Pain Management: Sickle Cell Disease
What Are the Symptoms of Sickle Cell Crisis?
Symptoms of sickle cell crisis include:
- Severe pain
- Chest pain and difficulty breathing
- Joint pain and arthritis and bone infarctions
- Blockage of blood flow in the spleen or liver
- Severe infections
Patients with sickle cell disease may develop severe pain in the chest, back, arms, legs, and abdomen. Pain can occur anywhere in the body. Sickle red blood cells in the lungs can cause severe illness with chest pain, fever, and difficulty breathing. Sickle cell disease can also cause permanent damage to the brain, heart, kidneys, liver, spleen, and bones. The severity and symptoms vary greatly from person to person, even within the same family.
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.