Infants and Children With Sickle Cell Disease

Sickle cell disease (SCD) is a lifelong inherited condition that affects red blood cells. Normally, red blood cells are round and flexible, so they can pass easily through blood vessels.¹

When a child inherits SCD, their body makes C-shaped, or sickle-shaped, red blood cells that are stiff. These misshapen red blood cells can cause many complications and even death when they get stuck in blood vessels. However, early diagnosis and treatment can help prevent infections and other health problems in children with SCD.¹

How does a child get sickle cell disease?

Sickle cell disease occurs when a baby inherits 2 sickle cell genes – 1 from each parent. People who inherit only 1 gene are said to be carriers of sickle cell trait. People with sickle cell trait often have no symptoms.^2,3

However, if 2 people with sickle cell trait have a baby, there is a 1 in 4 chance (25 percent) that baby will have full-blown sickle cell disease. There is a 1 in 2 chance (50 percent) the baby will carry sickle cell trait like their parents.^2,3

How common is sickle cell disease?

In the United States, SCD is most common in children whose parents are Black or of African or Caribbean descent. It also occurs in children whose parents are Hispanic, Middle Eastern, Asian Indian, or southern European. In the United States:^1,4

• About 1 in 13 Black babies are born with sickle cell trait (have 1 sickle cell gene).
• About 1 in 365 Black babies are born with sickle cell disease (have 2 sickle cell genes).
• About 100,000 adults and children are living with sickle cell disease.

Diagnosing sickle cell disease

If you know that sickle cell runs in your family, you may want to get a blood test to see if you are a carrier. Genetic testing may help diagnose what type of SCD you have. It can also help confirm a sickle cell diagnosis if the blood test is not clear.⁵

Prenatal testing can be done as early as 8 to 10 weeks into pregnancy. However, this test cannot predict the severity of disease.⁵

After birth, newborn screening takes a drop of blood from a prick on the baby's foot. This test will tell you if your baby has sickle cell trait or sickle cell disease. Most states require babies to be tested for sickle cell at birth.⁵

What are the first symptoms?

Symptoms and complications vary, and they can be mild to severe. The first symptoms of SCD usually include:²

• Low red blood cell count (anemia)
• Yellowing of the skin, eyes, and mouth (jaundice)
• Severe pain (sickle cell crisis)
• Acute chest syndrome
• Enlarged spleen (splenic sequestration or pooling)

Since blood moves through every part of the body, complications from sickle cell in older children can appear anywhere. Long-term anemia may lead to delayed growth and development. Severe pain may require hospital stays for treatment. Other possible complications include:²

• Lung damage from acute chest syndrome
• Scarred spleen
• Higher risk of infections
• Stroke
• Blocked blood vessels in the penis (priapism), which can lead to impotence

How is the disease treated in children?

Treatments have improved the life expectancy for children with SCD in recent years, but it is still a severe disease. Treatment depends on your child's symptoms and how severe those are. Some treatments include:²

 
It is important to aggressively prevent and treat infections in children with SCD. Infections are the most common cause of death in children younger than age 5.²

By age 8, many children with sickle cell will have had their spleen removed. For kids under 5 who have had their spleens removed, daily antibiotics to prevent infection are recommended for 1 year following surgery.^2,6

A stem cell transplant is another surgery that can cure sickle cell in some children. It is the only cure for SCD.²

How can I support children living with SCD?

It is impossible to prevent children from having complications of SCD. However, you can help them live as healthy a life as possible. Ways that parents, teachers, and caretakers can reduce complications for children with sickle cell include:²

• Ensure they eat a healthy diet, get enough sleep, and drink plenty of fluids
• Keep them away from high altitudes, cold weather, and swimming in cold water
• Help them avoid people who are sick
• Make sure they wash their hands often
• Get regular checkups and all recommended vaccines

Talk to your child’s healthcare team regularly about any concerns or symptoms. They can give you advice on healthy activities for your child and ways to support them.