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Genetic and Rare Diseases Information Center (GARD)

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Sickle beta thalassemia


Other Names for this Disease
  • Hb S beta-thalassemia
  • Hemoglobin sickle-beta thalassemia
  • Sickle cell - beta-thalassemia disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

What is the best pain management regimen for a 50-year-old with sickle beta thalassemia experiencing sickle cell crisis? Lately, the onset has become more frequent, about every three months. Is this normal?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is a sickle cell crisis?

A sickle cell crisis occurs when sickle-shaped red blood cells block the small blood vessels that carry blood to the bones. This causes pain that can begin suddenly and last several hours to several days. Affected people may experience pain in their back, knees, legs, arms, chest or stomach. The pain can be throbbing, sharp, dull or stabbing. The severity and frequency of the pain varies greatly from person to person and from crisis to crisis. Recurrent episodes may cause irreversible organ damage.[1][2]
Last updated: 12/22/2014

Can a sickle cell crisis be prevented?

There are many factors that contribute to the development of a sickle cell crisis and in most cases, the exact trigger for the crisis is unknown. However, taking the following measures may reduce the risk of experiencing a crisis:[2]
  • Eating a balanced diet
  • Limit alcohol consumpation
  • Quit smoking
  • Exercise regularly
  • Drink plenty of water
  • Reduce or avoid stress
  • Avoid temperature extremes (too hot or too cold)
Last updated: 12/22/2014

How might a sickle cell crisis be treated?

The best options for pain management vary from person to person. Some affected people may be able to use over-the-counter pain relievers (acetaminophen, aspirin, ibuprofen, or naproxen), while others may need a prescription medication to help reduce pain.

There are also several non-drug options for pain control such as:[3][2]
  • Physical therapy can relax and strengthen muscles which can help reduce pain. A physical therapist may also be able to suggest gentle exercises and heat/cold treatments for pain relief.
  • Transcutaneous electrical nerve stimulation (TENS), a low intensity electrical current, can sometimes be effective for pain relief.
  • Relaxation techniques and psychotherapy may help affected people cope with pain.
  • Massage can decrease muscle stiffness, which may help reduce pain.
  • Acupuncture and accupressure can help relieve stress and pain.
  • Distraction through engaging hobbies and fun events can help redirect the focus away from pain.

Please speak with your healthcare provider before trying any new medications or methods of pain relief.
Last updated: 12/23/2014

References
Other Names for this Disease
  • Hb S beta-thalassemia
  • Hemoglobin sickle-beta thalassemia
  • Sickle cell - beta-thalassemia disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.