I used to write more. Maybe I used to have more to say. Maybe writer’s block is hanging around longer than usual. Maybe my ego has cooled and I don’t think anyone cares about what I have to say anyway.  But I used to write more.  

I used to care more about people across communities with needs. Maybe having two kids made my world shrink a lot.  Maybe ten years as a prosecutor made me numb. Maybe I’ve fallen into the trap of knowing I can’t change much anyway, and therefore should only worry about myself and my family – even though it is their world that is on fire. But I used to care more.  

I used to be more patient. Especially with my son.  Maybe parenting a 7 year old is harder than parenting a 3 year old, but I don’t really think so. It certainly isn’t his fault.  

I used to be better at ignoring odds that say I shouldn’t be here and not constantly worry about my longevity. Maybe that’s what happens to everyone in their 40’s because we’re all getting older, and everyone is experiencing loss now, not just people with CF, and it isn’t just me.  But I used to live without that anxiety, even when my outlook was worse.  

I used run and bike more. Sometimes it was easy and sometimes it was hard, but fast or slow I used to do it more. Now I have to drag myself out the door.  Maybe that happens to parents with young kids, but I used to run and bike more. And I used to set more personal goals. I’ve done a lot. Maybe marathons are bad for my knees now. Maybe I don’t have to prove anything to anybody, myself included.  But I used to set more goals.  

I used to be quick to express concerns about misguided (in my opinion) policies and their unintended consequences on people with CF and their families to my care team and leadership at the CFF.  It’s been awhile since I’ve done that.  Maybe we’re all cynical now.  Maybe it was the pandemic. Maybe it was the political vitriol and damage of the last 8 years. But I used to engage. 

But maybe it is something else.  I’m not the only person with CF taking modulator therapies to experience emotional detachment, brain fog, lack of focus, and increased anxiety and depression.  It is not hard to find individual accounts of severe personality changes, depression, self-harm, and even suicidal ideation. I am not the first person to express these concerns.  I was in the Promise Study.  It was not designed to catch longer term or lagging negative side effects of Trikafta, and if it was, it failed. The FDA has not responded to filed concerns about these drugs. Vertex has no more  incentive to address potential serious side effects of its blockbuster drug than it does to ensure global access to people in need. And now that drug is approved for use in the youngest of children with CF. CFF was deeply invested in making these drugs work. No matter that Europe has changed its warning label. It messes up a really good story. We all want these drugs to work. No one wants to feel like they are being forced to pick between their brain and their lungs. But here we are. 

For many drug-eligible people with CF it is obvious that these medications improve lung function, and for many without serious side effects.  Thousands of people’s lives have been extended and changed on Trikafta.  This would be easier to write if that weren’t the case. But nothing is black and white.  The world is complicated, and Trikafta has been no exception. I don’t have a problem with that. That’s life. These modulators are first in class drugs. That means risk. I get it. However, that risk is all the more reason for clinicians and researchers to have been listening closer to patients expressing these serious concerns, and giving these patients appropriate validation in clinic, and raising some eyebrows and alarm more broadly.  In my experience and opinion, voicing these issues in my clinic, to care teams at other institutions, and to CFF leadership, it has taken years longer than it should have to begin to take these patient accounts seriously.  We spent two years saying it might be the pandemic. It was not the pandemic.  And then we threw our hands in the air saying its too late, we can’t unwind and study the drug correctly to know what’s really going on. We don’t have any data about how to reduce prescribed amounts and maintain efficacy. And nothing has been done. And the years went by, and prescribing eligibility expanded, and now this drug is approved and being prescribed to otherwise healthy 2 year olds with CF.  

At this point, can a pediatric CF physician credibly discourage parents of an otherwise healthy, thriving 2 year old with CF from starting Trikafta at such a young age?  The drug is FDA approved. These issues are not listed side-effects. So now this 2 year old, who does not know what her normal emotional stasis is, or what memory should feel like, or how her brain usually functions will be given this drug.  For years. This is a failure. Even if only 5-10 percent of people with CF are experiencing these side effects on these drugs. This is what failure looks like. 

I have heard CF Center Directors state that their goal for their clinic is a “one hundred percent prescribing rate for modulator eligible patients.”  Maybe that is a sound bite and an easy bullet on a powerpoint.  But it demonstrates zero interest in listening to patients who are not tolerating these drugs. And this is a failure too.    

 To every person living with CF who has had these experiences with modulator therapy, you are not alone.  To every parent of a child with CF who thinks their child became a different kid, unhappy, distracted, and withdrawn, you are not alone.  Your experiences are valid, you are right to question whether or not modulator therapy has caused at least some of these changes.  And to any clinicians reading this, it is not too late to do better tomorrow. 

-If you or someone you know is experiencing negative medication related side effects, please speak to your care team or a provider you trust as soon as possible.-

***The views and opinions stated are those of the contributing author and do not represent any collective position of Breathe Bravely , the organization. Breathe Bravely encourages this community to use their voice boldly, understanding that we each have a different story to tell. What’s most important to Breathe Bravely is that each voice can find a place of belonging - one that is rooted in encouragement, reflection, and respect.