By Manon Goulet
It’s not easy to share his journey. There is the fear of being judged or of being accused of wanting to attract attention … But what prompts me to tell you my story is the desire to give a little hope to those who , like me, have cystic fibrosis.
I am in my sixties and have cystic fibrosis. I am one of over ten Canadian adults who are diagnosed with cystic fibrosis each year. In fact, it was in 2016 that my condition was discovered. It was a shock but also a relief. Everything was becoming more logical. Multiple sinusitis and sinus surgeries from childhood, coughs, pneumonia, asthma, secretions and FATIGUE! It must be said that in the sixties, the life expectancy of children with cystic fibrosis did not exceed the age of kindergarten. I believe that despite all these symptoms, which today would undoubtedly have been linked to cystic fibrosis, this diagnosis seemed less and less possible for the doctors I consulted, since I was still alive. Despite everything, I had a fulfilling family and professional life. I have a partner with whom I have shared my life for forty years and two children whom I adore. Not to mention two grandsons that I love madly. When my pulmonologist told me about my diagnosis, my first thought was for my children. What a dreadful feeling to have passed on such a terrible gene without my knowledge!
Over time, the symptoms got worse, making my daily life more and more difficult. Then suddenly, in my late fifties, I had to stop working. It was as if I didn’t have the strength anymore. This insurmountable fatigue, coupled with repeated infections, made me feel like everything was becoming a mountain I no longer had the strength to climb. I had to come to terms with a job that was demanding, but also so rewarding. I wondered what my life was going to become, what I was going to do. I had the feeling that I would not be able to overcome this fatigue. With an FEV1 below 50%, I felt that my life was all about doing activities that were judiciously chosen based on my fatigue.
Then I started to knit. It must be said that the arrival of a grandson was a great incentive. It allowed me to focus on a project, gain new knowledge and achieve something concrete. It was completely in keeping with what had become of my three daily goals: • Learn something new; • Do something for someone; • Laugh every day.
Laughter can do so much good. Especially when everything looks gray. You don’t have to laugh out loud for hours, just a little laugh can be beneficial. Having more time also allowed me to focus on physical activity to improve my lung capacity. In the past, my workouts were constantly interrupted due to respiratory infections and used up a lot of energy after a day’s work. Now I can take a nap after exercising, that helps a lot. I was even encouraged to start a running program, gradually, with one minute intervals to start. My goal is not to run a marathon, but rather to improve my cardio-respiratory function. It should also be mentioned that I benefit from modulator therapy. I feel very positive, particularly on my respiratory function, but also on my energy. Although it does not cure cystic fibrosis, and I must continue with the usual treatments and therapies, the positive impact on my health is significant. I still need a lot of sleep and napping is part of my daily life, but I have developed a routine that allows me to be diligent in my treatments, to include some physical activity, rest, fun and, of course, knitting! We must continue to hope that Canada will change the rules for the treatment of orphan diseases to allow pharmaceutical companies such as Vertex to submit new drugs such as Trikafta to Health Canada. It would allow 90% of Canadians with cystic fibrosis to improve not only their condition, but also their quality of life, in addition to prolonging it. I know I am lucky and wish that very soon we could all enjoy new treatments and live with the hope of a full life and of one day reaching retirement age!