Life with Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the digestive system, lungs, and other organs in the body caused to a rare genetic mutation. It affects cells that produce sweat, mucus, and digestive juices. These fluids are normally thin and slippery, but in people with CF, a defective gene causes the fluids to become sticky and thick, and instead of acting as lubricants, the secretions plug up tubes and passageways, especially in the lungs and pancreas. Although cystic fibrosis is progressive and requires daily care, people with CF are usually able to attend school and work. They often have a better quality of life than people with CF had in previous years. Improvements in screening and treatments mean that people with CF now may live into their mid-to-late 30s or 40s, and some are living into their 50s.

The disorder is extremely rare with it only affecting around 900 people in Scotland, one of which is Matthew McGill. Matthew is 19 years old and lives in Paisley, he has been affected by CF his whole life having been diagnosed at birth. Matthew said: “It's just something I've always had to deal with day to day, I sometimes have difficulty breathing and I have an insulin tube on me 24/7. I am used to it because it's been like that since I was born and I've had to send some time in hospital growing up when it got bad.”

Advances in understanding of CF mean that there are more effective treatments, so people with the condition are living longer. Around half of those with the condition live past 40 Matthew said: “I don’t like to think about my life expectancy because it’s not going to change, I shouldn’t worry about it, and I don’t need anyone to feel sorry for me. My family and friends are supportive so that’s all that matters in my eyes”. In 1964, people with cystic fibrosis would be lucky to live past five years old. Now, according to the UK Cystic Fibrosis Registry Annual Report for 2016, half of those with the condition in the UK will live to celebrate their 47th birthday, and a baby born with the condition today can expect to live even longer.

CF comes with a 98% infertility rate amongst males which can only be described as heart-breaking for those diagnosed and their families. This means that men cannot biologically father a child without help from fertility specialists. Matthew said: “I am not thinking about having children anytime soon but it is something that hurt me when I first found it out, there is still a chance that I can have kids later on in life though and id do anything for that chance, it does make me think about whether or not it would be a good idea or not to leave my kids without a dad for most of their life. I generally try not to think too far into the future and just to take each day as it comes and make the most of every day”.