From Emily:

sorry - i should have added that the WHO guidance doesn't mention sickle cell and the only previous question on en-net related to pregnant women. Many thanks for any advice.

There is a surprising absence of WHO guidance on the issue of the use of MNP with children who have a haemoglobinopathy, such as sickle cell anaemia.

Within the current WHO guidance on MNP for children a sachet is recommended to contain 12.5 mg of elemental iron (preferably as encapsulated ferrous fumarate). It states that ‘This guideline is not applicable to children with specific conditions…’ but sickle cell disease, other haemoglobinopathies, or thalassaemia are not discussed.
WHO. Guideline: Use of multiple micronutrient powders for home fortification of foods consumed by infants and children 6–23 months of age. Geneva, World Health Organization, 2011.
http://www.who.int/publications/guidelines/nutrition/en/

When looking at the question raised by Emily I think it is important to distinguish the 2 forms of Sickle cell disease, and try and get information on the prevalence of these in a population.

- People who are homozygous for Hb S have an abnormal ß chain, resulting in fragile, relatively inflexible RBCs that can plug capillaries, causing tissue infarction, and that are prone to haemolysis, causing anaemia.
- People with sickle cell trait are heterozygous (Hb AS) and do not experience haemolysis, painful crises, or thrombotic complications except possibly during hypoxic conditions (eg, at high altitudes, during sudden decompression in airplanes).
http://www.merckmanuals.com/professional/hematology-and-oncology/anemias-caused-by-hemolysis/sickle-cell-disease

CDC states on their web site that for homozygous people with sickle cell disease:
“Taking iron supplements will not help people with sickle cell disease. This type of anemia is not caused by too little iron in the blood; it's caused by not having enough red blood cells. In fact, taking iron supplements could harm a person with sickle cell disease because the extra iron builds up in the body and can cause damage to the organs.”
http://www.cdc.gov/ncbddd/sicklecell/treatments.html

This advice is not given for people with sickle cell trait.

I think that the discussion re a previous question on iron/folate also has some relevant information: http://www.en-net.org/question/1518.aspx
At the end of the day it is likely to come down to hard choices over the balancing of risks.

Thanks so much Andy - really helpful. The UNICEF regional office is in touch with WHO, so I'll let you know any response.

Emily.

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.

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