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Cystic fibrosis patients are meant to stay six feet apart. But these sisters are inseparable

By Kirsti Melville for Earshot

Sisters Ruby and Isobel Donaldson smile, Ruby has an arm around Isobel.
Sisters Isobel and Ruby Donaldson have bonded over their shared experience of cystic fibrosis.(Supplied: Matthew Casey/CFWA)

If sisters Ruby and Isobel strictly followed medical advice, they wouldn't ever be allowed in the same room. No touching, no playing, no hanging out.

And that's not because of coronavirus and social distancing — although the sisters are very vulnerable to the outbreak.

It's because of the "six-foot rule" — an everyday medical protocol that says people with cystic fibrosis (CF) should stay at least six feet, or two metres, away from each other.

It's so they don't spread bacteria like pseudomonas aeruginosa, which is potentially deadly — and sends shivers down the spine of anyone with CF.

It causes major infections in the lungs of people with CF and an irreparable decline in lung function.

At least half of people with CF harbour pseudomonas in their lungs and it's very hard to kill.

"It's in drains, it's in lakes, in dirt," says Caz Boyd, who has CF and is the president of Cystic Fibrosis WA.

"It's one of the really, really nasty bugs."

The bacteria are spread through direct and indirect contact; by kissing or holding hands, coughing, or by touching anything a person harbouring the bacteria has touched. Hence the six-foot rule.

But that rule is tricky for 11-year-old Ruby and her sister Isobel, 14.

Ruby and Isobel Donaldson sit on a couch with a medical device connected to a tube in their mouths.
Isobel and Ruby are inseparable, even when doing breathing exercises.(Supplied: Matthew Casey/CFWA)

The Perth sisters both have CF, but their mum Christine Donaldson says there's not much you can do to keep them apart or prevent them sharing bacteria.

"They've grown up sharing a car together, sharing a room together, picking up each other's toys," she says.

"You might as well try stopping the tide."

In a world that dictates that people with CF stay away from each other, Ruby and Isobel are impossibly close.

"We're best friends," Isobel says.

"You're not mine!" Ruby quips back, before they fall apart laughing.

Ms Boyd says a lot of young adults with CF self-isolate because that's what they've been taught.

"It's really sad because it's hard enough dealing with this disease, let alone being isolated," she says.

"They're missing out on the shared experience of living with CF that I had."

Before the six-foot-rule: camps and common rooms

That shared experience was possible because the six-foot rule didn't always exist.

CF patients used to share hospital rooms, play areas and kitchens.

"We'd all be hanging out together in the common room with the TV and a kitchen. We'd sit around the table and have a roast dinner together," Ms Boyd says.

Caz Boyd smiles at the camera in a close up photo.
Caz Boyd says the six-foot rule is isolating for many people.(Supplied: Caz Boyd)

And then there were the CF camps held all around the world.

"Kids were sharing bedrooms, playing games together, and then they were coming home sick with these bacteria in their lungs," says Jo-Anne Giles, who also has CF.

"Parents were chattering about this when I was a teenager in the 1980s. They were saying, 'My child didn't have this and now they do… I think it's the camp.'

"But doctors were saying that just wasn't possible."

But it was possible, and at 17 Ms Giles came home from her first camp with pseudomonas bacteria in her lungs.

It meant a long stay in hospital with intravenous antibiotics and irreversible damage to her already compromised lungs.

Two of her closest friends with CF died about the same time.

"I saw them die and I thought 'I don't want to die like that,'" Ms Giles says.

"It made me confront my own mortality. It really shook me."

More protected, but at what cost?

The introduction of the six-foot rule about a decade ago was devastating for many people with CF.

It meant renegotiating the parameters of friendships with the very people who just got you — those who shared the pain of not being able to breathe, of chronic coughing and the spectre of an early death.

"Some people even had relationships with other CFers and then all of a sudden the cross-infection issue came out," Ms Boyd says.

For many older people with CF, the thought of isolating themselves from old friends was too much. They continued to maintain contact, carefully managing the risk.

But it's isolating for young people who abide by the cross-infection protocol that dictates their world. It means no more camps. No more support groups. No more fundraising meetings. No mothers' groups.

People with CF are isolated in hospital wards. They wear protective masks when they go to CF clinics for check-ups.

Most clinics now use buzzers so patients don't have to sit in a waiting room together, mask or no mask.

Children often move schools if another child with CF is enrolled.

Parents phone ahead to check no other CFers will be present at parties, at CF association meetings, on sporting teams.

Ms Boyd worries about the impact this is having on the mental health of young people with CF.

"One of the things that got me through was being able to sit down and moan with CF friends.

"Social media platforms help but nothing's better than close contact with friends. I just think it's sad."

In 2019, the film Five Feet Apart, starring Cole Sprouse and Haley Lu Richardson, brought CF to the big screen.


It's about two teenagers with CF trying to bridge the six-foot divide by "stealing back" one foot of the distance they should stay apart.

That premise — which lends Five Feet Apart its title — is controversial.

Critics say the film promoted dangerous behaviour and showed a "Hollywood" version of CF.

But many people with CF were thrilled to see a major film portray the demands of living with the disease — even if it wasn't always entirely accurate.

But what about the 'six-foot-rule' if you have more than one child with CF?

Christine and Gerard Donaldson's first two children do not have CF.

But when Isobel, their third child, was born, they were called into the doctor's office and asked if they'd heard of CF.

"I knew it meant loads of physio, a lot of tablets, lots of hospitalisation and a load of being sick," Ms Donaldson says.

After having Isobel, the Donaldsons had a dilemma.

They had always wanted four children but trying for a fourth meant a one-in-four chance of having another child with CF.

They were considering genetic embryo testing, with all its prickly dilemmas — what to do if the embryo tests positive for CF, what to do with leftover embryos? — when they found out Christine was pregnant.

"I knew we wouldn't terminate if the baby had CF," Ms Donaldson says.

"I couldn't imagine what that would do to Isobel."

So they took the risk and had Ruby. They were sure she wouldn't have CF. She did.

Sisters Ruby and Isobel Donaldson smile while holding a number of pills in their hand.
The girls take about 40 to 50 tablets a day.(Supplied: Matthew Casey/CFWA)

Ruby and Isobel are inseparable, bonded by their shared experience of living with CF.

Twice a day, the sisters inhale 10 capsules of a mucus-loosening drug called Bronchitol before doing their breathing exercises together. Ten rounds of ten counts of five.

It takes a long time.

One, two, three, four, five.

Two, two, three, four, five.

Three, two, three, four five.

And on it goes. Morning and night. Every single day.

On top of this, the girls take about 40 to 50 tablets a day — Isobel can swallow "nine in one go", Ruby "only" six — and a range of antibiotics.

The kitchen bench is piled high with tablets and physio equipment and a whole cupboard is dedicated to their medication.

But it's this kind of physio and treatment, along with the new medication Orkambi, that means kids like Isobel and Ruby are likely to live well into adulthood, probably without needing a lung transplant.

A heartbreaking divide

Orkambi is a gamechanger. It's the first drug to treat the underlying causes of the disease, not just its symptoms.

But it's extraordinarily expensive — hundreds of thousands of dollars, per patient, for life.

Box of medication
Orkambi treats the underlying causes of the disease, not just its symptoms.(ABC Tropical North: Melissa Maddison)

It took a long, bitter fight and three rejections before the Australian Government finally agreed to put it on the PBS in 2018, making it readily accessible here.

But it's simply unaffordable for those living in countries where it's not subsidised, and it's creating a heartbreaking divide globally.

"If people in the US want to go on life-changing medications like Orkambi they have to pay full price for them, hundreds of thousands of dollars a year," Ms Boyd says.

And it's the same with lung transplants.

"I have friends with CF in the US who've had to try and fundraise something like $500,000 to pay for their lung transplant," says Ms Boyd, who had a double lung transplant in 1994.

"If you can't afford to buy the drugs or pay for a transplant, you miss out. It's horrific to think about."

By accident of birth, the future looks promising for Ruby and Isobel.

Sisters Ruby and Isobel Donaldson hug each other.
Physio, treatment, and a new medication mean kids like Isobel and Ruby are likely to live well into adulthood.(Supplied: Matthew Casey/CFWA)

As well as benefiting from the early trial of Orkambi, they're part of a very small group of Australian children with CF trialling a new drug.

Isobel's lung function improved by 19 per cent after just two weeks.

"The hope is that in 10 years, people with cystic fibrosis will no longer be dying from CF lung-related disease," Ms Boyd says.

"I was diagnosed in 1971 and I've seen so many changes and this, finally, is almost like the cure we've all been waiting for."

For the first time, people with CF are having to think about old age and superannuation.

"When I was born, the life expectancy was 15. By the time I was 15, it had gone up to 20," Ms Giles says.

Ms Giles is a relative anomaly in the CF community. She had a double lung transplant in 2015 and now, at 52, she's "old".

"I used to think I wouldn't make 30. Then I got to 30 and I thought I might make 40. Now I think I might make 60," she says.

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