Research breakthroughs needed for IPF

Oct 13 2016

As a former gym instructor, busy mother of two teenagers and non-smoker, 53-year-old Leanne Porter was the exemplar of good health.

Yet as she tried walking up a hill to her daughter’s school a year ago, she ran out of breath halfway and had to rest. With her lungs feeling leaden, she finally hailed a taxi to drive her the rest of the way.

“I felt like I was drowning, but without the water,” Leanne says. “All of a sudden I couldn’t do the simplest of tasks.”

Leanne has since been diagnosed with a degenerative lung disorder known as Idiopathic Pulmonary Fibrosis (IPF). Idiopathic means “cause unknown”, which is exceptionally worrying for patients and researchers alike.

What doctors know, sadly, is that the disease mostly proves fatal within two to three years of diagnosis. Two drugs that potentially slow the progress are not available on Australia’s Pharmaceutical Benefit Scheme, leaving lung transplantation as the only real alternative for those, like Leanne, who can withstand such major surgery.

“The lung tissue stiffens to the point where it has the same tensile strength as aluminium, so imagine trying to blow up a crushed soft-drink can with a straw. That's what it's like to breathe with end-stage IPF,” explains HMRI respiratory researcher Professor Darryl Knight.

“We think – and I stress, we think – there are a series of microscopic injuries to the lung that cause a wound response which just doesn’t stop. We’re now trying to understand how the disease progresses, using sophisticated proteomic imaging and genome sequencing.

“I'd like to think that we can repurpose existing drugs to fight IPF but I'm not ruling out developing a whole new range of therapies.”

While the disease is a devastating concern for Leanne's family she remains hopeful that new treatments can be found. “As a young mum I want to be around for a lot more years,” she says. “My daughters need me and, to be honest, I need them as well.”

IPF is one of many life-threatening conditions that urgently need more funding. You can help make life better for people like Leanne. Click here to donate today.