Patient education: When your child has sickle cell disease (The Basics)

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What is sickle cell disease? — Sickle cell disease is a condition that affects the red blood cells, which carry oxygen to organs in the body. Normally, red blood cells are round. When people have sickle cell disease, some of their red blood cells can have an abnormal shape (figure 1). The abnormally shaped cells get stuck in the blood vessels easily, and so they don't bring enough oxygen to the body's organs. This can cause pain or organ damage.

What should I do first? — If your child is diagnosed with sickle cell disease, it's very important that they see a doctor who specializes in treating the disorder as soon as possible. Doctors who are experts in sickle cell disease are usually "hematologists." When you find a sickle cell disease expert, talk to them about all the things you will need to do at home to take care of your child. Also, make sure your child keeps seeing the sickle cell disease expert, and not just their regular doctor.

Doctors have learned a lot about sickle cell disease in recent years.

To keep your child as healthy as possible, in most cases the key will be to start a medicine called hydroxyurea. Also, it is very important to know how to spot problems early and to know what to do about them.

How can I help my child stay as healthy as possible? — You can do this by making sure they take their medicines and get regular check-ups with their sickle cell doctor. It's also important to make sure you know how to contact the doctor or nurse in case of an emergency.

You can help your child by making sure they take any medicines the doctor has prescribed, such as:

●Hydroxyurea – This medicine helps your child's body make red blood cells that work better. It comes as a pill, but there are pharmacies that can make it into a liquid that is easier for children to swallow.

For children who cannot take hydroxyurea, there are other medicines that can help the red blood cells work better.

●Penicillin – This is an antibiotic that helps prevent infections. Children with sickle cell disease should take penicillin until they are at least 5 years old

During routine visits your child will be checked for problems that can happen because of sickle cell disease. This includes blood tests. Your child will also get vaccines to help prevent infections.

Some children have more serious problems with blood flow, such as strokes or blood getting stuck in the spleen (see 'The spleen' below). For those children, blood transfusions may be needed. Blood transfusions are also given before surgery.

It's important to be aware of the problems that can happen in children with sickle cell disease. Below is a list of the most important things to watch for. These things are also listed in the table (table 1).

Preventing and treating infections — Any infection in a child with sickle cell disease can be serious. This is why it's so important for children to get all of their vaccines. This will include a flu shot every year as well as some vaccines that children without sickle cell disease do not get. To further help prevent infection, your child should take penicillin (an antibiotic) twice a day starting soon after birth. The doctor will tell you when your child can stop taking daily penicillin.

You should bring your child to a doctor right away if they ever get a fever. Talk to your doctor about a plan so you know what to do when your child gets a fever, and what temperature is high enough for you to call. You should see a doctor or go to the emergency room even if the fever comes down quickly with or without medicines to bring down fever.

You should also call the doctor if your child shows other signs of infection, even without a fever. This includes feeling very tired, loss of appetite, stiff neck, headache, trouble breathing, or coughing.

Coughing and breathing problems — Coughing and trouble breathing can be signs of infection in the lungs. But they can also be signs of a dangerous condition called "acute chest syndrome." This condition sometimes happens in children with sickle cell disease. If your child ever has a bad cough or any wheezing or trouble breathing, take them to the doctor or emergency room right away.

Asthma is also common in children with sickle cell disease. If your child has asthma, it's important to make sure they see their doctor regularly and follow their treatment plan.

The spleen — The spleen is an organ in the body that filters blood and helps fight infections. It is in the upper left part of the belly (figure 2). People with sickle cell disease can have two problems with the spleen:

●Sickled red blood cells can damage the spleen. After the first few months of life, the spleen no longer works well to help fight certain infections. This is why people with sickle cell disease need to take extra care to prevent infections and to treat infections right away. (See 'Preventing and treating infections' above.)

●Blood can get trapped in the spleen and not be able to move to the rest of the body. When this happens, the spleen can get very large. If your child gets dizzy or passes out, call the doctor right away or go to the emergency department. There, doctors can check to see if a spleen problem is the cause.

Your child's doctor might also tell you to check your child's belly every day so you will know if the spleen is getting bigger.

Managing your child's pain — Children with sickle cell disease can have episodes of pain or swollen, painful fingers or toes (called "dactylitis"). You can learn to spot the signs of pain in your child. Your child's doctor will talk to you about ways to reduce pain and how to treat pain at home. They might prescribe pain medicines. The doctor will also tell you what to do if the medicines aren't helping enough, including when to call for advice or get medical care. Some areas have special hospitals or clinics just for treating pain related to sickle cell disease. If available, these places are often better able to treat pain than the emergency department.

Pain can look different in children of different ages:

●A baby who is in pain will not always cry. Instead, they might be less active or just lie still.

●A school-aged child who is in pain might lose interest in their usual activities or try to ignore the pain. If the pain is not treated early, it can become hard to control. Teach your child to always tell you or another adult if they are having pain.

●An older child may complain of pain or may have trouble concentrating in school because of pain.

There are also some things you can do to help prevent pain episodes, or make the episodes a little easier for your child to handle. The main one is to take hydroxyurea or other medicines your child's doctor has prescribed. (See 'How can I help my child stay as healthy as possible?' above.)

Other things that might help include making sure your child drinks enough fluids, eats a healthy diet, avoids very cold temperatures, takes their medicines, and gets enough sleep. If your child is dealing with stress at school or at home, it's also important to try to address this and help them handle the stress.

The risk of stroke — Children with sickle cell disease are at risk of stroke. If your child ever has symptoms of a stroke, call the doctor who treats your child's sickle cell disease and take your child to the hospital. The symptoms of a stroke can include:

●Severe headache

●Seizure

●Not being able to wake up

●Trouble speaking

●Drooping of the face on one side

●Weakness in an arm or leg

Your child's doctor will do regular ultrasound tests called "transcranial Doppler" to find out your child's risk of getting a stroke. This test is usually done every year starting when a child is 2 years old. If the test shows that your child has a high risk of stroke, your doctor will suggest additional tests and/or treatments that can help prevent a stroke.

Long-lasting erections in boys — Boys with sickle cell disease can get a problem called "priapism." Priapism is an erection that happens when blood gets trapped in the penis. It can be very painful and is not related to having sex or wanting to have sex. If your son gets an erection that is painful or does not go away, talk to his doctor. An erection that lasts more than 4 hours can cause long-term damage to the penis. If your son gets an erection that lasts 4 hours or more, take him to an emergency room right away.

If I have another child, will they have sickle cell disease too? — If you are planning to have more children, there is a risk that your other children could also have sickle cell disease. Your doctor or a genetic counselor can talk to you about these risks.

There is also a chance that your child could pass sickle cell disease on to their future children. When your child gets older and starts thinking about planning a family, they should talk to a doctor or genetic counselor about the risks.