Told she had a mysterious, incurable disease that kills as many people a year as breast cancer, suburban teenager Lilianna Aguirre reacted the way many people diagnosed with pulmonary fibrosis do.
"I had no idea what it was until I Googled it. I had never heard of it," says Aguirre, who now is waiting for a lung transplant.
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Pulmonary fibrosis factsŸ About 40,000 Americans die each year from the disease, about the same as breast cancer.
Ÿ While the disease sometimes runs in families, and smoking can be a factor, most cases are idiopathic -- meaning they have no known cause.
Ÿ A recent study shows that up to 50 percent of patients may be initially misdiagnosed.
Ÿ Median age at the time of diagnosis is 63, but it has been found in patients from teenagers to octogenarians.
Ÿ Symptoms include a chronic cough, shortness of breath, fatigue, loss of appetite and weight, and chest discomfort.
Ÿ There is no cure or FDA-approved therapies, but treatments include medicines to lessen the symptoms, supplemental oxygen, pulmonary rehabilitation therapy, and lung transplantation.
Source: Pulmonary Fibrosis Foundation
"I just thought I was getting old and getting fat," admits Jeffery Peters, 67, a former runner from Streamwood who learned that pulmonary fibrosis was the reason a short hike left him out of breath. "When you find out it's incurable and can kill you fast, it's scary."
"Until a family has been given a diagnosis of pulmonary fibrosis, they've never heard of it," says Jennifer Bulandr, director of community affairs for the Pulmonary Fibrosis Foundation, which is hosting an inaugural international summit today through Saturday in Chicago. The summit, with a live webcast at ipfsummit2011.com, is drawing experts from Peru, China, India, Japan, France and Nigeria.
Bulandr, of Barrington Hills, first heard of pulmonary fibrosis in 2002, nine weeks before it killed her father, John A. Lukasik, at age 58. The lack of information and support spurred her to join the movement to educate people about the disease.
Pulmonary fibrosis is a scarring or thickening of the lungs. The disease, which kills 40,000 Americans each year, has no cure or even a standard treatment. In most cases, it is idiopathic, which means that doctors don't know what causes it.
It damages the lung tissue that transfers oxygen to the bloodstream and other organs, and many patients live only three to five years after diagnosis, according to the National Institutes of Health. Some live more than a decade.
"Anything strenuous is more and more difficult all the time," says Peters, who used to walk his Streamwood neighborhood with his wife, Michele. Unemployed since the company where he worked closed, Peters takes drugs as part of a research study and uses his prescribed round-the-clock oxygen tank only when he feels the need.
"You find your level and then you drop off. You stay at that level for a while and then you drop off again," Peters says of his disease.
"When I tell people what my dad has, I get that blank stare: 'He has what?'" says social worker Courtney Firak, Peters' stepdaughter, who recently joined the Pulmonary Fibrosis Foundation staff. "That's sort of a frustrating aspect to it."
While the breast cancer community has built an impressive support system that encompasses everything from donations at the checkout line to football players wearing pink cleats, pulmonary fibrosis is using its 10th-anniversary dinner Friday at the Field Museum to launch its campaign: "New Decade, New Reach: Together we'll make a difference in pulmonary fibrosis."
"I tell my mom that this disease just kind of grabs you when it wants to," says Aguirre, now 22 and living in Northlake with her mom and family. She has faced adversity before. A victim of another lung ailment as a toddler, Aguirre suffered a brain hemorrhage when she was pregnant and delivered a stillborn daughter in 2008.
While many people her age are consumed with holiday shopping deals and gift lists, the only list that matters to Aguirre is the transplant list of patients waiting to receive new lungs.
"On that list, I'm No. 34 nationally and I'm No. 1 in Illinois," says Aguirre, who will attend the IPF Summit unless she gets a call from doctors that they found donor lungs. "I started crying when my doctor told me (about the need for a lung transplant), but now I'm really, really ready for it. I'm not afraid to die. I'm not scared at all."
The more research and attention given the disease, the better, says Bulandr, who notes that the support helps patients go on with their lives.
"They go on cruises. They go on vacation. We have one who goes swimming with her oxygen. We have another who goes roller skating. Another rides her bicycle everywhere," Bulandr says. "But we don't have that happy story, such as a 30-year-old with two small children who beat her breast cancer and is going to live a long and happy life now."
Publicity -- even from the deaths of personalities such as Evel Knievel, Robert Goulet, Lura Lynn Ryan and the fathers of U.S. Sen. Mark Kirk and State Sen. John Cullerton -- can be used to educate the public. Patients use whatever breath they have to speak out about the disease.
"I'll do whatever I can," Aguirre says, "to spread the word."