Thursday September 16, 2004
PUBLISHED
IN THE ITEM
Baffling disease
Keith Gedamke / The Item
Nellie Welch and Mary Johnson look over autoimmune disorder literature. The
two women have a very rare liver disorder called primary
biliary cirrhosis (PBC) that causes debilitating
fatigue and a progressive scarring of the liver with bile duct
deterioration.
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By IVY MOORE
Item Features Editor
ivym@theitem.com
Nellie Welch
and Mary Johnson likely would never have met except for very rare circumstances
— an obscure illness that affects them both.
“I hadn’t heard of it until I got it,” Johnson said, as Welch
nodded.
“It’s just one of those things
that’s kind of rare,” Welch said.
Johnson, 52, and Welch, 65, have primary biliary
cirrhosis (PBC), a chronic liver disease that causes slow, progressive
destruction of the bile ducts in the liver. Since the liver then has difficulty
secreting bile, bile acids and cholesterol build up in the blood causing
inflammation, which in turn leads to destruction of the bile ducts, scarring
of the liver and, eventually, cirrhosis.
“When people hear the word cirrhosis, they think we must be heavy
drinkers,” Welch said. “Actually we’re not at all. PBC is
not related to how much alcohol you drink. We just usually say PBC (to avoid
negative response). I have had a couple of very good friends who said ‘I
didn’t know you drank.’”
PBC is very rare, and about 90 percent of those who have it
are women. Johnson and Welch said they find it pretty
amazing that there are two “PBCers,” as they call themselves, living
in
Since the time of Welch’s diagnosis, a Web site,
http://www.pbcers.org/,
has been established. That’s how the two got together.
“Actually I had joined the PBC group on the Web site,” Johnson
said, “and Nellie happened to post one day and I went ‘There’s
somebody else in
Welch added, “I had posted, like Mary said, and I thought I cannot be
the only person in the world who has this thing, and when I found the PBCers
Web site, I posted for anyone in
Since that time, the online PBCers have established a support group.
“We meet quarterly,” Welch said. “We have 23 people. That’s
not all necessarily in
Because it’s so rare, PBC is often difficult to diagnose. It took “two
or three years” for Welch to get a diagnosis for the illness that was
causing extreme, debilitating fatigue and pain.
“Total exhaustion,” she said. “Such fatigue you cannot imagine.
I had had chest pain, had gall bladder surgery, but I still would have pain
that nothing would ease it until it absolutely wore off. My doctor treated
me for everything in the book because they could find nothing. And then we
went to
Coping with the pain was difficult, Welch said. “I just learned when
it hits, you just have to try to grin and bear it, because nothing helps.
There’s nothing to do. They gave me Demerol shots, all sorts of pain
medication and nothing would do until it wore off itself.”
As if the pain weren’t enough, Welch said, “The next thing that
happened was I started itching. The kind of itching you cannot describe —
no rash, nothing on your body to indicate you’ve got a problem, but
you just itch. I got a loofah sponge (to scratch
with), and I carried it with me. I am on a medication to keep the itching
under control now. It’s not anywhere near what it was, but if I sit
down I’ll be scratching like a dog with fleas.”
Suspecting a food allergy, Welch saw an allergist, who did “all sorts
of blood work.” The tests revealed a high
“sed rate,” the rate at which red blood
cells settle.
“She sent me to a rheumatologist, and he couldn’t find anything.
I was just in tears by then,” Welch said. “I had $800 worth of
tests. I just looked at him and said, ‘I know there is something
wrong.’
“He said, ‘Well if I had to guess, I’d guess a smoldering
liver disease.’”
While waiting to see a gastroenterologist in
“I got my hands on a Mayo Clinic health book, looked up the causes of
itching, and when I came to primary biliary cirrhosis
I went down the list of symptoms, and it had everything I had,” she
said. “So I went in to my doctor and I said, ‘This is what I think
I have. I’d like for you to prove it or disprove it.’ He did a
lot of research, another blood test, and he called me and said, ‘You
have it.’”
Johnson’s diagnosis took only a few months.
She explained, “Mine came about different than Nellie’s. In fact,
I feel I’m very fortunate to find it when I did. I went in for a regular
gynecology checkup.”
Johnson had mistaken her fatigue for a normal symptom of aging.
“I’d been going to school at the time and had been tired. You know,
when you hit your late 40s and early 50s, you start to slow down. You get
tired. My doctor talked me into the ‘executive panel’ (20 different
blood tests).”
Neither the gynecologist nor her primary physician were very concerned about
her elevated liver enzymes — even acetaminophen can cause the high reading
temporarily — but when the enzyme levels came back even higher on a
subsequent test, Johnson’s primary care physician suspected hepatitis.
When hepatitis was ruled out, she was sent to a gastroenterologist who found
a cyst on her liver and a gallstone. During gall bladder surgery, her liver
was biopsied. That led to the diagnosis of PBC.
The cause of primary biliary cirrhosis is not
known.
“It’s thought to be autoimmune,” Welch said, “but there
is still a lot of debate about it and research as to what actually causes
it. But they think it’s autoimmune in nature like lupus, thyroid disease,
fibromyalgia — all of these things that they
can’t find a reason for.”
Johnson added, “They really haven’t stated it positively, but they
think it’s genetic.
“You read about sisters having it and mothers and daughters having it.
I have a sister who has rheumatoid arthritis, I
have a sister who has polymyalgia. A lot of times
when you have somebody that’s got that, and then you’ve got achy
joints, then you think ‘I’m probably getting what they
have.’”
Welch began taking a medication called Actigall
which gradually helped relieve her pain attacks.
“There’s another drug called URSO. It just slows it down, there
is no cure,” she said.
Johnson is taking URSO, “And I’m on
colchicine, which is usually used for gout,”
she said. “Anybody who’s got PBC is at risk for osteoporosis, so
now I’m doing my walks and my weights and all I can do. This disease
can attack your joints and your muscles, and you ache.”
“What concerns us is that there are women out there walking around who
have elevated liver counts who simply don’t know that they are prime
candidates for PBC,” Welch said. “Not everybody with elevated counts
has it, but a good portion do.”
Getting diagnosed is important for both quality of life and life expectancy,
Johnson said.
“From what I understand when I was first diagnosed, life expectancy
after diagnosis was approximately five years,” Welch added.
“They’ve done an awful lot of research, and with the
Actigall, the generic and the name brand, and the
URSO, they’ve slowed it down to where now you can live 20 years upward,
depending on how you respond to medication.”
Johnson said, “The idea is to slow the progression so you stay in the
lower stages a longer period of time.”
PBC is staged like many diseases, with stage one the earliest, four the last.
“Four is end stage, but it can range from early four on up,” Welch
said. “End stage means either transplant or liver failure. I got my
blood work back the other day, and it shows that mine is progressing
now.”
Johnson said, “I just think Nellie’s a hero in a lot of ways because
she’s gone through a lot. Getting on the URSO as soon as I did, I feel
that I have a good outlook as far as how long it might take to progress into
another stage.”
She said that, because PBCers look healthy, a lot of people don’t understand
how they can suffer. While Johnson herself is able to work full time, Welch
has had to give up her job and other activities.
“I think this thing is cyclical for some reason,” Johnson said,
“because I can go for a period of time where I feel great, and then
I’ll have a low ebb.”
Welch added, “You can’t dwell on it, because if you do, you’ll
drive yourself crazy.”
She reiterated their concern for people who may be suffering from undiagnosed
PBC. “We’d like to issue the invitation for anyone else out there
who’s got any kind of liver disease,” she said. “If they would
like to be a part of our support group, they can contact me for information.
Our next meeting will be in October.”
For more information about primary biliary
cirrhosis, see the Web site
http://www.pbcers.org/,
email pbcers@pbcers.org or
pbclinie@pbcers.org,
or write to: PBCers Organization,
Reach Features Editor Ivy Moore at
ivym@theitem.com or
803-774-1221.