Breakthrough for young man treated for sickle cell disease
Sickle Cell Anemia Treatment
Treatment of sickle cell anemia includes taking measures to treat anemia, prevent sickle cell crises and other complications, and treat any crises or complications that occur.
Preventive Care for Children
Children with sickle cell anemia are at high risk of infection, so much of their routine care is aimed at preventing infection by the following:
The drug hydroxyurea is approved for use in adults with sickle cell anemia, and is sometimes used in children too.
Hydroxyurea causes the body to produce fetal hemoglobin, a type of hemoglobin that is normally produced only before and shortly after birth.
The presence of fetal hemoglobin lengthens the life span of red blood cells and makes them less likely to sickle.
It takes several months for hydroxyurea to start having any benefit.
People taking hydroxyurea should have their blood checked monthly to assess the effect of the drug and to help determine the correct dose.
Possible short-term side effects of hydroxyurea include gastrointestinal complaints and, rarely, darkening of the skin and nails.
Little is known about the long-term side effects of hydroxyurea.
Help for Pain
When treating pain associated with sickle cell anemia at home, the following can help:
In a hospital, sickle cell crises are generally treated with:
- Fluids, given by mouth or intravenously
- Pain relievers, including those used at home or, for severe pain, opioids
- Oxygen therapy if oxygen levels are low
Blood transfusions may be needed in some situations, such as:
- A sudden worsening of anemia due to an infection
- A condition known as splenic sequestration, in which large amounts of blood pool in the spleen
- Other acute complications of sickle cell anemia
Some people need blood transfusions only occasionally, while others need them regularly.
Blood transfusions have their own complications, including transfusion-induced iron overload.
Because the body does not have a way to get rid of iron received from blood transfusions, excess iron can build up in and damage vital organs.
Iron overload is prevented with a class of medications called iron chelators, drugs that attach to iron in the body so that it can be excreted in feces.
Stem Cell Transplants
Blood and bone marrow stem cell transplants may be used to treat sickle cell anemia and may offer a cure for a small number of people.
They are performed mainly in children with sickle cell anemia, although early studies suggest they may be feasible in adults as well.
Stem cells are found in bone marrow and, in smaller quantities, in blood and in the umbilical cord.
Stem cells can develop into red blood cells, white blood cells, and platelets.
The stem cells used for a transplant must come from a closely matched donor — usually a family member who does not have sickle cell anemia.
Maintaining a healthy lifestyle with sickle cell anemia can help with symptoms and includes:
- Eating right
- Exercising — but talk to your doctor about an appropriate amount
- Staying hydrated
- Getting enough sleep
- Not smoking
- Talking to your doctor about how much alcohol, if any, is safe to drink
People with sickle cell anemia can also help to prevent sickle cell crises by following these precautions:
- Don't take decongestants, which tend to tighten blood vessels.
- Avoid very hot or cold temperatures, both of which can trigger a crisis.
- Be cautious at high altitudes — you may need extra oxygen.
- Avoid choosing a job that requires a lot of physical labor, exposes you to extreme temperatures, or involves long work hours.
- Don't travel in unpressurized airplanes (speak to your doctor if you must fly in such a plane).
Video: Defeating Sickle Cell Disease with Stem Cells + Gene Therapy: Stem Cells in Your Face, Episode 2
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