Pain Management: Sickle Cell Disease

Sickle cell disease is a genetic (passed from parent to child) disorder that affects the body's blood cells. In this disease, faulty hemoglobin (a substance that carries oxygen in the blood) causes the red blood cells to change shape when oxygen is released to tissues.

Normal red blood cells are round and are able to move through small blood vessels in the body to deliver oxygen. In sickle cell disease, a chemical change in hemoglobin causes the substance to form long rods in the red blood cell as the hemoglobin releases oxygen. These rigid rods change the shape of the red blood cell into a sickle shape.

The disease gets its name because the faulty blood cells are shaped like sickles used to cut wheat. When the deformed cells go through blood vessels, they clog the blood flow and can break apart.

Sickle cells can cause pain, damage the blood vessels or other areas of the body, and cause anemia (low blood count).

Sickle cell disease is found most often in African-Americans and Africans. However, other ethnic groups also can have sickle cell disease. In many states, the law requires newborn babies to be tested for sickle cell disease, regardless of ethnic background.

What Causes Sickle Cell Disease?

A chemical change in hemoglobin causes the substance to form long rods in the red blood cell as the hemoglobin releases oxygen. These rigid rods change the shape of the red blood cell into a sickle shape.

Sickle cell disease is not contagious. Children are born with sickle cell hemoglobin, which they inherit from their parents. Individuals may be carriers who have sickle cell trait or may actually have sickle cell disease. People who inherit only one sickle cell gene are carriers, but people who inherit two sickle cell genes have sickle cell disease.

What Are the Symptoms of Sickle Cell Disease?

Symptoms of sickle cell disease include:

• Fatigue
• Pain
• Anemia
• Swelling and inflammation of the joints
• Blood blockage in the spleen or liver

Sickle cell disease can also cause damage to the heart, lungs, kidneys and bones.

How Is Sickle Cell Disease Diagnosed?

Sickle cell disease is diagnosed by examining a sample of blood under a microscope. When the abnormal sickle-shaped cells in the blood are identified, a diagnosis is made. Also, a blood test called the hemoglobin electrophoresis can determine if a person has the disease or is a carrier.

How Is Sickle Cell Disease Treated?

Currently, there is no cure for sickle cell disease. Treatment of sickle cell disease depends upon the symptoms a person is experiencing. Some medications that can be used to treat sickle cell disease-related pain include:

• Opioid medications
• Hydroxyurea (for example, Hydrea and Droxia)
• Anti-inflammatory medications such as aspirin and ibuprofen
• Tricyclic antidepressants such as Elavil
• Anticonvulsants such as Gabapentin

What Is the Outlook for People With Sickle Cell Disease?

People who have sickle cell disease have a reduced life expectancy. Some people can survive beyond their 40's while others do not survive beyond infancy or early childhood.

Reviewed by the doctors at The Cleveland Clinic Pain Management Department.