"The thoughts of a Father" by Joel Goodrow

Hello friends! This is something that my Dad wrote for me in honor of CF Awareness Month. I hope you enjoy hearing his thoughts. :) - Elena Goodrow

This is a letter to those people with cystic fibrosis that grew up during a time of incredible progress for the disease. Many of you were the first to receive incredible drugs that treat the symptoms and underlying causes of CF.  I am a parent of one of those people and I wanted to share my experiences in hopes that it helps you understand your own Generation X or Millennial family members.  The relatives that supported you as a child can never truly understand what it’s like to have a life threatening genetic disease, but they were around you witnessing the transformation of CF medicines and living life on the edge of breakthroughs that would forever impact you and the whole CF community.

I was completely ignorant when you were born.  Progress in gene science was steady during the 1990s, but technology was still being developed and wasn’t widely used as part of routine healthcare until the mid 2000s.  Genetic testing of pregnant people to screen for common diseases wasn’t regular at our doctor’s office back then, so we had no idea that we were carriers of CF. Thankfully, most people are now screened for common genetic problems during routine care. When you were tested for CF as a baby, we waited several agonizing weeks for results because the specialized process took so long.  Concerned friends and coworkers were surprised that we were blindsided by this disease, and everyone was shocked to learn that CF was the most common fatal genetic disease in North America. Universal newborn screening wasn’t instituted nationally until 2010.

The average age of survival for a person with CF was about 30 when you were born. There were medicines on the horizon, therapies in the pipeline, and whispers of miraculous cures within 5-10 years. We didn’t know how you would benefit from new drugs or if it would make a difference in your life.  When you were a child, our effort to extend your lifetime was focused on administering three new treatments. Inhaling Pulmozyme to thin the sticky mucus in your lungs, inhaling TOBI to fight lung infections, and hooking you up to a violently shaking high-frequency chest wall oscillation vest.  You were young, innocent, and irritated that your evenings were spent hooked up to machines. We were fighting a battle with new weapons and the promise of a brighter future.

When you were a little kid, we found out more about your disease and how it was impacting your lungs and digestion specifically.  Learning how to feed you the best foods and ratio of digestive enzymes was a daily experiment. Going to CF specialists and visiting our pediatrician was part of our routine as we dealt with concerning respiratory infections, digestion medication doses, and general follow up about your well-being.  Sometimes it was terrifying since relatively small concerns like a childhood cold or being slightly underweight could be the beginning of a serious issue that would take months of recovery effort. Managing social time with other families was hard because sharing a minor sickness was a major deal for us and even well-meaning friends exposed us to illnesses that meant yet another round of antibiotics for you that year.  How much constant medical attention can a child take? At what point is being sickly the new normal? How were these challenges going to affect your overall health and lifespan? You were innocent and didn’t know that you carried a different burden than those around you.

We know now that new treatments did cause a huge statistical increase in life expectancy of those with CF, but that trend was just starting when you were young. Watching many young people struggle and die was a sobering reminder that the future was very uncertain and the impact of CF varied widely for each individual. We are all mortal, of course, but the expectation that you’ll outlive your own child is traumatic. Processing the mortality of your own child is not a natural feeling for a parent, and for many years your birthday was a reminder that the clock was ticking faster for you and your life expectancy was in flux. Annually processing this grief was emotional and lonely, but each year brought new hope that you would someday grow old as a result of medicine that addressed the root cause of your disease.

Later in your formative years as a teen and young adult, the individual drugs for what would become Trikafta were in the FDA approval process and being rolled out. Early recipients in the CF community were reporting improved health as they took drugs that would be combined to form effective CFTR modulators. It was a very exciting time. It was supposed to be a cure. You were already growing up and changing so much as a person, it was hard to identify which changes were the result of taking new medicines and what changes were just plain you! It was going to take a while to figure that out, and there was so much going on in your life.

Whether you liked it or not, becoming an expert at navigating the insurance and healthcare system was necessary to make sure you had the tools to thrive as an adult.  Every year we were in the middle of a battle between the pharmaceutical companies, the insurance companies, and the government.  Why must those born with a genetic disease fight so hard for fair healthcare while they are also fighting hard for good health?  It was stressful and didn’t feel fair.  Sometimes small oversights in the system meant delays that created dwindling supplies of critical medicines that were part of your daily regimen.

It’s difficult to fathom that in our lifetimes the disease that caused such urgency and fear is being transformed into a condition that is managed well by taking some pills. Children born with CF today could expect to live into traditional retirement years, and many of you can see that future unfolding before you as well. I feel so privileged to be a witness of the incredible transformation of CF treatments and the growth of the next generation. You are living the future of cystic fibrosis and my sincerest wish is for continued amazement at how the world around you changes next.