Cystic fibrosis from different angles: the patient, the doctor, the parent, the sibling
In Ireland, about 1,300 suffer from the life-threatening inherited disease
Rhonda Daly, Brian McCarroll and Meabh Henderson. Photograph: Alan Betson
Cystic fibrosis is the most common life-threatening inherited disease in Ireland. One in 19 Irish people carry the cystic fibrosis gene, but both parents need to have it to pass on the disease to a child. It affects the absorption of salt and water in the lungs, sweat glands, pancreas and gastrointestinal tract.
This results in the build up of a thick sticky mucous which can clog the airways and harbour harmful bacteria. Symptoms vary from person to person but the most common symptom is recurrent chest infections which result in lung damage. There are about 1,300 people with cystic fibrosis living in Ireland.
THE PATIENT: Brian McCarroll
Brian McCarroll (29) was diagnosed with cystic fibrosis at birth. He is the third of four children who grew up in Walkinstown, Dublin, and his older sister, Sinead also has cystic fibrosis.
“I don’t remember much of when I was a child except having to use a nebuliser when out playing on the road. I had to go to Our Lady’s Children’s Hospital, Crumlin a few times a year with chest infections when I was a teenager. I missed 97 days of school in fifth and sixth year in secondary school. The other teenagers with cystic fibrosis that I met in hospital are still some of my best friends. You grow up earlier than other people because of the things you have to face.
“I fought against having cystic fibrosis when I was a teenager and it was only when I was 21, did I begin to realise that I had a serious illness and had to look after myself. I got disability allowance from when I was 16 and I have a free travel pass but I work part time now for an events management company.
“I think cystic fibrosis is a hidden illness and people don’t have a clue how much time you have to spend looking after yourself. I spend about three hours a day managing my condition – taking nebulisers [to break down mucous in the lungs], doing physiotherapy exercises and taking digestive enzymes and vitamins with my food. I go to the gym five times a week and I started swimming in January 2018. I did the Malin2Mizen four-day cycle for cystic fibrosis in May 2017. Looking after yourself well is about finding the balance between exercise, work and rest.
“I was lucky to go on the clinical trial for Orkambi five years ago [the drug was approved for people with cystic fibrosis in April 2017] and I’ve seen a big improvement since then. I knew immediately that I wasn’t on the placebo as I’ve much more energy and fewer chest infections since I went on the drug. I currently live with my mother and sisters. Jen is my long-term partner – we’ve been together eight years – and we’d really like to have our own place to live and have a family. Getting a mortgage with cystic fibrosis is very difficult but we are currently on the medical waiting list for a house.”
THE PARENT: Rhonda Daly
Rhonda Daly is the mother of 10-year-old Darcy Grainger who has cystic fibrosis.
“Darcy was always ill from aged two onwards. We were constantly at the GPs and in A&E once a month. We were told that she had asthma but she was constantly battling chest infections, stomach problems, vomiting and coughing and couldn’t maintain weight. When she was six, I brought her to the private clinic in the Children’s University Hospital, Temple St, Dublin and she was diagnosed with cystic fibrosis and admitted to hospital for one month.
Darcy’s dad, Chris, and I are carriers of the cystic fibrosis gene which we didn’t know at the time.
“Although it was a huge shock to get the diagnosis, it was also a relief to know what was wrong with her after all the years of being so unwell. Darcy is in fourth class now in St Francis Senior School in Coolock. She is brilliant at managing her physiotherapy and all her medications, vitamins and digestive enzymes. I don’t think the other children understand it but she missed 30 days of school last year. When she’s well, she swims once a week and plays camogie twice or three times a week. When, she’s ill, she rests a lot. She recently started on the new cystic fibrosis drug, Orkambi.
“Every three months, Darcy has to take intravenous antibiotics three times a day for two weeks. The cystic fibrosis nurses in the Children’s University Hospital, Temple St have been very supportive. They showed me how to give Darcy the intravenous antibiotics at home but two years ago, she got a port put in and now she does it herself. It’s much better doing this at home because she would be at risk of cross-infection in the hospital. We’re very vigilant about where Darcy goes to avoid her coming in contact with people who are sick but our families are a great help in all of this.
“It’s very stressful having a child with cystic fibrosis. There is a lot of anxiety and worry about how she’s doing and there’s a lot of planning but Chris, Darcy and I work as a team. We’re the three musketeers. It’s amazing what you can do when you have to. I’m Darcy’s carer so I only work a few hours a week. You have to fit normal life around cystic fibrosis.
“For years, we decided not to have another baby but then last year, we tried and we lost a baby. All our friends are aware of cystic fibrosis and a group of us do the mini-marathon every year for Cystic Fibrosis Ireland. Chris did the Dublin Marathon in 2016 for Cystic Fibrosis Ireland and Darcy joined him in the last 200 metres of the race.”
THE EXPERT: Prof Gerry McElvaney
Prof Gerry McElvaney is consultant respiratory physician at Dublin’s Beaumont Hospital.
“Cystic fibrosis is the most aggressive lung disease we see and there is a big treatment burden on people with it. However, in the last 20 years, there have been great advances in medicines and the opening of specialist cystic fibrosis centres with multi-disciplinary teams in Cork, Galway and Dublin.
“Most people with cystic fibrosis come to these centres every three months and also on a drop-in basis if they need more treatment or support. Every three months, we do tests including a pulmonary function test to check their lungs and a sputum test to check for chronic and new infections.
“Cystic fibrosis is a multi-system disease so we also check for diabetes, liver disease and osteoporosis. Complications generally occur as people get older so it is important for people with cystic fibrosis to have regular physiotherapy, anti-biotic treatment and good nutrition.
“Cystic fibrosis used to be a childhood disease but we’re now seeing people live much longer. Most people with cystic fibrosis will need a lung transplant in their 20s or 30s but it can be very difficult to find the right match. We were behind other countries in our treatment approach to cystic fibrosis 15-20 years ago but now, the median survival age for people with cystic fibrosis is the same in Ireland as it is in the United States. ”
THE SIBLING: Meabh Henderson
“It was the norm for me growing up that my sister, Allsun had cystic fibrosis because she is seven years older than me. I was born into this family with Allsun and my brother, Cormac, who is four years older than me. I shared a room with Allsun for the first five years and the hum of her nebuliser would put me to sleep. To this day, I’ll fall sleep to anything with a hum – like on a bus. I remember how we could never physically play with her because she was weaker than us and always so skinny.
“I don’t remember trips to the children’s hospital but I do remember her going into St Vincent’s hospital for three weeks at a time, twice a year. I remember being at home when she coughed – and the whole family would almost stop breathing – waiting for her to breathe again. I also remember she could eat anything she wanted and I couldn’t which I thought was very unfair.
“Anytime I was unwell, I wouldn’t say anything because it was nothing compared to what she was going through. I felt envious of her special treatment yet at the same time, I felt guilty and protective of her. I was always told she wouldn’t live past the age of 20 and when I was 12, I used to prepare the song I’d sing for her at her funeral.
“I remember how stressful it was for my mother bringing Allsun into St Vincent’s hospital [before the special cystic fibrosis units were opened] worrying about the risks of cross-infections. Allsun was always so private about her cystic fibrosis. She did well in school and studied social sciences at UCD. She moved to Jersey after college where she met her husband-to-be, Rui Ramos.
“However, when Allsun was in late 20s, her lung function started to drop and she was in hospital for eight months of each year for about five years. She wasn’t even able to walk upstairs. These were really tough years. I studied finance and accounting in Maynooth University and then studied to be an accountant. [I now work as an accountant for a pharmaceutical firm]. I had planned to go to Australia for two years but in the end only went for two months. My parents talked about converting a room in their house in Castleknock for Allsun and in some ways, we began to prepare for her passing away.
“She waited for three years for a double lung transplant and had three false hopes. For the family, it was an emotional rollercoaster. Finally, she flew over to Newcastle with my mother for a double lung transplant in 2011. Allsun’s lung function is now in the normal range but she has to cope with a lot of medications following her transplant. She has a very positive attitude and is dedicated to being as healthy as she possibly can be.
“My relationship with my sister completely changed when her health started to deteriorate. I realised how much I loved her and our bond has grown since then. We see each other about twice a week and we speak every day. However, I also realised that during my 20s, I kept my head down and swallowed my own tears a lot. I did a diploma in psychology and mindfulness courses to help make sense of my repressed emotions and numb feelings. No counselling or psychological support was ever offered at any stage to Allsun or any of the family. Luckily, we are good open communicators and used humour to mask all the fear and pain that cystic fibrosis can bring to a family.”
Cystic Fibrosis Ireland’s annual fundraising 65 Roses Day is on Friday, April 13th. Donate at cfireland.ie or LoCall 1890 311 211.