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Pain in Sickle Cell Disease (Sickle Cell Anemia)

What is sickle cell pain?

Sickle cell disease if one of the most common inherited diseases worldwide, and pain is the most important symptom of the disease.  The pain is often described as deep, gnawing and throbbing.  The skin may be tender, red, and warm in the painful areas.  Kids with sickle cell disease may experience a wide variety of pain ranging from mild to severe, starting as early as six months old and continuing throughout their lives.

The pain of sickle cell anemia is very frustrating.  You never know when it will strike and it is often difficult to get it under control.

How can you avoid triggering a pain crisis?

Most pain episodes do not have an obvious reason for their occurrence.  But here are some possible causes and things that your child should avoid if possible:

What are the particular dangers of smoking around kids with sickle cell disease?

Research shows that kids with sickle cell disease who are exposed to environmental tobacco smoke (ETS) at home have more than twice as many pain episodes (pain crises) as sickle cell patients who were not exposed. Sickle cell crises involve serious symptoms, and often require hospitalization. Researchers in one study estimate that ETS exposure increases the risk of crisis by 90% among children with sickle cell disease.  If you smoke, either quit or don’t smoke around your child.

When should my child see the doctor for sickle cell pain?

If your child has sickle cell disease, is under two years old, and has pain or fever, they should see a doctor right away.  Other serious warning signs that signal a need for immediate medical treatment are: 

How can I recognize pain in my child? Why is describing pain so important?

Everyone can feel pain, even babies and young children.  Sometimes children have a hard time expressing themselves and may find it hard to tell you where it hurts and what it feels like. 

For this reason, doctors and nurses are using new tools to help define pain in the kids they care for.  Pain charts and scales for children use pictures or numbers to describe their pain.  Describing the pain can help parents, doctors and nurses understand how bad the pain is, and how to best treat it.  Talking to your child’s doctors and nurses about pain is important.  The more they know about your child’s pain, the more they can help.  Pay attention to how your child acts.  For example, when your child is in pain, they may be restless or unable to sleep.

The first step in treating your child’s pain is to tell your child’s doctor or nurse about it.  Your health care provider will ask several questions about the pain, including where it hurts, what it feels like, and how it has changed since it started. 

Your child’s doctor may ask you to keep a pain diary with your child, which keeps track of when your child has pain throughout the day.  This diary can also document how the pain changes after taking pain medications.  If medications do not seem to work, or if your child has a bad reaction, tell the doctor and keep a list of these problem medicines for future reference.

How can we treat the pain at home?

Many pain crises can be managed at home.  Your child will need to increase their fluid intake and should use the pain pills prescribed by their doctor.  If you can’t take care of the pain at home with these measures, or if the warning signs listed above occur, then get medical care right away.

In addition to drinking lots of water and taking pain pills, there are many other ways to help your child fight the pain of sickle cell disease.  As with other kinds of pain, it’s best to use many different approaches at once to get the best pain relief.  Biofeedback, cognitive strategies, self-hypnosis, and progressive relaxation all show great promise as useful treatments to add in to the usual sickle cell pain treatment. 

What kind of treatment is my child likely to receive in the hospital?

If treating the pain at home didn’t work, you would bring your child to the hospital.  At the hospital, your child will probably receive treatment similar to this:

What are the challenges of pain treatment in the ER or hospital?

A fear of addiction, by patients, families, and health care workers can sometimes interfere with appropriate treatment of pain.  However, there are detailed treatment guidelines that outline the right way to treat sickle cell pain. 

What about meperidine (Demerol)?

Meperidine (Demerol) has been a controversial medicine.  Meperidine should not be taken by mouth for chronic or acute pain in sickle cell disease.  Intravenous (IV) meperidine should not be used as a first-line medication for sickle cell pain, because it can be toxic to the nervous system at higher doses.  However, if your child has a morphine or hydromorphone allergy, or when they have improved in the past using meperidine briefly, meperidine may be a good choice.

What about hydroxyurea?

The medicine hydroxyurea has been used for over a decade to treat sickle cell disease in adults.  It is not yet approved for use in children, but is showing promise in many studies.  It looks like it may be both effective and safe for children, and is proven to help with pain and other complications in both children and adults.  Continuing research looks at long-term effects and safety of hydroxyurea treatment in kids.

How does sickle cell pain affect my child's life?

Sickle cell disease is a hard condition to cope with.  Controlling the pain can be very frustrating.  Because there are no outward signs of the pain, sometimes people don’t believe a child is suffering as much as they really are.  In addition to the pain, there are many ways kids with sickle cell have to change the way they live.  For example, they may not be able to go swimming with their friends if the pool is too cold, and frequent hospital stays interrupt their lives.  These kinds of restrictions can really cramp a kid’s style.

You may need to work with your child’s school to be sure your child is getting the help and support needed. With proper supports in school, kids with sickle cell pain can participate in most activities.  Find out how to work with the school system to get the help in school that your child needs.  This information includes tips for parents, school nurses and teachers.

Sickle cell disease, like any chronic condition, challenges kids and their families in all kinds of ways.  When they find helpful coping strategies, kids and their families can rise to the occasion.

Where can I find more information on sickle cell pain and coping strategies?

Where can I find more information on sickle cell disease?

What organizations are available?

References

Written and compiled by Kyla Boyse, R.N. and Brendan P. Kelly, M.D.  Reviewed by Andrew D. Campbell, M.D.
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Updated May 2009

U-M Health System Related Sites:
Department of Psychiatry
U-M Pediatrics

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