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, or sickle.
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
Pediatric sickle cell complications
Sickle-shaped red blood cells stick together and block blood vessels, which can cause complications like:
- Acute chest syndrome: Sudden damage to lung tissue is common in children with sickle cell disease.
- 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 disease has more bile (a liquid used to break down the fats we eat) than his gallbladder can hold. The extra bile causes gallstones. Gallstones can cause jaundice, which is when the skin or eyes are yellow in color.
- Jaundice: Fragile sickle cells can break open, leaking bilirubin (a yellow pigment in bile) into the bloodstream and causing the skin and eyes to turn yellow.
- Nephropathy: Sickle red blood cells can get trapped inside the blood vessels of the kidneys and cause kidney damage.
- Pain: Sickle red blood cells can 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 disease 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, causing a stroke. Strokes occur more frequently in children ages 2 to 5, but can occur at any age.