Sickle cell disease is a genetic blood disorder in which a protein within red blood cells called hemoglobin is irregularly shaped. Healthy red blood cells exist in the shape of an O, while cells affected by sickle cell disease resemble the shape of a sickle, or a C. This not only affects the ability of hemoglobin to transport oxygen throughout your child’s body, it can also create blockages within blood vessels, as sickle cells are stiff and sticky.
The sickle cell clinic at the Dell Children’s Blood and Cancer Center provides the most advanced treatment and long-term care methods available, and our specialized team is dedicated to providing the education and resources that children affected by sickle cell disease and their parents need throughout each step of the treatment process.
Wellness and Prevention
Sickle cell disease is an inherited condition that typically affects children of African, Hispanic or Caribbean decent, although its trait can also be found in those of Middle Eastern, Latin American and Mediterranean heritage.
There are varying degrees of sickle cell disease, and your child’s specific type can play a role in determine the best treatment and management strategies. Each type, however, may present with similar symptoms and complications:
- Anemia, which is the most common symptom associated with sickle cell disease. This occurs when there are not enough red blood cells in your child’s blood to function properly.
- Pain crisis, also called vaso-occlusive crisis, occurs when there is a blockage in your child’s blood vessels due to clusters of sickle cells. The resulting pain or swelling can occur anywhere in your child’s body, depending on where the blocked vessel is located.
- Acute chest syndrome occurs when the blood vessels that supply oxygen to the lungs become blocked, and symptoms can resemble those of pneumonia.
- Enlarged spleen can occur when blood pools in the spleen.
- Jaundice, which is the yellowing of your child’s skin, eyes or mucous membranes, happens when unhealthy sickle cells die so quickly that bilirubin (a yellow compound in blood) builds up.
Early detection is an important step in caring for children with sickle cell disease, as this can help to prevent serious complications that can surface. To accurately diagnose or rule out the possibility of sickle cell disease in your child, doctors will perform a serious of tests, including:
- Physical exam to check for any physical symptoms such as pale skin due to anemia, pain, swelling or infection
- Newborn screening tests are currently in place in all 50 states in the U.S. to check for hemoglobin disorders in infants
- Hemoglobin electrophoresis can determine whether your child is a carrier of the sickle cell trait or if he or she has the disease
- Blood tests to measure hemoglobin levels, red blood cell count and to check for the presence of sickle cells
Treatment for children and teens with sickle cell disease is centered on prevention strategies to avoid serious complications that stem from the disease. The type and severity of your child’s sickle cell disease can also play a role in determining the most effective and appropriate treatment plan.
Hematopoietic Stem Cell Transplantation
Currently, hematopoietic stem cell transplantation is the only known cure for sickle cell disease. During a stem cell transplant, your child will first receive radiation therapy or chemotherapy to reduce or destroy the sickle cells in his or her body. Then, healthy cells taken from a donor, usually from a sibling or close relative, are injected into your child’s bloodstream to replace unhealthy cells.
Hydroxyurea is an oral medication that can reduce or prevent the complications associated with sickle cell disease by increasing the amount of fetal hemoglobin that your child’s body can produce. For children with severe symptoms and frequent pain crises, doctors may suggest taking hydroxyurea daily.
Blood transfusions can also help to prevent complications and manage symptoms of sickle cell disease, and your doctor may recommend either acute or chronic transfusions, depending on your child’s individual needs.
Children with sickle cell disease require routine monitoring and ongoing preventative care to help manage both acute and chronic symptoms. Working with a pediatric hematologist who understands your child’s medical and history allows for effective long-term treatment and a smooth transition into adolescent and adult care.