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.