 Scleroderma
By
Janice Lynn
Upon learning that fellow Noodler Maryanne Cappelluti died of
scleroderma, I changed my topic for this month's article. Despite
the fact that I work within the health care field, I knew very
little about scleroderma except the basics. The more I researched,
the more humbled I became.
Scleroderma stands for "hard
skin". It's a chronic disease of unknown cause that's characterized
by diffuse fibrosis, degenerative changes and vascular abnormalities
in the skin, joint structures and body organs. It's often thought to
just be one disease when really it's a symptom of interrelating
diseases that involve the abnormal growth of connective tissue. This
connective tissue supports the internal organs and the skin. The
majority of symptoms have vascular features, but frank vasculitis
(inflammation of the blood vessels) is rarely seen. It can range
from a mild disease, affecting only the skin, to a systemic disease
that can cause death in a few months.
Scleroderma is divided into two major clinical groups.
"Localized" is generally restricted to the fingers, hands and face.
CREST syndrome is closely associated with localized scleroderma. (Calcinosis
- calcium deposits in the connective tissues, which when they break
through the skin cause painful ulcers; Raynaud's phenomenon -
small blood vessels in hands/feet contract in response to cold or
anxiety with the skin turning white, then blue, then red, leading to
ulcers, scars and/or gangrene, present in 90 percent of cases; Esophageal
dysfunction - swallowing difficulties and/or chronic heartburn; Sclerodactyly
- thick or tight skin on fingers, which makes it hard to bend or
straighten the fingers; Telangiectasia - small red spots on
the hands and face caused by tiny blood vessels swelling.) The
second group is "Systemic." In this group, the disease has distal
and maximal extremity and truncal skin thickening.
Prevalence
is one in 100,000. It predominantly affects young adults (16 to 40
years) but can be seen in middle age as well (40 to 70 years).
Symptoms of scleroderma usually appear between 30 to 50 years old.
It's rare in children and individuals over 70. The female to male
ratio is 4:1. It affects people of all races and ethnic groups
although systemic scleroderma affects more African-American women
than those of European descent.
It's unknown what causes
scleroderma, but it's believed to be from an alteration in the
immune system. There is some association/increased risk with quartz
mining, quarrying, vinyl chloride, hydrocarbons, toxin exposure and
rapeseed oil. Treatment of other diseases with bleomycin has caused
a scleroderma-like syndrome.
Signs and Symptoms:
Skin:
-
ulcers on
the fingers and toes
-
tightness,
swelling and thickening of the fingers and toes
-
hyperpigmentation (darkening) and hypopigmentation (lightening) of
the skin
-
itching
-
scaling
-
hardening
of the subcutaneous tissue
Peripheral vascular system:
-
Raynaud
phenomenon
-
telengiectasia
Joints, Tendons and Bones:
-
muscle
cramps
-
hand
swelling
-
joint
stiffness
-
pain in
multiple joints
-
hardening
of fingers
Muscle:
Digestive
Tract:
Kidney:
Lungs:
Nervous
system:
The skin
becomes edematous, hardens, thickens, and in the late stage
deteriorates. Lymph fluid builds around the sweat glands. The hair
follicles deteriorate. The tendons harden. The interlobular arteries
in the kidneys thicken in the intimal linings, and albumin and
microscopic amounts of blood are spilled into the urine. The heart
enlarges, and the heart muscles thicken/harden and quit functioning
properly, leading to ischemia (the heart not getting enough oxygen).
Frequently, cysts form in the lungs and the lungs harden. The
esophagus deteriorates and hardens and has decreased functioning.
Depending on the symptoms, diagnosis can be difficult and
time-consuming. There are multiple lab tests that can be done to
help with diagnosis. Two antibodies that are commonly found in
scleroderma patients are Antitopoisomerase-1 or Anti-Sc1-70 (about
40 percent of cases have this) and Anticentromere (found in 90
percent of those with limited systemic scleroderma). Unfortunately,
people who do not have scleroderma sometimes have these antibodies,
so they alone cannot determine diagnosis, just assist in diagnosis.
Sometimes, it takes years and numerous doctor visits as disease
processes are ruled out prior to an accurate diagnosis being made.
Possible complications with scleroderma are renal failure,
respiratory failure, disability, severe muscle cramps and
contractures, heart arrhythmias, obstructive bowel, cardiomyopathy
and death.
Scleroderma is not currently curable. The
treatment is of the symptoms and by offering support. Research is
being done in search of more effective treatments and a cure.
Maryanne will be greatly missed.
References:
Bryan C
et al: Prediction of five-year survival following presentation with
scleroderma. Arthritis Rheum 1999: 42:2660 {NLM Cit ID: 20081761}
Kelley WN, Harris ED, Ruddy S, Sledge CB, eds: Textbook of
Rheumatology. 5th Ed. Philadelphia, W.B. Saunders, 1997
Koopman WJ, eds: Arthritis and Allied Disorders. 13th
Ed. Philadelphia, Lea & Febiger, 1997
Tierney, LM, McPhee SJ,
Papadakis MA: 2001 CURRENT Medical Diagnosis & Treatment.
40th Ed. New York, Lange Medical Books/McGraw-Hill, 2001.
Web References:
www.scleroderma.org (Scleroderma Foundation)
http://www.nlm.nih.gov/medlineplus/scleroderma.html
(National Institutes of Health)
http://www.srfcure.org/srf/home.htm (Scleroderma Research
Foundation)
http://www.mayoclinic.com/health/scleroderma/DS00362
http://www.sclero.org/
Janice Lynn is a
nurse practitioner in a small practice. To find out more about her
debut, award-winning novel JANE MILLIONAIRE or to join her readers
group to be eligible for her awesome freebie giveaways, visit
www.janicelynn.net, or to read Janice's ramblings about a
writer's life and her obsession with Matthew McConaughey, visit
http://janicelynn.blogspot.com.
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 by
Dr. Debra Holland
Dear Dr. Debra,
In January's column, you mentioned reading something uplifting or
motivational each day. With the beginning of a new year, I think
it's a good time to start this myself with the aim of maintaining a
good outlook on life and a positive attitude. What are some of the
books you'd recommend?
A Curious Reader
Dear Curious,
I commend you for taking on a course of positive reading. Many
people don't feel they have a lot of time to read, or they aren't
too interested in nonfiction. The nice thing about a motivational
book is that you only need to spend a few minutes a day reading it
to receive a positive message. As I said in a previous column, I
spend five to fifteen minutes first thing in the morning reading a
motivational book so I can begin my day on a positive note. Taking a
few minutes to read before you go to sleep is also a good idea. As
you hover on the edge of sleep, the positive concepts you're reading
penetrate into your subconscious mind. However, if neither of those
times works for you, anytime you can catch a few minutes is fine. Or
you also can listen to the book on tape or CD while you are working
around the house or driving in your car.
In the following list, a few of the books have been around for a
long time. However, even though the examples in these books may be
outdated and quaint, the concepts are classic. Although you can find
many of these books in your local library, I like to have my own
copies so I can underline sentences that have meaning to me. I also
like to write comments in the margins. To invest in a motivational
library doesn't have to be expensive. Often these types of books can
be found at secondhand bookstores, library sales, thrift stores or
garage sales. Most of the authors listed below have written more
than one book, so you also might check out his or her other volumes.
Easier Than You Think...because life doesn't have to be so hard:
The Small Changes That Add Up to a World of Difference by
Richard Carlson
Life Makeovers: 52 Practical and Inspiring Ways to
Improve Your Life, One Week at a Time by Cheryl Richardson
Awaken the Giant Within: How to Take Immediate
Control of Your Mental, Emotional, Physical and Financial Destiny
by Anthony Robbins
How to Think Like Leonardo da Vinci by Michael
J. Gelb
Codes of Love: How to Rethink Your Family and
Remake Your Life
by Mark Bryan
The Seven Habits of Highly Effective People by
Steven Covey
Nine Steps To Financial Freedom by Suze Orman
Think and Grow Rich by Napoleon Hill
The Sky's The Limit by Wayne Dyer
The Winner's Edge by Denis Waitley
Simple Abundance: A Daybook of Comfort and Joy
by Sarah Ban Breathnach
More spiritual/metaphysical book suggestions are:
Ethics for the New Millennium by the Dalai
Lama
Conversations with God by Neale Donald Walsch
The Writings of Florence Scovel Shinn by
Florence Shinn
Creating Money: The Keys to Abundance by
Sanaya Roman and Duane Packer
I'm currently reading The Call to Create: Celebrating Acts of
Imagination by Linda Schierse Leonard, which I think would be
good for anyone who has doubts about starting a creative project or
is feeling blocked in their artistic endeavors. Other books that aid
in creativity are:
Bird By Bird by Anne Lamott
Pencil Dancing by Mari Messer
Connecting to Creativity: Ten Keys to Unlocking
Your Creative Potential by Elizabeth Bergman and Elizabeth
Colton
The Artist's Way by Julia Cameron
The next books on my morning reading list are The Power of Your
Subconscious Mind by Joseph Sherman and Five Good Minutes:
100 Morning Practices to Help You Stay Calm and Focused All Day Long
by Jeffrey Bantley.
I also subscribe to a weekly motivational e-mail newsletter by
Cheryl Richardson. To subscribe, go to
www.cherylrichardson.com/newsletter/subscribe.htm.
I hope as you embark on your course of motivational reading, you are
able to have an increase in your positive outlook and generate more
success in your life.
Dr. Debra
Debra Holland, Ph.D., is a licensed psychotherapist
who specializes in relationships and communication techniques. You
may visit her website at
www.drdebraholland.com. E-mail her your questions at
drdebra@drdebraholland.com.
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Living Well article, click
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