Sickle Cell Disease Program

Some symptoms that may suggest sickle cell disease include:

Complications
Sickle cell disease is a condition that affects each child differently. It can affect nearly every major organ of the body, putting children at risk for developing a wide variety of complications.

To promote the well-being of children with sickle cell disease, doctors in the hematology department work closely with patients, parents and other specialists at Children's Hospital Boston to quickly identify and treat these conditions. Some of the physicians and nurses who may also treat your child at Children's may specialize in cardiology, pulmonary and gastrointestinal disorders.

A short list of associated sickle cell disease complications treated with the help of specialists outside of the hematology department is listed below.

Acute Chest Syndrome (ACS) - Caused by sickled cells blocking the flow of oxygen in the lungs as a result of stress from infection, fever or dehydration. ACS resembles pneumonia and can include fever, pain, and cough or difficulty breathing. Multiple episodes of acute chest syndrome can cause permanent lung damage. Sickle cell patients who also suffer from asthma are at particularly high risk of ACS and need to be followed closely by pulmonary specialists.

Splenic Sequestration (pooling) - Caused by sickle cells pooling in the spleen, where this results in an enlarged and tender spleen and a sudden, severe life-threatening anemia. Chronic poor perfusion of the spleen results in most patients having a scarred, permanently non-functioning spleen. Splenic sequestration may necessitate surgical removal after age 8. The loss of spleen function increases the risk of overwhelming bacterial infection, which is the major cause of death in children under the age of 5 years in this population.

Stroke - A sudden, severe complication of children with sickle cell disease caused by blockage of blood flow and oxygen delivery to the brain by sickle cells. Any interruption in the flow of blood and oxygen to the brain can result in devastating neurological impairment. Up to 1 percent of sickle cell disease children may have a stroke per year. Having had one stroke, a child is much more likely to have repeated strokes and thus must start chronic red blood cell transfusion therapy.