Victims of pulmonary fibrosis raise funds, and hope, for a cure from the fatal lung disease.
By Nora Fleming / Special to The Malibu Times
The 100 or so people gathered at the fourth annual Butterfly Garden Party in Malibu raised their arms, holding in their hands small packets that contained live Monarch butterflies. In unison, they opened the cage doors and a flurry of the large, orange and black butterflies filled the air, fluttering back into the wild.
The action represented loss, as well as hope for the future of those who suffer from pulmonary fibrosis, a fatal lung disease that has no known treatments and no known cure.
The event, sponsored by the Coalition for Pulmonary Fibrosis, a nonprofit founded in 2001 to raise awareness of the disease, raised roughly $125,000, and was held at Deirdre Roney and John Cadarette’s home in Malibu for the second year. Roney, a member of the coalition’s board of directors, has lost seven family members to the disease, spanning three generations.
“We tried to raise awareness [at the event]; I think we did that successfully,” Roney said. “We tried to let each guest know how important he or she is to our cause. So little is known and so little is being done [for the disease]. When you give to this cause, it makes a difference.”
Pulmonary fibrosis causes lung inflammation, which, in turn, causes scarring of the lungs that does not heal. This scarring prevents the lungs from taking in an adequate amount of oxygen to disperse to the rest of the organs. Because of this, other bodily functions also shut down in patients with PF. Many have been and continue to be misdiagnosed with resulting problems from the disease that can include pneumonia or heart attacks, among others.
To date, roughly 48,000 people are diagnosed with the disease annually, 40,000 of whom die. There are at least 128,000 people in the United States living with PF, though coalition CEO Mischa Michon said most health care professionals estimate the number at 200,000 to 1 million, due to a lack of a patient registry and many misdiagnoses.
Sandra and Jim Rock of Rancho Murrieta were presented with the Frank Cabral Humanitarian Award at the event for their community service work involving PF. Sandra Rock was diagnosed in 2001 and told she had three to five years to live, if she was lucky. Seven years since the diagnosis, Rock is still fighting, and still running a patient support group she started after she was diagnosed.
Rock’s group, which now has 60 members, meets monthly, but, she said, while new members keep joining, members also continue to pass away, due to the short life expectancies of PF patients post-diagnosis. The average life expectancy of PF patients after they are diagnosed is 2.7 years. Roughly 100 members have died since the group began, Rock said.
“It makes you realize it could be you at any time,” Rock said, of experiencing the loss of group members.
There are no known definite causes of PF, and while there have been signs of genetic links in families, like Roney’s, this only accounts for 10 to 15 percent of PF patients, Michon said.
It is this lack of knowledge on behalf of both patients and doctors that the coalition aims to change. A lack of funding has directly contributed to a lack of research, which has meant there is still a 50 percent misdiagnosis rate, Michon said. It’s also been challenging to rally the activist and support base for pulmonary fibrosis that many other diseases have due to the lack of widespread knowledge.
Roney said while researchers seem to have new ideas for possible treatments, they can’t be tested due to poor funding. She said she sees part of the problem as the varying agendas of stakeholders involved in disease research, such as universities, pharmaceutical companies and health organizations, whose interests often conflict.
Coalition staff and volunteers recently returned from their annual Washington D.C. trip where they worked to rally support for a House of Representatives bill, HR 6567, that will go into hearing at the beginning of the next congressional session.
The bill would provide $16 million for research funding during the course of five years. It would also provide for the creation of a patient registry to account for all the national patients affected with the disease and for the creation of a voluntary committee with members of national health organizations who could continue to lobby for research funding and disease education.
This would, in turn, Michon said, lead toward developing treatments and, optimistically, a cure for the disease.
“There are such few diseases in the country with no treatment. [Pulmonary fibrosis] is such a step child,” Michon said.
For families like the Rocks, the research cannot come soon enough.
“The hardest part of the disease is the uncertain, fatal disease factor,” Jim Rock said. “You wish against wish you could do anything to prevent it and are always living with a cloud overheard, waiting for the shoe to drop.”
More information about the Coalition for Pulmonary Fibrosis can be obtained online at www.coalitionforpf.org