Sickle Cell Disease
What Happens
Normal red blood cells have a 120-day life span. But people born with sickle cell disease have sickle-shaped
blood cells that usually live no more than 20 days. These sickled cells can get stuck in blood vessels, blocking blood flow. See a picture of sickle cells blocking a blood vessel
Lesser blood flow can damage the body's organs, muscles, and bones, sometimes leading to life-threatening conditions. Sickle cell disease may cause problems such as:
- Painful events (vaso-occlusive complication), which result when blood vessels are blocked. This is a common condition of sickle cell disease.
- Splenic sequestration, usually seen in children age 6 months to 2 years during or after a simple respiratory infection. Large numbers of sickled red blood cells become trapped in the spleen
- Acute chest syndrome, most common in children but more severe in adults. Coughing and chest pain are symptoms of acute chest syndrome, which may occur after an infection or painful event.
- Severe infections, especially in children younger than 3.
- Aplastic crisis, which may occur after infection with some viruses. During an aplastic crisis, bone marrow stops producing red blood cells, which results in sudden and severe anemia.
When a child is born with sickle cell disease, it's impossible to predict which problems will develop, when they will start, or how bad they will be. During the first 6 months of life, infants have a high level of fetal hemoglobin (HbF) in their blood, which protects them from red blood cell sickling. But dangerous complications of sickle cell disease may quickly develop between ages 6 months and 5 years, after levels of fetal hemoglobin decrease. Normal red blood cells have a 120-day life span, but sickled blood cells usually live no more than 20 days. When bone marrow can't produce enough red blood cells to keep up with sickled blood cell loss, severe anemia may develop.
Older children and adults with sickle cell disease may have few problems or have a pattern of ongoing complications that shortens their lives. The most common and serious problems caused by sickle cell disease are anemia, pain, and organ failure. Stroke affects around 10% of children with sickle cell disease.2
Other complications of sickle cell disease include:
- Growth slowdown. Children with sickle cell disease often grow more slowly than normal and go through puberty later than children who don't have sickle cell disease.
- Open sores (ulcers) on the legs and feet, commonly during adulthood. These ulcers can be very painful and heal slowly. Some may last for years.
- Eye damage. Long-term vision problems may result from blocked blood flow in the inner lining of the eye (retina).
People who have sickle cell disease live to age 50 to 60, on average.3 A lot depends on the type of sickle cell disorder and how it affects a person's health.
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| Author: | Debby Golonka, MPH | Last Updated: December 9, 2008 |
| Medical Review: | Anne C. Poinier, MD - Internal Medicine Martin Steinberg, MD - Hematology | |
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