We are home to the country’s largest pediatric sickle cell disease program, caring for more than 1,800 children and young adults.
What is sickle cell disease?
Sickle cell disease is a genetic blood disorder that affects hemoglobin in red blood cells. Hemoglobin is a protein that carries oxygen to all parts of the body. Normal red blood cells are round and smooth. In a child with sickle cell disease, red blood cells are hard, sticky and shaped like a crescent.
The three most common types of sickle cell disease include:
- Hemoglobin SS disease (also called sickle cell anemia)
- Hemoglobin SC disease
- Sickle cell beta thalassemia
Sicke cell complications
Sickled red blood cells stick together and block blood vessels, which case cause complications such as:
- Acute chest syndrome: Sudden damage to lung tissue is common in children with sickle cell.
- Aplastic crisis: The body stops making red blood cells for a short time, causing a drop in your child’s red blood count.
- Fever and infection: a fever is usually a sign of infection. Infections are a medical emergency for a child with sickle cell disease.
- Gallstones: A child with sickle cell has more bile (a liquid that is used to break down the fats we eat) than his gallbladder can hold. The extra bile causes gallstones. Gallstones can cause a yellow color of the skin or eyes (jaundice).
- Jaundice: When fragile sickle cells break open, bilirubin (a yellow pigment in bile) leaks into the blood stream causing the skin eyes to turn yellow (jaundice).
- Nephropathy: When sickle red blood cells get trapped inside the blood vessels of the kidneys, causing kidney damage.
- Pain: Sickle red blood cells get stuck and block the flow of blood to parts of the body, such as the bones, lungs, spleen, brain, eyes and kidneys, causing pain.
- Retinopathy: Sickle cell can damage the retina in the eyes.
- Splenic sequestration crisis: The spleen suddenly gets swollen because sickle red blood cells block the blood vessels inside the spleen. This can lead to shock and is an emergency for children with sickle cell disease.
- Stroke: A blockage or bleeding of the blood vessels can cause a loss of blood supply to part of the brain (a stroke). Strokes occur more often in children ages 2 to 5, but can occur at any age.