Living with idiopathic pulmonary fibrosis (IPF)

In 2018, Mal Doyle was diagnosed with idiopathic pulmonary fibrosis (IPF), the most common type of pulmonary fibrosis and an area of significant unmet medical need, affecting approximately five million people worldwide. The disease is fatal, as it causes lung tissue to become thickened, scarred and stiff, leading to irreversible lung damage, severe breathing difficulties and, ultimately, respiratory failure. There is no cure for IPF; currently approved therapies can slow the decline in lung function, but unmet needs remain.

Diagnosed With IPF: A life-altering experience

To a passerby, Doyle looks like a healthy 85-year-old. But not every patient with IPF is like Doyle. Many are physically impaired, have difficulty performing simple daily activities due to profound breathlessness and require continuous supplemental oxygen to ease the burden of normal breathing. 

IPF symptoms include fatigue, gasping for breath, coughing and more. In Doyle’s case, he says he has more bad days than good. But despite his ailments, Doyle has a design for living.

"You have to look forward to something," he said. "Whether it’s your next meal, a cup of coffee, a phone call with a friend — you just have to look forward to tomorrow. That’s the key."

Supporting patients with pulmonary fibrosis

Doyle works on behalf of the Pulmonary Fibrosis Foundation (PFF) — a national organization dedicated to acting as a trusted resource for all who are affected by this disease. He started a PFF support group with hopes it would help others struggling with the condition.

At Bristol Myers Squibb, the wheels are in motion. 

Building a better future for PF patients

Several studies are underway to develop treatments that might slow the progression of fibrosis, according to Aryeh Fischer, Lung Fibrosis clinical development lead, who has extensive clinical care and research experience in lung fibrosis. "We’re researching multiple mechanisms and approaches, with the goal of helping those living with IPF and other forms of lung fibrosis."

One of these mechanisms is a lysophosphatidic acid receptor 1 (LPA₁) antagonist. When the lungs are injured, LPA is produced which leads to scarring (fibrosis) that reduces functioning of the lungs. This reduced function can lead to respiratory failure, which is fatal. Research has shown that blocking LPA₁ receptors may be beneficial in treating pulmonary fibrosis.

The high need for treatment for IPF and patients like Doyle propel Bristol Myers Squibb scientists to drive research forward.

"The thing about Mal that really strikes me is his positivity and how motivated he is to get the word out about this disease. He educates people and, in his engagement with Bristol Myers Squibb, he encourages us to advance our research in this area," said Bruce Ellsworth, PhD, scientific executive director, Medicinal Chemistry, Bristol Myers Squibb. "It’s really inspiring to our team to keep driving forward to find solutions for patients."