According to the Centers for Disease Control and Prevention, approximately 70,000 to 100,000 Americans have sickle cell disease where a mutation in hemoglobin creates sickle-shaped red blood cells when they do not have oxygen. These red blood cells are hard, yet fragile, and they clog up the small blood vessels. This causes a great deal of pain as well as damage to the tissues and organs. Physicians use several medications to help people with this disease.
Analgesics
Analgesics are medications that relieve pain. According to "Harrison's Principles of Internal Medicine," morphine and meperidine, both types of opioids, stimulate the opioid receptors in the tissues and central nervous system, or the brain and spinal cord. Receptors are proteins on or within cells that wait for instructions on what to do. When morphine and meperidine stimulate the opioid receptors, they release substances to ease the pain.
A medication called ketorolac is used to relieve bone pain. It is a NSAID, or nonsteroidal anti-inflammatory drug. It eases pain, is anti-inflammatory and is not made from steroids.
Hydroxyurea
Hemoglobin is made of four parts, called chains. The majority of adult hemoglobin has two alpha chains and two beta chains. The red blood cells sickle because of a mutation on the beta chain. Fetal hemoglobin is likewise made of four chains, but it has two alpha chains and two gamma chains. Infants do not start making the adult form of hemoglobin until they are approximately six months old. Hydroxyurea is a medication used for sickle cell disease because it increases the fetal hemoglobin. The Merck Manual notes that it has some serious side effects: it decreases the number of white blood cells and platelets, and causes birth defects. Physicians do not give hydroxyurea to women who are within the age range where they can have children.
Folic Acid
Folic acid, or vitamin B9, is essential for the cells to be able to make DNA and RNA. The cells must have DNA and RNA to divide. Any cells of the body--and particularly developing blood cells--that are rapidly dividing need folic acid in large amounts. Since sickle cell disease destroys the sickled, fragile red blood cells, people with this disorder especially need folic acid.
Penicillin
According to The Merck Manual, physicians prescribe penicillin for children from four months to six years old. Penicillin is an antibiotic; it kills bacteria. Children take penicillin to prevent getting bacterial infections due to sickle cell disease.
References
- Centers for Disease Control and Prevention: Sickle Cell Disease
- "Handbook of Pathophysiology"; Elizabeth Corwin, MSN, Ph.D., FNP; 2000
- "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
