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Together is a new resource for anyone affected by pediatric cancer - patients and their parents, family members, and friends.
Learn MoreSickle cell disease is a serious medical condition that affects the red blood cells. Normal red blood cells are round and flexible. People with sickle cell disease have red blood cells that are hard, sticky, and shaped like a banana.
People with sickle cell disease are born with it. It is the most common inherited blood disorder in the United States.
Sickle cells clump together and clog blood vessels. This can cause problems throughout the body. Children with sickle cell disease can face many health problems. They need to see doctors throughout their lifetime.
Sickle cell disease can be treated. In some cases, it can be cured with a stem cell transplant.
Symptoms and complications of sickle cell disease vary from patient to patient. They range from mild to life-threatening.
Sickle-cell related health problems may begin when your baby is about 6 months old. As your child grows older, different health problems related to sickle cell disease can happen.
Here are some common complications and symptoms your child may have as they age:
Your child’s care team can help you treat and manage these issues.
Sickle cell disease is a group of blood disorders that affect hemoglobin. Hemoglobin is the protein in red blood cells that carries oxygen through the body.
Sickle cell disease is passed through families. It is inherited from your parents like hair color or eye color. Each child has 2 hemoglobin genes — 1 from their mother and 1 from their father.
To be born with sickle cell disease, children must inherit 2 sickle cell genes – 1 from their mother and 1 from their father. A child must inherit a sickle cell gene from 1 parent and another abnormal hemoglobin gene from the other parent.
Healthy red blood cells have hemoglobin A. Hemoglobin A helps keep the red blood cells soft and round so they flow easily through small blood vessels.
People with sickle cell disease have red blood cells that contain hemoglobin S. Hemoglobin S causes blood cells to become sticky and banana shaped. The cells block tiny blood vessels and slow the delivery of blood and oxygen to the body’s organs.
Sickle cells die faster than normal red blood cells. Fewer red blood cells are available to deliver oxygen. Lack of oxygen can lead to many health problems such as pain crises, enlarged spleen, and blood infections.
Sickle cell disease is more common in people who have ancestors from:
All newborn babies in the United States get a blood test to see if they might have sickle cell disease. This test is done in the hospital within 1 to 2 days of birth – before the child would show any symptoms.
A health care provider pricks the baby’s heel to get a few drops of blood. This blood sample is sent to the state’s newborn screening lab. A positive result means that the screening test detected hemoglobin S.
How parents find out about their baby’s screening test results varies from state to state. In general, the lab sends results to the baby’s pediatrician or a sickle cell disease treatment center. In some states, both receive the results.
If the baby does not have a pediatrician, the state department of health may get the result. The health care provider shares the results with the parents. Some providers share the news by letter. Others share it by phone. A phone call is a good time to address worries the parents may have.
A pediatrician can refer the family to a sickle cell center. The health care provider also schedules an in-person follow-up appointment at a sickle cell clinic.
If your baby is more than 2 weeks old and you have not gotten the newborn screening test results, call your child’s pediatrician, clinic, or hospital. Keep in mind that some states only report results when a baby has sickle cell disease.
It is also important to know if your child has sickle cell trait. In general, sickle cell trait does not cause health problems in children or adults. But in rare cases it can.
If your baby has sickle cell disease, care will begin soon after diagnosis.
At your baby’s first appointment, you will meet the hematology team. A health care provider may take another blood sample and order a different test to confirm the screening test results, depending on where you live. If the baby has sickle cell disease, the test will show what type it is.
The test will also show if the baby has sickle cell trait.
Your baby will also start treatment with penicillin. Taking penicillin twice a day helps prevent deadly infections in babies and young children with sickle cell disease.
Treatment should start before babies are 2 months old and continue until they are 5 years old. Some children will need to take it longer.
Several medicines are now available for children and adults with sickle cell disease. Remember that all medicines have risks as well as benefits. Your child’s care team can guide you.
Hydroxyurea helps red blood cells stay round and flexible. They can travel more easily through the tiny blood vessels.
Many children with sickle cell disease who take hydroxyurea have fewer:
Hydroxyurea might also prevent or slow down damage to the spleen, kidneys, lungs, and brain.
Hydroxyurea is for children 2 and older.
L-glutamine works by increasing the amount of glutamine in the blood. This may help reduce the damage that occurs in red blood cells. It is usually used with other medicines to reduce the chance of complications.
L-glutamine is approved to treat patients 5 and older.
Voxelotor helps hemoglobin hold onto more oxygen and may prevent red blood cells from sickling.
This medicine can be given to children 4 and older.
This medicine works by making blood cells less sticky. Sickled red blood cells flow more freely through the body. Crizanlizumab is given once a month through an IV.
It is approved to help decrease the number of pain episodes in people with sickle cell disease who are 16 or older.
Currently a stem cell transplant is the only known cure for sickle cell disease.
Most people with sickle cell disease are not good candidates for this treatment. A stem cell transplant can also cause serious health problems such as the loss of the ability to have children.
Doctors only use this treatment in children with serious health problems. Researchers are working to overcome these problems so that more people can be cured.
A transplant is a major medical procedure. Children who have a transplant must take strong chemotherapy drugs to destroy the bone marrow. They must spend several weeks in the hospital. It can take up to a year to know if the transplant worked.
No single treatment is right for every child. Regular appointments with a doctor who specializes in treating children with sickle cell disease can help.
Regular health and wellness checkups from a pediatrician are also an important part of care for children with sickle cell disease.
Sickle cell disease was once thought of as a childhood disease. But now most children live well into adulthood, thanks to early detection through newborn screening.
Keep in mind that children with sickle cell disease are not sick all the time. In fact, most of the time they can play and go to school like other kids.
When they can grow up, they can go to college, have careers, and lead a mostly normal life. For the best chance for a bright future, your child will need ongoing medical care.
Your child’s doctor is the best source of information on their case.
Talk to your health care provider if you have questions about sickle cell disease, your child’s treatment, or their prognosis. Always follow the instructions given by your care team.
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Reviewed: January 2023