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Sickle Cell Anemia

Also indexed as: Crescent Cell Anemia, Sickle Cell Disease

Illustration

Get support for sickle cell anemia by focusing on your unique nutritional needs. According to research or other evidence, the following self-care steps may help you manage this kind of chronic anemia:

What you need to know

  • Fight back with fish oil
  • Reduce the frequency of severe pain episodes by taking a daily fish oil supplement providing 33 mg of EPA and 23 mg of DHA for every 2.2 pounds of body weight
  • Think zinc
  • Under the supervision of a doctor, take a daily supplement of 100 mg of zinc, plus 2 mg of copper, to help prevent cell damage and speed healing of leg ulcers associated with sickle cell anemia
  • Fill up on fluids
  • Drink plenty of water and other fluids to maintain good circulation
  • Take a test
  • Have your blood homocysteine levels checked to find out if daily folic acid supplements of 1 to 4 mg are right for you, or to discover if you have a vitamin B12 deficiency that requires treatment

These recommendations are not comprehensive and are not intended to replace the advice of your doctor or pharmacist. Continue reading the full sickle cell anemia article for more in-depth, fully-referenced information on medicines, vitamins, herbs, and dietary and lifestyle changes that may be helpful.

About sickle cell anemia

Anemia is a deficiency of the oxygen-carrying capacity of red blood cells. Sickle cell anemia is an inherited chronic anemia in which the red blood cells become sickle or crescent-shaped. The symptoms of sickle cell anemia are caused by the clogging of small blood vessels by the sickle cells or by poor delivery of oxygen to the tissues due to the anemia itself.

A sickle cell crisis is a painful episode that occurs when the body becomes severely deprived of oxygen. The disease and the trait occur in people of African descent, as well as in people from Mediterranean countries, India, and the Middle East, but rarely in people of European descent.

Product ratings for sickle cell anemia

Science Ratings Nutritional Supplements Herbs
2Stars

Fish oil

Folic acid (for lowering homocysteine levels)

L-arginine (for pulmonary hypertension)

Vitamin B12 (for sickle cell patients with diagnosed B12 deficiencies)

Zinc

 
1Star

Beta-carotene and other carotenoids

Magnesium

Vitamin A

Vitamin B2

Vitamin B6

Vitamin C

Vitamin E

Garlic

3Stars Reliable and relatively consistent scientific data showing a substantial health benefit.
2Stars Contradictory, insufficient, or preliminary studies suggesting a health benefit or minimal health benefit.
1Star For an herb, supported by traditional use but minimal or no scientific evidence. For a supplement, little scientific support and/or minimal health benefit.

What are the symptoms?

Symptoms include fatigue, joint and abdominal pain, irritability, yellow discoloration of the skin and eyes, leg sores, gum disease, frequent respiratory infections, blindness later in life, and periods of prolonged, sometimes painful erections in males. People with sickle cell anemia can have episodes of severe pain in the arms, legs, chest, and abdomen that may be accompanied by fever, nausea, and difficulty breathing. These symptoms occur only in people who inherit copies of the sickle cell gene from both parents. People who inherit a sickle cell gene from only one parent have what is known as sickle cell trait and are without symptoms.

Medical options

In some cases, the prescription drug hydroxyurea (Droxia) is used to increase hemoglobin levels. Doctors might also recommend preventive antibiotics for common respiratory infections. Acute episodes may be treated with supplemental oxygen, intravenous antibiotics, prescription painkillers, and blood transfusions.

Healthcare practitioners typically advise people with sickle cell anemia to avoid high altitudes, to maintain an adequate fluid intake, and to receive vaccinations. Occasionally bone marrow transplants are recommended.

Dietary changes that may be helpful

People with sickle cell anemia suffer from many nutrient deficiencies, but preliminary research on dietary habits shows that food and nutrient intake by sickle cell patients in general meets or exceeds recommendations and is not significantly different from healthy controls.1 2 3 This suggests the higher rate of nutrient deficiencies may be due to an increased need for many nutrients in sickle cell patients. The effectiveness of dietary interventions in supplying adequate nutrition to meet these higher demands has not been examined.

Vitamins that may be helpful

In a preliminary study, individuals with pulmonary hypertension (a life-threatening complication of sickle cell anemia) received L-arginine in the amount of 100 mg per 2.2 pounds of body weight, three times per day for five days. L-arginine treatment resulted in a significant improvement in pulmonary hypertension, as determined by a 15% decline in the pulmonary artery systolic pressure.4 Longer-term studies are needed to confirm these preliminary results.

Sickle cell anemia may result in vitamin B12 deficiency. A study of children with sickle cell anemia found them to have a higher incidence of vitamin B12 deficiency than children without the disease.5 A study of 85 adults with sickle cell anemia showed more of them had vitamin B12 deficiency than did a group of healthy people.6 A subsequent preliminary trial demonstrated that for patients with low blood levels of vitamin B12, intramuscular injections of 1 mg of vitamin B12 weekly for 12 weeks led to a significant reduction in symptoms.7 Researchers do not know whether people with sickle cell anemia who are found to be deficient in vitamin B12 would benefit equally from taking vitamin B12 supplements orally.

In a preliminary trial, 20 patients with sickle cell anemia were given either 1 mg of folic acid per day or folic acid plus 6 grams of aged garlic extract, 6 grams of vitamin C, and 1,200 mg of vitamin E per day for six months.8 Patients taking the combination had a significant improvement in their hematocrit (an index of anemia) and less painful crises than those taking just folic acid.

Preliminary research has found that patients with sickle cell anemia are more likely to have elevated blood levels of homocysteine compared to healthy people.9 10 Elevated homocysteine is recognized as a risk factor for cardiovascular disease.11 In particular, high levels of homocysteine in sickle cell anemia patients have been associated with a higher incidence of stroke.12 Deficiencies of vitamin B6, vitamin B12, and folic acid occur more frequently in people with sickle cell anemia than in others13 14 15 and are a cause of high homocysteine levels.16 A controlled trial found homocysteine levels were reduced 53% in children with sickle cell anemia receiving a 2–4 mg supplement of folic acid per day, depending on age, but vitamin B6 or B12 had no effect on homocysteine levels.17 A double-blind trial of children with sickle cell anemia found that children given 5 mg of folic acid per day had less painful swelling of the hands and feet compared with those receiving placebo, but blood abnormalities and impaired growth rate associated with sickle cell anemia were not improved.18 In the treatment of sickle cell anemia, folic acid is typically supplemented in amounts of 1,000 mcg daily.19 Anyone taking this amount of folic acid should have vitamin B12 status assessed by a healthcare professional.

Iron deficiency is relatively common in people with sickle cell anemia, especially in pregnant women and in children.20 21 Iron deficiency in people with sickle cell anemia is best diagnosed with a laboratory test called serum ferritin.22 23 During sickle cell crises, however, serum ferritin is no longer useful as an indicator of iron deficiency.24 The value of iron supplementation for people with sickle cell anemia who are diagnosed with iron deficiency is unclear. Iron supplements have, in some reports, reduced the severity of anemia as measured by laboratory tests; however, some reports suggest they may increase the symptoms of sickle cell anemia.25 26 Moreover, a state of iron deficiency has been shown to reduce sickling of red blood cells in the blood of people with sickle cell anemia.27 A small trial of iron restriction in patients with sickle cell anemia found improvement in anemia and clinical symptoms as well as decreased red blood cell breakdown during iron restriction.28 A doctor should be consulted before deciding to supplement or restrict iron in sickle cell anemia.

Low concentrations of red blood cell magnesium have been noted in patients with sickle cell anemia.29 30 Low magnesium, in turn, is thought to contribute to red blood cell dehydration and a concomitant increase in symptoms. In a preliminary trial, administration of 540 mg of magnesium per day for six months to sickle cell anemia patients reversed some of the characteristic red blood cell abnormalities and dramatically reduced the number of painful days for these patients.31 The form of magnesium used in this trial, magnesium pidolate, is not supplied by most magnesium supplements; it is unknown whether other forms of magnesium would produce similar results.

In test tube studies, vitamin B6 has been shown to have anti-sickling effects on the red blood cells of people with sickle cell anemia.32 33 Vitamin B6 deficiency has been reported in some research to be more common in people with sickle cell anemia than in healthy people.34 35 In a controlled trial, five sickle cell anemia patients with evidence of vitamin B6 deficiency were given 50 mg of vitamin B6 twice daily. The deficiency was reversed with this supplement, but improvement in anemia was slight and considered insignificant.36 Therefore, evidence in support of vitamin B6 supplementation for people with sickle cell anemia remains weak.

Antioxidant nutrients protect the body’s cells from oxygen-related damage. Many studies show that sickle cell anemia patients tend to have low blood levels of antioxidants, including carotenoids, vitamin A, vitamin E, and vitamin C, despite adequate intake.37 38 39 40 41 42 Low blood levels of vitamin E in particular have been associated with higher numbers of diseased cells in children43 and with greater frequency of symptoms in adults.44 A small, preliminary trial reported a 44% decrease in the average number of diseased cells in six sickle cell anemia patients given 450 IU vitamin E per day for up to 35 weeks. This effect was maintained as long as supplementation continued.45

In another preliminary trial, 13 patients with sickle cell anemia were given two supplement combinations for seven to eight months each. The first combination included 109 mg zinc, 153 IU vitamin E, 600 mg vitamin C, and 400 ml (about 14 ounces) of soybean oil containing 11 grams of linoleic acid and 1.5 grams of alpha linolenic acid. The second combination included 140 IU vitamin E, 600 mg vitamin C, and 20 grams of fish oil containing 6 grams of omega-3 fatty acids. Reduction in diseased cells was observed only during the administration of the first protocol. The authors concluded that zinc was the important difference between the two combinations and may be a protector of red blood cell membranes.46

Fish oil alone has also been studied. In a double-blind trial, supplementation with menhaden oil, in the amount of 250 mg per 2.2 pounds of body weight per day for one year, reduced the frequency of severe pain episodes by approximately 45%, compared with placebo.47 This treatment may work by correcting an imbalance between omega-3 and omega-6 fatty acids that occurs in people with sickle cell anemia.48

The zinc deficiency associated with sickle cell anemia appears to play a role in various aspects of the illness. For example, preliminary research has correlated low zinc levels with poor growth in children with sickle cell anemia.49 In a preliminary trial, 12 people with sickle cell anemia received 25 mg of zinc every four hours for 3 to 18 months.50 The number of damaged red blood cells fell from 28% to 18.6%. Addition of 2 mg of copper per day did not inhibit the effect of zinc. (Zinc supplementation in the absence of copper supplementation induces a copper deficiency.) Patients with the highest number of damaged red blood cells had a marked response to zinc, but those with lower levels of damaged cells (less than 20% irreversibly sickled cells) had little or no response. Chronic leg ulcers occur in about 75% of adults with sickle cell disease. In a controlled trial, sickle cell patients with low blood levels of zinc received 88 mg of zinc three times per day for 12 weeks.51 Ulcer healing rate was more than three times faster in the zinc group than in the placebo group.

Are there any side effects or interactions?
Refer to the individual supplement for information about any side effects or interactions.

References:

1. Tangney CC, Phillips G, Bell RA, et al. Selected indices of micronutrient status in adult patients with sickle cell anemia (SCA). Am J Hematol 1989;32:161–6.

2. Phillips G, Tangney CC. Relationship of plasma alpha tocopherol to index of clinical severity in individuals with sickle cell anemia. Am J Hematol 1992;41:227–31.

3. Chiu D, Vichinsky E, Ho SL, et al. Vitamin C deficiency in patients with sickle cell anemia. Am J Pediatr Hematol Oncol 1990;12:262–7.

4. Morris CR, Morris SM Jr, Hagar W, et al. Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med 2003;168:63–9.

5. Osifo BO, Adeyokunnu A, Parmentier Y, et al. Abnormalities of serum transcobalamins in sickle cell disease (HbSS) in Black Africa. Scand J Haematol 1983;30:135–40.

6. al-Momen AK. Diminished vitamin B12 levels in patients with severe sickle cell disease. J Intern Med 1995;237:551–5.

7. al-Momen AK. Diminished vitamin B12 levels in patients with severe sickle cell disease. J Intern Med 1995;237:551–5.

8. Ohnishi ST, Ohnishi T, Ogunmola GB. Sickle cell anemia: A potential nutritional approach for a molecular disease. Nutrition 2000;16:330–8.

9. van der Dijs FP, Schnog JJ, Brouwer DA, et al. Elevated homocysteine levels indicate suboptimal folate status in pediatric sickle cell patients. Am J Hematol 1998;59:192–8.

10. Houston PE, Rana S, Sekhasaria S, et al. Homocysteine in sickle cell disease: relationship to stroke. Am J Med 1997;103:192–6.

11. Alpert MA. Homocysteine, atherosclerosis, and thrombosis. South Med J 1999;92:858–65 [review].

12. Houston PE, Rana S, Sekhasaria S, et al. Homocysteine in sickle cell disease: relationship to stroke. Am J Med 1997;103:192–6.

13. al-Momen AK. Diminished vitamin B12 levels in patients with severe sickle cell disease. J Intern Med 1995;237:551–5.

14. Lin YK. Folic acid deficiency in sickle cell anemia. Scand J Haematol 1975;14:71–9.

15. Natta CL, Reynolds RD. Apparent vitamin B6 deficiency in sickle cell anemia. Am J Clin Nutr 1984;40:235–9.

16. Alpert MA. Homocysteine, atherosclerosis, and thrombosis. South Med J 1999;92:858–65 [review].

17. van der Dijs FP, Schnog JJ, Brouwer DA, et al. Elevated homocysteine levels indicate suboptimal folate status in pediatric sickle cell patients. Am J Hematol 1998;59:192–8.

18. Rabb LM, Grandison Y, Mason K, et al. A trial of folate supplementation in children with homozygous sickle cell disease. Br J Haematol 1983;54:589–94.

19. Waterbury L. Anemia. In Barker LR, Burton JR, Zieve PD. Principles of ambulatory medicine, 4th ed. Baltimore: Williams & Wilkins, 1995, 605.

20. Pellegrini Braga JA, Kerbauy J, Fisberg M. Zinc, copper and iron and their interrelations in the growth of sickle cell patients. Arch Latinoam Nutr 1995;45:198–203.

21. Reed JD, Redding-Lallinger R, Orringer EP. Nutrition and sickle cell disease. Am J Hematol 1987;24:441–55 [review].

22. Vichinsky E, Kleman K, Embury S, Lubia B. The diagnosis of iron-deficiency anemia in sickle cell disease. Blood 1981;58:963–8.

23. Rao KR, Patel AR, McGinnis P, Patel MK. Iron stores in adults with sickle cell anemia. J Lab Clin Med 1984;103:792–7.

24. Brownell A, Lawson S, Bozovic M. Serum ferritin concentration in sickle cell crisis. J Clin Pathol 1986;39:253–5.

25. Vichinsky E, Kleman K, Embury S, Lubia B. The diagnosis of iron-deficiency anemia in sickle cell disease. Blood 1981;58:963–8.

26. Haddy TB, Castro O. Overt iron deficiency in sickle cell disease. Arch Int Med 1982;142:1621–4.

27. Rao KR, Patel ER, Honig GR, et al. Iron deficiency and sickle cell anemia. Arch Int Med 1983;143:1030–2.

28. Castro O, Poillon WN, Finkle H, Massac E. Improvement of sickle cell anemia by iron-limited erythropoiesis. Am J Hematol 1994;47:74–81.

29. De Franceschi L, Bachir D, Galacteros F, et al. Oral magnesium pidolate: effects of long-term administration in patients with sickle cell disease. Br J Haematol 2000;108:284–9.

30. De Franceschi L, Bachir D, Balacteros F, et al. Oral magnesium supplements reduce erythrocyte dehydration in patients with sickle cell disease. J Clin Invest 1997;100:1847–52.

31. De Franceschi L, Bachir D, Galacteros F, et al. Oral magnesium pidolate: effects of long-term administration in patients with sickle cell disease. Br J Haematol 2000;108:284–9.

32. Kark JA, Kale MP, Tarassoff PG, et al. Inhibition of erythrocyte sickling in vitro by pyridoxal. J Clin Invest 1978;62:888–91.

33. Kark JA, Tarassoff PG, Bongiovanni R. Pyridoxal phosphate as an antisickling agent in vitro. J Clin Invest 1983;71:1224–9.

34. Flores L, Pais R, Buchanan I, et al. Pyridoxal 5’-phosphate levels in children with sickle cell disease. Am J Pediatr Hematol Oncol 1988;10:236–40.

35. Reed JD, Redding-Lallinger R, Orringer EP. Nutrition and sickle cell disease. Am J Hematol 1987;24:441–55.

36. Natta CL, Reynolds RD. Apparent vitamin B6 deficiency in sickle cell anemia. Am J Clin Nutr 1984;40:235–9.

37. Tangney CC, Phillips G, Bell RA, et al. Selected indices of micronutrient status in adult patients with sickle cell anemia (SCA). Am J Hematol 1989;32:161–6.

38. Chiu D, Vichinsky E, Ho SL, et al. Vitamin C deficiency in patients with sickle cell anemia. Am J Pediatr Hematol Oncol 1990;12:262–7.

39. Jain SK, Ross JD, Duett J, Herbst JJ. Low plasma prealbumin and carotenoid levels in sickle cell disease patients. Am J Med Sci 1990;229:13–5.

40. Jain SK, Williams DM. Reduced levels of plasma ascorbic acid (vitamin C) in sickle cell disease patients: its possible role in the oxidant damage to sickle cells in vitro. Clin Chim Acta 1985;149:257–61.

41. Natta C, Stacewicz-Sapuntzakis M, Bhagavan H, Bowen P. Low serum levels of carotenoids in sickle cell anemia. Eur J Haematol 1988;41:131–5.

42. Essein, EU. Plasma levels of retinol, ascorbic acid and alpha-tocopherol in sickle cell anemia. Centr Afr J Med 1995;41:48–50.

43. Ndombi IO, Kinoti SN. Serum vitamin E and the sickling status in children with sickle cell anemia. East Afr Med J 1990;67:720–5.

44. Phillips G, Tangney CC. Relationship of plasma alpha tocopherol to index of clinical severity in individuals with sickle cell anemia. Am J Hematol 1992;41:227–31.

45. Natta CL, Machlin LJ, Brin M. A decrease in irreversibly sickled erythrocytes in sickle cell anemia patients given vitamin E. Am J Clin Nutr 1980;33:968–71.

46. Muskiet FA, Muskiet FD, Meiborg G, Schermer JG. Supplementation of patients with homozygous sickle cell disease with zinc, alpha-tocopherol, vitamin C, soybean oil, and fish oil. Am J Clin Nutr 1991;54:736–44.

47. Tomer A, Kasey S, Connor WE, et al. Reduction of pain episodes and prothrombotic activity in sickle cell disease by dietary n-3 fatty acids. Thromb Haemost 2001;85:966–74.

48. Tomer A, Kasey S, Connor WE, et al. Reduction of pain episodes and prothrombotic activity in sickle cell disease by dietary n-3 fatty acids. Thromb Haemost 2001;85:966–74.

49. Pellegrini Braga JA, Kerbauy J, Fisberg M. Zinc, copper and iron and their interrelations in the growth of sickle cell patients. Arch Latinoam Nutr 1995;45:198–203.

50. Brewer GJ, Brewer LF, Prasad AS. Suppression of irreversibly sickled erythrocytes by zinc therapy in sickle cell anemia. J Lab Clin Med 1977;90:549–54.

51. Serjeant GR, Galloway RE, Gueri MC. Oral zinc sulphate in sickle-cell ulcers. Lancet 1970;2:891–3.

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