Deirdre wasn't completely unprepared. "People were dying of this disease in my family since I was 9 so all of us understood it profoundly," she said. She left her job as a Los Angeles prosecutor and flew to Michigan to be with her mother, as did her four brothers and sisters.

A family history
Deirdre was the same age as Sheila had been when her own mother-Deirdre's grandmother-died of IPF. The disease may go back even further in her family. No one knows for sure. IPF is still a relatively unknown and little understood disease that is frequently misdiagnosed because of its similarities to other lung diseases involving inflammation and scarring of the lungs.

There is no cure and no known cause for IPF although researchers are investigating a number of potential triggers such as a virus or a potential genetic link.

For Deirdre and her family, the possible familial connection is a reality. After her grandmother died, all six of her children were subsequently diagnosed with IPF. Four died from it, one died of something else, and the last one, an aunt, is still living with IPF at age 75.

Most Galvins went with the standard treatment-high doses of steroids-and experienced its distressing side effects. Several died about a year after their diagnoses. Marty died at age 45, leaving a wife and five children, just a few months after their dream home was finished. Ten years later, John died at age 51.

Choosing how to die
After seeing what her brothers went through, Deirdre's mother decided against hospital care, opting to die at home. Shortly after her diagnosis she was bedridden and on oxygen. Sheila's husband and all her children learned how to give her pain medications and use oxygen machines. A hospice worker prepared Sheila and her family for her death.

Four months after her diagnosis, Sheila died with her husband, all of her children and many other family members at her bedside. She was 52.

A profound impact
Donny, another of Sheila's brothers, died of IPF in 1993. Michael, another IPF-diagnosed brother, died in 1995 at age 63 from unrelated heart problems.

"We are a tightknit group already. Our family became closer as a result of our shared experiences with the disease and the common threat we face. With each death, family members essentially re-live the previous deaths, making each a much more intense and painful experience," Deirdre said.

The family evolved from being largely in denial about the disease and death to openly discussing it. Deirdre's Aunt Mary Kay, who was unable at the time to discuss her husband Marty's impending death from IPF, eventually became director of spiritual counseling at a large hospice center.

"This disease has had a dramatic impact on my family," Deirdre acknowledged.

Living with the threat of IPF
For a time, Deirdre pursued physicians and researchers, trying to interest them in studying her family and the genetics of IPF. She gave up after being turned down numerous times. However, since then several research studies have been initiated to study a possible genetic link.

Recently, she's become involved in the Mary D. Harris Memorial Foundation (MDMHF). The small nonprofit group was founded by Jeff Harris in memory of his wife, Mary, who died from IPF at age 45, leaving behind three school-age children. Mary Harris's sister was subsequently diagnosed with IPF, and their father likely died of the disease. The group's primary goal is to raise money for IPF research.

The Foundation has given Deirdre and her relatives hope and a cause to rally around. In the meantime, they cope with the reality of possibly developing IPF. Deirdre, 39, has undergone baseline lung function tests and x-rays, as have other siblings and cousins. The tests reveal nothing, as physicians and researchers know little about what early-stage IPF might look like. The disease has a tendency to become apparent in its later stages, when a significant percentage of lung capacity is already lost.

Family members must make major life decisions about marriage and family with few clues as to what the future may hold. This may mean choosing not to have children or having children early so the children will be older when their parents succumb to the disease.

For Deirdre, a local civic leader and activist, it means cramming as much as possible into the time she has. "I can't put off things the way other people can," she said. She spends as much time as she can with her children, ages 6 and 9, who are aware that IPF is in the family. At the same time, she pursues her work promoting awareness of the disease and serving on the board of the MDHMF.

The key is to not let her work toward a cure for IPF take over her life and to avoid becoming obsessed with the disease.

"We won't let this disease get us before it gets us," she said.