Sickle Cell Disease
Normal red blood cells have a 120-day life span. But people born with sickle cell disease have Reference sickle-shaped Opens New Window Reference Opens New Window blood cells that usually live no more than 20 days. These sickled cells can get stuck in blood vessels, Reference blocking blood flow Opens New Window Reference Opens New Window.
Less 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:
- Reference Sickle cell crisis, which happens when blood vessels are blocked. This is a common condition of sickle cell disease.
- Reference Splenic sequestration, usually seen in infants and young children during or after a simple respiratory infection. Large numbers of sickled red blood cells get trapped in the Reference spleen Opens New Window Reference Opens New Window. It can cause sudden and life-threatening Reference anemia Opens New Window.
- Reference Acute chest syndrome, most common in children but more severe in adults. Symptoms include coughing and chest pain, which may occur after an infection or painful event.
- Severe infections.
- Reference Aplastic crisis, which may occur after infection with some viruses. During an aplastic crisis, bone marrow stops producing red blood cells, which causes 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 Reference fetal hemoglobin (HbF) Opens New Window 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.
Older children and adults with sickle cell disease may have few problems. Or they may 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 out of 100 children who have sickle cell disease.Reference 1
Other complications of sickle cell disease include:
- Growth slowdown. Children who have sickle cell disease often grow more slowly than normal and go through Reference puberty Opens New Window later than children who don't have the 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 Reference vision problems may result from blocked blood flow in the inner lining of the eye (retina).
- A lung problem called Reference pulmonary hypertension Opens New Window where blood pressure is very high in the lungs.
|By:||Reference Healthwise Staff||Last Revised: Reference October 1, 2012|
|Medical Review:||Reference E. Gregory Thompson, MD - Internal Medicine
Reference Martin Steinberg, MD - Hematology