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UW Hospital Support Group for People with Cystic Fibrosis is Controversial

DAVID WAHLBERG
Wisconsin State Journal
608-252-6125
September 14, 2007

Deirdre Marsh, 36, of Waunakee, has been married 10 years.

Jim Feldhausen, 37, of Fitchburg, has a 2-year-old daughter.

Shane Alloway, 21, of Spring Green, has a new job.

Not long ago, they wouldn't have been expected to finish elementary school.

Marsh, Feldhausen and Alloway have cystic fibrosis, a genetic disorder in which mucus builds up in the lungs and digestive system, frequently causing deadly infections.

Cystic fibrosis has been known for years as a fatal childhood condition. But now it is also becoming an adult disease, with 45 percent of patients today age 18 or older.

Medical advances - including newborn screening, available in most states largely because of a UW-Madison study - are keeping patients alive longer.

A few decades ago, patients usually died by age 10. The average lifespan now is 37. Doctors expect babies born with cystic fibrosis today to survive into their 50s and 60s.

Many patients are experiencing milestones never dreamed of before: high school, college, careers, marriage, children, even grandchildren. But with increased longevity comes challenges, including the isolation of living with a chronic illness still associated with frail children.

UW-Madison also has been active on that front. UW Hospital formed one of the nation's few support groups for adults who have cystic fibrosis, a seemingly routine step that is controversial.

Cystic fibrosis patients are susceptible to infections, and the bacteria some patients carry can become resistant to antibiotics. So doctors generally tell them not to intermingle. In the 1980s, camps for children with the disease were shut down for this reason.

"We don't recommend people with cystic fibrosis getting together," said Leslie Hazle, director of patient resources for the Cystic Fibrosis Foundation.

The UW Hospital support group, operated under strict hygiene rules, is sanctioned by the hospital's infection control doctors. Damien Wilson, a social worker at UW Hospital, and Sue Osmond, a nurse, formed the support group three years ago. They will present a DVD about it at the foundation's national conference in California next month.

"We could be ridiculed for what we're doing," Wilson said. Nurses and social workers at other hospitals have told him that doctors they work with wouldn't approve of such a group, he said.

Participants say the group offers an important outlet because they haven't had many role models to help them navigate their lives.

"I never thought I'd be married. I never thought I'd live to be 36," said Marsh, who is going back to school to become a nurse. "We have a lot of things to think about."

Feldhausen and his wife, Colleen, said they weren't sure if they should have children. Cystic fibrosis hinders male fertility, so expensive procedures could be involved.

The support group and hospital staff helped answer their question. The couple adopted Josephine, who turns 2 this month, from China last year.

"The last thing I wanted to do was get a child and then die," Feldhausen said. "But I've decided I'm going to make the most of it."

New treatments

Cystic fibrosis, which affects about 30,000 Americans, is mostly a lung disease. Mucus clogs the lungs, making breathing difficult and causing inflammation and infections.

Patients take antibiotics, often requiring intravenous delivery in the hospital. Most receive daily physical therapy - chest pounding from another person or use of a vibrating vest - to loosen mucus from their lungs.

But the disorder also affects the pancreas, causing digestion problems. Patients don't absorb important nutrients from food. To compensate, they take vitamins and enzymes and eat high-calorie, high-fat diets.

In 1989, scientists discovered the gene mutation that causes cystic fibrosis, leading to hope that gene therapy would cure the disease. But trials proved disappointing, in part because viruses used to deliver the corrective genes to patients caused inflammation.

New medications have helped, however. Pulmozyme, the first drug approved specifically for cystic fibrosis, in 1994, helps thin out mucus. An aerosolized antibiotic, called TOBI, was approved three years later.

A newer arrival is hypertonic saline, an inhaled saltwater solution. It was developed after doctors noticed that cystic fibrosis patients who were surfers had fewer lung problems.

Studies are under way of other drugs, including pills that would correct defective chloride channels in patients' cells. The defect, a hallmark of the disease, creates salt build up and leads to thick mucus.

Approval of such a pill could dramatically reduce lung problems.

"I think that will happen within five years," said Dr. Phil Farrell, a pediatric pulmonologist at UW-Madison who specializes in cystic fibrosis.

Newborn screening

Farrell, former dean of the UW School of Medicine and Public Health, helped oversee the main study that led to one of the biggest developments in cystic fibrosis: newborn screening. UW-Madison bioethicist Norm Fost was co-director.

From 1985 to 1994, researchers tested the 650,000 babies born in Wisconsin for cystic fibrosis, through blood samples taken by pricking their heels. The blood test isn't conclusive; a positive result must be followed by a sweat test. In that test, chloride, a component of salt, is measured in sweat.

For half of the babies in the study, parents were immediately notified of positive blood tests. For the other half, parents didn't hear the news until the children reached age 4.

By comparing the two groups, the study determined that early diagnosis of cystic fibrosis and early use of enzymes and other treatments lead to increased height, weight and cognitive function. Such measures also may improve lung function and longevity.

Wisconsin, by initiating newborn screening in 1985, became the second state to do so, after Colorado. In 2004, the U.S. Centers for Disease Control and Prevention cited the Wisconsin study as its main reason for recommending that all states begin such testing.

Today, 34 states conduct the testing; another eight are expected to join the list this year, Farrell said.

Of the 70,000 babies screened each year in Wisconsin, about 150 have positive blood tests, said Jessica Burda, a spokeswoman for the Wisconsin State Laboratory of Hygiene, which analyzes the samples.

About 20 of those babies are found to have cystic fibrosis, Burda said.

Most of the 100 children and young adults with the disease who remain in Farrell's study are thriving, he said, especially when compared to an 8-year-old girl who was his first cystic fibrosis patient in 1966.

The girl, half normal size, had blue skin and clubbed fingers from a lack of oxygen. She suffocated to death on her respiratory secretions.

"It was absolutely devastating," Farrell said. "It's incredible how things have changed."

"Next best thing"

When Marsh was diagnosed at age 4, doctors told her parents she wouldn't live past 8.

After she broke that barrier, they said she might last until 12. When she continued to survive, they said 16.

Though she had to roll an IV pole through her college dorm and be given a special discharge from the hospital to attend graduation, Marsh received a communications degree from UW-Whitewater in 1994.

She worked at the Wisconsin Historical Society before taking a job last year as a UW Hospital clerk. She has enrolled at Madison Area Technical College, hoping to join the nursing program.

"I've been blessed to hang on long enough to get to the next best thing," Marsh said.

She and her husband, Hans Marsh, a lumber salesman, celebrated their 10th anniversary last month.

He knew little about the disease when they met but has become a conscientious coach. He reminds her to go the doctor when her cough deepens and stays far away when he gets a cold.

They've managed to go on several canoeing trips in northern Minnesota. They bring inhalers for her medications instead of the nebulizers she typically uses, which require an air compressor.

"We work with her limitations, but we haven't really had to change our lifestyles that much," he said.

Motivated to live

Feldhausen, a Green Bay native, wasn't diagnosed until age 33, despite having respiratory infections and digestion problems throughout his life.

He appears to have a relatively mild form of the disease, though it still involves many medical complications, he said.

Shortly after the diagnosis, when he tried to check in for a doctor appointment, the clerk asked where his son was - a sign that even the medical system still views cystic fibrosis as a children's disease.

At 6 foot, Feldhausen weighed just 165 pounds. Now that he takes enzymes and supplements, he's up to 205 pounds.

Formerly a dental technician in the Navy, he works in human resources at UW-Madison's School of Education.

Feldhausen spends an hour every morning and 90 minutes every night doing physical therapy and inhaling medications to clear mucus from his lungs.

The hypertonic saline inspired by surfers has helped him stay out of the hospital, he said. He stays fit by playing in volleyball leagues.

"I'm pretty motivated to stay healthy," he said, looking at daughter Josephine one recent evening at his home. While he fingered her hair, she wiggled on his lap.

X-raying a sister

When Alloway was diagnosed at 15 months old, the prognosis still wasn't good, his mother Sherry Alloway said. Doctors said he might live to age 10 or 12.

"It was pretty grim," she said.

But her 21-year-old son, who plays football and basketball with friends and lifts weights, has stayed relatively healthy, having been hospitalized for cystic fibrosis treatment only once.

He graduated this summer from the radiology tech program at UW Hospital and has started working in that role there. His disease is one reason he chose a health-care field, he said.

Alloway soon might be taking X-rays of his 8-year-old sister Ashley, who also has cystic fibrosis and goes to the hospital every few months for check-ups.

Other than taking his medications and reminding friends not to smoke around him, Alloway said his life is fairly normal - regardless of the statistics he and other patients face.

"I've been pretty lucky," he said. "It makes you appreciate the life you have right now."

Cystic fibrosis

What it is: A genetic disorder producing thick mucus that clogs the lungs and digestive system.

Symptoms: Coughing, wheezing, lung infections, poor growth, salty skin, difficult bowel movements.

Who has it: About 30,000 Americans, most diagnosed as children; Another 10 million Americans are carriers.

The prognosis: The average lifespan is 37 and increasing.

The support group
To learn more about the UW Hospital support group for adults with cystic fibrosis: contact Sue Osmond, at 262-5307 or sosmond@uwhealth.org, or Damien Wilson, at 263-6525 or dwilson@uwhealth.org.

On the Internet

For more information: www.cff.org or www.uwcfcenter.org.

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Please Note: This article and the accompanying photos are an electronic clipping from the Wisconsin State Journal from September 17, 2007.

Related Stories:

• Why do Some Carry Cystic Fibrosis Mutation?

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