15 July 2021

The Day We Waited 11 Years For...

This was the moment we've waited his whole life for: On morning of July 1st, Bennett swallowed his first dose of Trikafta, the first medication approved by the FDA for children ages 5-12 that addresses the underlying cause of Bennett's specific type of cystic fibrosis.  

Each pill costs about $270 each.  Bennett literally swallows nearly $900 a day to keep himself healthy.  In total, Trikafta costs about $300,000 a year.

Out of the 8 daily medications Bennett takes, this is the only one that is not prescribed to stop the symptoms of CF.  This one is prescribed to actually stop CF itself.  

Trikafta is no cure.  But we have some real hope that this medicine will significantly slow down the progression of his disease.

Trikafta comes in a pill pack.  It's the combination of three meds (think trifecta): ivacaftor, elexacaftor and tezacaftor.  Bennett is instructed to take two pills in the morning.  He takes an evening pill exactly 12 hours later.  To absorb the medicine, he also has to eat something with fat in it.  Bennett is expected to take this medication for the rest of his life.

The expectation that Bennett would be getting on Trikafta soon has been a family experience.  We've spent lots of time talking about it and looking forward to it.

The night before Bennett's big day, Avonlea (age 7) spent an hour hiding in my bathroom to make Bennett a handmade "Trikafta" sign with which to surprise him the next morning.  

We had known this day was coming for months, even years.  I thought we were ready until a few nights before the medicine arrived.  One night, at dinner during a conversation about our anticipation of Bennett getting on Trikafta, Avonlea reflectively lamented, "I'm going to miss Bennett not doing his Vest anymore."  

It was such a sweet comment.  She loves her brother dearly.  His doing his CF treatments is all she has ever known since she was a baby.

I reassured her his treatments weren't going away, "oh, Bennett will still have to do his Vest.  The medicine will help but he won't be able to stop his treatments and taking his medicines for a while."  

Bennett abruptly put his fork down and curtly responded, "so this is a downgrade?!"

Part of me wanted to burst into laughter and tears at the same time.  It was so funny to me that my Generation Z child was using computer technology language to articulate the impact of Trikafta to his treatment regimen.  And part of me was so sad to think that, as exciting Trikafta is to all of us, the reality of the situation for Bennett would be that he would ultimately be adding *more* treatment to his already burdensome treatment regimen.

I reassured him that it wouldn't be like this forever.  We don't yet know how Trikafta affects the body long-term.  Doctors don't know yet what impact it will be to stop the medications prescribed for addressing the symptoms of CF.  When is too early to withdraw meds?  What's the impact if we take off this med versus that?  Nobody yet knows, not even the researchers.

The night before Bennett was to take his first Trikafta pill, another important conversation came up.  As we were saying prayers before bed like we always do, Bennett began to become emotional.  He shared about his fears of taking Trikafta.  He shared how he likes himself just as he is and how he didn't want to change. He shared how he was afraid of gaining weight, becoming "fat" and even losing his g-tube.  

It occurred to me that even though I've wished CF away all his life, CF is all he knows.  His body has CF and getting rid of CF is, in some way, risking getting rid of his body as he knows it.  I had to really breathe deeply and accept that even in good gifts, there can be losses.

I tried sharing with him why it was ok.  I wanted him to be ok.  But found the conversation to be futile.   He needed time to grief and nothing I said was going to speed that up.

I realized I needed to give him the control of this big change in his life.  As much as I had waited for 11 years for him to start this medication,  I told myself that we could wait a few days or weeks longer.  

I said, "I want you to take Trikafta tomorrow because I think it will make you feel better.  But I'm going to let you decide whether or not you want to take it.  It's your body.  If tomorrow isn't the day, let's wait.  You take it when you're ready.  It's up to you."

That seemed to help.  Bennett seemed to appreciate the space to make the decision that felt right to him.

But, I was pretty quickly reminded that while Bennett is now a pre-teen and has grown up moments, he's still a child who is a sucker for candy.  When I asked Bennett if he wanted me to get him a Snickers bar to take with his Trikafta, he quickly agreed tomorrow would be a great day to start. 

And that was it!  He was all in!

I first learned about the real possibility Bennett getting access to a medicine that could help him back in 2016 (see:  Here we are, five years later, with that medicine in our hand.  

I feel incredibly blessed by the gifts of science and the many blessings in forms of donations to the CF Foundation that came from people who care for Bennett and other people with cystic fibrosis.  Thank you to everyone who helped us get to this point.  The fight isn't over but we have a pretty powerful tool in the toolbox that we've never had before!