How cystic fibrosis impacts me: four people tell their stories

Almost 1,400 people are currently living with cystic fibrosis (CF) in Ireland. The incidence of this chronic inherited lung disease is higher in Ireland than anywhere else in the world. Yet, as new drugs come on the market, people are living longer than ever with the condition.

Ahead of 65 Roses Day – Cystic Fibrosis Ireland's annual fundraising day is on Friday, April 12th – here, a secondary school pupil, a third level student, a woman in her first professional job and a new mother speak about living with cystic fibrosis.

Benat Broderick

Benat Broderick is a willing spokesman for the chronic inherited lung disease, cystic fibrosis. The Junior Cert student speaks coherently about how CF affects, and doesn't affect, his daily life. "I have to spend time doing my nebulizer [a devise which transfers liquid medication into a mist to be inhaled] and clearing my lungs before school, after school and before bed but I don't do anything special in school, except I try to avoid germs as much as possible," he explains.

Relaxed about being in the first Junior Certificate class group in the recently opened Stepaside Educate Together Secondary School, Broderick says, "I can do everything everyone else does but it's a bit more challenging for me. For example, I get out of breath more easily in PE class so might have to take a break."

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He is conscious that when people hear or see him coughing that they might think he has the flu or a cold and could be infectious. “If I get a coughing fit, I go out [of class]but it’s important that people know that CF is not infectious,” he says.

He goes into hospital for intravenous antibiotics every three to four months but he feels a lot better since he started taking Orkambi, the new drug treatment for people with cystic fibrosis. “I’ve put on weight. I’ve grown. I’ve more energy and my lung function is better since I started taking Orkambi. It has made my life better in a good way,” he adds. Broderick would like to work as a journalist when he finishes his studies.

Alannah Shesgreen

Alannah Shesgreen was diagnosed with cystic fibrosis when she was two-and-a-half years old. "I have always been conditioned not to strive for things I knew weren't possible, so I never wanted to be a doctor or climb Mount Everest but I always aim to be the best version of myself and prove what I can do," says the 19-year-old student from Nenagh, Co Tipperary.

A second year student of Law and Accounting at the University of Limerick, Shesgreen shares a flat with three other girls. "Cystic fibrosis is a high-maintenance illness so the biggest impact on my everyday life is the time it takes in the morning and the evening to take my nebulizer and do my physiotherapy," she explains. In 2017, Shesgreen was also diagnosed with CF-related diabetes which adds extra demands onto her daily routine.

As well as keeping up with her studies, Shesgreen says that exercise is very important to her. “I go to the gym three times a week and I run twice a week. I’m training for the Great Limerick Run in May,” she says.

The eldest of four children, Shesgreen has a sister with CF and is conscious of the effort her parents make to keep them both well. “I spent time in hospital recently and I was aware of their worry. Yet I feel moving to college has been a huge change because it’s up to me now to look after my health and know what I’ve to do to keep out of hospital.”

Since I started college, the disability services offer me so much support

Shesgreen has been in hospital twice for intravenous antibiotics since she started college. Sometimes, she is breathless when walking to college in bad weather. But, she is keen to point out that registering with the disability services at UL has been a great support to her.

“I was hesitant about registering at first because I wanted to get into college on my own achievements – which I did – but since I started college, the disability services offer me so much support; things like being able to do my exams in a small room, being able to take breaks to check my blood and take insulin. I can also get a set of notes from lectures that I miss,” she explains. Shesgreen’s ambitions include getting a first in her final exams at UL and to spend some time travelling once her studies are complete.

Saoirse Perry

"I was relatively well as a child and teenager. I rarely got chest infections and had good lung function but when I was seventeen, my liver started to act up," explains Saoirse Perry, who recently started working as a library assistant in Maynooth University.

Within a year, she had lost weight and had several chest infections. This all happened when she was in her final year of college, studying Geography and Irish at Maynooth University (MU). Her symptoms got so bad that she was put on the waiting list to have a liver transplant in December, 2013. In April 2014, Perry had a liver transplant and following a period of recovery, she returned to college the following January. She graduated with a degree in Geography and Classics in 2017. The following academic year, she did a Masters in University College Dublin in Information and Library Studies and got a job as a library assistant at MU in September 2018.

"I live in Cabra with my boyfriend and I commute to Maynooth. The only difference in my daily routine is that I have to get up a lot earlier to do my nebulizers. I work shifts so if I have to be in for 8.30am, I have to get up at 5.30am. The work itself is fine, if anything it's the commute that's a bit tiring," she explains. "I love working in an academic library and engaging with the students. Once I get a good nights sleep and I eat well I'm fine. I'm conscious not to run myself down."

While she didn’t disclose that she had CF in the interview for the job, once she settled into the job, she spoke to her manager. “The Access Department at MU was fantastic when I was sick as a student and when I came back after having a transplant, everyone was very open and welcoming. The library has the same ethos so when I told my manager, she organised a meeting with the health & safety officer who organised a specific room for me to go to do my nebulizer three times a day,” explains Perry.

She says that the support she has received is very reassuring for her. “I was told that I was hard working and to take my time and not worry and that I could have these extra five minutes as well as my break. That was such a relief.”

Perry believes that it’s important to speak out about living with CF. “It’s important that people understand what’s involved so that employers and government bodies can meet the needs of people with cystic fibrosis,” she says.

Josephine Carey

Josephine Carey and her partner, Michael decided to do genetic testing to see if Michael was a carrier of the cystic fibrosis gene before they became parents. "One in 19 people in Ireland carry the CF gene so it wasn't surprising that he turned out to be a carrier. With me having CF and him a carrier, we had a one in two chances of having a baby with CF so we opted for in vitro fertilization (IVF) with preimplantation genetic diagnosis," explains Carey.

Tests on the seven embryos collected following IVF revealed that four out of seven of the embryos were suitable for implantation. One healthy embryo was then transferred to Carey and the couple’s daughter, Ada was born nine months later in October 2018.

“My pregnancy went surprisingly well. Beforehand, I focused a lot on getting as healthy as possible and then during my pregnancy, I did a lot of walking. My biggest thing was to get my lung function as good as I could,” explains Carey.

The second youngest of eight children, one of Carey’s older brothers, Christy was also born with CF but died at the age of 24 in 2001. Carey herself was diagnosed with CF when she was just a couple of months old and although she was hospitalised a few times for intravenous antibiotics as a teenager, her health was relatively stable during her twenties.

“In 2013, I went on Kalydeco, which was the first high-tech drug for CF. That really brought my lung function back up and I gained weight. Before that, I had a lot of chest infections and took oral antibiotics and home intravenous antibiotics as well,” says Josephine.

I'm also in an international Facebook group for mothers with CF so I get good support there too

Working as a waitress in her family’s pub and restaurant business in Michelstown, Co Cork, Carey managed well throughout her pregnancy and stopped work about a month before Ada was born. “The birth went smoothly considering she arrived 10 days early. I was well too. During my pregnancy, I had reduced the dose of Kalydeco and then I stopped taking it while I breastfed Ada,” she says.

When Ada was 12 weeks old, Josephine decided to go back on her medication as her lung function was dropping and she was losing weight. “It took a couple of weeks to take effect but then it did. I was well looked after with Michael cooking meals and the CF team from Cork University Hospital coming to the house to check up on me,” she says.

She is thrilled to be a mother. "I'm loving it. I'm very happy and Ada is brilliant. I'm also in an international Facebook group for mothers with CF so I get good support there too," she says.

In terms of giving advice to other women with CF keen to become parents, she says, “the most important thing for me was to keep my body going. There will be days that you are exhausted and have to rest but keeping active is the key.”