Trikafta Update

Hello, hello!  I need to write a post on our vacation (quite possibly the most amazing vacation I've ever been on!!).  But that will have to wait until I have more time.

For now, a quick Trikafta update will have to suffice.  I (think) I mentioned previously that we were a bit concerned about the effects of Trikafta on A's liver (actually, it was probably just myself who was concerned, but anyway...).  A's liver enzymes were slightly elevated and had been for a year or so.  He had the necessary blood work done before starting the drug and has had two appointments with a hepatologist since then.  Good news!  A's liver enzyme levels actually decreased since starting Trikafta.  A has no scarring and the test results were "reassuring".  He has mild fat levels that the doctor was not concerned about.  Such a relief and we're so thankful for this drug!!

Trikafta Update

Just before Christmas, I shared that A finally got insurance approval for Trikafta.  It was a frustrating month as A followed up with his clinic and Vertex.  He was initially denied approval – twice.  When the approval finally came, I was so excited, but also nervous.  What if it didn’t work for him?  What if he experienced adverse reactions and had to stop taking it?  What if it affected his liver function?

The approval also came after a really disappointing clinic visit.  I shared a bit here.  In a nutshell, A had his lowest pft results ever.  I was really scared and upset.  A needed a new nebulizer and he was prescribed an oral antibiotic.  He was also to start on Cayston (earlier than planned).  The nurse practitioner wanted to admit A, but he declined thinking that once he had a new nebulizer and the antibiotics, things would improve.  He also wasn't feeling particularly bad despite the pft results.

A started on Trikafta just before Christmas – 12/21 to be exact.  He wanted to wait until the weekend because we’d heard so much about the “purge” so many CFers have experienced - lots of productive coughing, headaches, fatigue, body aches, etc.  We were prepared for the worst.  I think I asked A about 50 times over the course of that Saturday how he was feeling.  He kept telling me “fine”, that he didn’t feel much of a difference.  Hmmm.  The following day, we again prepared for the worst.  Again, A said maybe he felt like he could breathe a bit better, but for the most part, he didn’t feel much different.  Day after day I’d ask and day after day, A felt fine.  I wasn't convinced the Trikafta was even working.

On 12/23, A started Winter Break.  The timing couldn’t have been better.  We all needed some down time after a few really busy weeks.  We slept in, spent time with family, and ate well.  We also went on a few really long family walks.  We often walked for an hour in chilly weather and I noticed A didn’t seem tired – at all.  I also noticed he was coughing less (not at all). 

Because of the low pft results, A had a follow up appointment 3 weeks after his clinic appointment (2.5 weeks after starting Trikafta).  If his numbers didn’t improve, he would likely be admitted.  He seemed to be feeling better, he was eating well, and he seemed to have energy, but I was still nervous about the appointment.  On the day of, I asked A if he was nervous.  He responded with a flat out “no”.

A texted me a photo of his results.  The results page contained his numbers for the past 11 years.  On first glance, I could see the numbers were good and I was so relieved.  Upon further examination, I realized that these were his highest numbers in 4 years!!  And with a 10% improvement over his results 2.5 weeks ago, this was the biggest jump in pft percentage that he’d experienced in 11 years!! Fortunately, A's pft numbers are typically very stable.  He may go up or down 1 to 2 percentage points from clinic visit to clinic visit, but for the most part, they're stable.  This is also why there was concern when he was down 5% at his last clinic appointment.  10 percent is huge for him.  I’m so grateful.  And I’m still cautiously optimistic.  A meets with a hepatologist later this month.  I’m still worried about his liver enzyme levels and the effects of Trikafta on his liver.  But for now, I’m incredibly thankful.  Approval came just when we really needed it!

One other point of note, for the first time in a REALLY long time, A could not provide a sputum sample.  I've known A for over 16 years and he's always been able to cough something.  This time, the NP had to swab the back of his throat!  I'm pretty amazed.

Trikafta Update

It's been a week... a very challenging week.  One that consisted of a pretty terrible clinic visit for A (more on that in a bit), Charlie ruining the quilt on my bed (thanks to the boys letting him in our bedroom while I was showering), the boys accidentally recycling the pricey Christmas gift I purchased for W's teacher, my company announcing they were moving further away (only about 15-20 minutes, but that means an extra 30-40 minutes of driving per day), and I was supposed to have Friday off, but a surprise email from the FDA on Friday morning meant I worked all day and through the weekend (and will work tomorrow).  UGH.  I think I was near tears more than once.

But one really great thing happened....

On Tuesday, A had clinic.  He picked me up for lunch and when I asked how it went he responded with "ehhh...".  I knew that wasn't good.  I asked what happened, what was wrong?  Apparently A's lung function was the lowest it has ever been.  Wait, what?  A had a cold, but didn't seem to be affected by it.  He seemed normal for the most part.  I've seen him at low points and expected that his clinic appointments wouldn't be good, but this one was a bit of a surprise.  A admitted that he'd run out of one antibiotic and his nebulizer wasn't working.  He didn't think it would make much of a difference to wait for clinic to get replacements and he was wrong.  I was really upset and told him his health had to be his first priority.  No more waiting.  The NP suggested a hospital admission and A declined.  He thought his PFT scores would improve once he had his antibiotic and working nebulizer.  He was also prescribed an additional antibiotic.

I was also really angry.  We've been fighting with insurance to get approval for Trikafta for a month.  Maybe if A was on it, he wouldn't have experienced such a decline.

I arrived home from work feeling pretty defeated.  And then A held out his phone and played a message he'd received just before I got home.  Insurance FINALLY approved Trikafta!!!!  I was so excited!!

We're both hopeful the drug will have a positive impact, but also cautious.  We found out that A's liver enzymes are slightly elevated so another concern.  Things can never be easy, but we're thankful that A is able to try this new drug.