MNPs for children and sickle cell anaemia

Hi Emily,
I also found this "in-depth report" in the New York Times, an excerpt of which says:

NUTRITION AND DIETARY SUPPLEMENTS
Foods. Good nutrition, while essential for anyone, is critical for patients with sickle cell disease. Some dietary recommendations include:

Fluids are number one in importance. The patient should drink as much water as possible each day to prevent dehydration.
Diet should provide adequate calories, protein, fats, and vitamins and minerals. Patients and families should discuss vitamin and mineral supplements with their doctors and nurses.
Some studies claim that omega-three fatty acids, found in fish and soybean oil as well as dietary supplements, might make red blood cell membranes less fragile and possibly less likely to sickle, although no studies have proven this definitively.
Vitamins. Patients should take daily folic acid and vitamin B12 and B6 supplements. Vitamin B6 may have specific anti-sickling properties. Some doctors recommend 1 mg folic acid, 6 microgram vitamin B12, and 6 mg vitamin B6. Foods containing one or all of these vitamins include meats, oily fish, poultry, whole grains, dried fortified cereals, soybeans, avocados, baked potatoes with skins, watermelon, plantains, bananas, peanuts, and brewer's yeast. Of note, folic acid can mask pernicious anemia, which is caused by deficiency of vitamin B12 and is more common in African-Americans than other populations.

Note on Iron. Although sickle cell disease is often referred to as anemia, patients should avoid iron supplements or iron rich foods when receiving multiple transfusions, which increase the risk for iron-overload.

Although there is no guidance from UN agencies or other expert bodies, there is some medical literature supporting the idea that persons with sickle cell trait do indeed frequently have iron deficiency anemia and respond well to iron supplementation.

See:
Vichinsky E et al. The diagnosis of iron deficiency anemia in sickle cell disease. Blood 1981; 58(5):963.
Mohanty D et al. Iron deficiency anaemia in sickle cell disorders in India. Indian J Med Research 2008;127:366-369.
Rodrigues PC et al. Iron deficiency in Brazilian infants with sickle cell disease. J Pedatr (Rio J) 2011;87(5):405-411.
Terlouw DJ et al. Relation between the response to iron supplementation and sickle
cell hemoglobin phenotype in preschool children in western Kenya. Am J Clin Nutr 2004;79:466-72.

Naturally, most papers show that children with sickle cell disease who receive transfusions do not have iron deficiency, and iron supplementation in such children should probably be avoided because of the risk of iron overload. The Terlouw paper also showed a greater tendency of iron supplemented children with sickle cell trait to develop malaria parasitemia and disease, but then the Pemba trial showed this in children without sickle cell trait as well. Another paper (Menendez C et al. The response to iron supplementation of pregnant women with the haemoglobin genotype AA or AS. Trans Royal Society of Trop Med 1995;89:289-292.) showed that pregnant women in The Gambia with sickle cell trait who are given supplemental iron were more likely to have malaria parasitemia at 36 weeks and placental parasites at delivery. The newborns of these women had lower birth weights.

In populations with a high prevalence of the sickle cell gene, the vast majority of carriers will be heterozygotes. These people with sickle cell trait may have a high risk of iron deficiency and may benefit from iron supplementation. In addition, there is little mention in these papers of an elevated risk of iron overload in people with sickle cell trait. However, a group of real experts need to consider all the available medical literature before making a recommendation based on more informed opinions. Any resulting recommendation should address iron supplementation in people with other hemoglobinopathies as well.