Outline of Key Messages from Cystic Fibrosis Ireland
To Joint Oireachtas Committee on Health and Children
Philip Watt, CEO
Cystic Fibrosis Ireland
February 2013
Cystic fibrosis (CF) is an inherited chronic disease that primarily affects the lungs and digestive
system of about 1200 children and adults in the Ireland (70,000 worldwide). A defective gene and its
protein product cause the body to produce unusually thick, sticky mucus that:
o
Clogs the lungs and leads to life-threatening lung infections; and
o
Obstructs the pancreas and stops natural enzymes from helping the body break down and
absorb food.
Cystic Fibrosis Ireland is the only national patient CF group in Ireland. We were formed in 1963 and
this year is our 50th birthday www.cfireland.ie Along with the Irish Cancer Society we are the oldest
patient group formed in Ireland.
In the 1950s, few children with cystic fibrosis lived to attend primary school. Today, advances in
research and medical treatments, including in Ireland, have further enhanced and extended life for
children and adults with CF. Median age of death is still around 25 years of age, but thanks to care
standards in our CF hospital centres, many people with the disease in Ireland can now expect to live
into their 30s and beyond. People with CF in Ireland are increasingly going on to attend third level
colleges, accessing employment, and living more independent lives, with the support of family and
friends. However this also puts major demands on adult CF services in particular.
The impact of CF can vary from one person to another. There are some people with CF who live until
their teens and there are others that live in to their 50’s. Ireland has among some of the most
severe manifestations of CF and also has the highest incidence (per head of population) of CF in the
world, with three times the rate of the United States and the rest of the European Union.
Cystic Fibrosis: Progress and Challenges
There have been some significant improvements in CF care in recent times, however CF care has
suffered from decades of underfunding in Ireland and the infrastructure is still ‘catching up’ with
accepted standards in the US and other parts of Europe.
The recent approval of the ground-breaking drug Kalydeco and the introduction of newborn
screening for CF in 2011 and the opening of the long-awaited unit in SVUH are some positive recent
developments, however there are huge challenges that remain, including for example:
1. Isolation facilities are still needed across the country. There is an urgent need for 13 adult
inpatient isolation rooms in Beaumont Hospital. There are only 4 isolation rooms at present
and there are around 130 adult patients attending Beaumont. Estimated cost of this project
is €2.5million for capital costs alone. There are major infrastructure deficits in other CF
Centres including Galway, Cork, Limerick, Dublin, Waterford and Castlebar. Some of these
are being addressed by fundraising through CF and locally based charities.
2. The need for an increased rate in double lung transplantation. Many of our patients need
double lung transplants as their lung function decrease. There were 10 Irish CF transplants
undertaken in 2012, we would urge that this figure doubles in 2013 to meet existing need. A
key priority would be the filling of the outstanding cardiac-thoracic surgeon post in the
Mater Hospital. The quality of transplantation in Ireland and safety standards is high in
Ireland, thanks to the expertise of clinicians involved in transplantation
3. The non-replacement or significant delays in the replacement of dietitians, specialised
nurses and physiotherapists on maternity and sick-leave is causing real hardship for many of
our patients. Annual assessments in some hospitals are being cancelled or deferred as a
result of the staff embargoes.
4. As a result of the recent budget, the prescription charge for Medical Card holders has
increased from 50c per item to €1.50 per item, up to a monthly limit of €19.50 per family
(increased from €10).CFI have received several calls from members in recent weeks
expressing their concern with this increased rate, which has essentially tripled. The Disability
Federation of Ireland, in conjunction. This further increases the burden of those already
trying to live with additional costs of disability.
5. The need to preserve research funding for new and innovative treatments is crucial,
including for example the joint funding scheme operated by the HRB and the Medical
Research Charities Group.
6. The need for lung health to become a much more important public health priority that it is
at present, consistent with the overall messages given by presentations today. This includes
public awareness messages to the general public and the need for those with long term
illnesses to be proactive for example in relation to the ‘flu jab and pneumococcal
vaccinations (pneumonia) especially for vulnerable groups. Partnership initiatives between
the State and NGO’s including CFI and the Lung Health Alliance and clinicians is crucial as an
effective approach going forward.