Sickle Cell Disease
Sickle cell disease (SCD) is a serious autosomal, recessive, genetic disease in which the body produces
sickle-shaped red blood cells, unlike the disc-shaped red blood cells that most people produce. Sickle
cells are stiff and sticky and often block blood flow to the vessels of the limbs and organs. This can cause
pain and organ damage, as well as increasing the risk of infection if the spleen is damaged. Normal red
blood cells live for 120 days, but sickle cells only live for 10 to 20 days and the bone marrow is not able
to keep up with the fast turnover. Therefore, there are not enough red blood cells to carry oxygen
throughout the body. This makes people with SCD fatigued. Pain develops when sickle-shaped red blood
cells block blood flow through tiny blood vessels to the chest, abdomen, and joints. Pain can also occur
in the bones. The pain may vary in intensity and can last for a few hours to a few weeks. This is referred
to as a sickle cell crisis. Hands and feet can become very swollen if the red blood cells block blood flow
out of the extremities. Sometimes the retina of the eye is damaged and serious vision problems may
result. A stroke can occur if sickle cells block blood flow to an area of the brain. Acute chest syndrome is
a life threatening complication of SCD that causes chest pain, fever, and difficulty breathing; it may
require emergency treatment. Adults with SCD may develop pulmonary hypertension. SCD can cause
ulcers to form on the legs. The breakdown of red blood cells in the body causes the release of bilirubin;
people with SCD are more likely to develop gallstones.
The gene is more common in families that come from Africa, India, Mediterranean countries, Saudi
Arabia, the Caribbean islands, and South and Central America.
Treatments for SCD
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Transplant: Stem cell transplant has a 5% to 10% risk of death, but patients with successful
transplants were completely cured of sickle cell disease, with no further episodes of pain.
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Hydroxyurea: When taken daily, hydroxyurea reduces the frequency of painful crises and may
reduce the need for blood transfusions. Hydroxyurea increases your risk of infections, and there is
some concern that long-term use of this drug may cause tumors or leukemia in certain people.
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Oxygen: People with SCD often need supplemental oxygen.
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Penicillin: Children will often take penicillin throughout their toddler years to prevent infection.
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Opioids: People with SCD often require opioid pain medications and anti-inflammatory medication.
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Transfusions: Blood transfusions are often necessary; sometimes the sickle cells are removed and
normal red blood cells are transfused.
Experimental treatments
Experimental treatments for SCD include:
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Gene therapy
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Nitric oxide (people with SCD have low levels of nitric oxide, which helps to dilate blood vessels and
reduces the stickiness of red blood cells)
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Drugs to boost fetal hemoglobin production
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Statins (may reduce inflammation and allow blood to flow more readily through vessels)
In 1973, the average life span for people with sickle cell disease was 14, but their life expectancy has
since increased to their 40s and 50s, and in some cases, beyond the age of 60.
Nutrition tips for people with SCD
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A diet containing up to 3 times the normal recommended nutrients can help to prevent nutrient
deficiency and help to prevent severe and frequent crises.
Patients were traditionally told to take folic acid supplements because bone marrow needs folic acid
to make new red blood cells. More recent studies have shown, however, that clinically significant
folic acid deficiency occurs only in a small minority of sickle cell patients.
Drink plenty of water; dehydration increases the risk of a sickle cell crisis.
Supplementation with omega-3 fatty acids can improve the membranes of red blood cells and may
decrease disease flare-ups.
Vitamin E deficiency could well be linked to immature sickle cell formation and hemolysis.
Most studies support a relationship between sickle cell disease and zinc deficiency. Etiologic
associations between zinc deficiency and complications of sickle cell disease such as poor ulcer
healing, growth retardation, delays in sexual development, immune deficiencies, and high,
immature, sickle cell counts have all been suggested.
Pharmacological amounts of vitamin B6 and certain of its derivatives possess in vitro ant sickling
activities. Nevertheless, a small clinical trial failed to demonstrate any consistent hematologic
effects of B6 supplementation.
Research on vitamin D deficiency among those with SCD show that children have less pain if they
begin supplementation around seven months of age.
The “dense” red blood cells in people with SCD allow them to adhere to neutrophils, platelets, and
vascular endothelial cells. This might trigger vasoocclusion and a painful crisis. In one study,
researchers found that a blend of antioxidants, hydroxyl radical scavengers, and iron-binding agents
inhibited the formation of these dense cells in vitro. Some nutritional supplements are effective and
can inhibit dense cell formation by 50%. These consist of 4.0 mg/mL for aged garlic extract, 0.38
mg/mL for black tea extract, 0.13 mg/mL for green tea extract, 0.07 mg/mL for Pycnogenol®, 930
μM for α-lipoic acid, 270 μM for vitamin E, 45 μM for coenzyme Q10, and 32 μM for β-carotene. Both
an ex vivo study and a pilot clinical trial demonstrated that a cocktail consisting of daily doses of 6
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grams (g) of aged garlic extract, 4-6 g of vitamin C, and 800 to 1200 IU of vitamin E may indeed be
beneficial to the patients.
When patients with SCD are in pain, they often do not eat enough. The following tips may help:
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Add olive, canola, peanut, or walnut oils and soft margarine spreads to your foods
Spread peanut butter on toast, muffins, crackers, waffles, pancakes, and fruit slices
Make your own fruit smoothies with fruit, low-fat vanilla yogurt, 2% milk, and protein
powder
Drink Carnation Instant Breakfast®, or blend a packet of chocolate Instant Breakfast mix, with
peanut butter and a banana
Prepare pasta, rice, vegetables, meat loaf, potatoes, and casseroles with shredded low-fat
cheese
Mix cottage cheese or plain yogurt with canned fruit
Try adding cottage cheese or ricotta cheese to casseroles and egg dishes
Stir nonfat dried-milk powder into soups, milk, casseroles, and hot cereals, adding the
powder into foods before heating to avoid the clumping that occurs when stirred into foods
that are already heated
Drink fruit juices
Put avocado and olives on your sandwiches
Mix cooked or canned beans or peas into casseroles, rice dishes, pasta, or salad
Sprinkle seeds or wheat germ on fruit, vegetables, salads, ice cream, pudding, custard,
cereal, or yogurt, or add to a muffin mix, pancake batter, waffle batter, or bread dough
Include dry fruits in muffins, cookies, breads, cakes, grain dishes, cereals, puddings, and
cooked vegetables, such as carrots or sweet potatoes
Substitute milk for water in recipes, whenever possible
Snack on a mix of dry cereal, dried fruit, nuts, seeds, and a few chocolate chips
References and recommended readings
Mayo Clinic Staff. Sickle cell anemia. Mayo Clinic website. http://www.mayoclinic.org/diseasesconditions/sickle-cell-anemia/basics/definition/con-20019348. Published July 11, 2014. Accessed
December 9, 2014.
Moore M. Nutrition for the child with sickle cell anemia. Kids Eat Right website.
http://www.eatright.org/kids/article.aspx?id=6442480398. Reviewed 2013. Accessed December 9,
2014.
Ohnishi S, Ohnishi T, Ogunmola G. Sickle cell anemia: a potential nutritional approach for a molecular
disease. Nutrition. 2000;16(5):330–338. DOI: http://dx.doi.org/10.1016/S0899-9007(00)00257-4.
December 9, 2014.
Pain management: sickle cell disease. WebMD website. http://www.webmd.com/painmanagement/pain-management-sickle-cell-disease. Reviewed August 1, 2014. Accessed December 9,
2014.
Reed J, Redding-Lallinger R, Orringer E. Nutrition and sickle cell disease. Am J Hematol. 1987;24(4):44155. PubMed website. http://www.ncbi.nlm.nih.gov/pubmed/3551592. Accessed December 9, 2014.
Sickle cell anemia and nutrition. BoneMarrow, mx website. http://www.bonemarrowmx.com/sickle-cellanemia-and-nutrition/. Accessed December 9, 2014.
Sickle cell disease: nutritional considerations. NutritionMD website.
http://www.nutritionmd.org/consumers/hematology/sickle_cell_nutrition.html. Accessed December 9,
2014.
What is sickle cell anemia? National Heart, Lung, and Blood Institute (NHLBI) website.
http://www.nhlbi.nih.gov/health/health-topics/topics/sca/. Published September 28, 2012. Accessed
December 9, 2014.