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Dublin: 1 °C Monday 18 November, 2019
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'No parent wants to see their child go before them': Living day-to-day with cystic fibrosis

Cystic fibrosis can be a life-changing condition for entire families.

The Crombie family (from left to right) - Hannah, Hughie, Priscilla and Dermot.
The Crombie family (from left to right) - Hannah, Hughie, Priscilla and Dermot.
Image: Shane O'Neill Photography

DERMOT CROMBIE HAS a daily routine that he never breaks.

Every morning when he wakes up, before he goes to school, nine-year-old Dermot has to perform a few daily rituals.

With the help of his parents, Dermot first spends about five minutes taking in a salt and water solution through his nebuliser. After this, he takes his inhaler, followed by some physio breathing exercises.

Dermot does five sets of breathing through an Acapella – device designed to clear out the excess mucus from his airwaves.

“What all of that does is it brings up the excess mucus that would have formed while he was sleeping at night-time,” Dermot’s mother Priscilla tells TheJournal.ie.

So if that mucus stayed there that’s what would encourage a chest infection, so the goal in the morning is to clear all of that so it just doesn’t settle in his lungs.

All this is done before Dermot has breakfast. At breakfast he takes Kalydeco with 10g of fat. Kalydeco is the medicine used to combat the effects of Dermot’s illness – cystic fibrosis.

Throughout the day, Dermot also takes the medicine Creon whenever he eats more than 10g of fat. This helps his body break down the food.

“This allows him to gain weight and grow in height as well,” says Priscilla.

At night, Dermot takes Kalydeco again and repeats his physio and nebuliser breathing exercises.

This is Dermot’s daily routine, and he carries it out every day since he was diagnosed with CF last year.

65 Roses Day

Today is 65 Roses Day, a fundraising appeal organised by Cystic Fibrosis Ireland.

The day will see hundreds of volunteers taking part in order to try to raise €100,000 for vital CF services.

65Roses18 Dermot and his sister Hannah at the launch of 65 Roses Day. Source: Shane O'Neill Photography

Cystic fibrosis is Ireland’s most common life-threatening disease. A genetic disorder, it affects the regulation of absorption and secretion of salt and water in the body.

This can result in a build up of sticky mucus which can clogs airwaves and harbour harmful bacteria. The most common symptom of CF is a recurrent chest infection which can lead to lung damage.

Respiratory failure is the most common way people die as a result of CF. This is why Dermot’s morning and evening exercises are so important.

“The stronger [Dermot's lungs] are, the stronger they can fight against all these bugs and infection as well,” says Priscilla.

The family live in Rhode, Co Offaly, and Dermot loves football and is a very active child.

Priscilla says that encouraging this and ensuring Dermot stay fit and healthy is also vitally important.

Exercise is such an important element of it as well, because obviously the more exercise you do the more you strengthen your lungs.
The fitness element is something we’d really advocate for.

Diagnosis

Dermot was diagnosed with CF in March of last year. Priscilla says this was quite late, and for much of his life they believed he had suffered from asthma.

“I always knew at the back of my mind something wasn’t quite right,” she says.

Last year, as Dermot approached his eighth birthday, Priscilla and her husband Hughie noticed his condition worsen. They also noticed that he had started to lose weight and wasn’t growing properly.

Dermot was sent for tests, and the results came back positive for cystic fibrosis. When he was sent for tests, it was the first time that CF had even mentioned as a possible condition.

“It was a very difficult thing to hear, ” says Priscilla.

“I was so devastated at the time and so angry that we had pushed for a number of years to try to find out what was wrong.

And we just couldn’t understand why it had taken so long for CF to come into the picture, and then all of a sudden he had it.

Mixed into the devastation and sadness was a strange sense of relief – that the family finally knew what was wrong with Dermot and that from then on he would be able to get the proper care he needed.

A full life

CF is a genetic disorder that people are born with. About one in 19 people are carriers of the gene, and when two carriers have a child there is a one in four chance of them having CF.

Hughie and Priscilla weren’t aware that they were carriers of the gene before Dermot was diagnosed with CF. On days where he isn’t feeling well, she says they sometimes feel guilty.

“There’s a certain amount of guilt on our side because we obviously both had the CF gene,” she says.

We weren’t aware of it and we kind of feel slightly guilty that we’ve given this horrible thing to him as well.

However, Priscillia says that on good days and bad, the parents work hard to make things as normal and easy as possible for their son.

“Sometimes when he does have days like that we take it in his stride and make it more relaxed for him so he doesn feel like it is a chore.

That’s important as well that he builds it into his routine especially when he gets older, and he doesn’t have mammy running after him the whole time.

The number of people with CF in Ireland has risen recently – from 1,025 people in 2011, to 1,219 people in 2015.

Ireland has the highest rate of CF in Europe – at almost three times the national average of other EU countries.

As treatments and medicines have improved, the life expectancy of CF patients has extended significantly, meaning people with the illness can live long lives in spite of their condition.

A deal was reached on Tuesday by the HSE for the ‘life-changing’ drug Orkambi, to treat people diagnosed with CF. Kalydeco – the medicine used by Dermot – was also approved by the HSE for more general use.

“Now with the new advancements in drugs in gives them a lot more prospects for the future and that they can live as long as a healthy being,” says Priscilla.

“No parents wants to see their child go before them.

You still worry about it, but the fact that he’s on this drug gives a little bit of hope that he will live as long as everybody else.

You can find out more about 65 Roses Day at www.65rosesday.ie 

Read: ‘We are so, so happy that it’s finally here’: Cystic fibrosis patients celebrate deal on Orkambi

Read: ‘Life changing’ cystic fibrosis drug Orkambi will be available from next month

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About the author:

Cormac Fitzgerald

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