She revealed what life is like living with the disorder to mark Cystic Fibrosis Day and the long-awaited delivery of Orkambi.
Cystic Fibrosis Day: Dublin mother shares son's experience of living with disorder
Today is Cystic Fibrosis Day, and CMRF at Crumlin Hospital is urging the importance of medical research into the disorder as families across the country receive access to the life-changing Orkambi drug.
A Dublin mother has shared her family's story as they deal with their son Alex's, 6, experience living with cystic fibrosis in support of the day.
Julie O'Herlihy was told that Alex would be born with cystic fibrosis during her pregnancy, and spent the last 14 weeks of her pregnancy learning about what life would be like with a child who has cystic fibrosis.
She said: "Alex was born in the February and there was a big team waiting to meet him. The good thing about knowing he had CF before he was born was that they had everybody on standby.
"After his arrival we did what all new parents do, try and figure out having a new-born, and after two weeks we started our daily trips to Crumlin Hospital to meet his team of doctors, physios, dieticians and nurses. He started on his digestive enzymes as his pancreas didn’t function properly and we learned how to do physio with him.
"So coupled with having our first baby we had to come into Crumlin most days for the first few weeks. As parents we become even tighter and self-sufficient."
Despite his condition, Alex has the same life as any other six-year-old - just with his regime of physio and medication added in.
Julie continued: "He’s six years old now and he’s on a strict regime of physio and takes up to 30 tablets a day, every day. There are no breaks. He’s never known not having CF so he does this easily. It’s routine.
"Before breakfast he does a nebuliser of hypertonic saline, to break down the mucus in his lungs, and follows that by PEP (Positive Expiratory Pressure) which clears the mucus out. Then he has his breakfast with his enzymes and vitamins. He doesn’t break down fats properly, due to the blockage in his pancreas, so he takes some of his vitamins, A, D, E and K, in tablet form.
"Then he goes off to school. He knows to take out his little enzyme package at lunch and take them then. After school he does another set of physio along with a second nebuliser, this time pulmozyme. He takes more enzymes with dinner and indeed all snacks containing fat throughout the day and lots of exercise is encouraged."
Although they are a positive family and don't allow Alex's cystic fibrosis to stop him enjoying life, Julie admits that it's tough and that there is a big element of fear involved.
She said: "Even though we’re positive, as parents, it’s tough. Fear is a big emotion in our house. Fear of infections. Fear of the unknown. We’re constantly on alert. He’s had a lot of antibiotics this winter, but thankfully we’ve avoided any hospital admissions.
"Orkambi won’t change his daily routine but we hope he will require less antibiotics and be able to deal with everyday coughs and colds better. We’re hoping Orkambi will keep Alex out of hospital and as well as he is today going forward.
"His teacher described him at his parent teacher meeting as ‘an extremely empathetic little fella.’ He’s very intelligent, caring, kind, adaptable and he never moans or complains. We’ve taught him to be resilient.
"I think when he starts Orkambi one of the dark clouds will be lifted from over my head. Alex was born in the right age and I’m so hopeful about the future and the drugs that will be created.
"The thing for us as parents is that we need to keep Alex as well as we can now so that when the future drugs come out his lungs will be in the best shape possible."
Professor Paul McNally is leading research into Cystic Fibrosis at the National Children’s Research Centre and hopes to better understand the evolution of lung disease in children with CF with the SHIELD Cystic Fibrosis study.
The study has been funded through the Children’s Medical and Research Foundation (CMRF) Crumlin and the National Children’s Research Centre (NCRC) from its inception and involves over 250 children who have attended Our Lady’s Children’s Hospital, Crumlin, Tallaght and Limerick.
“Survival in Cystic Fibrosis over the last 20 years has come a long way from when I was a medical student where the average predicted survival was 25 years. Now it’s 41 years.” Professor Paul McNally said.
“That is a result of the combined efforts of hundreds of research teams all around the world. A recent study published in the European Respiratory Journal forecasted that the total number of patients will grow by 50% by 2025. The number of CF adults will increase by 75% and the number of children by 25%. Because of new born screening with the heel prick test, most babies with CF should now be diagnosed quickly.”
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