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

Written by the doctors and editors at UpToDate

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What is sickle cell disease? —

This is a condition that affects red blood cells, which carry oxygen to organs in the body.

Normally, red blood cells are shaped like half-filled water balloons. In sickle cell disease, a protein called hemoglobin does not form correctly. This causes some of the red blood cells to "sickle," which means they become stiff with sharp edges (figure 1).

The stiff cells can get stuck in blood vessels easily and cause blockages. When this happens, the red blood cells don't bring enough oxygen to the body's organs. This can cause pain and other medical problems.

A person who has sickle cell disease is born with it. Babies with sickle cell disease do not have any symptoms right away. This is because the red blood cells use a different type of hemoglobin for the first few months of life, then start using the sickle hemoglobin later. Symptoms might start to appear around 6 months of age.

What should I do first? —

If your child is diagnosed with sickle cell disease, it's very important they see a doctor who specializes in treating the disorder as soon as possible. The type of doctor is called a "hematologist." When you find one, talk to them about all the things you will need to do at home to care for your child. Also, make sure your child keeps seeing the hematologist as well as their regular doctor.

The hematologist will help you learn about sickle cell disease and how to take care of your child. They will talk to you about the problems that can happen in children with sickle cell disease, and what to do about them.

How can I help my child stay as healthy as possible? —

Make sure they take their medicines and get regular check-ups with their hematologist. It's also important to make sure you know how to contact the doctor or nurse in an emergency.

Steps you can take to keep your child as healthy as possible include:

●Treatments to prevent problems related to sickle cell disease – Your child's doctor will talk to you about a medicine called "hydroxyurea" (sample brand names: Droxia, Hydrea). It helps stop red blood cells from "sickling." This can prevent problems. Most children with a severe form of sickle cell disease will live a longer and healthier life, with less pain, if they take hydroxyurea. Make sure your child takes it exactly as instructed. It comes as a pill, but some pharmacies can make it into a liquid that is easier for children to swallow. Your child will need regular blood tests to make sure the dose is right.

Doctors are studying other treatments for sickle cell disease. These include bone marrow transplant, gene therapy, and new medicines. Your doctor can answer your questions. You can get information about these treatments even if you do not plan to try them.

●Vaccines and medicines to prevent infection – To protect your child from infections, they should get all the recommended childhood vaccines. They should also get some extra vaccines that are especially important for children with sickle cell disease. Most children with sickle cell disease should also take penicillin during early childhood. There is more information about this below. (See 'Preventing and treating infections' below.)

●Screening for common problems – Your doctor will recommend special tests and other ways to check for problems children with sickle cell disease can have. One example is a screening test called "transcranial Doppler." It looks at the blood vessels in the head and is used to screen for risk of stroke. There is more information about this below. (See 'The risk of stroke' below.)

Another example of screening is checking for problems with the spleen. Your doctor might have you feel your child's belly every day. This way, you will know if their spleen is getting bigger. There is more information about this below. (See 'The spleen' below.)

●When problems do happen, respond quickly – It's important to be aware of the problems that can happen in children with sickle cell disease. There is a list of the most important things to watch for below. 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 because sickle cell disease affects the immune system and can make it harder to fight certain infections. This is why it's so important for children to get all their vaccines. This includes a flu vaccine every year and the COVID vaccine as recommended by their doctor. Children with sickle cell disease also get some vaccines that children without sickle cell disease do not get.

Most children with sickle cell disease should also take penicillin (an antibiotic) twice a day, starting around 1 or 2 months of age. The doctor will tell you when your child can stop taking daily penicillin. Usually, they keep taking it until they are at least 5 years old.

In a person with sickle cell disease, fever can be a sign of a serious infection. 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 if your child gets a fever, and what temperature is high enough for you to call. See a doctor or go to the emergency department, even if the fever comes down quickly with or without medicines.

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 a lung infection. This can cause a dangerous condition called "acute chest syndrome." If your child ever has a bad cough, chest pain, wheezing, or trouble breathing, take them to the doctor or emergency department right away.

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

The spleen —

The spleen is an organ 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 2 problems with the spleen:

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

●Blood can get trapped in the spleen and not 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 show you how to check your child's belly every day, so you will know if their spleen is getting bigger.

Managing pain —

Children with sickle cell disease can have episodes of pain or swollen, painful fingers or toes. This is called "dactylitis." You can learn to recognize the signs of pain in your child.

Your child's doctor will talk to you about ways to reduce pain and treat it 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 might cry. Sometimes, 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 might complain of pain or might have trouble concentrating in school.

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 thing is to take hydroxyurea or other medicines your child's doctor prescribes. (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, and gets enough sleep. If they are dealing with stress at school or at home, try to help them cope.

When your child gets older, the doctor can work with them to create a "transition plan." This is a way for the child to begin to take a role in their own health and learn about pain care. This typically starts during the teen years, but each child is different. There are many websites and other sources of information that can help.

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 their sickle cell disease and take them to the hospital.

Symptoms of a stroke can include:

●Severe headache

●Seizure

●Not being able to wake up

●Trouble speaking

●Drooping of the face on 1 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. It does not hurt.

The doctor might also do a brain scan to see if there is any sign of a "silent" stroke. This is when a stroke happens without causing any symptoms. If tests show your child has a high risk of stroke, the doctor will suggest treatments that can help prevent a stroke, like regular blood transfusions.

Long-lasting erections —

Males with sickle cell disease can get a problem called "priapism." This 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 child gets an erection that is painful or does not go away, talk to the doctor. An erection that lasts more than 4 hours can cause long-term damage to the penis. If an erection lasts 4 hours or more, take your child to the emergency department 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 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 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.

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