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:
- Swimming in cold water
- Being out in cold weather
- Getting too hot
- Getting dehydrated from not drinking enough fluids
- Colds and infections
- Overdoing it—not getting enough sleep and rest
- Drinking alcohol or smoking
- Menstruation (getting your period)
- Being around second-hand smoke
- Find out more: Practical Tips for Preventing a Sickle Cell Crisis
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:
- Pain that doesn’t go away with home treatment
- Severe abdominal pain (stomachache) or swollen belly
- Chest pain
- Sudden vision problems
- Headache that’s different from usual
- Feeling more and more tired
- Fever over 101º –always check temperature with a thermometer if your child seems sick
- Problems with breathing or lungs
- Not being able to move (paralysis)
- Sudden weakness
- Sudden loss of feeling (numbness)
- Swollen joints
- Painful erection of the penis that won’t go away (priapism). For more on priapism, or in Spanish: Priapismo.
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.
- Pain management for children has an example of the faces pain scale, and general information about pain and non-drug pain relief.
- The Oucher is a pain measurement scale with photographs of children’s faces that is especially helpful with younger kids who may have trouble using a number scale.
- All about how to know if your child is in pain, measuring pain on pain scales, and using your child’s behavior to tell how bad the pain is.
- Measuring pain in cognitively impaired children.
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.
- To find out more about all the different options in pain-control strategies, see YourChild: Pain and Your Child or Teen.
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:
- Morphine by IV in the ER.
- Hydromorphone may also be used by IV.
- An anti-inflammatory medication like Toradol by mouth (pills) or by IV, or ibuprofen pills is added to help treat the inflammatory pain.
- Medical staff should assess your child’s pain every 15-30 minutes at first. More morphine or hydromorphone will be given until your child has moderate pain relief.
- Pain medications should be scheduled at defined times and should not be given only once the pain is worse. It is much harder to get out-of-control pain back under control than it is to keep pain controlled.
- Intramuscular (IM) injections can scar or cause abscesses. They are also not a good, consistent way to get the pain medicines absorbed into the body. For these reasons, they are not used to deliver sickle cell pain medicines.
- If your child has complications or the pain can’t be brought down to a level where the pain pills alone are working, your child may need a stay in the hospital. During a hospital stay, your child may use Patient-Controlled Analgesia (PCA). Once pain pills alone keep the pain in check, your child is ready to go home from the hospital.
- Common side effects of pain medications used to treat sickle cell pain include constipation and retaining urine. Your child may need other medications to treat these problems and may have to have a urinary catheter to drain their bladder.
- If your child gets itching, hives, or bad reactions to the medicines, your doctors might have to switch the pain medications.
- In addition to the medication, you, the hospital staff, and your child should be teaching, practicing and using other pain control strategies, such as self-hypnosis, progressive relaxation, deep breathing, distraction, guided imagery, and play. Combining many strategies at once is much more effective than just using medication alone.
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.
- See YourChild: Chronic Conditions for more information and ideas for helping your child and your family cope with this chronic condition.
Where can I find more information on sickle cell pain and coping strategies?
- Pain Assessment and Pain Management in Sickle Cell Disease: A Guidebook for Patients and their Families
- YourChild: Chronic Conditions
- YourChild: Pain and Your Child or Teen
Where can I find more information on sickle cell disease?
- The Pediatric Comprehensive Hemoglobinopathy Program at the University of Michigan has a team approach to managing sickle cell disease, and serves patients across Southeast Michigan.
- The Management of Sickle Cell Disease from the National Heart Lung and Blood Institute. In this book, available online (click on PDF to view the 206 page book), the section on pain is based on the guidelines from the APS.
- Health Supervision for Children with Sickle Cell Disease is a policy statement from the American Academy of Pediatrics.
- Sickle Cell Disease—quick reference and fact sheet from the March of Dimes has a good overview of the disease.
- The Sickle Cell Information Center provides information, news, and research updates.
- Sickle Cell Kids is a fun, educational website with games and other activities for kids.
- The Infant and Young Child with Sickle Cell Anemia, from the Texas Department of State Health Services, provides an overview of managing fever, effects on the spleen, medications, painful episodes and inheritance of the disease.
- Sickle Cell Anemia: A Parent’s Guide for the School Age Child, part of the same series as above, has useful information for parents.
- For kids: Do you know about sickle cell anemia?
- For teens: Sickle Cell Anemia
- Sickle Cell Disease and Sickle Cell Trait from the Texas Department of State Health Services
- The National Heart Lung and Blood Institute has Sickle Cell Anemia information.
- Sickle Cell Disease information from Harvard Medical School.
- Sickle Cell Disease information with interactive tutorials, animations and video clips.
- Hemoglobin S Allele and Sickle Cell Disease is an article about sickle cell genetics and epidemiology from the Centers for Disease Control and Prevention.
What organizations are available?
- The Sickle Cell Disease Association of America (SCDAA) has as its goal to promote finding a universal cure for Sickle Cell Disease while improving the quality of life for individuals and families where sickle cell-related conditions exist.
- The Sickle Cell Foundation of Georgia, Inc. is dedicated to providing education, screening and counseling programs for Sickle Cell and other abnormal hemoglobin.
- The American Sickle Cell Anemia Association serves the Cleveland, OH area.