Over the course of 10 years, a woman watched as five of her family members died from a perplexing lung disease with no cure that can kill patients as soon as a year from diagnosis. Despite its severity and the thousands of people affected by the disease every year, it remains widely unknown to the American public.
During a disastrous period starting in 1996, Teresa Barnes of Memphis, Tennessee, lost her father, aunt and three uncles to idiopathic pulmonary fibrosis, or IPF syndrome, an incurable disease with no known cause which affects 30,000 to 40,000 new people every year in the United States. The syndrome increasingly scars and thickens the tissue of the lungs, making breathing laborious. As the lungs struggle to distribute oxygen to the bloodstream, patients experience worsening coughs and shortness of breath as the brain and other vital organs struggle for nutrients.
“Imagine taking a pillow case and putting it over your face and trying to breathe,” Barnes tells PEOPLE. “Patients are struggling and there’s not one thing they can do to make things better.”
According to the Mayo Clinic, symptoms of the disease include a dry cough, fatigue, unexplained weight loss, aching muscles and joints, and a clubbing of the fingertips. Most people who are affected are between the ages 50 to 70.
On average, patients with IPF succumb to the disease within three to five years after first showing signs of symptoms, according to the National Institute of Health. Some patients can die sooner, or much later, depending on how the disease progresses for them. Though the progression can vary from patient to patient, none will see their health return to the status it once was. Approximately 132,000 people in the U.S. have the disease and about 40,000 die annually from it, according to NIH.
“It was torment to watch a family member suffering like that,” Barnes says. “It takes a toll every day.”
One by one, Barnes’ family went from experiencing mild breathlessness to becoming completely dependent on an oxygen tank in a matter of months. Barnes says her father, who was the first to exhibit symptoms, was not able to walk across the room without feeling as if he was going to faint in the time leading up to his death. Barnes has not been diagnosed with IPF, but the fear of it remains in the back of her mind, she says.
“Unfortunately, when people get IPF, they die, it’s just a matter of when and how,” Bill Vick, 78, tells PEOPLE. “It’s the terror and horror of feeling like you’re being waterboarded day after day, and week after week, until you die.”
Vick, of Plano, Texas, has dedicated his time to raising awareness for IPF research after he was diagnosed with the disease in September 2011 and told he had three years to live. He started PF Warrior, a volunteer group based out of Texas that works to spread awareness about the disease and provide tools and resources to patients. One of the issues that Vick and Barnes have worked on is getting NIH to invest more funding into research for the disease. But without name recognition, federal funding is hard to come by, and the general public remains uninformed.
“For most patients, if they’re telling a friend about the disease, most will not know what they’re talking about,” Barnes says. “It’s difficult for them because they suffer in isolation.”
Because IPF patients die so quickly after showing symptoms, it’s difficult for a patient to become the face of the disease and raise awareness and name-recognition. Yet, notable people have died from the disease, such as Jerry Lewis, Evil Knievel, Marlon Brando and Congressman Charlie Norwood of Georgia.
As of today, Barnes and Vick say the only way to survive the disease is with a lung transplant. But there have been recent developments in therapies that may slow the progression of the disease and research into stem-cell therapy has proven promising. Until these therapies come to market, much of the IPF community is now concentrating on sustaining the quality of life of patients. Advancements can include modernizing portable oxygen machines to be controlled with a smartphone or designing them to be less obtrusive.
Three Lakes Partners, a philanthropic organization that raises funds for IPF research, launched a $1 million competition for entrepreneurs and designers who have promising ideas for improving quality of life for patients. The American Lung Association recently announced a partnership with the organization to accelerate the development of resources for patients. In the face of a lack of funding, it is efforts like this that will “spark” progress toward a better future for patients.
“We have to make sure people don’t give up, patients don’t give up, and the families who are left behind when patients are lost don’t give up,” Barnes says. “We have to keep fighting.”