Cystic fibrosis (CF) patients and their families in Ireland have been given hope following the revelation that a woman with the condition here was diagnosed for the first time at the age of 76.
CF is Ireland’s most common genetically inherited disease and, with 1,100-plus patients, Ireland has the highest proportion of CF patients in the world.
Doctors at the Cork Adult Cystic Fibrosis Centre at Cork University Hospital (CUH) report in the latest edition of the Irish Medical Journal (IMJ) that a woman aged 76 was diagnosed with CF at their unit two years ago and now, aged 78, she remains stable.
The chief executive of Cystic Fibrosis Ireland, Philip Watt, yesterday admitted to being gobsmacked and amazed by the case.
He said: “It is truly remarkable that a woman has lived to 78 with CF and went undiagnosed until she was 76.”
Mr Watt said that the case “adds to the hope for CF patients here who are already benefiting from new and innovative drugs and improvement in treatment centres in Ireland”.
The IMJ report states that, previously, the oldest patient diagnosed with CF in Ireland was aged 61. The oldest patients with CF in the US and the UK are 82 and 79 respectively.
The report said the 76-year-old was referred to CUH for assessment of possible lung disease. The woman reported a history of recurrent childhood pneumonia, asthma and had a remote smoking history. She also had a daily cough and frequent hospital admissions with lower respiratory tract infections.
Based on the clinical findings and investigations, a diagnosis of CF was firmly established “and at 76 years of age, this patient represents the oldest ever first presentation of CF in Ireland”.
The authors of the case report state that advances in dealing with CF “have led to significant increases in life expectancy. Additionally, effective nebuliser antibiotic and mucolytic therapies mean that the predicted median life expectancy of someone born with CF today is greater than 50 years of age.
“Our case report highlights that in those with appropriate clinical findings, investigations to exclude CF should be instigated at any age.”
‘Not unheard of’
Prof Charles Gallagher, director of the Adult Cystic Fibrosis Centre at St Vincent's University Hospital, Dublin, said the woman's case was "rare but not unheard of".
“It’s not something we see every day but it is becoming more common to diagnose people who are in their 50s, 60s or 70s with cystic fibrosis,” he said. “Cystic fibrosis is no longer a disease for young people, it’s fast becoming a disease of middle age. Slightly more than half of Irish people with CF are adults.”
Dr Gallagher predicted that more people in their 60s and 70s would be diagnosed with the disease in the next 10 to 15 years. “The good news is more people with CF are living for longer and that treatments and screening for the disease are improving all the time,” he said.
Mr Watt said: “It is disappointing that the woman slipped through the net for so long in terms of diagnosis, but it is a remarkable story and very positive that a woman with CF can live for so long. She has a milder form of CF and it is unrealistic to believe that all CF patients will live to this age, but it is another flicker of hope for patients.”
