There have been some positive developments in the care of people with cystic fibrosis (CF) in Ireland in recent years, however, major concern has been expressed about poor staffing levels in this area.

According to Cystic Fibrosis Ireland (CFI), there is a staffing crisis in hospitals, with some CF centres 20 staff short of what is required by European standards.

Around 1,200 people in Ireland have CF, which is a hereditary disease that primarily affects the lungs and digestive system. It is caused by a defective gene, which along with its protein product, causes the body to produce unusually thick and sticky mucous.

This mucous clogs the lungs, leading to potentially life-threatening lung infections. Ireland has the highest prevalence of the disease in the developed world. 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 that child will have CF.

Speaking at the launch of CFI's 65 Roses Day fundraising appeal, which takes place on April 15, CFI chief executive Philip Watt, highlighted that a number of positive developments have taken place in recent years. These include:

-The lung transplant programme in Ireland carried out successful transplants on 37 people with CF between 2013 and 2015
-New groundbreaking drugs, such as Kalydeco are helping to tackle the underlying cause of CF in around 11% of the CF population in Ireland.

However, Mr Watt noted that it is unclear whether the HSE will pay for an innovative new drug, Orkambi, which has the potential to positively impact on 50% of the CF population in Ireland.

He also called on the HSE to ensure that staffing levels in CF services are adequate.

"The HSE needs to future proof services. All CF staff must be replaced when they resign or go on maternity or sick leave. Even where the filling of posts is agreed, there is often a delay in recruiting replacements and so this needs to be factored into recruitment processes to ensure that there is no loss of service," he insisted.

He also noted that some CF centres still require adequate inpatient facilities. For example, Beaumont Hospital in Dublin requires 20 inpatients rooms, but only has seven.

However, he said that this is a period ‘of real hope for people with CF' and this is reflected in the increase in the average age of death of people with the disease, which has doubled since the late 1980s, when it was just 13 years, to 27 years in 2014.

CFI's 65 Roses Day aims to raise €65,000 for much-needed services for people with CF.

"Funds raised on 65 Roses Day go to support CFI's range of services ,which include grants to support people with CF, the development of new CF health facilities, research and education.

"By way of example, in 2015, more than 300 grants amounting to €147,000 were awarded to people with CF to support them in a wide range of areas, from fertility treatment and transplant assessment, to exercise equipment and education support. Without the support of the public, the support grants provided to people with CF simply wouldn't happen," explained CFI ambassador, Keelin Shanley.

This event will take place as part of CF National Awareness Week 2016, which runs from April 11-17. For more information, click here