Cystic fibrosis no longer just a childhood disease as Perth advocate Carolyn Boyd approaches 60

When Carolyn Boyd was born with cystic fibrosis in 1967, her parents were told she would not survive past her 10th birthday. Now, as she approaches her 60th, new data shows that more Australians with the genetic disorder are adults than children. The understanding of cystic fibrosis in the 1960s was extremely limited; Ms Boyd was not diagnosed until her fourth birthday despite numerous bouts of pneumonia. She spent her childhood having daily physiotherapy, breathing through a nebuliser and unable to walk and run around with other children. She never planned for a future because she was always told she would never make it to adulthood. Cystic fibrosis is the most common life-shortening genetic condition affecting Australians. It causes thick, sticky mucus to build up in the lungs and other organs, making it harder to breathe. A lung transplant became Ms Boyd’s only hope of survival. At 27, she moved from Perth to Sydney as transplants weren’t being done in Perth at the time. She was admitted to hospital on a Friday and her mum was told that if she didn’t get the call for a transplant by Sunday they would have to intubate her the week after and there would be no hope of a transplant. “I had very poor lung health,” she said. “I was hooked to a respirator and that was doing the breathing for me. “I didn’t have energy to move. Nobody gives you a book and says ‘this is how it’s going to feel when your body’s dying’. I got to the point where I thought if I don’t get this call (for a transplant) soon, then so be it.” On the Sunday evening she got the call that she was eligible for a double-lung transplant the next day. The transplant changed everything, giving her decades filled with travel, full-time work and the simple joy of breathing without difficulty. “I woke up from the transplant, and for the first time I could remember I had pink nails and pink lips. I used to always wear a red lipstick and put nail polish on because my lips were blue and my fingernails were blue,” Ms Boyd said. “Once I’d recovered, I loved doing the normal things — like working full-time and going night-clubbing and being able to climb 100 stairs and get up and dance on a dance floor for hours without having to be coughing and trying to gasp for my last breath.” Ms Boyd celebrates her transplant anniversary every November as a second birthday. On the 16th anniversary, she marked the milestone with her first skydive. She is now a board director of Cystic Fibrosis Australia and has remained close to her donor’s family, becoming godmother to one of the donor’s children. While Ms Boyd’s transplanted lungs are no longer affected by cystic fibrosis, she still lives with other complications of the disease. She is immunosuppressed, has pancreatic insufficiency and sinus disease. As she approaches her 60th birthday, Ms Boyd said she never forgets how “incredibly lucky” she is and is determined to ensure children diagnosed with cystic fibrosis today can benefit from treatments that were not available when she was growing up. “A child born with cystic fibrosis today, due to the treatments that we have, will only have it as a label,” she said. “They won’t know what it’s like to live with lung damage and the things that could eventually lead to their death. They will lead a normal life due to the treatments that we’ve got today.” Some of the latest developments in treatment include newborn screening, improved antibiotics, better nutritional support and, most significantly, CFTR modulator therapies that target the underlying genetic cause of the disease. The latest Australian Cystic Fibrosis Data Registry Report shows a record 3916 Australians are living with the disorder because people are living longer than ever before, not because more babies are being born with the condition. Adults now make up 62 per cent of Australians with it, compared to 56 per cent in 2020 and only one third when the registry was first published in 1999. Dr Jo Armstrong, CEO of Cystic Fibrosis Australia, said Ms Boyd represented a generation of Australians diagnosed with cystic fibrosis who were given a limited life expectancy. “Just a generation ago, many Australians born with CF were not expected to live into adulthood. Life was often shaped by repeated hospital stays, malnutrition, social isolation and the unnerving knowledge that time could be unfairly limited. Today, what is possible for people living with CF is a changing story,” she said. Coinciding with the latest data is the launch of CF: There’s More To The Story, a national multimedia exhibition by Melbourne photographer and filmmaker Alexandrena Parker, who lives with cystic fibrosis, featuring six Australians, including Ms Boyd. “This campaign asks people to look closer at the stories behind each face, the strength, complexity, resilience and hope, and the parts of CF that often remain invisible. There is no single story of CF anymore and that is what I wanted this work to show,” Ms Parker said.