A family in Teaneck, NJ, lost two adult sisters in three years. An EMT who once breezed through her physical exams now can’t walk up a flight of stairs. A 9/11 volunteer will die soon if he doesn’t receive a lung transplant.
These people have succumbed to or are battling a disease that doctors don’t fully understand and some have only recently learned to diagnose: pulmonary fibrosis.
Literally “scarring of the lungs,” pulmonary fibrosis affects 128,000 Americans and kills as many people every year as breast cancer but garners only a fraction of the attention. There is no known treatment, and its victims typically die within five years of diagnosis.
While researchers have no hard data yet on whether New Yorkers represent a disproportionate number of cases, physicians have noted a high number of diagnoses among 9/11 first responders.
More than 50,000 first responders were exposed to a host of poisons that hung in the air after the World Trade Center was attacked. Tissue analysis of seven first responders found everything from asbestos to small shards of glass, according to a study by Mount Sinai School of Medicine and Memorial Sloan-Kettering.
One of the problems with identifying pulmonary fibrosis is that not everyone has heard of it.
“It’s not like lung cancer or emphysema. I would say the general population does not know about it,” said Dr. Marvin Schwarz, a pulmonologist at the University of Colorado and a leading expert on pulmonary fibrosis. “That’s slowly changing. The awareness of physicians is changing. It is now something that they look for when somebody has a family history or an exposure like 9/11.”
The swiftness of the disease also makes it difficult to study. So much scar tissue builds up in the lungs of people with pulmonary fibrosis that they can no longer exchange oxygen and carbon dioxide. Within two to five years, patients simply lose the ability to breathe. The only cure currently available to patients is a lung transplant, and even that offers a five-year survival rate of only 40%.
Environmental toxins are not the only causes of pulmonary fibrosis. Chemotherapy, smoking, diseases such as lupus and even the antibiotic nitrofurantoin have been found to cause the disease.
Advocates say that doctors need to use caution before prescribing medication known to cause pulmonary fibrosis. And patients, particularly cancer patients taking bleomycin or other chemotherapies associated with the disease, need to be aware of family history and other risk factors before plunging ahead with potentially harmful treatments.
“People who have survived cancer think that what they’ve overcome is the worse thing they’ll ever overcome. And then they get the pulmonary-fibrosis diagnoses, and it’s worse,” said Teresa Barnes, vice president of patient outreach and support for the Coalition for Pulmonary Fibrosis.
While the causes are many, the treatments are sadly few and far between, said Barnes, who became involved in pulmonary-fibrosis advocacy to help find a cure for the disease that killed her father 15 years ago.
“Fifteen years later, the treatment options are still the same, and that is not acceptable,” Barnes said.
Ongoing clinical trials for at least 10 different drugs meant to prevent or reverse scarring in the lungs have shown some effect, but no cure besides a lung transplant currently exists. Until a cure is found, researchers are trying hard to improve the quality of life of people with the disease.
One drug, pirfenidone, has shown promise in improving lung function in early clinical trials here and in Europe and became available for pulmonary-fibrosis treatment in Europe last year. Another drug, BIBF 1120, also seems to prolong lung function in patients, according to a study published last year in the New England Journal of Medicine.
“People who suffer from IPF are in great need of a safe and effective treatment to preserve lung function so they can maintain physical activity and reduce the impact on their independence for as long as possible,” Dr. Luca Richeldi, lead author and director of the Research Centre for Rare Lung Diseases at the University of Modena and Reggio Emilia, in Italy, said in a written statement. “The positive trends in slowing the decline in lung function over time . . . and improving the quality of life.”
The research is heartening for Schwarz, who has watched the field gain more attention as patients and physicians learn about the disease.
“I remember when our group here [in Colorado] was one of the only ones that had any interest and were studying it,” Schwarz said. “People are recognizing that they have a lot of patients with this problem and that there is no treatment for it. Doctors are recognizing the frustration and the sadness of this disease.”