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!

29 June 2021

Goodbye Waco, Hello Dallas!

The worldwide pandemic changed the world.  Our world included.  

The kids and I faired really well during a year of COVID. No one got sick and everyone was able to make it through a year of homeschooling while I worked from home (thanks to amazing neighborhood friends and a fantastic babysitter).  But our time staying safe from COVID gave me an opportunity to recognize some unmet needs in our family during those months of isolation.

After careful consideration, in January, I began the process of moving the children and myself to Dallas, Texas - closer to Bennett's CF care, closer to more educational opportunities and closer to more family support.  

I was pregnant with Bennett when we moved to Waco twelve years ago. We had expected the move to be temporary and it became permanent, which I loved. Waco is such a great place to live and raise a family.  I am so very grateful for the many years and many friends we made in Waco. We have so many sweet memories. 

Our move to Dallas is not too far from Waco, only two hours "up the road". We plan to visit as much as we can. The kids will continue to spend time with their dad there.  

This season of change for our family goes beyond just moving. Bennett is currently getting access to Trikafta, the medication that we hope will change his life.  And this Fall, Bennett is moving into his last year of middle school while Oliver begins a final year of junior high.  

While we are feeling very positive about these changes, we know grief is also a real part of change. Loneliness has a way of settling as the newness of moving in fades away.  

What keeps me grounded is knowing that I've felt God's prompting throughout the move.  While I was initially hesitant to consider moving away, every curious step I have taken has been met with open doors and a sense of peace.  

I've thought several times about Isaiah 43:19 where God says: "Behold, I am doing a new thing..."

I don't totally know what new thing God is doing in this season of our lives.  But I do know, whatever God is doing, it is good.

09 June 2021

Trikafta Approved!

Joy!  That is what our family feels today!! 


This morning, with one eye open and the other still shut, I woke up, rolled over and opened my smartphone.  I clicked a notification to find a link sent to me by another mother of a child with cystic fibrosis. My heart lept: 

It's approved!!!!!! 

A few minutes later, Bennett walked in my room. I was delighted to share with him the good news, "Hey buddy, your medicine has been approved!"  He grinned and gave me a high five!  It was the news we were waiting for.

Trikafta, the first medication approved by the FDA that benefits Bennett's age group and Bennett's genetic mutation by addressing the underlying cause of his cystic fibrosis, is now available for Bennett to take!  We are now within weeks of starting this new potentially life-changing therapy.

If you've prayed for Bennett, walked for Bennett, raised money on behalf of Bennett and/or followed Bennett's blog, THANK YOU!!!  It is our hope that you will celebrate this huge gift with us!!  You are part of his story!  You are part of *this* story!!

We have so much to be grateful for.  Bennett is 11 years old and has stayed very healthy so far.  His lung function currently sits at 89%.  However, he has never had a cough or acquired a lung infection that required IV antibiotics.  We hope the Trikafta will increase Bennett's lung function to 100%, which would be considered normal for any child.

Bennett is in the 40th percentile for his height but he struggles most with his weight.  Despite a g-tube and daily tube feedings, Bennett's weight is in the 13th percentile (his doctors prefer his weight to be at 50 percentile).  So, we hope Trikafta will help Bennett's GI system work more efficiently allowing him to keep the nutrients he takes in.  And, if possible, we hope Trikafta might even allow him to get rid of the g-tube (something he's never lived a day in his life without).  Additionally, we hope Trikafta will extend Bennett's life expectancy.

Trikafta, which has been approved for people ages 12+ since October 2019, affects every person differently.  We don't know if it will work well for Bennett.  There have been some side effects that have been difficult enough for some people that they've had to stop taking the medication.  But clinical trials seem to indicate that children have tolerated the medicine well.  So, we are very hopeful.

I wrote in my blog many years ago about what it feels like when the CF community rejoices at the approval of a drug for those with cystic fibrosis, but a drug in which your child cannot benefit.  This was the case in 2012.  There was so much excitement and so much hope.  But, we couldn't benefit because Bennett didn't have the right gene mutation. 

I am very aware that, today, there are around 10% of people with cystic fibrosis and their families for which today is a painful day.  They are excited for the CF community.  But they know their random genetics mean they must wait longer until they can benefit from a drug that will increase their life expectancy and give them relief from this cruel disease.  They are on a clock, a race for a medication like Trikafta and each day that goes by is another day lost.  This is also the case for so many CF families around the world who do not yet have this medication available to them in their country.  For these families, I just want to say, as exciting as today is, there is a sadness I feel with you, as well.  Your time is coming too and I will be anxiously waiting along with you, working to help us find a medication that benefits your child, your family member, you.  We are in this together, as the CF Foundation's tagline says, "Until it's done."

Lastly, I want to thank the hard working people at Vertex and the Cystic Fibrosis Foundation who worked so hard to make this dream a reality.  Bennett's story began many years before Bennett's born, before we knew we would become a part of the cystic fibrosis community.  Thank you to all the scientists, investors and brilliant minds that set forth this venture philanthropy model of discovery to bring this medication forward.  Thank you to those clinicians and clinical trial participants who tested this medication so we can feel confident about it's safety.  Today's joy isn't only because Bennett has hope like never before.  It's because we realize the gift we've been given through the work and sacrifice of so many before us.

We can't wait to share when Bennett receives Trikafta in hand.  And we can't wait to see what happens.  Trikafta is not a cure.  CF is not going away.  But it's hope that CF will have just a little less control over Bennett's life, and for that, we are eternally grateful!

Today is a very exciting day!!!  We hope you will celebrate with us!!  CHEERS!!!!

18 February 2021

Emergency Preparedness and Cystic Fibrosis

Texas is in the middle of a historic winter storm event right now.  And I'm in the middle of Texas.

For many many Texans, tonight brings another day and night with no electricity and no heat.  On the heels of no heat or electricity, there is an emerging water crisis.  While the upcoming weather looks to return to our normal above freezing temperatures in the coming days, we are holding our collective breaths that our frozen pipes won't destroy our homes, businesses and schools.  This is day 7 of historic low temperatures.  We are weary.  Lest we forget we're all in the middle of a COVID pandemic that has forced us to withdraw from our normal life for nearly a year.  

The children and I are some of the luckiest ones having not lost heat and electricity during this Winter Storm.  I can only assume our power is near a critical city infrastructure and the reason why we have continued to have heat and electricity so far.  But I have gone most of the week fully expecting and prepared to lose electricity and a way to warm ourselves.  

I wanted to share some of the ways I prepared for this winter storm and some of the ideas I've learned from our CF community about how to cope in an emergency while having cystic fibrosis.  While this list won't likely help most of our CF community suffering in Texas right now, I hope it can help my future self and others who randomly find this blog at some point after googling "managing CF during crisis." 

Here are ways to prepare for an emergency when considering cystic fibrosis:

* When there is no electricity and you can't do your percussion Vest, use hand Chest Percussion Therapy (CPT).
* Consider getting an battery-powered Afflovest percussion vest.  Insurance will usually cover some portion of a new medical device every 5 years.  This is a good investment in times like these.
* Keep a list of your medications and access to your medical record/hospital portal on your phone.  
* In anticipation of an event, try to fill all prescription medications.  Even better, ask your doctor to write a 90 day prescription so you never run out of medicine.
* Store up supplies overtime.  While they take up closet space, they can also give you extra time to get supplies if something unexpected happens.
* Boil masks and nebulizers if electricity doesn't allow for baby bottle sterilization
* Remember hand hygiene is so important with CF.  Keep baby wipes on hand.
* Consider asking your doctor for a prescription for an albuterol inhaler in case you can't use your nebulizer to do albuterol.
* The eRapid can be a battery powered way to do breathing treatments.
* In an emergency, if you don't have electricity and need to do your treatments from home, try going to your local fire station.
* Remember to reach out to your CF Clinic for help or just to let them know what you're dealing with.  A CF social worker's job is to help find community resources to help during a crisis. 
* Consider purchasing a generator for backup power at home.
* Use a power inverter to do nebulized medicines in the car. 
* If on oxygen, arrange for extra oxygen tanks on hand.
* Create an emergency plan.  This link offers some really great suggestions, as well as some good scenarios to think through when you're not in the midst of an emergency.
* Connect to your community.  You don't have to fight CF alone.
* Let your utility company know you have a person with a medical condition in your home.  Some utility companies will prioritize your home's access to electricity in a crisis, if they know ahead of time.
* Have a 3-day supply consisting of one gallon of water per person per day (or more!).  Since electrolyte levels are important for people with CF, consider also having gatorade on hand as well.
* When you're in a difficult situation (emergency or not) and need help related to life with CF,  call the CFF's Compass.  They are there to help connect you to communicate organizations and resources.  
* Read about how the CF Foundation suggests families with CF prepare for emergencies.
* Feeding tube feeds can be hard without electricity.  Pumps have batteries but they need to be charged eventually.  One electricity-free feeding tube Bennett loves and uses is the bFed bolus system.

Bennett uses the bFed bolus feeding tube system.  This feeding bag works through gravity, rather than electricity.  It can easily be hands-free with the addition of the "Free Arm."  We really love not having to depend on electricity/batteries to do his feeding treatments.