Cystic fibrosis patient, thriving 18 years after double lung transplant, credits ‘amazing’ medical advancements

Kate Willis was just 11-weeks-old when doctors diagnosed her with cystic fibrosis and told her parents she didn’t have long to live.

Her mum and dad were warned not to expect her to survive past childhood.

More than four decades later — thanks to advances in science and medical care — she’s now the busy mum of eight-year-old Adaline and works four days a week as a naturopath and dietician in the Central Queensland city of Rockhampton.

In 2006, she received a lifesaving double lung transplant at The Prince Charles Hospital in Brisbane.

Then two years ago, she started taking a triple combination of drugs — known collectively as Trikafta — which she says changed her quality of life, and “probably lengthened” it as well. 

“They’re amazing. I’ve put on 12 kilos. I’ve got so much more energy. Before I was on them, I was 45 kilos,” Ms Willis says. 

“I had really bad diabetes and … I didn’t have enough nutrition and fat on me. I could only work one day a week. I had liver disease as well.

Trikafta works by correcting the malfunctioning protein produced by the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which is mutated in people with the condition.

‘So many new medical treatments’

Cystic fibrosis affects the lungs, pancreas and other organs.

When the protein is not working correctly, it’s unable to help move chloride, a component of salt, to the body’s cells. without the chloride to attract water to the surface of cells, the mucus in various organs becomes thick and sticky.

In the lungs, the mucus clogs the airways and traps bacteria, which can lead to infections, inflammation and respiratory failure.

But Trikafta thins mucus in the lungs, so it can be coughed up easily.

The 43-year-old is one of tens of thousands of patients that have benefited from The Prince Charles Hospital’s respiratory investigations unit, which recently celebrated its 60th anniversary.

Queensland’s first thoracic physician

Unit founder Kevin Kennedy — who is in his 90s and still living near the hospital — was Queensland’s first thoracic physician, or respiratory specialist, having trained at London’s Brompton Hospital before returning to the sunshine state in the early 1960s.

These days, Queensland Health employs 55 thoracic physicians.

But in the early stages of Dr Kennedy’s career — years before man had even walked on the moon — sub-specialties, such as thoracic medicine, were only just starting to emerge in Australia.

“At that time … most of the physicians were general physicians,” he recalls.

So much so, that after sharing his plans to pursue a career in thoracic medicine with then-Queensland director-general of health and medical services, Dr Abraham Fryberg, he was told: “I don’t see much future in that. You should go away and think about whether you really want to go ahead with this.”

Dr Kennedy was advised that, if he did continue with plans to pursue thoracic medicine, he should seek some work as a university lecturer, otherwise “you could starve”.

But after returning from London in 1962, he started working at the Brisbane Chest Hospital, which would later become The Prince Charles Hospital.

The next year, as Beatlemania swept the world, Dr Kennedy established the state’s first respiratory investigations unit there.

Cystic fibrosis largely off the radar

In those early days of specialist thoracic medicine in Queensland, most of the hospital’s inpatients were being treated for tuberculosis (TB), an infectious disease caused by bacteria.

“When I came back to the Brisbane Chest Hospital, the day I started, there were about 200 people in hospital with TB, about 11 cardiac patients and about five other non-TB respiratory patients,” Dr Kennedy recalls.

Cystic fibrosis was largely off the radar of adult respiratory medicine then.

“There’s certainly much more effective treatment for them now and they’re diagnosed at birth, so treatment can be started very early — whereas going back many years, it would only have been when they were getting in their terminal phases that they were diagnosed.”

In the days before effective antibiotics to treat TB, Dr Kennedy recalls patients with the bacterial infection being hospitalised for six to 12 months.

“Those patients were only allowed visitors twice a week for two hours,” he says.

“Children weren’t allowed into the hospital because of the risk of TB.”

In an era without smart phones and FaceTime, the only way parents could see their children was to go to one end of the ward and look down at the playground.

They could wave to their children from there.

After years working in thoracic medicine, Dr Kennedy became medical superintendent of The Prince Charles Hospital in the 1970s, living in a house on hospital grounds with his wife and children.

“It was handy, of course,” he says with a grin.

“At the time, it was a requirement that the medical superintendent had to agree to live in the premises provided.”

He lived there until he retired in 1990.

First lung transplant

But his legacy continues on through the respiratory investigations unit.

In 1996, Queensland’s first lung transplant was performed at The Prince Charles Hospital. The success of the operation was aided by the unit’s extensive testing capacity to assess the patient’s suitability for the surgery.

Respiratory scientist Annette Dent, the longstanding director of the unit, joined in 1981.

Ms Dent says the unit assessed 7,803 patients in 2022-23, performing 17,714 tests — that’s more than 15 times the number carried out in its first year of operation, with 1,169 tests.

The unit sees a wide range of patients including those with asthma, lung cancer, cystic fibrosis, emphysema, chronic obstructive pulmonary disease, interstitial lung disease and, more recently, silicosis.

Thoracic physician Philip Masel, who has worked at The Prince Charles Hospital since 1997, says he’s amazed with the strides made in respiratory medicine in that time.

“I’ve seen incredible advances … which just shows the benefit of research and people doing research, got to keep that alive,” he says.

“Colleagues of mine are in that really academic field and I take my hat off to them — incredibly committed people battling away for a decade for these advances to come.

‘Transformed the field’

Advances such as the triple-therapy Trikafta, for instance, have been a game changer for many cystic fibrosis patients.

“It’s transformed the field,” Dr Masel says.

“It’s keeping people off the transplant list.”

Kate Willis admits she sometimes gets sad when she thinks about other children with cystic fibrosis that she saw in hospital growing up, who were not as fortunate as she was.

“I’ve had a lot of friends die,” she says.

After years of taking immunosuppressant drugs to avoid rejecting her donated lungs, Ms Willis has developed kidney disease and eventually, she’ll need a kidney transplant.

She’s hopeful her twin sister Brooke, or husband John, will be a live donor.

In the meantime, life is good.

“We’ve come so far,” she says.