DO YOU KNOW...

Pain and sickle cell disease: What you can do

 

About pain

A child with sickle cell disease will likely have pain at times. The pain can keep your child from being active, from sleeping well, from enjoying family and friends, and even from eating. Pain can also make your child feel afraid or depressed. When your child hurts, it will help for him to feel a sense of control over the pain. Fortunately, the more you know about pain, the better you will be able to help your child.

It is important for you to understand the kind of pain your child is having, what influences the pain, and the best way to help your child cope with pain. Rather than feel helpless, you can help your child learn skills that will help reduce the pain.

If your child is feeling pain, it is important that you tell his doctor or nurse case manager as soon as you can. With treatment, most pain can be reduced.

What causes pain?

Pain can have many different causes. The most common cause of pain in children with sickle cell disease is the sickling of red blood cells. Sickle cell disease is a genetic disorder that affects the red blood cells.

The main purpose of red blood cells is to deliver oxygen to the body. The part of the blood that carries oxygen is called hemoglobin. People with sickle cell disease have abnormal hemoglobin. This abnormal hemoglobin is called sickle hemoglobin or hemoglobin S.

A normal red blood cell is round or doughnut shaped (without the hole in the center), flexible, and smooth. A sickle red blood cell is sickle (banana or quarter moon) shaped, hard, and sticky. As the hemoglobin releases oxygen, it causes the red blood cells to change from a normal round shape to an abnormal sickle shape. This causes the red blood cells to stick together. This slows the delivery of oxygen to the cells and causes pain. This is known as a vaso-occlusive or pain crisis.

Also, do not forget that your child may have pain that has nothing to do with sickle cell disease. Like everyone else, your child can get headaches, muscle strains, and other aches and pains. You should check with your child’s doctor or nurse about what to do for your child for these everyday aches and pains before giving him more medicines.

Assessing pain

If your child is in pain, he may do one of the following:

  • Complain of pain
  • Cry, moan, be irritable, or withdraw quietly
  • Be restless or not want to move at all
  • Hold or guard the area of discomfort
  • Not eat or drink as much as usual
  • Have trouble sleeping or sleep too much to avoid the pain

The doctor and nurse will ask your child to tell how much they are hurting by using one of three methods:

  • The FACES pain scale-revised (FPS-R): 
Faces pain scale
  • If your child is old enough, he may be asked to rate how much it hurts from 0 to 10 without the FACES pain scale.
  • If your child is too young to rate his pain, the doctor or nurse will use a scale (called the FLACC) as they watch for behaviors that might mean your child is in pain.

You know your child better than anyone, so tell the doctor or nurse if your child is not acting normal. Talk to your child about what he is feeling and thinking.

  • Your child's thoughts about what he hears are important. (“I was told this will hurt the last time, so it will hurt this time.”)
  • The beliefs he has learned influence what he tells you about his pain. (“I'm a big boy, and big boys don't cry!”)
  • Emotions influence the amount of pain. (“Where's my Mommy? I'm scared without my Mommy!”)
  • Attitudes from other people play a role. (“It's expected to hurt at least some.”)

Be careful not to reinforce negative thoughts, beliefs, emotions, and attitudes. Below are some helpful responses to your child's pain:

  • Tell your child about what is happening to him, if he wants to know more about it.
  • Acknowledge the pain; do not minimize or deny it.
  • Make physical contact with your child. Hold his hand or give him a hug. (Avoid the painful area)
  • If possible, remain with your child until the pain is controlled.
  • Talk about the positive steps that are being taken to reduce the pain.
  • Keep your own anxiety under control and remain calm.
  • Support your child's way of coping.

Treating pain

If your child has a fever above 100.9º F, do not give pain medicine. A fever of 100.9 or greater is considered an emergency. Get medical treatment right away and call the clinic at 901-595-5041. If it is after hours, call 901-595-3300 and ask the operator to page the hematology doctor on call.

Mild to moderate pain episodes often can be managed at home.

Home treatment guidelines

  • Give pain medicine as directed by your doctor or nurse case manager.
  • Mild pain can be treated with ibuprofen (Motrin®, Nuprin®, Advil®).  
  • Tylenol with codeine is often used for moderate pain.
  • Severe pain requires treatment by a doctor.

Pain treatments work differently for different people. You need to fully understand how your child’s medicines should be taken and always follow those directions carefully. Because some actions can lead to serious or even dangerous results, please follow these guidelines:

  • Do not give your child more medicine than is prescribed by the doctor.
  • Do not give pain medicine more often than prescribed.
  • Do not crush pills that cannot be crushed, cut, or chewed.
  • Do not add any other medicines or herbal remedies without first talking to the doctor or nurse case manager.
  • Do not continue pain medicine at home for more than 24 hours without talking with your child’s nurse case manager.

Your child might not get the pain relief he needs from just using pain medicine at home. If the pain is severe, get medical treatment for your child and call your nurse case manager or hematology doctor on call (after hours). Do not try to give your child medicine at home for severe pain.

It is very important that you tell the doctor or nurse how your child feels and whether or not the treatments are helping. Sometimes patients worry that their doctor or nurse will think that they are complaining, but this is not true. Your child’s doctor and nurse need this information, so they can find the right pain medicine and treatments for your child.

Here are some things to help your child. These techniques may not be a substitute for pain medicine, but they can help the pain medicines work better.

Techiue How it works Examples

Relaxation

  • Relieves anxiety
  • Reduces muscle tightness
  • Good for episodes of brief and/or severe pain
  • Deep breathing (try soap bubbles or party blowers)
  • Muscle relaxation
  • Meditation
  • Soothing music or nature sounds
Distraction
  • Focus attention on something other than pain
  • Counting, singing, praying
  • Watching television or movies (especially comedies), talking to family, listening to someone read, playing video games
Reframing/ Thought Stopping
  • Evaluate negative thoughts and images and replace them with more positive ones
  • "I've had similar pain, and it got better"
  • “I’m strong. I can do this!”
Imagery/ Hypnosis
  • Concentrate on the image of a positive experience or situation or a favorite memory
  • Ask your child to be very specific in describing the details of the imagined experience (colors, sounds, smells, tastes, feelings)
Modeling
  • Learn from someone else's successes
  • Have your child observe another child managing his pain and anxiety by staying calm and talking through his coping techniques.
Heat
  • Increases blood flow
  • Warm baths or towels. Consult your doctor before using heating pads.

Common questions parents ask

Do infants and children feel pain?

Yes. The belief that they do not experience pain is not true. Even though they are unable to talk about it, infants and children do feel pain. Therefore, they need to be treated with the same care and concern given to adults in pain.

Do children always admit to having pain?

No. If children are having pain they may be afraid to tell someone about it. 

Can children really tell us if the treatment of their pain is working well?

Yes, children are the experts of their own pain. Even very young children can tell us when they are in pain and how much they are hurting.

Will my child become “addicted” to the pain medicine?

No, except in very rare cases. Some parents and children worry about becoming “addicted” or “hooked” if they take pain medicines. Drug addiction means that a person is taking a drug to get a mental “high” instead of relief from real pain. However, patients with sickle cell disease take pain medicine to relieve pain so that they can be as active and comfortable as possible. The truth is addiction is very rare when medicine is taken for pain control.

If my child takes large doses of pain medicine now, will it stop working later when he really needs it?

No, the medicine will not stop working. Sometimes, however, your child’s body might get used to the medicine, which is called “tolerance.” If this happens, your child will need to be prescribed a different type of pain medicine.

Can’t my child just “tough it out”? What about the old saying, “No pain, no gain”?

It is unacceptable for a child ever to be in pain. Being sick is scary enough for anyone. Untreated pain does not make them “tough” or help them to build character.

What can I do to help my child with his pain?

  • Be calm and have a positive attitude.
  • Control your own anxiety and do not show negative cues such as gasping, flinching, and cringing.
  • Do not scold or punish your child for not cooperating.
  • Avoid helping to hold your child down during a medical procedure. Instead, hold his hand or offer ways to distract him from the procedure.

Questions?

If you have other questions or concerns about your child’s pain, call the clinic at 901-595-5041. If you are calling after hours or on the weekend, dial 901-595-3300 and ask the operator to page the hematology doctor on call. Outside Memphis, dial toll-free 1-866-2STJUDE (1-866-278-5833) and press 0. 


 

This document is not intended to take the place of the care and attention of your personal physician or other professional medical services. Our aim is to promote active participation in your care and treatment by providing information and education. Questions about individual health concerns or specific treatment options should be discussed with your physician.

St. Jude complies with health care-related federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex.

ATTENTION: If you speak another language, assistance services, free of charge, are available to you. Call 1-866-278-5833 (TTY: 1-901-595-1040).

ATENCIÓN: si habla español, tiene a su disposición servicios gratuitos de asistencia lingüística. Llame al 1-866-278-5833 (TTY: 1-901-595-1040).

تنبيه: إذا كنت تتحدث باللغة العربية فيمكنك الاستعانة بخدمات المساعدة اللغوية المتوفرة لك مجانا. .يرجى الاتصال بالرقم. 5833-278-866-1  (الهاتف النصي: 1040-595-901-1).