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Health Condition

Sickle Cell Anemia

About This Condition

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.

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.

Other Therapies

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.

References

1. 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.

2. 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.

3. Daak AA, Ghebremeskel K, Hassan Z, et al. Effect of omega-3 (n-3) fatty acid supplementation in patients with sickle cell anemia: randomized, double-blind, placebo-controlled trial. <em>Am J Clin Nutr</em> 2013;97:37-44.

4. El-Beshlawy A, Abd El Raouf E, Mostafa F, et al. Diastolic dysfunction and pulmonary hypertension in sickle cell anemia: is there a role for L-carnitine treatment? Acta Haematol 2006;115:91-6.

5. 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.

6. 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.

7. 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.

8. 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.

9. 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.

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

11. 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.

12. 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.

13. 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.

14. 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.

15. 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.

16. 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.

17. 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.

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

19. Bao B, Prasad AS, Beck FWJ, et al. Zinc supplementation decreases oxidative stress, incidence of infection, and generation of inflammatory cytokines in sickle cell disease patients. Transl Res 2008;152:67-80.

20. 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.

21. 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.

22. 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.

23. 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.

24. 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.

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

26. 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.

27. 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.

28. 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.

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

30. 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.

31. 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.

32. 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.

33. 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.

34. 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.

35. 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.

36. 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.

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

38. 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.

39. 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.

40. 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.

41. 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.

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

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

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

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

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

47. 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.

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

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

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

51. 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.

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

53. 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.

54. 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.

55. 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.

56. 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.

57. 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.

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

59. 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.

60. 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.

61. 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.

62. 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.

63. 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.

64. 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.

65. 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.

66. 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.

67. 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.

68. 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.

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

70. 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.

71. 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.

72. 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.

73. 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.

74. 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.

75. 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.

76. 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.

77. 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.

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The information presented by Healthnotes is for informational purposes only. It is based on scientific studies (human, animal, or in vitro), clinical experience, or traditional usage as cited in each article. The results reported may not necessarily occur in all individuals. Self-treatment is not recommended for life-threatening conditions that require medical treatment under a doctor's care. For many of the conditions discussed, treatment with prescription or over the counter medication is also available. Consult your doctor, practitioner, and/or pharmacist for any health problem and before using any supplements or before making any changes in prescribed medications. Information expires December 2018.