Testimonial: Being CF at sixty

By Manon Goulet

Sharing your journey is not easy. There is the fear of being judged or being accused of wanting to get attention... But what motivates me to tell you my story is the desire to give hope to those who, like me, have cystic fibrosis.

I am in my sixties and have cystic fibrosis.
I am one of 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, cough, pneumonia, asthma, secretions and FATIGUE! It must be said that in the sixties, the life expectancy of children with cystic fibrosis did not exceed kindergarten age. I think that despite all these symptoms, which today would undoubtedly have been linked to cystic fibrosis, this diagnosis seemed less and less feasible for the doctors I consulted, since I was still alive.
Despite everything, I had a full family and professional life. I have a spouse with whom I have shared my life for forty years and two children whom I love. Not to mention two grandsons that I love madly. When my pulmonologist announced my diagnosis, my first thought was for my children. What a terrible feeling to have transmitted such a terrible gene without my knowledge!

Over time, the symptoms worsened, 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, combined with repeated infections, gave me the impression that everything was becoming a mountain that I no longer had the strength to climb. I had to grieve a job that was demanding, but also so rewarding.
I asked myself 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 under 50%, I had the feeling that my life consisted of doing activities that were carefully chosen according to the fatigue that inhabited me.

Then I started knitting. It must be said that the arrival of a grandson was a great incentive. It allowed me to focus on a project, to acquire new knowledge and to achieve something concrete. It was completely in line with what my three daily goals had become:
• Learn something new;
• Do something for someone;
• Laugh every day.

Laughter can do so much good. Especially when everything seems gray. No need 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 required a lot of energy after a day at work. Now I can take a nap after doing some physical effort, that helps a lot.
I was even encouraged to start a running program gradually, with one-minute intervals to get started. 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 the effects very
positive, especially on my respiratory function, but also on my energy.
Even though it does not cure cystic fibrosis, and I need to continue with the usual treatments and therapies, the positive impact on my health is significant.
I still need a lot of hours of sleep and napping is a part of my daily routine, but I have developed a routine that allows me to be diligent in my treatments, to include a bit of 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 like Trikafta to Health Canada. This would allow 90% of Canadians living with cystic fibrosis to improve not only their condition, but also their quality of life, in addition to extending it.
I know that I am lucky and I hope that very soon we will all be able to benefit from new treatments and thus live with the hope of a full life and of all reaching retirement age one day!

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