If you have learned that your newborn or toddler has sickle cell anemia, you – and other family members and friends – may have many questions.
Today, most cases of sickle cell disease in the United States are diagnosed by newborn screening. It’s important to make the diagnosis early, so babies can start taking penicillin (or another antibiotic) to prevent infection. Connecting early with a pediatrician for primary care – and blood disorder specialists who can work closely with the child as they grow older and with their family – can help prevent complications from the disease.
Hemoglobin is the part of the red blood cell that carries oxygen. In sickle cell anemia, hemoglobin can change the rounded shape of red blood cells to a C shape that is twisted, like the tool called a sickle. When this happens, the cells become sticky and can clog small blood vessels. It also makes the red blood cells more fragile and susceptible to breakage, causing anemia.
Millions of people around the world suffer from sickle cell anemia, or SCD. In the United States, the Centers for Disease Control and Prevention (CDC) estimates that it affects around 100,000 people. It occurs in about one of 365 Black or African American births and one in 16,300 Hispanic American births.
Is SCD a genetic disease?
Yes. We all have two genes that determine the type of hemoglobin we have, one from each parent. People with sickle cell disease have two copies of the sickle cell gene that are inherited from both parents. If someone has a copy, they have a ‘sickle cell trait’, which means they carry the gene.
The sickle cell trait is common, affecting one in 13 black children. Although there are some problems associated with the sickle cell trait, people with the sickle cell trait are generally healthy; in fact, they are thought to be less likely to develop severe cases of malaria, which is why the disease is thought to have evolved in people from areas around the equator where malaria is common.
There are other types of genetic hemoglobin diseases, such as thalassemia or hemoglobin C. Sometimes people are born with a sickle gene and a gene for a different hemoglobin problem. The severity of their condition depends on the particular combination of hemoglobin.
Currently, there is no cure for sickle cell anemia or other genetic hemoglobin diseases except bone marrow transplant or stem cell transplant.
What health problems could CDS cause?
When sickle cell disease clogs blood vessels, it can lead to pain and damage to organs or tissues due to lack of blood flow. When combined with the effects of anemia, it means babies and children with sickle cell disease are more likely to have complications from infections, such as pneumonia; eyesight problems; blood clots; and respiratory problems. Children with sickle cell disease may also have swelling of the spleen if the sickle cells remain there.
How can you help your child to lead healthy lives as they grow older?
Children with sickle cell disease and their families can work together to help prevent complications by taking the following steps:
- Stay well hydrated, as dehydration can make cells more susceptible to sickle disease.
- Avoid extreme temperatures, especially cold, as this can also trigger cell sickles.
- Avoid areas of high altitude, 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 if possible.
- Get all recommended vaccinations.
- Learn all the signs and symptoms of blocked blood vessels, anemia, swollen spleen, infection, blood clots, and lung problems, so they know when to seek immediate medical attention. Ask your child’s doctor or medical team to help you understand the main warning signs of each of these signs based on your child’s age.
Also check out this helpful toolkit created by the CDC and the American Society of Hematology. It explains the common complications of sickle cell disease and what you can do for better health.
Being closely linked to medical care is crucial, because the earlier complications are diagnosed, the earlier and successfully they are treated. There are also treatments such as 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 are showing symptoms or not, so they can work together to help live the healthiest life possible.
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