Fragile Scleroderma patients
require special care

March 11, 2013 9:51 am Views: 6913
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Theresa has Scleroderma. In the past two years she was had numerous hospital stays and ER visits associated with complications of her disease that range from gastroparesis to kidney failure. All too often, when in hospital, she encounters physicians and nursing staff that have limited knowledge, if any, of Scleroderma or Systemic Sclerosis and the special care considerations patients require. “Hospitals need to know how to manage common symptoms of this disease. For me it’s about knowing that my care-givers understand the basics – how to manage my digital ulcers, the painful skin and joints and GI issues. It’s a safety issue,” said Theresa, recently discharged from hospital for an intravenous infusion to open up her blood vessels.”

Scleroderma or SSc (systemic sclerosis) is a chronic and progressive autoimmune disease of the connective tissue. For unknown reasons, the immune system triggers the over-production of collagen (scar tissue) which damages the cellular matrix of connective tissue found in skin, tendons, ligaments, cartilage, bone, blood vessels, the gut and other internal organ surfaces. Cold fingers (known as Raynaud’s Phenomenon) is often the first symptom but the most characteristic feature of Scleroderma is the presentation of hard, hide-bound thickening of the skin. Less visible but of major consequence are the lesions that develop in small blood vessels (vascular lesions), which may involve major organs such as the heart, lungs and kidneys. The disease is incurable but not untreatable.

Who Is Diagnosed with Scleroderma?

Scientists are certain that Scleroderma is not contagious, not infectious, and not cancerous. Studies of twins also suggest that Scleroderma is not inherited. Although more common in women, with an average age of disease onset around 40 years, Scleroderma also occurs in men and children, from infants to the elderly. It affects people of all races and ethnic groups.

36% of Patients Hospitalized

Based on the 2010 Canadian Scleroderma Patient Survey, an initiative of the Scleroderma Society of Canada (SSC) and the Canadian Scleroderma Research Group (CSRG) and funded in part by the Canadian Institutes of Health Research (CIHR) and the SSC, 36% have been hospitalized for Scleroderma or Scleroderma-related symptoms. The main reasons for hospitalization were heart/lung, gastrointestinal, vascular and infection issues.

While there are no Canadian statistics currently available about hospital stays for Scleroderma, Hospital Episode Statistics (2002-03) from the Department of Health, England reported:

  • 14% of ER visits for systemic sclerosis required emergency hospital admission
  • 10 days was the mean length of stay in hospitals for systemic sclerosis
  • 5 days was the median length of stay in hospitals for systemic sclerosis[1]

Managing the Hospitalized Scleroderma Patient

Too often, when Scleroderma patients are hospitalized for surgery or treatment of other conditions or complications of their disease, they encounter staff with limited knowledge of Scleroderma. While the manifestations of the disease can vary greatly from patient to patient, the following checklist[2] may assist hospital staff when developing a care-plan for hospitalized Scleroderma patients.

  • Increased sensitivity to cold and painful spasms in extremities (Raynaud’s Phenomenon)

Provide extra blankets. Avoid drafts. Maintain warmth particularly pre & post-op.

  •  Ulceration, Impaired healing

Assess for signs of infection. Provide appropriate dressings.

  • Tight skin

Provide protection during surgery and/or procedures. Assess for signs of skin breakdown. Extra care required with venipuncture and blood pressure measurement.

  •  Dry skin

Assist with moisturizing as required.

  •  Painful hands and feet

Avoid injury, e.g. during transfer and ambulation.

  • Painful joints

Assist with repositioning. Provide extra pillows. Use massage and the application of heat. Assess pain and anti-inflammatory medications. Encourage ambulation as tolerated.

  •  Impaired movement and mobility

Assistance may be required with feeding and oral hygiene.

  • Dry mouth, dry eyes (Sicca Symptoms, Sjogrens Syndrome)

Ensure drinking water is readily accessible. Maintain good oral hygiene especially when patient unable to drink.

Assist with application of eye drops or ointment if patient to self-administer, particularly pre and post-op and prior to sleeping.

  • Esophageal involvement including difficulty swallowing

Elevate head of bed. Provide extra pillows. Position upright when eating and after meals. Administer antacids as required.

Discuss food preferences while ensuring adequate and appropriate dietary intake. Consult with dietician as necessary.

  • Bowel involvement diarrhea and/or constipation; fecal incontinence

Assess for dietary requirements and medication regime. Consult with dietician as necessary.

  • Shortness of breath on exertion

Allow patient to set the pace during physical activity. Assess O2 saturation and need for oxygen. Refer to respiratory therapist as needed.

  • Reduced capacity to cope

Create calm, supportive environment. Encourage stress reduction and relaxation techniques. Refer for social work assessment.

  • Increased skin sensitivity

Take extra care as patient has a low pain threshold.

  • Blood pressure irregularities and/or pulmonary arterial hypertension

May signal kidney impairment requiring immediate intervention. If patient has pulmonary arterial hypertension, the anaesthetist needs to be alerted well in advance as anaesthesia carries increased risks. Extra care required with intubation.

Maintain warmth to allow for optimal venous access.

Want to learn more?

The Scleroderma Society of Ontario (SSO) will be hosting the 2013 Scleroderma Canada Congress on September 27 and 28 in downtown Hamilton, Ontario. This year’s theme “Whole patient care – Treating the patient not the disease” is expected to attract several hundred patients, providers and researchers. In conjunction with the Congress, the Scleroderma Patient-centered Intervention Network (SPIN) will be holding its annual working meeting and will be active contributors to the Congress. SPIN is comprised of clinicians and researchers from around the world with expertise in Scleroderma. The Network also includes patient representatives from the Scleroderma Society of Canada, Scleroderma Society of Ontario, Sclerodermie Quebec as well as the Scleroderma Foundation of the United States and the Federation of European Scleroderma Associations (FESCA) with members from Belgium, Croatia, Cyprus, Denmark, Finland, France, Germany, Hungary, Ireland, Italy, Netherlands, Norway, Poland, Portugal, Spain, Sweden and the United Kingdom. For more information, contact info@sclerodermaontario.ca or call toll free 1-888-776-7776.



[1] Hospital Episode Statistics, Department of Health, England, 2002-03

[2] Adapted from (1) A HOSPITAL CHECKLIST A Guide to Nursing Patients with Scleroderma published by Scleroderma Australia and (2) Hospital Checklist A Guide to Nursing Patients with Systemic Sclerosis (Systemic Scleroderma) published by The Scleroderma Society UK

 

 

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11 Comments

  • I would like to mention that many scleroderma patients need intravenious fluids warmed before administered. It is really painful and makes the blood vessels cramp when they are cold.

  • sherrill Heerwagen

    Amen amen amen, thank you. I’m sending this to all my family and Dr.s!!!!!

  • Thanks!
    It’s clear and easy to understand.
    I’m a nurse and 18 months ago I was diagnosed: sclerodema.
    It’s too hard to explain the same each time because it’s unknown disease.
    Greetings from Barcelona,
    Maria

  • Hi Maureen, Great article & particularly liked the checklist for nursing staff. I just wish there was an option on the page to print or at least save this article for printing at a later stage if necessary.

    I have huge issues with some of the staff that are supposed to be caring for my health & the root cause is lack of knowledge of the condition & its accompanying overlaps, of which I have many.

    The trick is approaching these people on their own terms when they have a starting position of supreme superiority. I’m thinking of consultants in particular, who are academically brilliant but have zero people skills, lack patience/empathy or are just downright rude.

    Some serious guidance on the later would be useful. I’m sure lots of us who have experienced it would be more than willing to contribute the coping strategies that have worked for us & thse that definitely don’t.
    Best regards

    Marie

  • Denise Gough Peters

    Thank you. Information very educational.

  • Ma. Renebel dizon

    Thanks for the imformation.. I’m from the Philippines I have systematic scleroderma it’s very hard to diagnos this my sick even our doctor here don’t know what is my case that’s why I plan to go Dubai to do some laboratory test.. My doctor said I have scleroderma at the age of 27 until now it’s very hard to accept my situation I try to finish my life.. Pls!! Help me.. I don’t know what I will do..

  • Thanks for this article. I have had scleroderma 16 years and have found few people (especially doctors) neither know nor care about my condition. I have experienced that a few more people seem to know the term,at least. I have recently developed lung involvement and now have systemic sclerosis.

  • Very helpful& welcome, Maureen. Thanks! Bob Buzza, VP, Scleroderma Association of BC

  • Thank you for this informative,concise and helpful article on caring for hospitalized Scleraderma patients. I would constantly have to brief almost every health provider that attended to my husband who had rapid onset diffuse Scleraderma from hospitals to rehab facilities including phlebotomists who always mentioned ” wow his skin is so tight or so hard”. I am printing this information out and sending it to all hospitals and facilities that my husband was treated at. Perhaps these institutions could instruct their employees and staff before arrival of a Scleraderma patient instead of running out to their computers and “Googling” Scleraderma for a quickie refresher course before treating the fragile Scleraderma patient.

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