The head of the local branch of a national cystic fibrosis support and advocacy group says while there have been some positive developments in the care of people with CF the organisation is still playing “catch up” due to years of under-investment in facilities.
Mary Lane Heneghan, the chairperson of the Cystic Fibrosis Ireland Galway Branch, outlines that key frontline healthcare staff are not being replaced when they are on sick or maternity leave.
“We desperately need to ensure that people with cystic fibrosis have access to centres across the country with adequate facilities to avoid cross-infection. Appropriate facilities and staffing are not optional extras, they are critical to the safe care of patients and need to be resolved right now.”
Speaking at the charity’s annual conference which took place in Galway at the weekend, she praised GAA commentator Joe Brolly for his support for cystic fibrosis care. He received a special recognition award from the organisation in appreciation of his efforts.
Ms Lane Heneghan stated he had mounted an unprecedented campaign to raise the need to change the consent law in relation to organ donation at the highest Government levels.
“Some people with cystic fibrosis need a double lung transplantation and will rely on the generosity of organ donors. A change in the consent law would mean that potential organ donors would be presumed to have given their consent for organs to be donated unless they have opted out or family members decline.”
She pointed out that the award was for his “courageous and selfless work” for people requiring organ donation.
A number of eminent medical professionals spoke about developments in CF care from lung transplantation to new drug therapies.
Speaking prior to his presentation Professor Jim Egan, a consultant respiratory and lung physician at the Mater Misericordiae University Hospital in Dublin and the director of the National Organ Donation and Transplantation Office, said in 2012, 14 double lung transplants were carried out at the hospital. Four of these were patients with cystic fibrosis.
“Last year, we more than doubled these numbers to 32 transplants, nine of which were for people with the disease. Recent recruitment of specialist surgeons has underpinned the ability to complete the numbers. In 2014, we hope to sustain these numbers and look forward to an enhanced organ donation infrastructure within intensive care units following the allocation of funds towards organ donation to allow the deployment of a ‘soft opt-out’ donation legislation.”
Dr Barry Linnane, a paediatric respiratory consultant at University Hospital Limerick, told the conference he was excited about the therapies and treatments being developed for some of the more common genetic mutations of CF.
“The recent breakthrough in cystic fibrosis care, with the success of Kalydeco in correcting the underlying molecular defect causing the disease, has not only improved the lives of the 10 per cent of patients here who can benefit from it but potentially heralds a new treatment era. Research efforts are increasingly focusing less on generic symptom-based treatment and more on bespoke care, tailored and targeted to the individual.
“Fundamental to this personalised care is the use of treatments designed to correct the specific genetic defects, orcombination of genetic defects, causing the disease. There are already clinical trials under way on developing additional therapies which will target the separate mutations of the disease for other people with cystic fibrosis and we look forward to seeing these enter clinical care over the next number of years.”
Cystic fibrosis is Ireland’s most common life threatening inherited disease with one in 19 people carrying the gene. Ireland has the highest rate of the condition in the world. CF affects many bodily functions such as breathing, digestion and reproduction. The lifelong condition usually becomes more severe with age - some people with it live until their teens while others live until their 50s. It affects men and women in equal proportion.
There are about 1,110 people with cystic fibrosis in Ireland. Fifty new cases are diagnosed each year.
For further information on Cystic Fibrosis Ireland’s 65 Roses national awareness week or on the condition contact LoCall 1890 311 211 or visit www.cfireland.ie