PBC afflicts women with pain,
alienation
by Mary
Helen YarboroughPublic Relations
Through no fault of their own, people suffering from primary biliary cirrhosis (PBC) endure a continual decline in physical health and comfort. Their pain is often exacerbated by uncompassionate reactions from family, friends and associates who are ignorant of the disease’s root causes.
Shirley Edwards,
left, and Nancy Dougherty discuss life with PBC.Like cirrhosis, PBC impairs liver function. But unlike cirrhosis, PBC is not a result of alcoholism or any other substance abuse by its victims. An autoimmune disorder, PBC is a chronic liver disease that slowly destroys the ducts that drain bile from the liver. In PBC patients’ livers, the bile ducts are inflamed and inhibit the elimination of bile.
MUSC currently sees more than 50 PBC patients, according to Ira Willner, M.D., of MUSC’s Gastroenterology and Hepatology division. Willner said that PBC is treatable but incurable.
“We can slow down the progression of the disease with [the drug] URSO, but that’s about it,” said Willner. “It’s not curable and there is no research on it at MUSC right now.”
URSO, the commercial name for a bile acid drug or ursodiol, is the only ursodiol approved by the Food and Drug Administration for the treatment of patients with PBC. It is administered in pill form and is taken orally. The drug works by helping the liver eliminate bile.
Greg Buck, a physician assistant for MUSC’s Digestive Disease Center, said that PBC is not an uncommon disease, although little is known about its causes. While some people with PBC may survive indefinitely with little difficulty, many others suffer and die prematurely.
“It appears to affect middle-aged women by a ratio of 9-to-1,” said Buck, who has worked with an area Southeastern PBC support group. “Some folks with PBC need a liver transplant. It’s a slowly progressive disorder, but it varies in its rate of progression. Some folks have it and never have a problem. If it’s slow enough, people with PBC may die of some other problem.”
While some may survive, they often suffer from pruritus that causes intense itching. Others are affected by bone density loss or osteoporosis, and are at an increased risk of having breast cancer.
A familial path
Adding worry to agony, PBC tends to run in families, often passing from mother to daughter.
Shirley Edwards, a Charleston realtor, was diagnosed with PBC in 1993. Of her three daughters, she fears that her oldest, at 35, is showing the same early signs of PBC that she had experienced 14 years ago.
“She started breaking out with blood blisters,” Edwards said. “She’s fatigued. That’s the worst of this disease. It’s bad enough for me to have it, but for my daughter to have it is just awful for me.”
Edwards is not alone. Nancy Dougherty, a retired civil service worker for the U.S. Air Force living in North Charleston, was diagnosed with PBC in 1981. She said she believes that her sister also had it, but eventually succumbed to amyotrophic lateral sclerosis, or Lou Gehrig’s disease. She believes her mother, who died of a heart attack at 54, may have had the disease, as well. None of her three sons have shown any sign of the disease, she said.
But at 60-plus years of age, the perky, blonde Dougherty considers herself blessed. A few years ago, she was to be placed on the list to receive a liver transplant at the advice of Adrian Reuben, M.D., an MUSC gastroenterologist.
“He set me up for a liver transplant, but I never went on the list, because I got better,” Dougherty said, “through prayer—how else?”
Lunching on a plate of fresh fruit and vegetables, Dougherty enjoys what even some doctors have called a miracle.
“I guess you could say mine is in remission,” said Dougherty, who had quit smoking cigarettes in 1986 and began eating more healthily after diagnosis.
The two women have become friends through the years through the common bond of having PBC. They are part of a support group called the PBCers. Members of the group all have PBC and they come together to share their feelings and fears, but moreover to report on wellness tips, research, new drugs and their own progress.
“We’re not getting together to complain or be down on life,” Edwards said. “We’re not sitting around saying, ‘Oh, we’re going to die.’ It’s not like that at all. We’re a positive group.”
Edwards’ PBC has progressed from a level 1 to a level 2 cirrhosis. At levels 3 and 4, a person is referred for a transplant.
For Edwards, a former school teacher, daily life is a grind; a painful and tiring existence.
“I told the doctor that I can’t function without something to alleviate the pain and discomfort; otherwise, I would sit in the chair and cry,” Edwards said. “I’m not going to stop living. I have to take six URSO pills a day because I start itching so bad. I have a lot of fatigue, but I keep going. I talk to my body. I’m so tired, but I say, ‘you’re going to walk.’ I push myself.”
Edwards also has adjusted her lifestyle. “I stay away from greasy food and meat. I eat vegetables and fruit, and I exercise and walk a mile each night,” she said.
Coming together
Roughly 20 people with PBC came together in the Southeast region that includes Georgia, South Carolina, North Carolina and Florida. The group, which Dougherty helped start two years ago, is part of a national support group.
The group became necessary because people suffering from PBC were being alienated by friends and their communities.
“People hear us say primary biliary cirrhosis and they immediately think it’s something we’ve brought on ourselves,” Edwards said. “Some of my family and friends don’t understand it. There is a lot of ignorance about PBC. They thought it was contagious. It’s not contagious.”
So, the PBCers was formed so people suffering from the disease can be with people who will not judge them.
“When I found out I had PBC, I said, ‘Oh my God, I don’t know anyone else I can talk to,” Edwards said. “So I went to the Web and I found the PBCers group. I wanted to find someone who understood what I was going through. I was horrified and distraught.”
Dougherty said that the group’s goals are to not only offer support for people suffering from the disease, but to raise awareness in communities and members of the U.S. Congress about the disease.
“We must raise awareness of the disease. More money is needed or should be made available by Congress so we can find ways to cure it,” Dougherty said. “We want to raise money like Jerry’s Kids, which helps raise money for roughly 2,500 children with muscular dystrophy. We have many more people suffering from PBC and many of those don’t even know it. …[And] we’re dying more than anyone else.”
All of the money raised by the support group through various community activities: craft sales, walks and doctors; goes to the American Liver Foundation to help raise awareness of PBC.
Symptoms of PBC
Both Edwards and Dougherty said they were misdiagnosed several times before being properly diagnosed with PBC. Because the disease is relatively uncommon and shares signs of other physical conditions, blood and liver function tests are required for proper diagnosis.
Signs of possible PBC include:
- Blood blisters on the skin and in the mouth
- Intense itching
- Fatigue
- Low platelet count
- Calcium deposits on the skin
- Dry eyes, mouth and vagina
- Hoarseness
- Pancreatic impairment
- Raynaud’s phenomenon (paling of fingers and toes)
- Schleroderma (i.e., tightness of the skin, esophageal malfunction)
Friday, Aug. 11, 2006
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