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Benign Hematology Conditions
Sickle cell disease (SCD) is a blood disorder that is inherited genetically. In the United States, it predominantly affects African Americans. Globally, the disease also affects people of Mediterranean descent and other racial and ethnic backgrounds. About 1 out of every 400 African American births is affected by SCD. There are approximately 100,000 people with SCD in the United States.
There are several different forms of SCD. The most common and usually the most severe form is sickle cell anemia.
Symptoms of Sickle Cell DiseaseRed blood cells contain hemoglobin, a protein that enables the blood to carry oxygen from the lungs to other parts of the body. SCD is marked by an abnormal hemoglobin that causes the blood cell to become shaped like a sickle or crescent. These sickled cells also become stiff and sticky compared to normal red blood cells. Sickled cells are less able to flow smoothly within the blood vessels. They end up blocking the smallest blood vessels, causing decreased blood flow to various parts of the body. Symptoms and complications of SCD include:
Bone painDecreased blood flow to the bones leads to periodic spikes in bone pain known as bone crises.
Acute chest syndromeSickled cells in the lungs cause decreased blood flow, inflammation and acute lung injury. Patients may need to be on a ventilator.
InfectionsThe spleen fails in SCD patients at a young age, decreasing the body’s ability to fight infections.
AnemiaSickled blood cells are less able to carry oxygen, leading to anemia. Symptoms can include fatigue, weakness, dizziness, headache, shortness of breath and chest pains.
Eye diseaseSickled blood cells can damage the fragile blood vessels in the back of the eye, leading to retina damage.
Lower extremity ulcersOlder children and adults with SCD can develop sores on the lower leg.
End organ diseaseDecreased blood flow can lead to organ failure, potentially affecting the kidneys, liver and heart.
StrokeChildren between the ages of 6 and 10 are at high risk of stroke. Older patients have a lower overall risk of stroke, but they have a higher risk of dying from a stroke.
Children with SCD tend to experience symptoms on an episodic basis. As patients enter adulthood, they tend to have chronic symptoms and organ dysfunction.
Call the Sickle Cell Clinic (414-805-2220) right away for:
- Fever greater than 101.5 degrees
- Stomach swelling
- Headache with no known reason
Call 911 right away for:
- Trouble breathing, seeing, walking, speaking, standing or understanding
- Dizziness or difficulty using hands
- Loss of feeling of the face, arm or leg, mainly on one side of the body
- Chest pain
Sickle Cell Disease TreatmentPatients with SCD require comprehensive care to manage the symptoms and complications of their disease.
Blood transfusionEmergency blood transfusion is used to treat stroke and acute chest syndrome. Patients may also receive transfusion to treat acute anemia. Patients who have had a stroke may receive chronic transfusion therapy (blood transfusions every 4 to 6 weeks) to prevent a second stroke and other SCD complications.
Drug therapy (hydroxyurea)In people without SCD, a small percentage of the hemoglobin in the blood is fetal hemoglobin. The drug hydroxyurea stimulates the production of fetal hemoglobin in patients with SCD, which decreases sickle hemoglobin.
Supporting careA variety of therapies are used to treat symptoms and complications of SCD. Bone crises are treated with pain medications and intravenous fluid. Antibiotics are used preventatively to reduce the risk of infection in the young. Patients should receive frequent eye exams to detect retina damage early.
Blood and marrow transplant (BMT)A small percentage of SCD patients can be treated with a stem cell transplant. Healthy stem cells collected from bone marrow or circulating blood can potentially cure SCD in younger patients. Stem cell transplant does carry significant risks, so candidates are carefully evaluated based on their prognosis, their current health and the availability of a suitable donor. Adult patients are generally ineligible for this therapy. Learn more about the Blood and Marrow Transplant (BMT) Program.
Adult Sickle Cell ClinicThe Sickle Cell Clinic at Froedtert & The Medical College of Wisconsin provides comprehensive care for adults with sickle cell disease. Patients receive ongoing care including drug management and pain management. Infusion services, including automated red cell exchange, are overseen by a physician who is board-certified in transfusion medicine. Patients manage all their acute health concerns through the clinic, without the need to go to a hospital emergency room. All patients receive education, coaching and follow-up support.
Date: March 8, 2013 Online Editor(s): Shannon Krause |
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