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 red blood cells, which carry oxygen to organs in the body.

Normally, red blood cells are disc-shaped, sort of like a half-filled water balloon. In sickle cell disease, some of the red blood cells become stiff and can have sharp edges (figure 1). These cells get stuck in blood vessels easily and can cause blockages. 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. The type of doctor is called a "hematologist." When you find a hematologist, talk to them about all of the things that you will need to do at home to take care of your child. Also, make sure that your child keeps seeing the hematologist as well as their regular doctor.

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

Steps that 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. This medicine helps protect red blood cells from getting damaged. Most children with a severe form of sickle cell disease will live a longer and healthier life, with less pain, if they take hydroxyurea. If your child is prescribed hydroxyurea, make sure that they take it as instructed. It comes as a pill, but there are pharmacies that can make it into a liquid that is easier for children to swallow.

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 about these treatments. You can get information about these treatments even if you do not plan to use them.

●Vaccines and medicines to prevent infection – To protect your child from infections, they should get all of 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 that children with sickle cell disease can have. One example is a screening test called "transcranial Doppler." This test 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 the 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 of their vaccines. This includes a flu shot every year, as well as some vaccines that children without sickle cell disease do not get. Getting the vaccines as a child, when the immune system is young, is very important.

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.

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 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 infection in the lungs. Lung infection 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 that 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:

●Over time, the spleen stops working in people with sickle cell disease. 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 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 their spleen is getting bigger.

Managing pain — Children with sickle cell disease can have episodes of pain or swollen, painful fingers or toes. The medical term for this is 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 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 because of pain.

There are also some things that 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 that 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 that your child drinks enough fluids, eats a healthy diet, avoids very cold temperatures, and gets enough sleep. If your child is dealing with stress at school or at home, try to address this and help them handle the stress.

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 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 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 is not painful. 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 that your child has a high risk of stroke, your doctor will suggest treatments that can help prevent a stroke.

Long-lasting erections — Males 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 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 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.

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