Cystic fibrosis patients ‘now have longer lives’

PEOPLE with cystic fibrosis are living longer and have a better quality of life, a new report has found. Adults with cystic fibrosis, which is also known as CF, tended not to be in employment, or have children, 20 years ago. But there have been major changes in quality of life, the report, Independent Living And Cystic Fibrosis, has found.

The average age of those dying from the disease rose from 17 years to 30 in the space of just two decades. The publication of the report comes ahead of the national fundraising day for Cystic Fibrosis Ireland, 65 Roses Day, on Friday, April 13.

Philip Watt, chief executive of Cystic Fibrosis Ireland, said access to ground-breaking drugs such as Orkambi had made a great impact on people’s quality of life but ‘key gaps’ still remain.

People with cystic fibrosis were not expected to be in any employment in 1998; however, last year, as many as 54% of those surveyed were in full-time or part-time work, according to the report.

Additionally, the percentage of those obtaining third-level qualifications has increased from 38% in 1998 to 49% in 2017.

As many as 74% of respondents lived with their parents in 1998, but that has reduced to 43%, while improved health and the introduction of IVF has resulted in 26% of people with CF having their own children.

Only 8% of those surveyed were married 20 years ago compared with 26% last year, according to the study

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Average lifespan up from 17 to 30

Cystic Fibrosis Ireland.

Mr Watt said that improvements in lifespan and quality of life are linked to improvements in services, such as CF hospital centres; higher rates of double lung transplantation; and, more recently, access to new ground-breaking drugs such as Kalydeco and Orkambi.

‘The report shows that with sustained investment over a number of years, people with a chronic and fatal disease such as CF can lead longer and better lives. However key gaps remain,’ he said.

‘The Government promised a dedicated cystic fibrosis in-patient unit in Beaumont Hospital commencing in 2017.

‘There has been no progress on this commitment in the [current] programme for government, and understaffing in major CF centres remains an issue.’

Around one-quarter of Ireland’s adult cystic fibrosis population participated in both surveys, while corroborative data was also drawn from other sources such as the Cystic Fibrosis Registry of Ireland.

Cystic fibrosis is an inherited, chronic disease that affects the lungs of about 1,300 children and adults across the country.

Last month, Dancing With The Stars judge Julian Benson discussed living with cystic fibrosis on RTÉ’s The Late Late Show, telling host Ryan Tubridy: ‘I have CF, but CF doesn’t have me.’

He recounted how he had been diagnosed with cystic fibrosis at two years of age, having spent a year in Temple Street Children’s Hospital.

‘Cystic fibrosis affects the lungs and the pancreatic system,’ he said. ‘It builds up mucus so... you’re getting constant chest infections.

‘So, you’ve got to mind yourself and, again, it’s all about you have to be on a regime, a permanent regime of antibiotics and medication to keep you as well as you can [be].’