Born with a disease that kills but not at death’s door: Living in that in between

At summer camp, the number of my cabin mates dwindled each year. Not because of the lack of activities or negative social atmosphere but because each year more and more of my cabin mates passed away. I would think “where’s Holly?” and be told “Oh, she didn’t make it back this year.”

I became conditioned to death in my Cystic Fibrosis summer camp. I knew we all shared the same disease that affected us from birth that limits our breath and would ultimately end our lives.

I first heard the words “Cystic Fibrosis” at 5 years old. I remember the pale-yellow walls of a depressing hospital room. I remember playing with my new pencil sharpener that was in the shape of a little globe. And I remember the gravity of the room – being scared, sad and confused. After five years of struggling with malnutrition even though I was eating, and other peculiar symptoms, I was diagnosed with Cystic Fibrosis (CF).

CF is a genetic disorder that impacts multiple organs, but mainly affects the digestive system and lungs, where unusually thick mucus becomes a breeding ground for infection, degrading lung function over time. The destruction of the lungs leads to death in the majority of people with CF.

I quickly learned that having CF meant I had to breathe in medicine through a mask apparatus; I needed my lungs thumped for drainage therapy; and I needed to take tens of pills every time I ate. I had to do therapies three times a day for a total of five hours, every single day.

I’ll always remember her reaction, how her mouth hung wide open in complete and utter shock.

Early in my childhood, my best friend saw me with my mask apparatus on and I’ll always remember her reaction, how her mouth hung wide open in complete and utter shock.

That was a pinnacle moment in which I knew I was different.

I went through awkward teenage years when I didn’t want to be the girl with CF. I stopped taking my medication at meals to steer clear of attention. It wasn’t until I started coughing up blood in university that I started to take my disease more seriously.

I had this epiphany that I should only be ashamed of the choices I’ve made but being ashamed of a disease that I was born with is ludicrous. I then sought out my first support group for people with CF and began doing volunteer work for CF Canada.

At CF Canada, I help with fundraising initiatives and do advocacy work as a spokesperson. It’s also the place where I found community within this complicated disease. Twenty years ago, we learned people with CF can’t be around each other because we transmit bacteria from our lungs to one another. We knew masks and six-feet-apart well before COVID-19 days. Our community was devastated but resilient through the pandemic, and we continued to find ways to stay in touch despite not being in the same room.

As a child with CF, I was hit with this adult issue of mortality – something no 5-year-old should be worrying about. When I was born in 1971, the median survival rate for women with CF was 20 years old. I had a fundraising poster in my room that said, “Julia has CF and she turned 12 years old, she has reached middle-aged.” I’ve chased that median age of survival my whole life.

There was a period as a teenager when I engaged in high-risk behaviour because what’s the point in caring if I’m going to die so soon. But then when I thought I saw the finish line, it kept getting pushed; I had to start thinking about university, then marriage and now planning for retirement.

I am living this full and happy life, but I’m always on the edge. Every time I have a weird cough, I’m reminded of this issue of mortality. I think, “Will this cause a hospitalization or lung transplant?” I worry about what will happen to my husband and the other people I would leave behind.

Ten years ago, I started running, which progressed from walking on the treadmill to running half marathons. Running means everything to me because I’m breathing despite the statistics that have told me I wouldn’t be able to at my age. I’m sure any runner finds it empowering, but the knowledge that I can run with this disease that plagues my lungs has a whole other level of meaning to me.

It’s not about the speed, and it’s not about winning – it’s just about being able to run.

I do not represent the whole CF community. I’m one of the lucky ones – this statement comes with immense gratitude but even more survivor’s guilt. I have a master’s in creative writing. I work a full-time job and have stayed out of the hospital. I only have three hours of treatments a day, whereas the average person with CF endures six hours. I’ve made it to 51 years old, which is sadly not a reality for many people with CF. I’ve celebrated 25 years of marriage and live with my husband, dogs and cat.

However, I still have this disease: I have all its mental health challenges, if not all the physical ones, so I’m left piecing out where I belong in this community and in my relationship with this chronic illness.

I live alongside my disease, but it doesn’t overtake me. Despite its challenges, CF has brought positivity to my life: How to live in the awareness that life is short, to help people suffering with disease and to take my own health seriously.

You can’t separate CF from me because it is part of who I am, but it is not exclusively my self-identity. I am empowered to live in this existence, knowing that I live alongside this disease, yet I still am a multi-dimensional person with my own talents, passions and zest for life.