Sickle cell disease, an inherited genetic disease, is characterized by abnormal hemoglobin production, which causes red blood cells to sickle, or change shape to resemble a "C" when exposed to low oxygen conditions. The abnormally shaped red blood cells cannot carry oxygen and the cells clump together blocking blood flow through arteries and veins. No cure exists for sickle cell disease. Treatments aim to control symptoms, prevent sickle cell crisis and improve the quality of life.
Nonsteroidal Anti-Inflammatories
Patients with sickle cell disease experience muscle, joint and organ pain. Chronically low hemoglobin and clumping of red blood cells damage tissues, causing inflammation throughout the body. The National Heart Lung and Blood Institute reports that over-the-counter anti-inflammatory medications such as ibuprofen help relieve mild to moderate pain.
Billie Ann Wilson, Ph.D., Margaret Shannon, Ph.D., and Kelly Shields Pharm.D., authors of "Pearson Nurse's Drug Guide 2010" list common side effects of NSAIDs, or nonsteroidal anti-inflamatory drugs such as ibuprofen, which include nausea, indigestion and gastrointestinal bleeding. Patients should take NSAIDs as directed by a physician to prevent serious side effects like kidney and liver toxicity.
Acetaminophen
Acetaminophen relieves mild to moderate pain symptoms without causing gastrointestinal upset. Liver toxicity can occur in overdose situations. The maximum adult dose is 4g in a 24-hour period. To achieve continual pain relief coverage and to avoid toxic effects of medications, patients can alternate between ibuprofen and acetaminophen throughout the day.
Narcotics
Physicians frequently prescribe meperidine, morphine and oxycodone to manage moderate to severe pain. Patients may have prescriptions of narcotics to manage pain at home. However, sometimes patients require hospitalization with intravenous administration of narcotic pain relievers to get severe pain under control.
Narcotics cause drowsiness, suppressed respirations, low blood pressure, nausea, vomiting and constipation. To avoid constipation, sickle cell patients should drink at least eight 8-oz. glasses of water a day and eat a high fiber diet. While taking narcotic pain relievers patients should not drive or operate machinery. Patients need to take narcotics exactly as prescribed to avoid overdose, which can cause respiratory arrest and death.
Hydroxyurea
Hydroxyurea is an anti-neoplastic or cancer drug. Hydroxyurea works on a cellular-level to stop cell division and proliferation. This action stops red blood cell sickling, preventing the chain reaction of cell clumping which decreases serious pain symptoms and complications caused by tissue damage. The NHLBI states that patients with sickle cell disease take hydroxyurea to prevent sickle cell crises; the drug is not effective as a pain reliever during crisis.
Because hydroxyurea affects cell proliferation it diminishes numbers of all blood cells and suppresses bone marrow function. Circulating white blood cells, necessary to fight infection, are decreased, placing patients at risk for infection. Platelets help the blood clot and hydroxyurea lowers platelet production too, placing patients at risk for bleeding. These serious side effects require careful monitoring by a physician. Patients must take hydroxyurea as directed.
References
- National Heart Lung and Blood Institute: Sickle Cell--Treatment
- "Pearson Nurse's Drug Guide 2010"; Wilson, B. A., Shannon, T. M., & Shields, K. M.; 2010
