Sickle Cell Disease
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What is Sickle Cell Disease?
Sickle cell disease (SCD) is a genetic disorder of the blood that is passed down from parents to a child.
In children with sickle cell disease, the protein (hemoglobin) in red blood cells that carries oxygen to all parts of the body is abnormal. Instead of round, they’re shaped like the letter C (and like a “sickle” farm tool). These damaged cells clump together, blocking blood flow and potentially damaging major organs.
Symptoms of Sickle Cell Disease
Most children with sickle cell disease will start showing symptoms during the first year of life, often around five months.
Signs and symptoms can include:
- Pain, the most common symptom. When sickled cells move through small blood vessels, their shape can cause them to get stuck. This blocks blood flow and causes pain, most often in the chest, arms, and legs. Babies and young children may have painful finger and toe swelling. Blocked blood flow may also cause tissue injury.
- Anemia. Also common, this causes the child to be pale and tired.
- Yellowing of the skin, eyes, and mouth (jaundice). Sickle cells die faster than normal red blood cells, and faster than the liver and spleen can filter them out. The yellow color is caused by a substance (bilirubin) that is released when the red blood cells die.
- Acute chest syndrome. This is when sickle cells stick together and block oxygen flow in the tiny vessels in the lungs. It often occurs suddenly and can be deadly. It can look similar to pneumonia and can include fever, pain, and a violent cough.
- Pooling (splenic sequestration). The spleen becomes enlarged and painful when sickled cells get stuck and build up. Fewer red blood cells are able to move, which can cause a sudden drop in hemoglobin. If severe, this can be deadly.
The symptoms can look like other health problems, so always see your primary care provider for a diagnosis.
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UR Medicine's Treatments for Sickle Cell Disease
Golisano Children’s Hospital’s Pediatric Hematology/Oncology Division brings together skilled physicians and advanced practice nurses who are dedicated to caring both for the physical and emotional needs of children and their families.
Treatment will depend on your child’s symptoms, age, and general health. It will also depend on the severity of the condition.
Early diagnosis and prevention are important in treating this disease. Treatment may include:
- Pain medication
- Hydration, including IV fluids in some cases
- Blood transfusions to treat anemia, chronic pain, acute chest syndrome, and splenic sequestration, and to prevent stroke
- Vaccines and antibiotics to prevent infections
- Folic acid to prevent severe anemia
- Eye exams to screen for an eye condition called retinopathy
- Hydroxyurea, a medicine that can reduce the number of sickle cells in the blood
What Sets Us Apart?
Our providers form a coordinated team to provide complete care for each child, based on the type and stage of the disease. Primary team members include:
- Pediatric hematologists/oncologists
- Pediatric hematology/oncology nurse practitioners
In addition, each child's medical team includes specialists in their specific condition. This team approach creates an ideal exchange of knowledge and expertise, ensuring that the best possible treatment plan is developed for each child.
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