Understanding sickle cell disease: how it is inherited, what to watch for, and how to live well.
Sickle cell disease (SCD) is a group of inherited red blood cell disorders. Healthy red blood cells are round and move easily through small blood vessels to carry oxygen to every part of the body. In someone with SCD, the hemoglobin is abnormal, causing red blood cells to become hard, sticky, and shaped like a C-shaped farm tool called a "sickle."
Sickle cells die early, which causes a constant shortage of red blood cells (anemia). They can also get stuck in small blood vessels and block blood flow, causing pain and other serious problems such as infection, acute chest syndrome, and stroke.
Sickle cell anemia (HbSS) is the most common and usually most severe form of SCD.
SCD is not contagious. A child is born with it when they inherit two sickle cell genes, one from each parent.
A person who inherits one sickle cell gene and one normal gene has sickle cell trait. Most people with SCT do not have symptoms, but they can pass the gene to their children. Know your status: a simple blood test can tell you.
With each pregnancy there is a 1 in 4 (25%) chance the child will have SCD, a 1 in 2 (50%) chance the child will have the trait, and a 1 in 4 chance the child will have neither.
All states, including Louisiana, test newborns for SCD. Early diagnosis lets families begin preventive care, such as penicillin and vaccines, right away.
Call a doctor or go to the emergency room right away for:
Drink plenty of water every day. Dehydration can trigger a pain crisis.
Avoid getting too hot or too cold, and avoid high altitudes and very strenuous activity without rest.
Wash hands often, keep vaccinations up to date (including flu and pneumonia), and take medicines as prescribed.
See a doctor who knows SCD regularly. Children need yearly transcranial Doppler screenings to check stroke risk.
Treatments include hydroxyurea, L-glutamine, blood transfusions, and, for some people, a bone marrow transplant or newer gene therapies. Ask your care team what is right for you.
Living with a chronic illness is hard. Lean on family, faith, and community support groups. Our church is here to pray with you.
The information on this page was summarized from the following sources. Visit them for complete, up-to-date details.