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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Limited scleroderma


Other Names for this Disease

  • Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia syndrome
  • Calcinosis-Raynaud phenomenon-sclerodactyly-telangiectasia
  • CREST syndrome
  • Limited cutaneous systemic scleroderma
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

My mom is dying from this very painful and agonizing disease. She lost both of her legs 4 years ago due to the disease and it has now progressed to her hands, and it's looking as though it's now going to take her hands from her. The pain she bears is very difficult to endure. Is there anything that could help her?

Our Answer

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

How might CREST syndrome be treated?

Unfortunately, CREST syndrome has no known cure. The condition carries both physical and psychological consequences, so a holistic approach to management should be taken.[1] Treatment generally focuses on relieving signs and symptoms and preventing complications.[2] Heartburn may be relieved by antacid medications that reduce the production of stomach acid. Medications that open small blood vessels and increase circulation may help relieve Raynaud's symptoms and reduce increased pressure in the arteries between the heart and lungs. Drugs that suppress the immune system have shown promise in preventing interstitial lung disease (a condition in which excess collagen collects in the tissue between the lungs' air sacs) in some people with CREST syndrome. To prevent loss of mobility, stretching exercises for the finger joints are important. A physical therapist can also show affected individuals some facial exercises that may help keep the face and mouth flexible. If CREST syndrome is making it difficult to perform daily tasks, an occupational therapist can help individuals learn new ways of doing things. For example, special toothbrushes and flossing devices can make it easier to care for the teeth. Surgery may be necessary for some affected individuals. Large or painful calcium deposits sometimes need to be surgically removed, and amputation of fingertips may be necessary if skin ulcers progress to gangrene.[2] Depression affects approximately 45% of patients with systemic sclerosis and 64% also develop anxiety, so early assessment and treatment of these psychological issues is recommended.[1]

For pain management, studies have shown that oxycodone is effective and safe for pain due to severe skin ulcers, while topical lidocaine helps reduce pain of digital ulcers in individuals with systemic scleroderma.[3][4]
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There are also some lifestyle changes and home remedies that may be helpful for some individuals with CREST syndrome. To reduce Raynaud's symptoms, individuals may consider wearing gloves or mittens outdoors when the weather is cool, and indoors when reaching into the freezer, for example. To maintain the body's core temperature, individuals may dress in layers and wear a hat or scarf, thermal socks, and well-fitting boots or shoes that don't cut off  the circulation. Individuals who smoke should talk to their doctor about the best ways to quit. Nicotine constricts the blood vessels, making Raynaud's phenomenon worse. Individuals who have difficulty swallowing may consider choosing soft, moist foods and chewing food well. To minimize acid reflux individuals may eat small, frequent meals; avoid spicy or fatty foods, chocolate, caffeine, and alcohol; and avoid exercising immediately before or after eating. Sitting upright for a couple of hours after a meal may also help. To help keep skin soft, individuals may avoid harsh soaps and detergents, while choosing gentle skin cleansers and bath gels with added moisturizers. Individuals may also consider bathing less frequently and taking brief baths and showers, using warm rather than hot water. Moisture levels in the home may be improved by using a humidifier to ease skin and breathing symptoms.[2]

For additional information about how CREST syndrome may be treated, the following article from eMedicine may be helpful:  http://emedicine.medscape.com/article/1064663-treatment#showall

The information provided here is for general educational purposes only. Individuals interested in learning about specific treatment options for themselves or family members should speak with their healthcare provider.
Last updated: 8/11/2011

What might relieve the pain associated with CREST syndrome?

CREST syndrome is a multi-system condition, and therefore pain may be experienced with several of the signs and symptoms associated with the disease. For a general description of how each individual sign and/or symptom may be treated, please refer to the question "How might CREST syndrome be treated?" For pain associated with:

  • Heartburn - this may be relieved by antacid medications that reduce the production of stomach acid.
  • Raynaud's phenomenon - this may be relieved by medications that open small blood vessels and increase circulation.
  • Calcinosis -localized surgery to remove the calcium deposits may be indicated.[2]
  • Skin ulcers - studies have shown that oxycodone is effective and safe for pain due to severe skin ulcers, while topical lidocaine helps reduce pain of digital ulcers.[3][4]
Last updated: 8/11/2011

References
Other Names for this Disease
  • Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia syndrome
  • Calcinosis-Raynaud phenomenon-sclerodactyly-telangiectasia
  • CREST syndrome
  • Limited cutaneous systemic scleroderma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.