Sustained investment in better services and medications for people with cystic fibrosis (CF) has made a major difference to those affected over the last two decades, however there is still much more that needs to be done, a new report has found.

The report, Independent Living and Cystic Fibrosis, which was compiled by Cystic Fibrosis Ireland (CFI), shows that between 1998 and 2017, there have been big improvements in the quality and length of lives of people with CF.

CF is Ireland's most common life-threatening inherited disease. Around one in 19 people are carriers of the CF gene and when two carriers have a child there is a one in four chance their child will be born with it. There are almost 1,200 people in Ireland with CF and this country has the highest prevalence and the most severe types of CF in the world.

The condition affects the regulation of absorption and secretion of salt and water in various parts of the body including the lungs, sweat glands and pancreas. This defect inhibits the flow of salt and water through the body's cells, causing a build-up of thick, sticky mucus, which can clog airways and harbour harmful bacteria.

Symptoms can vary in severity. The most common symptom is recurrent chest infections, which results in lung damage.

The report includes the results of two surveys - one taken in 1998 and one in 2017. Around one-quarter of the adult CF population took part in both surveys. Other data was taken from sources such as the CF Registry of Ireland.

Some of the key findings include:

-In 1998, the average age of death in Ireland among people with CF was 17 years. By 2015, this had increased to 30 years for the first time, with many living longer than this
-In 1998, adults with CF were not expected to have children of their own. In 2017, 26% of those aged over 21 had children of their own. This is thanks to better health and access to IVF. However, there is currently no State support for IVF and it costs around €6000 a time, restricting who can avail of it
-In 1998, 87% of adults were single compared with 39% in 2017. Just 8% of those surveyed in 1998 were married. In 2017, 26% were married
-In 1998, no adults with CF were expected to be in any type of employment. In 2017, 54% of those surveyed worked part time or full time
-In 1998, 74% of adults with CF lived in their parents' house. By 2017, this figure had fallen to 43%
-In 1998, 38% had obtained third-level qualifications. In 2017, this had risen to 49%.

"The findings are in many ways remarkable and send out a strong message of hope. The main message is that adults with CF are living much more fulfilled and independent lives in 2017 compared with 1998. They are much more likely to have a job, have better education attainment, to have their own home and to even have a family of their own," commented CFI chief executive, Philip Watt.

He said that the improvements in the length and quality of life were closely linked to service improvements, such as dedicated CF hospital centres, improved 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. The Government promised a dedicated CF inpatient unit in Beaumont Hospital commencing in 2017. There has been no progress on this commitment in the Programme for Government, and understaffing in major CF centres remains an issue," Mr Watt noted.

The report also pointed out that 14% of those surveyed in 2017 were ‘too ill to work', while many others said that the potential loss of a medical card or sudden loss of Disability Allowance when entering a job was a major deterrent to employment and independent living.

The publication of the report comes ahead of CFI's national fundraising day, 65 Roses Day, on Friday, April 13. People are urged to buy a purple rose for €2 or donate online here. Funds raised will be used to help people with CF to live independently.

The report, Independent Living and Cystic Fibrosis, can be viewed here