Pain Management: Sickle Cell Disease
How Is Sickle Cell Disease Diagnosed?
Sickle-shaped red blood cells can be seen when a blood sample is examined under a microscope. But sickle cell disease is diagnosed by a blood test called hemoglobin electrophoresis, which measures the amount of the abnormal sickle hemoglobin. The amount of sickle hemoglobin determines whether the person is a carrier (sickle cell trait) or has sickle cell disease.
There are also rapid screening tests that detect the formation of sickle red blood cells or clumps of abnormal sickle hemoglobin when oxygen is removed from the blood. These tests are less commonly used, because they cannot distinguish between sickle cell trait and sickle cell disease.
Prenatal testing for sickle cell disease is possible by examining the DNA of fetal cells obtained by chorionic villus sampling or amniocentesis.
Testing newborns for sickle cell disease is required by law in all 50 states. Early detection and treatment reduces the risk of serious infections and other complications.
How Is Sickle Cell Disease Treated?
Treatment of sickle cell crisis includes:
- Opioid pain medications (for example, morphine)
- Anti-inflammatory medications (for example, ibuprofen)
- Antibiotics for infection
- Intravenous or oral fluids
Transfusions of red blood cells are given for severe anemia, to prevent strokes, and before surgery. Sometimes an exchange transfusion is performed with a special machine that removes the abnormal sickle red blood cells and replaces them with normal red blood cells.
Hydroxyurea is the only FDA-approved medication that prevents painful episodes in sickle cell disease. Studies of patients with sickle cell disease show that the regular use of hydroxyurea decreases the frequency and severity of sickle cell crises and reduces the number of blood transfusions and hospitalizations.
Stem cell transplant is the only curative treatment for sickle cell disease. More than 200 patients with sickle cell disease have undergone stem cell transplants from a matched sibling donor. Stem cell transplant has a 5%-10% risk of death, but patients with successful transplants were completely cured of sickle cell disease, with no further episodes of pain.
Stem cell transplant is performed in young patients with severe sickle cell disease who have a matched sibling donor. Stem cell transplant using umbilical cord blood from a related donor has also been curative in a small number of patients.
Scientists are making progress in the development of new medications that prevent sickling of red blood cells and improve blood flow and delivery of oxygen to the body. Some of these new treatments are already being tested in patients with sickle cell disease.
What Is the Outlook for People With Sickle Cell Disease?
People who have sickle cell disease have a reduced life expectancy. But because of improvements in treatment, people with sickle cell disease now live longer and have a better quality of life. In 1973, the average life span was 14, but people with sickle cell disease now live into their 40s and 50s, and in some cases, beyond the age of 60. Long-term treatment with hydroxyurea may prolong survival.