About RTÉ Television
The Afternoon Show
The Afternoon ShowRTÉ One, Weekdays, 4.00pm

Cystic Fibrosis

Thursday, 8 April 2010

Cystic Fibrosis is Ireland's most common life threatening inherited disease, with one in 19 of us carrying the gene, and approx forty children a year are born with the condition.
Ireland has the highest prevalence of Cystic Fibrosis in the World and Ireland has the most severe types of Cystic Fibrosis in the World.

What is Cystic Fibrosis?

CF affects the glands, damaging many organs including the lungs, the pancreas, the digestive tract and the reproductive system. It causes a thick, sticky mucus to be produced, blocking the bronchial tubes and preventing the body's natural enzymes from digesting food.

Cystic Fibrosis primarily affects the lungs and the digestive system.
A build up of mucus can make it difficult to clear bacteria and leads to cycles of lung infections and inflammation, which can eventually lead to damage of the lungs.

CF can also make it difficult to digest and absorb adequate nutrients from food. Mucus blocks the duct of the pancreas, preventing enzymes from reaching the intestines to digest food.
As a result, persons with CF must consume artificial enzymes with food, to help them absorb adequate nutrition from their food. They must also follow a demanding daily routine of physical therapy to keep the lungs free of congestion and infection.
Additional organ systems involved are the reproductive tract and the sweat glands. Other organ systems may be involved to lesser degrees.
The result is that people with CF are prone to constant chest infections and malnutrition. However as therapeutic options have expanded over the last decade; significant advances have been achieved in both life expectancy and quality of life.

Dr. McKeon - Consultant Respiratory Physician, St Vincent's University Hospital

Why does Ireland have the highest rate of CF?

We don't really know. There tends to be a higher number of CF patients in an island country because the gene is more likely to be reproduced, but with most genetic conditions, we don't know the origin or how it was caused. Northern Europe has the highest incidence of the condition in the world, and Iceland is another country that has a high incidence, so it seems to be something that is common on island countries, but there is no definitive answer.

It's a genetic disease, is there any way of preventing it, or reducing the occurrence?

The most beneficial thing for CF is to be able to determine it as young as possible and so that we can treat it straight away. There is discussion in North America, and there are some private clinics that practice screening for the gene, so that two people with the gene will not procreate to have a child with CF. However this is controversial because it can cause a lot of worry for people who have the gene. However, it is a worthwhile discussion but probably won't be something we'll see here in the near future. There is a screening process for partners of CF patients to see if they have the gene.

How is someone diagnosed with Cystic Fibrosis?

It is normally diagnosed within the first two years of their life, mainly after 6 months and any age up to two years.

It is diagnosed by doing a sweat test. This is where a band placed around the arm and then the sweat goes into gauze and is sent for testing. It is in no way painful to a child. A GP will detect the symptoms and send the child to a paediatrician for this testing.

Can someone with CF have children?

Women with CF can reproduce naturally, although we would test the partner for the gene. If it is just the woman that has the gene then it means that baby won't have the condition.

Is exercise the key to Tomas's (a CF patient) good health, or is he an exception?

Tomas is an example of how important physical exercise is for CF patients. We recommend that someone with the condition do a mixture of physio and exercise to clear the lungs and help movement of mucus. People who are active do tend to be much healthier and spend less time being sick than people who don't. However some people may be unable to do exercise because of the condition, which can make it hard for them.

What quality of life should they expect to lead?

Daily medication is generally what holds people back as their medication, nebulizers and exercises can take up a lot of their day. However, most patients who are well enough can get a great education and a great career that suits them. It's not quite so simple because there are times when they will need to be in hospital, and some patients, due to a variety of reasons my have a worse condition than others, and may find it difficult not to be completely dependent on the health care system. Although Tomas is exceptional because he's really made the most of his disease, there are a lot of sufferers who live normal lives.


Symptoms are usually diarrhoea, no weight gain and regular chest infections. In some case the symptoms are picked up on within a few days after childbirth by the medical professionals, but in most cases it is well after the baby is brought home from hospital. There are proposals for testing at childbirth, but this has not started yet.

Fundraising pays for new dedicated Out Patient Unit at Temple Street Children's University Hospital

Temple Street Children's University Hospital officially opened a brand new Cystic Fibrosis and Respiratory Out-patient Unit at the Hospital today. The new unit is the culmination of intense fundraising through the hospital Fundraising Office and will literally be life-changing for the patients for whom it has been developed. The total cost of this project was €3.2M, over €2.5m of which was raised through donor support and corporate partnerships.

The Fundraising Office partnered with Friends First and a charitable foundation run by its parent company, The Eureko Achmea Foundation, to fund the new respiratory laboratory which is a vital component of the service. Speaking about the partnership Adrian Hegarty, CEO, Friends First Group said, "Friends First employees played an important role in the decision to make the donation towards this Cystic Fibrosis unit; it is tremendous to see this partnership come to fruition today. We are delighted to be part of this life changing service which will play such a significant role in the treatment of Cystic Fibrosis patients."

Cystic fibrosis is the most common, life threatening, recessively inherited disease with an average national yearly incidence of 1 in 1500 live births and a carrier rate of 1 in 20 people in Ireland. Cystic fibrosis affects many organs of the body but causes most problems in the lungs, intestines, liver and pancreas. Following advances in treatment, the majority of CF patients are surviving into their late twenties.

Children with CF need to be isolated from other CF sufferers so that they do not cross infect each other with the bacteria they grow in their phlegm. The new dedicated Out-Patient Treatment Unit will facilitate this. Thanks to generous nationwide support, the new unit is a reality and the very best standard of care is available in Ireland to the children who need it.

IPPA Happy Faces Campaign

This year, CFAI have been chosen by the Irish Professional Photographer's association to be the charity to receive funds raised by the Happy Faces campaign which will run from April 1st to 18th.

IPPA Happy Faces is the annual fundraising project, now in its fifth year, organised by the Irish Professional Photographers Association (IPPA). The fundraising this year is to assist the CFAI in providing isolation facilities for people living with Cystic Fibrosis in Ireland. People young and old, are encouraged to visit their local IPPA photographer and have a professional portrait taken for just €25, where they will receive a mini portrait session and a desk portrait.

All the photographers involved are providing their services free of charge and the monies raised will go towards the goal of raising €100,000 for much needed isolation facilities for people living with CF. A selection of the 'Happy Faces' photographed throughout the campaign will be featured in a 2011 calendar, with all proceeds from the sale of this going towards CFAI.

CF is Ireland's most common life threatening inherited disease. Ireland has the highest prevalence of CF in the world, with 1 in 19 carrying the CF gene. Ireland also has the most severe strain of the CF gene in the developed world. Despite this, facilities for CF patients in our hospitals are below European standards. Today people with CF can expect to live a near normal, full and productive life, provided that the correct facilities are in place, particularly isolation facilities, which IPPA Happy Faces 2010 is raising funds to improve.

For further information, go to