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http://www.medscape.com/viewarticle/473349_1

From Topics in Advanced Practice Nursing eJournal

Scleroderma: A Fascinating, Troubling Disease

Posted 05/03/2004

Elaine A. Furst, RN, MA

Abstract and Case Study

Abstract

Scleroderma is classified as a rare, autoimmune, collagen-vascular

disease of unknown etiology that can be difficult to diagnose and

treat. Its onset can be insidious or rapid; the symptoms range from

challenging and painful to life threatening. Individuals may

experience a range of symptoms different from the classic

description of the disease, resulting in confused health

professionals and frightened patients. The toll on lives, careers,

and disrupted family and social roles can be overwhelming as well as

destructive both to the patient and to loved ones. Because it is

rare (the incidence rate for the systemic form is estimated to be 19

new cases per year per 1 million adults), many nurses and physicians

have only read about scleroderma.[1] This article seeks to

familiarize advanced practice nurses (APNs) and other clinicians

with the major manifestations of scleroderma and to provide the

information necessary for its recognition and symptom management. A

list of resources is provided to assist with care planning.

Case Study: Sara Has Unusual Symptoms but No Clear Diagnosis

Sara is a 40-year-old woman who is ordinarily very active and busy

with her family and her community, but her busy life is at a

standstill. She has severe fatigue, arthralgias, and tight, itchy

skin on her face, arms, hands, and trunk. She spends most of her

days sitting up in the lounger in the living room because she is

both fatigued and has constant heartburn. She has had frequent

dental problems because of excessive dryness of her mouth. She

describes a dwindling appetite and thus has had very poor

nutritional intake for months.

Sara is always cold, and her fingertips and nails are often numb,

even in the summertime. There are 2 small, painful, digital ulcers

on the knuckles of her right hand and 1 under the thumbnail on her

left. Her fingers exhibit the beginnings of sclerodactyly, making it

difficult for her to perform daily activities and household duties.

Her internist has prescribed an antihistamine for the itching and a

protein pump inhibitor for dyspepsia and told her to rest

and " reduce the stress in your life. " She has been to see quite a

few specialists about her complaints, including a psychologist, and

none of them were definitive about the diagnosis.

Sara is finally referred to a rheumatologist at a nearby university

medical department who diagnoses systemic sclerosis. She and her

husband are frightened, especially when she is prescribed

methotrexate, which they know is a drug used in cancer. She types

scleroderma into her computer search engine and discovers

advertisements for herbal remedies for scleroderma, the site for the

Scleroderma Foundation, and articles about scleroderma in women's

magazines. This only adds to her confusion and worry.

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Thanks for sending this article, Kathi. It's quite good.

And here's some rheumatology trivia - Elaine Furst is married to

rheumatologist E. Furst - world-class expert on systemic

sclerosis. What a team!

I'll tell you where to go!

Mayo Clinic in Rochester

http://www.mayoclinic.org/rochester

s Hopkins Medicine

http://www.hopkinsmedicine.org

[ ] RE:Lets try this again...

> http://www.medscape.com/viewarticle/473349_1

>

>

>

>

> From Topics in Advanced Practice Nursing eJournal

>

> Scleroderma: A Fascinating, Troubling Disease

> Posted 05/03/2004

>

> Elaine A. Furst, RN, MA

>

> Abstract and Case Study

> Abstract

> Scleroderma is classified as a rare, autoimmune, collagen-vascular

> disease of unknown etiology that can be difficult to diagnose and

> treat. Its onset can be insidious or rapid; the symptoms range from

> challenging and painful to life threatening. Individuals may

> experience a range of symptoms different from the classic

> description of the disease, resulting in confused health

> professionals and frightened patients. The toll on lives, careers,

> and disrupted family and social roles can be overwhelming as well as

> destructive both to the patient and to loved ones. Because it is

> rare (the incidence rate for the systemic form is estimated to be 19

> new cases per year per 1 million adults), many nurses and physicians

> have only read about scleroderma.[1] This article seeks to

> familiarize advanced practice nurses (APNs) and other clinicians

> with the major manifestations of scleroderma and to provide the

> information necessary for its recognition and symptom management. A

> list of resources is provided to assist with care planning.

>

> Case Study: Sara Has Unusual Symptoms but No Clear Diagnosis

> Sara is a 40-year-old woman who is ordinarily very active and busy

> with her family and her community, but her busy life is at a

> standstill. She has severe fatigue, arthralgias, and tight, itchy

> skin on her face, arms, hands, and trunk. She spends most of her

> days sitting up in the lounger in the living room because she is

> both fatigued and has constant heartburn. She has had frequent

> dental problems because of excessive dryness of her mouth. She

> describes a dwindling appetite and thus has had very poor

> nutritional intake for months.

>

> Sara is always cold, and her fingertips and nails are often numb,

> even in the summertime. There are 2 small, painful, digital ulcers

> on the knuckles of her right hand and 1 under the thumbnail on her

> left. Her fingers exhibit the beginnings of sclerodactyly, making it

> difficult for her to perform daily activities and household duties.

>

> Her internist has prescribed an antihistamine for the itching and a

> protein pump inhibitor for dyspepsia and told her to rest

> and " reduce the stress in your life. " She has been to see quite a

> few specialists about her complaints, including a psychologist, and

> none of them were definitive about the diagnosis.

>

> Sara is finally referred to a rheumatologist at a nearby university

> medical department who diagnoses systemic sclerosis. She and her

> husband are frightened, especially when she is prescribed

> methotrexate, which they know is a drug used in cancer. She types

> scleroderma into her computer search engine and discovers

> advertisements for herbal remedies for scleroderma, the site for the

> Scleroderma Foundation, and articles about scleroderma in women's

> magazines. This only adds to her confusion and worry.

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