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Sickle Cell Disease

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Principal Proposed Natural TreatmentsOther Proposed Natural Treatments


Sickle cell disease is an inherited blood disorder. Normally, red blood cells are disc-shaped and flexible. In sickle cell disease, however, hemoglobin (the chemical within red blood cells that carries oxygen around the body) is abnormal. This defect causes red blood cells to collapse into a crescent, or sickle, shape.
These abnormal blood cells are destroyed at an unusually high rate, causing a shortage of red blood cells (anemia). In addition, they can suddenly clump together and clog up small blood vessels throughout the body. This clumping causes what is called a sickle cell crisis.
When blood vessels are blocked by sickle-shaped red blood cells, parts of the body are deprived of oxygen. This can cause severe pain and damage to the organs and tissues that are deprived.
Common triggers of sickle cell crisis include smoking, exercise, exposure to high altitudes, fever, infection, dehydration, and the drop in oxygen or changes in air pressure that can occur during airplane travel.
Diagnosis of sickle cell disease and sickle cell trait (a condition in which a person has one of the two genes necessary to develop sickle cell disease) can be done through blood testing, using a technique called hemoglobin electrophoresis. Treatment involves managing the anemia, chronic pain, and organ damage caused by sickle cell disease. In addition, the drug hydroxyurea can reduce occurrences of sickle cell crisis. Of course, it is also important to minimize exposure to conditions or situations that can trigger sickle cell crisis.

Principal Proposed Natural Treatments

Children with sickle cell disease often do not grow normally. Zinc deficiency can also cause delayed growth, and there is some evidence that people with sickle cell disease are more likely than others to be deficient in the mineral zinc . 1 For this reason, zinc supplementation at nutritional doses has been suggested for children with sickle cell disease.
In a placebo-controlled study , 42 children (ages 4 to 10) with sickle cell disease were given either zinc supplements (10 mg of zinc daily) or placebo for a period of 1 year. 1 Results showed that by the end of the study, the participants given zinc showed enhanced growth compared to those given placebo. Curiously, researchers did not find any solid connection between the severity of zinc deficiency and the extent of response to treatment.
Zinc is thought to have a stabilizing effect on the cell membrane of red blood cells in people with sickle cell disease. For this reason, it has been tried as an aid for preventing sickle cell crisis. In a double-blind, placebo-controlled study of 145 people with sickle cell disease conducted in India, participants received either placebo or about 50 mg of zinc 3 times daily. 2 During 18 months of treatment, the zinc-treated subjects had an average of 2.5 crises, compared to 5.3 for the placebo group. However, zinc didn't seem to reduce the severity of a crisis, as measured by the number of days spent in the hospital for each crisis.
Sickle cell disease can also cause skin ulcers (non-healing sores). In a 12-week, placebo-controlled trial, use of zinc at 88 mg 3 times per day for 12 weeks enhanced the rate of ulcer healing. 3 And, in another placebo-controlled trial, 25 mg of zinc 3 times per day for 3 months reduced the frequency of infections in children with sickle cell disease. 29
Warning: The high dosages of zinc used in the last two studies can cause dangerous toxicity, and should be taken—if at all—only under the supervision of a doctor. The nutritional dose described in the first study, however, is safe.
For more information, including detailed dosage and safety issues, see the full zinc article.

Other Proposed Natural Treatments

A 1-year-long, double-blind, placebo-controlled crossover study of 82 people with sickle cell disease tested a combination herbal treatment made from plants indigenous to Nigeria. 28 The results indicate that use of the herbal mixture reduced the incidence of sickle cell crisis.
A very small double-blind, placebo-controlled trial found intriguing evidence that fish oil may reduce the frequency of painful sickle cell episodes, possibly by reducing the tendency of the blood to clot. 4
Numerous other herbs and supplements have been suggested for people with sickle cell disease, including alpha-linolenic acid, beta-carotene , coenzyme Q 10 , folate , garlic , green tea , lipoic acid , magnesium , OPCs (oligomeric proanthocyanidins) , suma , vitamin B 2 , vitamin B 6 , vitamin B 12 , vitamin C , and vitamin E , but as yet the supporting evidence for these treatments remains far too preliminary to be relied upon at all. 5–27

References

1 Zemel BS, Kawchak DA, Fung EB, et. al. Effect of zinc supplementation on growth and body composition in children with sickle cell disease. Am J Clin Nutr. 2002;75:300–307.

2 Gupta VL, Chaubey BS. Efficacy of zinc therapy in prevention of crisis in sickle-cell anemia: a double-blind, randomized controlled clinical trial. J Assoc Physicians India. 1995;43:467–469.

3 Serjeant GR, Galloway RE, Gueri MC. Oral zinc sulphate in sickle-cell ulcers. Lancet. 1970;2:891–893.

4 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;85:966–974.

5 Ajayi OA, George BO, Ipadeola T. Clinical trial of riboflavin in sickle cell disease. East Afr Med J. 1993;70:418–421.

6 al-Momen AK. Diminished vitamin B12 levels in patients with severe sickle cell disease. J Intern Med. 1995;237:551–555.

7 Ballas SK. Short Report: Hydration of sickle erythrocytes using a herbal extract ( Pfaffia paniculata ) in vitro. Br J Haematol. 2000;111:359–362.

8 Chiu D, Vichinsky E, Ho SL, et al. Vitamin C deficiency in patients with sickle cell anemia. Am J Pediatr Hematol Oncol. 1990;12:262–267.

9 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;100:1847–1852.

10 De Franceschi L, Bachir D, Balacteros F, et al. Oral magnesium pidolate: effects of long-term administration in patients with sickle cell disease. Br J Haematol. 2000;108:284–289.

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

12 Flores L, Pais R, Buchanan I, et al. Pyridoxal 5’-phosphate levels in children with sickle cell disease. Am J Pediatr Hematol Oncol. 1988;10:236–240.

13 Jain SK, Ross JD, Duett J, et al. Low plasma prealbumin and carotenoid levels in sickle cell disease patients. Am J Med Sci. 1990;229:13–15.

14 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;149:257–261.

15 Kark JA, Kale MP, Tarassoff PG, et al. Inhibition of erythrocyte sickling in vitro by pyridoxal. J Clin Invest. 1978;62:888–891.

16 Kark JA, Tarassoff PG, Bongiovanni R. Pyridoxal phosphate as an antisickling agent in vitro. J Clin Invest. 1983;71:1224–1229.

17 Muskiet FA, Muskiet FD, Meiborg G, et al. Supplementation of patients with homozygous sickle cell disease with zinc, alpha-tocopherol, vitamin C, soybean oil, and fish oil. Am J Clin Nutr. 1991;54:736–744.

18 Natta C, Stacewicz-Sapuntzakis M, Bhagavan H, et al. Low serum levels of carotenoids in sickle cell anemia. Eur J Haematol. 1988;41:131–135.

19 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;33:968–971.

20 Natta CL, Reynolds RD. Apparent vitamin B6 deficiency in sickle cell anemia. Am J Clin Nutr. 1984;40:235–239.

21 Ndombi IO, Kinoti SN. Serum vitamin E and the sickling status in children with sickle cell anemia. East Afr Med J. 1990;67:720–725.

22 Ohnishi ST, Ohnishi T, Ogunmola GB. Sickle cell anemia: A potential nutritional approach for a molecular disease. Nutrition. 2000;16:330–338.

23 Osifo BO, Adeyokunnu A, Parmentier Y, et al. Abnormalities of serum transcobalamins in sickle cell disease (HbSS) in Black Africa. Scand J Haematol. 1983;30:135–140.

24 Phillips G, Tangney CC. Relationship of plasma alpha tocopherol to index of clinical severity in individuals with sickle cell anemia. Am J Hematol. 1992;41:227–231.

25 Reed JD, Redding-Lallinger R, Orringer EP. Nutrition and sickle cell disease. Am J Hematol. 1987;24:441–455 .

26 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;32:161–166.

27 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;59:192–198.

28 Wambebe C, Khamofu H, Momoh JA, et al. Double-blind, placebo-controlled, randomised cross-over clinical trial of NIPRISAN in patients with Sickle Cell Disorder. Phytomedicine . 2001;8:252–261.

29 Bao B, Prasad AS, Beck FW, 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.

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