If you’ve learned that your baby or toddler has sickle cell disease, you may have many questions, both you and other family and friends.
Currently, most cases of sickle cell disease in the U.S. are diagnosed by newborn screening. It is important to make the diagnosis early, so that babies can be started with penicillin (or another antibiotic) to prevent infection. Connecting early with a primary care pediatrician and blood disorder specialists who can work closely with the child as they grow up and with their families can help prevent complications from the disease.
Hemoglobin is the part of red blood cells that carries oxygen. In sickle cell disease, hemoglobin can change the rounded shape of C-shaped red blood cells that is crooked, like the tool called a sickle. When this happens, the cells get stuck and can clog small blood vessels. It also makes red blood cells more fragile and susceptible to breakage, causing anemia.
Millions of people worldwide have sickle cell disease or SCD. In the United States, the Centers for Disease Control and Prevention (CDC) estimates it affects about 100,000 people. It occurs in approximately one in 365 black or African American births and one in 16,300 Hispanic births.
Is SCD a genetic disease?
Yes. We all have two genes that decide what type of hemoglobin we have, one from each parent. People with sickle cell disease have two copies of the sickle gene, inherited from both parents. If someone has a copy of it, they have “sickle cell traits,” that is, they are carriers of the gene.
Sickle cell shooting is common and affects one in 13 black children. Although there are some problems associated with sickle cell disease, people with sickle cell disease are usually healthy; in fact, they are believed to be less likely to develop severe cases of malaria, which is why the disease was thought to have evolved in people from areas of the equator where malaria is common.
There are other types of hemoglobin genetic diseases, such as thalassemia or hemoglobin C. Sometimes people are born with a sickle gene and a gene for a problem other than hemoglobin. The severity of your condition depends on the particular combination of hemoglobin.
There is currently no cure for sickle cell disease or other genetic diseases of hemoglobin, except for bone marrow or stem cell transplantation.
What health problems can SCD cause?
When sickle cells clog blood vessels, they can cause pain and damage organs or tissues due to lack of blood flow. Combined with the effects of anemia, this means that babies and children with sickle cell disease are more likely to have complications from infections, such as pneumonia; vision problems; blood clots; and respiratory problems. Children with sickle cell disease may also experience swelling of the spleen if sickle cell cells become attached to it.
How can you help your child live a healthy life as he or she grows up?
Children with sickle cell disease and their families can work together to help prevent complications by doing these steps:
- Stay well hydrated, as dehydration can cause cells to have more sickle.
- Avoid extreme temperatures, especially cold ones, as they can also cause cell sickle.
- Avoid high altitude areas, where there is less oxygen.
- Be careful with very vigorous exercise, which can decrease the amount of oxygen available in the blood.
- Wash your hands regularly and avoid sick people as much as possible.
- Get all recommended vaccines.
- Know all the signs and symptoms of blood vessel blockage, anemia, swollen spleen, infection, blood clots, and lung problems, so they know when to attend immediately. Ask your child’s doctor or medical team to help you understand the key warning signs for each of them according to your child’s age.
Also, find it useful tool set created by the CDC and the American Society of Hematology. Explain the usual complications of sickle cell disease and the steps to follow to improve health.
Being closely connected to medical care is crucial, as the sooner complications are diagnosed, the more successfully they are treated. There are also treatments like hydroxyurea that can help prevent complications in the first place. Children with sickle cell disease should see their primary care pediatrician and specialist regularly, whether they have symptoms or not, so that they can work together to help live the healthiest life possible.
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