Sickle cell anemia is a blood disorder that affects people of all ages. It is passed from parents to their children. SCD causes irregular hemoglobin development in your body. Hemoglobin is a protein that is round and versatile in your red blood cells. They move through the small vessels of your blood and bring oxygen to all parts of your body. With sickle cell anemia, the red blood cells are poorly shaped, hard, and sticky. They get stuck inside and start clogging your blood vessels. This can lead to pain, infection, damage to organs, low blood count, stroke, and other serious health problems.

Preventing infections

  • Children with sickle cell anemia might receive penicillin between the ages of about 2 months old until at least age 5 years. Doing so helps prevent infections, such as pneumonia, which can be life-threatening to children with sickle cell anemia.
  • Adults who have sickle cell anemia might need to take penicillin throughout their lives if they’ve had pneumonia or surgery to remove the spleen.
  • Childhood vaccinations are important for preventing disease in all children. They’re even more important for children with sickle cell anemia because their infections can be severe.
  • Your child’s doctor should ensure that your child receives all the recommended childhood vaccinations, as well as vaccines against pneumonia, meningitis, hepatitis B and an annual flu shot. Vaccines are also important for adults with sickle cell anemia.

During the COVID 19 pandemic, people with sickle cell anemia should take extra precautions, such as staying isolated at home as much as possible and for those who are eligible, getting vaccinated

Treatment

Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. Treatments might include medications and blood transfusions. For some children and teenagers, a stem cell transplant might cure the disease.

Surgical and other procedures

Blood transfusions. 

These are used to treat and prevent complications, such as stroke, in people with sickle cell disease.

In a red blood cell transfusion, red blood cells are removed from a supply of donated blood, then given through a vein to a person with sickle cell anemia. This increases the number of normal red blood cells, which helps reduce symptoms and complications.

Risks include an immune response to the donor blood, which can make it hard to find future donors; infection; and excess iron buildup in your body. Because excess iron can damage your heart, liver and other organs, you might need treatment to reduce iron levels if you undergo regular transfusions.

Stem cell transplant. 

Also known as bone marrow transplant, this procedure involves replacing bone marrow affected by sickle cell anemia with healthy bone marrow from a donor. The procedure usually uses a matched donor, such as a sibling, who doesn’t have sickle cell anemia.

Because of the risks associated with a bone marrow transplant, including death, the procedure is recommended only for people, usually children, who have significant symptoms and complications of sickle cell anemia. A stem cell transplant is the only known cure for sickle cell anemia.

Clinical trials are on-going to address stem cell transplantation in adults and gene therapies.

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