Advocate’s brother died awaiting lung transplant

Cahir O’Hagan, a 25-year-old resident of Clonoe in County Tyrone, passed away while awaiting a double lung transplant. He suffered from cystic fibrosis (CF), a hereditary illness characterized by the accumulation of thick mucus in the lungs and digestive system. Due to the specific genetic composition of Cahir’s condition, he was not responsive to life-extending modulator medications like Kaftrio, Symkevi, and Orkambi. While these pharmaceuticals are effective in extending and enhancing the lives of approximately 90% of individuals with CF, they are ineffective for the remaining 10% of the CF population who possess distinct genetic mutations. One year following Cahir’s passing, his brother, Paul O’Hagan, has urged increased efforts to ensure that all individuals afflicted with CF can access vital medications. “Ten percent – it isn’t a number, it isn’t a percentage, it isn’t a statistic.” “It’s real people, families, worrying, struggling, not knowing if their loved one is going to survive.” In Northern Ireland, over 500 individuals are living with cystic fibrosis, with approximately two-fifths of this group being adults. Although Cahir was one of hundreds in the region with the condition, Paul characterized him as “one in a million.” Paul stated, “He was a very charismatic, driven, motivated individual.” “Whatever was threw at him, whether it be in life or with CF, he just always bounced back.” In July 2020, an announcement confirmed that cystic fibrosis patients in Northern Ireland would gain access to the drug Kaftrio for treatment via the health service. This arrangement succeeded earlier agreements with the same company for the medications Orkambi and Symkevi. Robin Swann, who was the health minister at the time, commented that Kaftrio would provide individuals living with cystic fibrosis a “better quality of life.” Kaftrio, Orkambi, and Symkevi are medications designed to address the underlying cause of the disease by circumventing the genetic errors that lead to it. Damian Downey, a professor of respiratory medicine at Queen’s University Belfast and a cystic fibrosis specialist within the Belfast Trust, stated that the creation of these drugs represents “truly a golden era of CF research.” He added, “It has transformed the lives of those with CF beyond all recognition, beyond anything that we could have hoped for the development of these drugs.” However, these treatments are not effective for all individuals with cystic fibrosis. The Cystic Fibrosis Trust reports that approximately 90% of the UK’s CF population possesses mutations that will eventually qualify them for these medications. Nevertheless, the organization emphasized: “Ninety percent is not everybody, and we are very aware of the 10% of the UK CF population who have genetic mutations that are not eligible for any of these new treatments.” Paul O’Hagan indicated that medical professionals attempted to treat Cahir with these drugs, but they proved ineffective for him. “The only other option for the ’10 percenters’ is a double lung transplant,” he said. Cahir required oxygen continuously, 24 hours a day, seven days a week, due to his lung capacity being only 18%, and he was placed on the transplant list in March 2023. Paul stated that the transplant “would have given him a much better quality of life – a second chance of life.” “Cahir was just waiting on the call,” he said. However, six months subsequent to this, Cahir passed away on 4 October 2023. “They tried everything for Cahir. And Cahir gave he gave it his best shot,” his brother continued. “He was due to get married in June of this year.” “Unfortunately, he did make it up the aisle but not the way it was supposed to be.” Paul expressed that for his family, it was “very disheartening” and “heartbreaking” that the existing CF medications, capable of extending lives, were unable to assist Cahir. “There’s so much good work has been done so far, but 90% have access to modulators, and 10% of people do not.” “You don’t stop a job when it’s only 90% done.” “If you fall into the 10% that shouldn’t mean that you’re falling under a death sentence.” “We all live in hope that one day there is a modulator, there is drugs out there that the other 10% of CF patients can avail,” he said. Professor Downey acknowledged the immense frustration experienced by that segment of the CF population, but noted that narratives such as Cahir’s motivated him and fellow clinicians and researchers to persist with their research endeavors. “We’ve had fantastic news with these drugs, but they are not a cure.” “Our aim, of course, is that nobody is left behind, and this is to ensure that 100% of people, those diagnosed with CF have access to drugs that can change their lives.” He further stated that “The science has dramatically improved over the last number of years,” adding that initial data appears promising. “We are very hopeful, with a degree of some confidence that we will find in the coming years a drug that’s impactful for all.”