid-soluble antioxidant in the
as well as increasing anti-inflammatory proteins in sickle cell
cell. The vitamin plays an indispensable role in protecting the
patients has also been documented. Experimentally, the min-
body against the damaging effects of ROS that are formed meta-
eral has also been shown to exhibit almost ninety percent inhi-
bolically or encountered in the environment. Vitamin C is a
bition of sickle cell hemoglobin polymerization (Nwaoguikpe
nutrient required in very small amounts to perform a range of
and Braide, 2012a), which is quite high compared to other anti-
essential metabolic functions in the body. It is mainly recognized
oxidants such as vitamins A, E, and C. A Cochrane review on
by the role it plays in collagen synthesis which is required for
this subject indicated that zinc-supplemented SCD patients
connective tissue formation, as an antioxidant, and the preven-
showed reduction in both frequency of crises and infections
tion of scurvy. Sickle cell patients are known to be oxidatively
(Swe et al., 2013). Thus, available evidence suggests some bene-
stressed and deficient in antioxidant micronutrients (Allard et al.,
fits for SCD patients who receive zinc supplements. However,
1998). Sickle erythrocytes and their membranes are susceptible to
most of the studies reviewed involved only adults and so multi-
endogenous free-radical-mediated oxidative damage due to
center trials that include children will be needed before this can
chronic redox imbalance in red cells that often results in continu-
be included in the management of pediatric SCD.
ous generation of reactive oxygen species (ROS) with clinical
manifestations of mild to severe hemolysis (Nwaoguikpe and
Folic acid Braide, 2012a). The production of ROS can be grossly amplified
in response to a variety of patho-physiological conditions such as
Folic acid is an essential vitamin needed for the production of hypoxia, inflammation, infection, dehydration and deficiency in
red blood cells and the formation of neurotransmitters in the antioxidant vitamins. There have been reports of low circulating
brain. As part of routine management of SCD, folic acid is levels of vitamins C and E in SCD patients (Arruda et al., 2013;
being prescribed in almost all health centers in both developing Bhoi et al., 2014; Tukur et al., 2015) and increased utilization of
and developed countries. It has been recommended that diet the vitamin for disease process could account for this deficiency.
for sickle cell patients should be high in folate as needed in Amer et al. (2006) reported 20–50% lower levels of reduced glu-
much as pregnancy (400–600 mcg daily) because of the tathione (GSH), the major intracellular scavenger of ROS and
increased production of erythrocytes needed to replace the cells 10- to 30-fold higher production of ROS in sickle cell patients
being continuously destroyed and to prevent megaloblastic compared to Hb AA controls (P < 0.005). They further showed
erythropoiesis (Mahan et al., 2012). There are however, incon- that exposure of blood samples of sickle cell patients to antioxi-
sistencies in literature on both folate status and the clinical ben- dants such as N-acetylcysteine, vitamin C, and vitamin E
efits of supplementation on the disease manifestations. Some decreased oxidative stress by 2-fold compared to the control
reports have cited low serum and erythrocyte folate levels in group (P < 0.05). Other supplementation benefits include in
pediatric sickle cell patients and high incidence of megaloblastic vitro inhibition of formation of dense cells and Heinz bodies
CRITICAL REVIEWS IN FOOD SCIENCE AND NUTRITION  2303 
(denatured Hb; Ohnishi et al., 2000). However, a randomized among sickle cell patients, there are no specific dietary recom-
controlled trial that evaluated the effect of vitamins C and E sup- mendations for these patients. However, it has been suggested
plementation in adults with sickle cell anemia reported no that, SCD patients should
improvement, but rather an increase in hemolytic markers 
 Take in enough water as possible each day, at least 8–10
(Arruda et al., 2013). Also, supplementation with these antioxi- glasses to prevent dehydration.
dant vitamins did not reduce lipid peroxidation among SCD 
 Avoid caffeine-containing drinks, such as energy drinks,
patients in Nigeria (Nku-Ekpang et al., 2016). This study also cola or coffee, and alcohol. These cause frequent urination
recorded increased activities of scavenging enzymes such as cata- which results in dehydration.
lase, superoxide dismutase, and glutathione peroxidase. A possi- 
 Avoid icy products which could cause blood vessels to
ble reason for the lack of improvement is the observation that narrow, causing difficulty in blood flow through vessels,
the reduced vitamin E antioxidant capacity of SCD was related to hence pain.
transfusion status, but not sickle crises (Marwah et al., 2002). 
 Consume protein-dense foods with emphasis on plant-
Studies that have had children as the target in this area are not sources of proteins such as beans, peas, pulses, lentils,
common. Thus, the available evidence do not support the idea soybeans, groundnuts, cashew, and mushroom.
that supplementation with vitamins C and E can improve the life 
 Increase consumption of food sources of citrulline, a
of children with SCD. nutrient that promotes blood vessels relaxation and
improves oxygen and blood circulation. Some sources
Vitamin D include watermelon, milk, legumes, and cucumber.
 Consume more food sources of folate, vitamin B6, B12,
Vitamin D is a fat-soluble vitamin responsible for the mainte- and zinc, which are involved in red blood cell production
nance of calcium and phosphate homeostasis and is vital for and maintenance of their membrane integrity.
bone health. It is normally referred to as “the sunshine vitamin” 
 Take iron-rich foods, such as liver, iron-fortified formula,
because modest exposure to sunlight is usually sufficient for its iron-fortified cereals, and iron-fortified energy with cau-
synthesis by the skin. This makes deficiency of the vitamin less a tion due to risk of iron overload as well as vitamin C
problem in tropical Africa. Deficiency of the vitamin can lead to which increases iron absorption (Mahan et al., 2012).
bone deformities such as rickets in children. It can also result in
bone pain and tenderness (osteomalacia) in adults (Mahan et al.,
2012). In reference to SCD, there is increasing data demonstrat- Conclusion
ing low serum levels of vitamin D among both healthy and sickle There are indications that children with SCD generally present
cell children mainly in the developed countries where there is suboptimal levels of most nutrients when compared with their
less sunshine. Other possibility may be due to decreased dietary non-SCD counterparts. There is, however, paucity of informa-
intake and in some cases to seasonal variability in food intake tion especially from Africa, where the condition is relatively
(Hyacinth et al., 2010). A study by Buison et al. (2004) showed prevalent. For polyunsaturated fatty acids, zinc, folate, and vita-
that HbSS children had a higher risk of low vitamin D status min D, trials with small numbers have suggested potential ben-
compared to healthy children aged 5–18 years. Similarly, efits. However, there is lack of trials with large sample size that
Martyres et al. (2016) and Lee et al. (2015) found half of their support the suggestion that supplementation with various
pediatric SCD subjects to be vitamin D deficient, and this defi- nutrients that have been considered in this review will be help-
ciency was associated with acute pain. HbSS patients may benefit ful. Despite these limitations, dietary supplementations need to
from routine vitamin D and calcium supplements, to reduce risk be considered as an integral component of the management of
of suboptimal peak bone mineral density (BMD) and consequent SCD, especially in view of the suboptimal status of nutrients
fragility. Another nutrient intervention documented is the sup- observed among SCD children. Dietary diversity is a recom-
plementation of high dose of vitamin D to children and adoles- mended approach to achieving nutritional requirements and
cents with SCD in a 6-month randomized double blind pilot adequacy and this may also be beneficial to SCD children.
study to prevent pathologic chronic bone pain (Osunkwo et al., Future studies on the role of diet in the management of SCD
2012). The investigators observed an increase in serum vitamin should therefore include this aspect of dietary intake.
D among the supplemented group compared with placebo. Addi-
tionally, they observed decrease in the number of pain days
among the supplemented group compared with the placebo. References
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