Cystic fibrosis sufferer Cody Sheehan reveals what it’s like to ‘breathe through a straw’

A man born with a crippling lung condition that made it feel like he was “breathing through a straw” has been given a second chance at life with a surprise lung transplant.

Cody Sheehan was six months old when his parents received the devastating news that their first child had been born with cystic fibrosis, a life-limiting genetic condition that causes severe damage to the lungs and digestive tract.

Mark and Bridget Sheehan’s world came crashing down when doctors said their beloved son was unlikely to make it to primary school.

But their ‘miracle’ boy defied the odds by fighting until the age of 28, when he underwent a double lung and liver transplant in March 2017 in an operation that lasted 17 hours at Westmead Hospital in Sydney.

“I didn’t struggle anymore, I just lay there and let my lungs do the work. It was amazing, just incredible,” Cody, now 32, told the Daily Mail Australia.

Mr. Sheehan (pictured in 2019) wasn't expected to make it to primary school, but he braved the odds by fighting until he got a double lung and liver transplant

Cody Sheehan (left, as a teenager, and right, in 2019) is one of 3,500 born with cystic fibrosis, a life-limiting genetic condition that causes severe damage to the lungs and digestive tract

Before his transplant, Cody’s life revolved around daily cycles of drugs, therapies and treatments.

He was so weak that he relied on a breathing machine called a nebulizer, often spending weeks in the hospital to restore his lung function.

His condition deteriorated so badly by age 25 that he developed hypoxia, a dangerous condition in which the blood doesn’t carry enough oxygen to your tissues to keep you alive.

Cody was put on the transplant list in early 2016, but after two ‘dry runs’ – in which a patient is called for a transplant but the surgeon decides the organ is not a good match – his family quickly lost hope.

It took a year for Cody to get the phone call that changed his life.

“I remember waking up like it was yesterday,” he said.

“It felt like there was an elephant-like pressure on my chest, but I could finally breathe.”

In the four years since, Cody has taken every opportunity and has done everything he can to support fellow patients by launching CF Avengers, a charity that supports people with cystic fibrosis.

“I knew I had to get as strong and as healthy as possible so that I could get out there and take on the world in any way I could. I now live for two people,’ he said.

Papa Mark, a travel writer from Charleston, South Carolina, sometimes struggles to believe how his sick little boy became the strong, determined man he knows today.

Papa Mark (right), a Charleston, South Carolina travel writer, sometimes struggles to believe how his sick little boy became the strong, determined man he knows today

Papa Mark (right), a Charleston, South Carolina travel writer, sometimes struggles to believe how his sick little boy became the strong, determined man he knows today

Cystic Fibrosis Explained

Cystic fibrosis is a genetic disorder that affects the way cells produce mucus and sweat, causing severe damage to the lungs, digestive system, and other organs of the body.

Jackie Fraser is one of only 3,500 Australians living with CF, according to Victoria’s statistics Better health database.

People with CF have a faulty gene that causes body fluids to become thick and sticky. Instead of acting as lubricants, as they should, the fluids block the airways, ducts and passageways, most severely in the lungs and pancreas.

CF is incurable and progressive, meaning it worsens with age. It requires daily care, but patients can still work and enjoy a fulfilling life if their condition is treated appropriately.

Improvements in screening and treatment mean that people with CF now have a significantly higher quality of life than in previous decades. Most patients with cystic fibrosis now live in their late 30s and 40s, and some live well into their 50s.

Life expectancy for patients with cystic fibrosis in Australia is only 37 years, but Cystic Fibrosis Australia is working to extend this to 50 years by 2025 through clinical quality improvement programs, research and advocacy to ensure equal access to treatment.

Source: Better health Victoria

“When we got Cody’s diagnosis, I wanted to pull the covers over my wife, my son, and myself,” he said.

“But Bridget said we won’t talk about Cody’s future until he’s 21. She kept me going because she didn’t even want to entertain the idea that he might not survive.’

While his donor’s identity has been suppressed under Australian law, Cody has learned that the person who gave him a second chance at life was a 17-year-old boy.

A year after his transplant, he was given the opportunity to write an anonymous letter to his donor’s next of kin to express his deep gratitude.

He was overjoyed to receive two letters from his donor’s mother.

Every year on the anniversary of his transplant, Cody sends her a note informing her of all he has accomplished.

Cody was put on the transplant list in early 2016, but his family quickly lost hope after two 'dry runs'

Cody was put on the transplant list in early 2016, but after two ‘dry runs’ his family quickly lost hope

“It was wonderful to have that bond with them. It’s not often people reply, so I’m just really grateful,” he said.

Mark is overcome with emotion as he considers how close he came to losing his son.

“There is no instrument to measure the sheer joy we have received from a family that must have suffered so much,” he said.

Cystic fibrosis is the most common genetic disease in Australia, affecting approximately 3,500 men and women across the country.

Sufferers develop an abnormal amount of thick, sticky mucus in the lungs and digestive tract, leading to recurrent infections and irreversible damage. Lung failure is the most common cause of death.

Cody Sheehan (pictured as a child) wouldn't make it to grade school

Cody Sheehan (pictured as a child) wouldn’t make it to grade school

The founder of CF Avengers (pictured) now lives for two, after receiving a double lung and liver transplant from a 17-year-old donor

The founder of CF Avengers (pictured) now lives for two, after receiving a double lung and liver transplant from a 17-year-old donor

There is no cure, but there is game-changing medication that can dramatically improve the lives of people with cystic fibrosis.

Cody is a passionate campaigner for the Trikafta grant, a breakthrough therapy drug manufactured by Vertex that has been shown to thin the mucus in the lungs of 90 percent of CF patients.

But because it’s not approved for government funding in Australia, the drug currently costs nearly $300,000 a year — a dazzling amount that puts it out of reach for the vast majority of people.

To make it affordable, the drug must be added to the Pharmaceutical Benefits Scheme (PBS) for anyone with cystic fibrosis.

Trikafta is already available and reimbursed in 17 countries, including Denmark, Finland, Germany, Ireland, Switzerland and the UK, a fact that Cody and other CF patients find “incredibly frustrating.”

Since June, four new agreements have also been signed with drug administrations in France, Italy, Austria and the Czech Republic.

Cody is a passionate campaigner for funding for Trikafta, a therapy drug manufactured by Vertex that has been shown to thin mucus in the lungs of 90 percent of CF patients

Cody is a passionate campaigner for funding for Trikafta, a therapy drug manufactured by Vertex that has been shown to thin mucus in the lungs of 90 percent of CF patients

The pharmaceutical company behind the medication, Vertex Pharmaceuticals, has three times applied for approval of Trikafta under the Australian PBS.

Some progress was made in August when the drug was given the go-ahead for addition to the PBS for people with a specific mutation of the condition.

But this is only true for about 20 percent of CF patients.

Vertex said the decision meant only 395 patients would be eligible to access Trikafta at a subsidized rate, a third of whom were already participating in clinical trials or part of the compassionate program.

Efforts by advocacy groups such as Cody’s previously led to the subsidization of Orkambi, a $250,000-a-year drug that was added to the PBS in August 2018.

After a protracted battle between CF support organizations and the federal government, costs for patients were reduced to just over $6 a month.

Cody believes that the same should now be done for Trikafta, with access for everyone.

Mark Sheehan (left) shared Cody's (right) incredible story in his new book, Breathing Through a Straw, Cody & I

Mark Sheehan (left) shared Cody’s (right) incredible story in his new book, Breathing Through a Straw, Cody & I

“Not only does this drug slow the progression of CF, it shows signs that it can repair parts of the damage,” he said.

“It’s going to be expensive, no doubt about that, but what’s life worth?”

Cody argues that paying for the drug would help not only thousands of CF patients, but also the Australian health care system, which has been stretched to its limits since the start of the pandemic in early 2020.

“A really easy way to look at why it’s important is that if patients don’t get access to Trikafta, they’ll end up needing multiple organ transplants,” he said.

“I don’t think you can imagine the pressure that hospitals and clinics and organ donation institutions will take off.”

Mark Sheehan has shared his son’s incredible story in his new book, Breathing through a straw, Cody & I, that is to order online for $29.99.

For more information on cystic fibrosis, treatment, and clinical trials, visit: Cystic Fibrosis Australia.

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