Infants and Children with Sickle Cell Disease

Sickle cell disease (SCD) is present at birth. It happens when a child inherits 2 genes for abnormal hemoglobin (1 from each parent). Every newborn in the United States is screened for SCD. Early diagnosis and treatment help prevent infections and other health problems in children with SCD.

Most children with SCD will start showing symptoms at around 5 months old. The type and severity of symptoms can vary. Treatment usually depends on their symptoms and may be done by a doctor with expertise in blood disorders. There are also ways to reduce the burden of the disease for your child.

How are newborns diagnosed with sickle cell disease?

Early diagnosis is important because it prevents complications, especially during early childhood. Every state in the United States, the District of Columbia, and all U.S. territories require that every newborn be tested for SCD.¹

The test is done by taking a blood sample from a baby’s finger or heel. The sample is then screened in a lab for abnormal hemoglobin. If a baby is diagnosed with SCD, doctors make sure the parents know the results. Parents and doctors can then take steps to prevent and treat complications of SCD.¹

What are the first symptoms?

Most newborns do not show symptoms until they are about 5 or 6 months old. This is because newborns have a different type of normal hemoglobin than children and adults. This type of hemoglobin is called “fetal hemoglobin” (HbF).²

HbF prevents red blood cells from becoming rigid and sickle-shaped. At birth, a newborn’s blood is made up of about 70 percent HbF. This percentage becomes lower as new red blood cells form and replace HbF with the adult form of normal hemoglobin, hemoglobin A. By 6 months old, less than 2 percent of total hemoglobin is HbF, and symptoms begin to arise.³

Each child’s symptoms vary, and can be mild or severe. The first symptoms of SCD usually include:¹

• Yellowing of the skin, eyes, and mouth (jaundice)
• Fatigue
• Painful swelling of hands and feet (dactylitis)

What are other complications of sickle cell disease for children?

Children can also experience complications of SCD, including:^4,5

• Acute chest syndrome
• Infections
• Long-term anemia
• Pain crisis (also called sickle crisis)
• Splenic sequestration (enlarged spleen)
• Stroke

These complications can be life-threatening for children with SCD. Call your child’s doctor right away if your child has:⁶

• Fever
• Sudden pain in the chest, belly, arms, or legs
• Sudden vision loss
• Symptoms of an enlarged spleen
• Symptoms of severe anemia, including fatigue and difficulty waking up
• Trouble breathing

How is the disease treated in children?

Improvements in prevention and treatment have reduced the life-threatening problems of SCD in children. Some preventions include:^4,6

• Drinking plenty of water to prevent pain crises
• Examination of the spleen for enlargement
• Eye exams to screen for vision loss
• Folic acid to prevent severe anemia
• Routine doctor's visits with lab checks to prevent stroke
• Vaccines and antibiotics to prevent infections

Children may also need treatment for complications, including:⁶

• Blood transfusions to treat anemia, chronic pain, acute chest syndrome, or enlarged spleen
• Hydroxyurea to reduce complications and painful episodes
• Pain medicines to treat pain crises

The only cure for SCD is a bone marrow transplant. This is only done for children with severe SCD who have minimal organ damage. They also need a donor who is a relative and a genetic match.⁷

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 healthy lives and reduce some burdens. They should be vaccinated and screened for vision loss, stroke, and spleen enlargement according to your doctor’s guidance. Other ways parents, teachers, and caretakers can reduce complications for children include:⁶

• Ensuring they eat a healthy diet, get enough sleep, and drink plenty of fluids
• Keeping them away from high altitudes, cold weather, and swimming in cold water
• Avoiding people who are sick
• Making sure they wash their hands often

Talk to your child’s doctor regularly about any concerns or symptoms. They can also give you specific advice on which activities your child should do and ways to support them.