Sickle-cell disease is a genetic disorder that causes normally flexible red blood cells to contort into a crescent-moon shape that makes them clump, blocking blood vessels and causing most patients bouts of intense pain. Normally, red blood cells, which carry oxygen throughout the body, are round and smooth. In sickle cell anemia, the red blood cells are sickle-shaped, which causes them to get caught up in smaller blood vessels, sometimes blocking blood flow. The complications of sickle cell anemia are numerous and include pain, infections, gallstones, leg ulcers, priapism in males (sustained and painful erections), pulmonary hypertension and stroke. For more research in the area of possible future research on natural ways to treat sickle cell disease or reduce the severity, consider signing up to a free email Newsletter.

Sickle Cell Anemia prevalence
About 1 in 650 African-Americans and up to 1 in 1,000 Latinos in the United States has some form of sickle cell disease including sickle cell anemia. Sickle cell anemia affects about 72,000 Americans, according to the National Heart, Lung, and Blood Institute (NHLBI). Those most at risk of the disease are blacks and people of Mediterranean and Middle Eastern descent.

Natural Options for Sickle Cell Disease
Glutamine - see study below. You can purchase Glutamine here. Discuss with your doctor before starting any supplement program.

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Sickle Cell and Blood Transfusions
Some patients with severe sickle cell disease may need a lifetime of blood transfusions to reduce the chances of suffering a stroke. A study, published in December 2005 New England Journal of Medicine, found that the stroke risk reappeared after blood exchanges were stopped. Regular transfusions of red blood cells typically cut the stroke risk by 90 percent.

Priapism and Sickle Cell Disease
Priapism, an unwanted painful erection of the penis, is a common complication of sickle cell disease. What is known about the prevalence of priapism, efficacy of management approaches, and outcome is drawn primarily from retrospective and single-center reports. Priapism occurs in two patterns: prolonged and stuttering (ie, recurrent brief episodes that resolve spontaneously). If priapism persists for 4 hours or more without detumescence, the patient is at risk for irreversible ischemic penile injury, which may terminate in fibrosis and impotence.

Lactate Dehydrogenase as biomarker
Lactate dehydrogenase levels are a biomarker for a subphenotype of sickle cell disease that includes pulmonary hypertension, leg ulcerations, priapism, and higher mortality risk.The lactate dehydrogenase level can be a convenient risk marker for clinicians to use in patients with sickle cell disease.

Sickle Cell Attack Trigger
Asthma is a risk factor associated with painful events in children with sickle cell disease. A sickle cell attack can occur a day or two after an asthma attack.

Sickle Cell Research Update
Oral glutamine supplementation decreases resting energy expenditure in children and adolescents with sickle cell anemia.
J Pediatr Hematol Oncol. 2004 Oct;26(10):619-25.
To determine the effects of orally administered glutamine on the resting energy expenditure (REE) and nutritional status of children and adolescents with sickle cell anemia. Twenty-seven children and adolescents (13 boys, 14 girls), 5.2 to 17.9 years old, received orally administered glutamine (600 mg/kg per day) for 24 weeks. Measures of REE and other nutritional parameters were compared at baseline and 24 weeks. RESULTS: After 24 weeks, the patients' median REE (kcal/d) decreased by 6%. Patients with less than 90% ideal body weight had even greater declines in REE after 24 weeks. Improvements in nutrition parameters and in two amino acids in the plasma were observed. CONCLUSIONS: After 24 weeks of orally administered glutamine, children and adolescents with sickle cell anemia had a decrease in REE and improvement in nutritional parameters. Those who were underweight had a greater decrease in REE than those of normal body weight. Lowering REE may be an effective way to improve the growth of these children and adolescents.

Sickle Cell Blood Test
A commonly ordered blood test may help doctors predict which sickle cell patients might develop serious complications, such as pulmonary hypertension and leg ulcers, and who's at risk for early death. When the enzyme lactate dehydrogenase (LDH) is present in high levels in the blood of someone with sickle cell anemia, the risk of pulmonary hypertension (high blood pressure in the lungs) is quadrupled compared to someone with low levels of LDH.
     LDH is an enzyme found in many cells throughout the body. Red blood cells, the heart, the kidneys, the liver and muscles are especially rich in LDH. Usually, the upper limit of LDH in healthy adults is 200 international units per liter (IU/L). However, when organs become diseased, LDH is released in increasing amounts. Low LDH levels in sickle cell patients conferr the lowest risk of developing leg ulcers, priapism and pulmonary hypertension.

Sickle cell disease questions
Q. Hello: I was doing some reading on L Arginine and found your site. I remember reading a number of years ago about nitric oxide being tested on sickle cell patients. After reading the effects of arginine; I wonder if supplementation would be advantageous for this population of folks. If too short acting for day to day use; could higher dosing help ease pain during a crisis? There should be a study.
   A. I have no idea if arginine would help patients with sickle cell anemia.