People who have sickle cell disease have a reduced life expectancy.
- Adults with the most severe forms of sickle cell disease have a life span that is 20 to 30 years shorter than people who don’t have the condition.
- The national median life expectancy of people who have sickle cell disease is 42 to 47 years.
A report from Blood, the Journal of the American Society of Hematology, showed that some people with mildly symptomatic sickle cell disease may live longer lives with proper management of the disease, including strong family support and strict adherence to medication and appointments. However, the report is an analysis of just four case studies so more study is needed.
What Is Sickle Cell Anemia?
Sickle cell anemia is a type of sickle cell disease, an inherited disorder that affects the body’s red blood cells which are responsible for carrying oxygen to the organs in the body using a protein called hemoglobin.
- Normal red blood cells are disc-shaped and flexible so they can fit through small blood vessels.
- Sickle cell disease causes some red blood cells to have an abnormal crescent shape that resembles a farming tool called a sickle, which is how the disease got its name.
- These sickle cells can latch on to one another or stick to blood vessel walls where they can accumulate and cause blood vessels to become blocked. Blood vessel blockages prevent organs from getting the oxygen they need, which can result in pain or organ damage.
What Are Symptoms of Sickle Cell Anemia?
Symptoms of sickle cell anemia often start when a baby is a few months old and may include:
- Pain crisis, also called sickle crisis
- May be mild or severe
- Can last hours to days
- Severe anemia
- Severe and sometimes life-threatening infections
- Lung problems
- Open sores on the skin (usually on the legs)
- Yellowing skin and eyes (jaundice)
- In men, a painful erection that lasts too long (priapism)
How Is Sickle Cell Anemia Diagnosed?
Sickle cell disease is diagnosed with a blood test called hemoglobin electrophoresis. All newborns in the U.S. are tested for sickle cell disease before leaving the hospital.
The same blood test can also show if a baby has sickle cell trait, though parents may not always be informed if a baby has sickle cell trait.
Parents may decide to have genetic testing for the sickle cell gene before attempting to conceive to determine the risk of having a baby with sickle cell disease.
What Is the Treatment for Sickle Cell Anemia?
Treatment for sickle cell anemia is aimed at treating symptoms and preventing organ damage and infection. Treatment is life-long and may include:
- Adequate hydration
- Drink 8 to 10 glasses of water daily to prevent and treat pain crises
- Intravenous (IV) fluids may be needed
- Hydroxyurea (Droxia, Hydrea) to produce more normal red blood cells
- L-glutamine (Endari), voxelotor (Oxbryta), and crizanlizumab (Adakveo) to decrease pain episodes
- Pain medicines
- Medicines to prevent and treat infections
- Folic acid (folate) supplementation to help make red blood cells
- Blood transfusions to treat anemia and prevent stroke
- Bone marrow transplant (also called a stem cell transplant)
- This procedure can cure sickle cell disease; however, it’s not routinely performed because it has numerous side effects and patients must meet certain criteria
- Gene therapy (under investigation)
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