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Sickle cell disease is an inherited blood disorder. This means it is passed down through the parent’s genes. It causes a problem with the hemoglobin, the protein in red blood cells that carries oxygen to the tissues of the body.
Sickle cell disease affects hemoglobin’s ability to carry oxygen. Normal hemoglobin cells are smooth, round, and flexible, like the letter "O." They can move through the blood vessels easily. Sickle cell hemoglobin cells are stiff and sticky. They are shaped like a sickle, or the letter "C." These sickle cells cluster together and can’t easily move through the blood vessels. They can block small arteries or capillaries. This stops the movement of healthy, normal oxygen-carrying blood. It also causes the painful and damaging complications of sickle cell disease.
Normal red blood cells can live up to 120 days. Sickle cells only live for about 10 to 20 days. The spleen helps filter the blood of infections. But, the sickled cells get stuck in this filter and die. When fewer hemoglobin cells are circulating in the body, it causes anemia. The sickle cells can also damage the spleen by blocking healthy oxygen carrying cells. Without a healthy spleen, children are more at risk for serious infections.
There are several complex variations of the sickle cell gene. Some don’t cause symptoms or serious complications, others do. Talk to your doctor about the specific form of sickle cell your child has.
Sickle cell is an inherited disease caused by a genetic defect. If a child inherits one gene from the mother and one from the father, he or she will be born with sickle cell disease. A person who has only one gene is healthy and said to be a "carrier" of the disease. They may also be described as having "sickle cell trait." A carrier has an increased chance of having a child with sickle cell disease.
Once parents have had a child with sickle cell disease, there is a1 in 4 or 25% chance with each later pregnancy, for another child to be born with sickle cell disease. This means that there is a 3 out of 4, or 75% chance, that another child will not have sickle cell disease. There is a 50% chance that a child will be born with sickle cell trait, like the parents.
Sickle cell disease can cause the following symptoms:
The symptoms of sickle cell disease may look like other disorders or medical problems. Always check with your child's doctor for a diagnosis.
Most states check newborns for abnormal hemoglobin as part of routine newborn screening tests. State newborn screening includes tests for all newborns within the first few days of life. These tests identify serious, life-threatening diseases.
Sickle cell disease may be found as part of newborn screening. Your family history, your child's medical history, and physical exam are all included in the diagnosis. If the screening test shows sickle cell anemia, hemoglobin electrophoresis or other blood tests are done. These tests look at the different kinds of hemoglobin in the blood.
Your child’s healthcare provider will figure out the best treatment based on:
Early diagnosis and prevention of complications is important in sickle cell disease treatment. Your child's health care provider will refer you to a hematologist. This is an expert in blood disorders. Other specialists may also be involved in your child's care.
Treatment options may include:
Complications of sickle cell anemia include:
Advances in preventive care and new medications have reduced the life threatening complications of sickle cell. However, it is still a severe, chronic, and sometimes fatal disease. A child with sickle cell disease should be carefully managed by specialists. How a child is managed depends on the following:
Also talk with your child's health care provider about the following:
Call your child's healthcare provider or get medical care right away if your child develops:
Tips to help you get the most from a visit to your child’s healthcare provider:
Bayhealth is Southern Delaware’s healthcare leader with hospitals in Dover and in Milford. Bayhealth provides a wide range of medical services, including cardiovascular, cancer, orthopaedics and rehabilitation, pediatrics, respiratory care, sleep care, surgical weight loss and women’s services.