Sickle cell anemia - Sickle cell disease (SCD)
It's a disease of the blood that gets its name because sickle cells look like a farm tool called a sickle. Sickle cell disease (SCD) and its variants are genetic disorders of mutant hemoglobin's.
There's no cure for sickle cell anemia, but your doctor can help you or your child live with the disorder, and treatments are improving. People with sickle cell anemia may require treatment from specialists at a hospital or a sickle cell anemia clinic. Regular visits to your doctor are a routine part of treatment. Treatment may include drugs to reduce pain and prevent complications, blood transfusions, supplemental oxygen and bone marrow transplants.
In a blood transfusion, red blood cells are removed from a supply of donated blood. These donated cells are then given intravenously to a person with sickle cell anemia.
Blood transfusions increase the number of normal red blood cells in circulation, helping to relieve anemia. In children with sickle cell anemia at high risk of stroke, regular blood transfusions can cut the risk of a first or second stroke by up to 90 percent.
Blood transfusions carry some risk. Blood contains the mineral iron. Regular blood transfusions cause an excess amount of iron to build up in your body. Because excess iron can damage your heart, liver and other organs, people who undergo regular transfusions must often receive treatment to reduce iron levels.
Bone marrow transplant
In a bone marrow transplant, your bone marrow is first destroyed using chemotherapy or radiation. A matched donor's healthy bone marrow is removed through a minor surgical technique. You then receive a transfusion of that healthy marrow, and the healthy marrow may start producing normal blood cells. Sometimes, however, the transplant doesn't work or the recipient's body rejects the new marrow.
The procedure requires a lengthy hospital stay. After the transplant, you'll need drugs to help prevent rejection of the donated marrow.
Before trying to conceive a child, you may decide to undergo genetic counseling if you carry the sickle cell trait.
An in vitro fertilization procedure is available that improves the chances that parents who each carry the sickle cell gene will have a child with normal hemoglobin. In the procedure, eggs are taken from the mother. Sperm is taken from the father. In a laboratory, the eggs are fertilized with the sperm. The fertilized eggs are then tested for the presence of the sickle cell gene. This procedure is known as preimplantation genetic diagnosis. Fertilized eggs free of the sickle cell gene can be implanted into the mother for normal development. However, this procedure is expensive and not always successful.
Taking steps to stay healthy is critical for anyone with sickle cell anemia. Good nutrition, adequate rest and protection against infections all are important to maintain good health.
Infants and children with sickle cell disease need to receive regular childhood vaccinations. Children and adults with sickle cell anemia also should have a yearly flu shot and be immunized against pneumonia.
To help stay healthy, follow these suggestions if you have sickle cell anemia:
If you or someone in your family has sickle cell anemia, you may need help handling the stresses of coping with this lifelong disease.
Your doctor can talk with you about your concerns. Sickle cell centers and clinics also can provide information and counseling. Many areas have sickle cell support groups for families affected by the disease.
The best way to help a child with sickle cell anemia is to learn as much as you can about the disease and to make sure the child gets the best health care possible. A child with sickle cell disease has special needs and requires regular medical care to stay as healthy as possible. Your doctor can explain how often to bring your child for medical care and what you can do if he or she becomes ill.