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Pain and teens with sickle cell disease

 

The most common clinical complication (health problem) of sickle cell disease is an acute vaso-occlusive crisis, also called a pain crisis. The pain from this crisis is unique and is different from that of cancer pain, pain after having surgery, and pain caused by trauma or injury.

As you know by now, pain related to sickle cell disease can recur many times throughout your lifetime. Mild to moderate pain can be managed at home. Severe pain often requires a visit to a specialized infusion center or emergency room, and sometimes you will have to be admitted to the hospital. Pain crises are the most common reason for emergency room visits and hospital admissions.

The pain can keep you from being active, from maintaining a normal work or school schedule, from sleeping well, from enjoying family and friends, and even from eating. Pain can also cause you to feel sad or afraid. The more you know about pain, the better you will be able to manage your pain.

If you are having pain, it is important for you to get treatment for the pain as soon as you can. With treatment, most pain can be reduced, and most of the time it can be controlled.  It is also important for you to understand the kind of pain you are having, what can cause the pain, and the best way for you to cope with the pain.

Types of pain in sickle cell disease: Acute and chronic pain

The acute pain crisis is the most common type of pain. Acute pain is unpredictable, comes on suddenly, and can occur without any other reasons. The extent of the pain varies, as well. It could be mild, or it could be severe and debilitating. Acute pain can last for several hours to a few days. The pain can go from one (1) part of your body to another part. If you have other health problems that are caused from your sickle cell disease, this can cause the pain crisis to last for weeks. Chronic pain is similar to acute pain except that it lasts 3 to 6 months or longer. 

Sickled cells blocking blood flow

What causes pain?

Pain can have many different causes. The most common cause of pain in people with sickle cell disease is sickling of red blood cells. When your body is exposed to certain conditions, red blood cells containing hemoglobin S can become banana-shaped, hard, and sticky. These conditions include too little fluid in the body, infection, and low oxygen levels. As the hemoglobin releases oxygen, it causes the red blood cells to change from a normal round shape to the abnormal banana shape, which is also called a sickle shape. The sickle-shaped red blood cells will stick together, which will clog tiny blood vessels and slow the delivery of blood and oxygen to the tissues. Not having enough oxygen reaching body tissues is called hypoxia. Hypoxia in tissues causes pain. This is known as a vaso-occlusion or pain crisis. 

Keep in mind that you may have pain that has nothing to do with sickle cell disease. Like everyone else, you can get headaches, muscle strains, and other aches and pains that are not related to your sickle cell disease. 

Assessing pain in sickle cell disease

When assessing acute and chronic pain, doctors and nurses will want to know:

  • Your current and past history of pain,
  • The length of time that the pain lasts, and
  • How the pain was treated before and how well you responded to pain medicines.

You will also be asked to rate your pain on a scale of 0–10, with zero meaning no pain and 10 being the most painful. 

Treating pain: Home treatment guidelines

  • Take pain medicine as directed by your doctor.
  • Mild pain can be treated with ibuprofen (Motrin®, Nuprin®, Advil®).
  • Acetaminophen (Tylenol® ) with codeine is often used for moderate pain.
  • Drink plenty of fluids, rest, and apply a warm blanket or towel to the painful area.
  • Severe pain requires treatment by a doctor.

Pain treatments work differently for different people. You need to fully understand how your medicines should be taken. Read the directions on the bottle carefully to make sure you take the pain medicine correctly. Because some actions can lead to serious or even dangerous results, please follow these guidelines:

  • Do not take more medicine than prescribed by your doctor.
  • Do not take pain medicine more often than prescribed.
  • Do not take pain medicine or any other medicine that was not prescribed by your doctor specifically for you.
  • Do not crush pills that cannot be crushed, cut, or chewed. If you are not sure if the pill can be crushed, ask your nurse, pharmacist, or doctor.
  • Do not add any other medicine, over-the-counter medicine, or herbal remedies without first talking to the doctor.
  • Do not continue pain medicine at home for more than 24 hours without talking to your doctor.

You might not get the pain relief that you need from just using pain medicine at home. If the pain is severe, call the Hematology Clinic at 901-595-5041 Monday–Friday 8 a.m.–4:30 p.m. Outside of these hours and on weekends and holidays, you will need to call 901 595-3300 and ask the operator to page the hematologist on call.

The earlier you call, the quicker we can evaluate and treat your pain.

It is important that you to keep your scheduled clinic appointments in an effort to stay well. Tell the doctor how you feel and whether or not the treatments are helping. The doctor needs this information to find the right pain medicine and treatments for you.

Other pain relief

Here are some things to help you when you are in pain. 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

Will I become “addicted” to the pain medicine?

Some people with sickle cell disease 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. Addiction is very rare when medicine is taken as ordered for pain control.

If I take large doses of pain medicine now, will it stop working later when I really need it?

No. The medicine will not stop working. Sometimes, however, your body might get use to the medicine, which is called “tolerance.” If this happens, you will need to be prescribed a higher dose of the same medicine or a different type of pain medicine.

Questions?

Remember to contact the Hematology Clinic or the hematologist on call if the pain medicine is not helping to relieve your pain.

If you have other questions or concerns about pain, call the Hematology Clinic. If you are calling Monday–Friday 8 a.m.–4:30 p.m., dial 901-595-5041. If you are calling after hours or on the weekend, dial 901-595-3300 and ask to speak to the hematologist 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).

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