Sickle cell disease


What is sickle cell disease?

Sickle cell disease is a chronic, inherited blood disorder in which red blood cells become crescent shaped, making it difficult for them to move through the bloodstream. Many times, these cells break down easily or the bone marrow is unable to produce enough new cells, which causes anemia. The disease affects 8 of every 100,000 people, but is more prevalent in African-Americans and Hispanic-Americans. Patients with sickle cell disease may experience recurring pain, damage to internal organs, severe infections, and in rare cases, life-threatening strokes.

Bone marrow transplantation in children with sickle cell disease

Bone marrow transplantation (BMT) is currently the only known cure for sickle cell disease. It involves replacing the abnormal stem cells residing in bone marrow with healthy cells from an eligible brother or sister. This procedure is also sometimes called a stem cell transplant. Learn more.

Acute chest syndrome in children with sickle cell disease

Caused by infections and/or a blockage of blood flow to the chest and lungs, acute chest syndrome (ACS) is a complication in sickle cell patients that can result in lung injury, breathing difficulty, low oxygen to the rest of the body and possibly also death. ACS is one of the most common causes of hospitalization for children with sickle cell disease and is the root cause for more than 25% of premature deaths in sickle cell disease. Learn more.

Brain complications in children with sickle cell disease

Children with sickle cell disease are at risk for brain damage because their irregularly shaped sickle cells can interrupt blood flow to the brain. Complete clogging of blood flow to the brain can lead to an “obvious” stroke. This occurs in about 10% of children with sickle cell disease before the age of 14 with the highest period of risk between 3 and 7 years. In addition, damaging strokes that do not cause obvious symptoms but can be seen on MRI images of the brain occur in at least 25% of school-age children with sickle cell disease. These so-called “silent strokes” may result in learning and/or behavior problems. Learn more.

Splenic sequestration in children with sickle cell disease

Parents should check their child’s spleen every day , particularly when the child is sick. They should know how their child’s spleen usually feels so that whenever the child seems sick they can check the spleen to see if it’s bigger. If the spleen suddenly becomes larger, a doctor should check the child as soon as possible. If the child also has symptoms of a low blood count, then the situation becomes an emergency. Learn more.

Sickle Cell Handbook

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