Life with CF: An Interview and Insight

Our current life events in shutdown and quarantine remind me how twenty-five years ago when my son was diagnosed with cystic fibrosis, my world stopped. My immediate, “protective mom” reaction was to create a bubble existence, a sanitary safety-net surrounding my child for as long as possible.

Our introduction to reducing cross-contamination of germs prepared us for our future. However, it was mixed with a new parent’s excitement and the dreams we held for this little person. Who will they become? What will they accomplish? Where will their lives lead? Unfortunately, life in a bubble leaves little space for such dreams to flourish. Our alternative was to face the reality of life with cystic fibrosis outside a bubble.

Interviewing my son who has CF

Watching the statistics of COVID leap and bound, coupled with the novelty of quarantining, I felt the need to inquire and, in some way, offer insight for what life in a CF world has meant for my son. We’ve had conversations about cystic fibrosis before, but never did he offer such sage understanding and personal perspective. The questions I posed led me down a path I wasn’t necessarily prepared to travel, but regardless, his responses were authentic, intense, and in some cases quite raw.

Mother: How do you view yourself?

Son: That changes based on how I’m feeling, but right now, I feel healthy and happy with a new job and new prospects for the future. At times, I can be very pessimistic based on the level of my health; not sure of what my future holds, feeling like I live in limbo. Trikafta has offered a great deal of optimism, as my health has improved. Before Trikafta, when I woke in the morning the first thing I had to do was a treatment because I couldn’t breathe. Now the first thing I have to do is eat.

Advice to others

Mother: What advice would you give people who have just been diagnosed with CF?

Son: Don’t let CF limit you. Know your limitations, but don’t let them stop you. While CF can be the reason why you can’t do something, it shouldn’t be the reason why you don’t try to do something; there’s a difference. Talking to other CF caregivers or patients can offer insight and support.

Remember to focus on self-care. It is vitally important for the patient and the family. Journaling and poetry are both effective tools when dealing with a chronic illness. It helps to get your thoughts out of your head and can even be healing looking back at the progress and growth. Explore your options with doctors and care teams, second opinions are at times helpful and important. Options aren’t always presented, so it’s really important to advocate for yourself or for your child; specifically, asking to explore different options for meds and/or tests. Also, it is important to take care of one’s mental health as much as CF.

Positives and negatives

Mother: What’s the hardest part of having your disease?

Son: Breathing, remaining positive, planning for the future; in that order. The daily healthcare routine is difficult and trying to remain positive varies on the level of intensity of the workload. Overall, cystic fibrosis affects my mental outlook, and that in turn can affect my physical health.

Mother: What is the biggest positive about having cystic fibrosis?

Son: I feel like I have a different understanding of time. Time is fragile.

CF and mental health

Mother: You have mentioned mental health a few times in our conversation. What are your thoughts about overall mental health and how it relates to CF?

Son: Memories of isolation at a young age, anger at limitations, physical pain, IV meds/therapies, and fear are all triggers for me and depression; they put me in a mindset that is unhealthy. Understanding how chronic illness can cause not only trauma, but pain, as it relates to both depression and anger is the first step to a healthier mind. Mental health needs to be a major component of CF care.

Mother: What role has fear played in your life?

Son: Fear is a double-edged sword, it can push me in a positive direction and can be a motivator, but the fear of dying can take over, it can play with your head. Entertaining the thought while at the same time fearing an early death, convincing oneself it’s just inevitable, is a dangerous path. The fear of not accomplishing all I want to in life; a family, home, career, makes me strive for more.

It’s like the perfect storm, there are factors all working together, both for and against you. They can either be motivating or discouraging. Trikafta has been a positive change. I am physically feeling better, my daily healthcare is lighter, and my mental health is in check. I’m more positive, less depressed, and less angry. Looking back before Trikafta, I can see how much control fear had in my life. Now my outlook is brighter, and I see life differently.

Motivations for the future

Mother: What should people know about 25-year-old you; what motivates you now?

Son: That’s a big question. A lot of things motivate me for different reasons, but overall I want to be sure that I am having an impact somewhere in my life. I want to have an impact on people’s lives both related to CF and not. I’m motivated by wanting to feel like I belong, with a job, friends, family; everyone wants to be included.

I’d like to be looked at differently, as a redemption story, having cards dealt and not knowing the outcome, yet persevering, pushing through, and remaining positive. People should know I tried; I work hard, I don’t let CF dictate my life. I want to prove that I deserve to be here and live a long life. I can make a difference with my life.

A final note

While the statistics of COVID-19 continue and the fear of what it means for all of us is ever-present, our bubble existence has eased somewhat. Small fractions of normalcy sneak back into daily life, albeit while donning a mask. We owe everything to Trikafta for the renewed hope it has offered and the possibilities it has revived. What the future holds is anyone’s guess, but in reality, our lives are left to chance, so that once again those dreams of long ago have new light and new space in which to flourish and grow.