As of 2010, according to the Centers for Disease Control and Prevention, approximately 90,000 to 100,000 people have sickle cell anemia, a genetic disease that involves abnormal red blood cells. People with this disorder have red blood cells that are shaped like a sickle, which makes it hard for them to freely circulate throughout the bloodstream. When the red blood cells get stuck in the blood vessels, they obstruct the flow of blood and oxygen.
What is Sickle Cell Disease?
Sickle cell disease is a type of anemia. It is a hereditary disease, where an amino acid called valine has taken the place of the amino acid glutamine in the hemoglobin of a red blood cell. This change creates a hemoglobin that is not stable. As a result, the red blood cells get hard and change into a sickle shape when there is not enough oxygen in the bloodstream or when there is too much acid, explains Charles Linker, M.D., Director of the Bone Marrow Transplant Program at the University of California in "Current Medical Diagnosis & Treatment."
Symptoms
Children with sickle cell disease have a large spleen at first, but the sickle-shaped red blood cells interfere with the flow of blood. This damages the spleen so much that it becomes very small, as explained in "The Merck Manual for Healthcare Professionals" by Alan Lichtin, M.D., of the Cleveland Clinic. The interference with the flow of blood in the blood vessels leads to pain crises, or severe pain in the joints, feet, hands and bones. If it happens in the blood vessels in the lungs, a sufferer may become short of breath and have chest pain. There can be pain in the lower back if the sickle cells block the blood flow to the kidneys.
Folic Acid
Folic acid, also called folate, is needed for the cells to make normal DNA. Thus, any cells in the body that quickly divide will especially be affected if there is not enough folic acid, writes Bertram Katzung, M.D., Ph.D., Professor Emeritus in the Department of Cellular and Molecular Pharmacology at the University of California in "Basic & Clinical Pharmacology." A folic acid deficiency especially affects the red blood cells, and since these cells are destroyed in sickle cell disease, people with this disease should take folic acid supplements every day.
Why Folic Acid Supplements?
The highest amount of folate is found in spinach and other greens, yeast, nuts and liver, but the body does not store much folic acid. Most of it is stored in the liver, but there is normally enough to last for three to four months. Also, the folic acid in food is destroyed by overheating, especially when the food is cooked in a large amount of water, per A. Victor Hoffbrand, D.M., Emeritus Professor of Haematology at University College in "Harrison's Principles of Internal Medicine." To ensure people with sickle cell have enough folic acid for their red blood cell production, they take folic acid supplements.
References
- "Basic & Clinical Pharmacology"; Bertram Katzung, M.D., Ph.D., Susan Masters, Ph.D., Anthony Trevor, Ph.D.; 2009
- Centers for Disease Control and Prevention: World Sickle Cell Day - 100 Years of Research
- "Current Medical Diagnosis & Treatment 2011"; Stephen McPhee, M.D., Maxine Papadakis, M.D.; 2011
- "Harrison's Principles of Internal Medicine"; Anthony Fauci, M.D., Dennis Kasper, M.D., Dan Longo, M.D. et al.; 2008
- The Merck Manual for Healthcare Professionals: Sickle Cell Disease
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