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.

Christmas Recap

Remember me?  I’m still around.  Work has been a bit crazy.  I can’t remember the last time I was THIS busy, but I’m learning so much and still really enjoying my job.  I was supposed to have a stretch of days off starting December 20, but I received an urgent request from the FDA so I didn’t have as much time off as anticipated.  I was so looking forward to that Friday before Christmas and had plans to take Charlie to the dog park, go out for lunch, help in W’s classroom, and wrap presents.  I still helped with W’s Christmas party (It was also his birthday!  Hello, 7!!), but the rest of my day was spent working.  I was disappointed, but had the wherewithal to keep in mind that I would have been SO much more miserable if I wasn’t in my current job.  Long hours aren’t quite as awful when you enjoy the work and I’m so grateful to have transitioned to a new role. 

Anyway, I managed to wrap presents and get things done just in the nick of time.  On Christmas Eve, we went to my cousin’s house for dinner.  It was great to see relatives I hadn’t seen in a long time.  The boys had fun running around the house and watching Frosty the Snowman.

On Christmas day, the boys were super excited to see if Santa visited.  They ran downstairs to find new scooters and several packages.  We went to church and grabbed coffee and then it was off to Nani and Papa’s house.  The boys enjoyed running around with cousins and opening presents.  W took time to examine each gift, while S really didn’t seem to care what he received, he just wanted to open everything!  We returned home with full bellies and two tired boys.

On the day after Christmas, my mom and Susana came over for brunch.  The boys opened their gifts from my mom, Santa, Susana, and A and me.  It was quite the scene as the room filled with torn wrapping paper and too many boxes to count.  The boys received Legos, Magnatiles, games, watches, and books along with their scooters – all of which were perfect for them.  The day happened to be warm – near 60 degrees, which is unheard of for late December.  We all went out for a family walk and enjoyed the fresh air.

The days after Christmas were my favorite.  We slept in, took long family walks, played too many board games to count (W is obsessed with Sorry!), visited friends, and took things slowly.  When the boys went to bed, A and I caught up on shows.  On one day, we spent at least 6 hours cleaning up and organizing, which was very much needed.  A and I managed to squeeze in a much needed lunch date.  We also took the boys to the library and they picked out lots of books.

 I also want to remember a couple things I made.  I was asked to bring a “cheese ball” for our Christmas Eve gathering.  My initial reaction was “a what?!”.  But then I remembered that my grandma (who passed away 12ish years ago – wow, typing that, I can’t believe it’s been that long) used to make this cheese ball with cream cheese and chipped beef.  I found the recipe and made it and love that it brought back memories.  I also made strata for our after Christmas brunch and used a recipe from my uncle who lives in Oregon.  I love that I was able to make a couple family recipes (even if they were easy). 😊

With work and only 4 weeks between Thanksgiving and Christmas, this Christmas felt especially chaotic and rushed.  As much as I wanted to slow down, I couldn’t.  I enjoyed Christmas, I always do, but I wish I had more time to prepare.  I’m really hoping the holidays will be less hectic this coming year (but doubtful!).

Stay tuned for a Trikafta update!

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.

Weekend Recap

Things have been a bit crazy around here.  'Tis the season, right?  So here I am popping on to write a quick update before I lose track.

We spent last weekend in Chicago.  We did this last year and loved it so much, we decided to do it again.  You wouldn't believe how many people tell me W is going to hate his birthday because it's so close to Christmas.  I find it really obnoxious.  After several rounds of IVF, I don't care when my kids' birthdays are - I'm just happy they're here.  Anyway, we've tried to make December extra special so W doesn't feel slighted.  Back to Chicago...

We arrived at the hotel around 3 and our room wasn't ready yet.  We left our luggage at the hotel and ventured out.  It was fun to walk along Michigan Avenue and we found a restaurant for an early dinner.

After dinner, we headed back to the hotel.  Our room was ready and we had a beautiful view of Michigan Avenue.  The boys decided to check out the pool and enjoyed swimming.

From there, we headed to Zoo Lights at Lincoln Park Zoo.  Although, the zoo wasn't far, traffic was horrendous and it took awhile to get there.  We had a great time seeing all the lights and checking out some of the animals.  We returned to our hotel exhausted.

Sunday, we visited the world's largest Starbucks.  It was fun to see, but definitely intimidating.

We headed to breakfast at the Grand Lux Cafe and were seated right by a large window.  It was so fun for the boys - they enjoyed people watching and even got to see a garbage truck.

Such a fun weekend!

Weekend Recap

We had the busiest weekend!  I love this time of year, but gosh, there’s so much to do and I’m not sure how to cut anything out.

Friday.  We went to Christmas Craft Night at W’s school.  The boys enjoyed making three different crafts and decorating cookies.  We got to catch up with W’s kindergarten teacher – I just love her so much.  The boys also picked out a couple new books.  All in all, a fun night.

Saturday.  W’s 7^th Birthday Brunch!  I tried to talk W out of a home birthday party, but failed.  That meant that I spend much of Friday trying to clean and organize things around the house.  In retrospect, this was much needed, but it was a lot of work.  Anyway, W loves his Starbucks bagels and apple juice which influenced the brunch idea.  We served donuts, bagels, cinnamon rolls, breakfast casserole, and fruit along with coffee and juice.  I really love how it turned out.  And since it started early, our guests still had the afternoon/evening for other activities.  Most importantly, W loved having his family and friends over for his favorite foods.

Saturday evening, my high school played A’s high school in basketball.  We all went to the game.  W and S really haven’t showed much interest in sports, but they’ve enjoyed the couple basketball games we’ve gone to.  Sadly (for me), A’s school won, but we all had fun watching.

Sunday.  Church followed by a pancake breakfast with Santa.  After filling up on pancakes, sausage, bagels, and juice, W and S greeted Santa.  I’m not sure what W requested – possibly miniature traffic signals?  S requested a toy car wash.  Ugh.  Why can’t my kids request something easy??

 

From there, W attended a birthday party for a classmate while S and I dropped off gifts for our adopted family.  We all ended up at my mom’s house where the boys played with Legos and we ordered Chinese food for dinner.  So lots of fun, but I was so tired on Monday.

Weekend Recap

I still have that darn cough that I mentioned previously.  Ugh - I can't seem to get rid of it!  We also have no updates on Trikafta, which is really frustrating.  On the bright side, we all enjoyed our time off…

Wednesday.  Our fifth annual Kindness Day!  This year was very similar to previous years with one exception – we went out to breakfast as a family.  We often stop at Starbucks, but I can’t remember the last time we actually went out for a real breakfast.  I think we all loved it and it was a great way to start the day.  From there, we paid for someone’s coffee at Starbucks, left a tip for the baristas, brought my mom coffee, handed out gift cards at the grocery store, left our mail carrier a treat, and prepared a basket of snacks for our delivery people.  It was a great day!

Thursday.  Coffee and the Macy’s Thanksgiving Day Parade (which the boys weren’t really into).  Our morning was slow and peaceful – my favorite kind of morning.  Then we headed to Nani and Papa’s for Thanksgiving.  Dinner was delicious as always and we enjoyed playing Uno Attack until it was time to leave.  The boys and their cousins received Christmas pjs from Nani and they all looked so cute.

For the record, that's a picture of a cornucopia. :\

Friday.  Shopping with my mom.  I was honestly dreading this.  Leaving the house at 7am was not very appealing.  I like spending time with my mom, but I do most of my shopping online.  My mom, however, doesn’t own a computer.  Once we were out and had coffee in hand, I actually had a lot of fun.  We went to a different shopping center and I enjoyed visiting stores I wouldn’t usually go in – Anthropology, J Crew, and Crate and Barrel to name a few.  We met the boys for lunch at our favorite pizza restaurant.

Saturday.  We found our Christmas tree!  Oh, how I love it.  And I’ll give A and I kudos because we finally figured out the easiest way to get the tree standing:  Cut off a couple inches on the very top so the angel can sit better, cut the netting off just the trunk so it doesn’t get stuck in the tree stand, make sure the tree is in the very middle of the stand so it doesn’t topple over… all of this probably seems common sense, but we’d made mistakes previously.  Anyway, I think our tree is bigger than what we’ve had in previous years and I love it.  I tried to convince the boys to wait until Sunday for ornaments, but they weren’t having it.

Sunday.  After church and Starbucks, we mostly relaxed.  We played games with the boys and watched tv.  I asked what we should have for dinner and S replied, “chicken and pasta”.  I actually had ingredients around the house and made up an instant pot recipe with chicken, penne, lemon, chicken broth, cream cheese, Parmesan cheese, a splash of milk, and spices.  You should have seen the look of horror when I mentioned that I made up a recipe.  And when I served the chicken and pasta with lemon cream sauce, W announced that it was his favorite meal ever and he even liked it better than pizza.  Say what?!?  The child who never likes anything loved my dinner.  So I ended the weekend feeling pretty darn good. 😊

Last Weekend's Recap (and a little Trikafta update)

Oops.  I actually wrote this a week ago and forgot to publish so here is last week's weekend recap...

I’m going to attempt a brief weekend recap.  I’ve been fighting a cold and I’m struggling today.  The worst part of this darn cold is that it came with a cough and I can’t sleep at night.  I’ve been fighting a headache since I woke up.  A has conferences this week so I’m in charge of the boys in the evening, which is usually fine, but with this cough and headache, it’s more challenging.  I’m hoping I can sleep in tomorrow.  Anyway, the weekend…

Friday.  The good:  We received W’s report card and he’s doing amazingly well.  We’re crazy proud of him.  He loves math and scored in the 89^th percentile on the standardized test.  He scored top marks in all subjects with the exception of Language Arts.  In that subject, he received a satisfactory.  He enjoyed Kindergarten, but he’s really taken off in first grade.  We went out to dinner to celebrate and let W choose the restaurant.  

The bad:  A’s insurance denied Trikafta.  I was incredibly disappointed.  How can they deny the only drug approved for his mutation?!  All that effort to get the drug approved and now he can’t even get it?!  Apparently the insurance company told his care team that the drug wasn’t on the formulary yet.  But we know of others with the same insurance (including A’s brother) who have gotten the drug.  Then we received a letter on Saturday stating that A first needed to try the alternatives – Orkambi and Symdeko.  Nope.  Not approved for A.  We’re a bit confused – was he denied because it isn’t on the formulary or because there are supposed alternatives?  Or both?  We’re appealing and pulling out all of the stops, but still very frustrating.  I’m holding out hope that once his care team explains there are no alternatives, he can get the drug.  We also found out that A's liver enzymes are "mildly elevated", which is worrisome to me.  I was really angry because apparently they've been "elevated for some time" (months), but it was never brought to our attention.  So more testing for A on that regard.  Admittedly, this whole process has been disappointing and discouraging after the high of finding out Trikafta was approved.  Stay tuned.

Saturday.  We invited a friend of S’s over for a play date.  All three boys had a really great time and A and I enjoyed catching up with S’s friend’s mom (Lauren).  Lauren is one of those dynamic people you want in your circle.  Super outgoing, driven, and kind.  I really like her and I’m glad that our kids are friends.

Sunday.  After our usual morning routine, A dropped the boys off at my mom’s so we could work on assembling an early birthday present for W.  He has been requesting a new bed for some time and he specifically wanted bunk beds.  His current bed was inherited from his cousin and he loved it for awhile, but we understood that he wanted to choose his own bed.  So I caved and purchased bunk beds that consist of a full size bed on the bottom, twin size on top, and can be taken apart and used as single beds.  The only problem?  It had about 10,000 pieces and took A and I over 6 hours to build.  So far, the boys love it!  They play on it, read on it, have sleep overs.  My rule is that sleepovers are only allowed on weekends because I want them to be well rested for school.  I will admit that it’s cute to walk passed W’s room and hear them chatting and giggling when they’re supposed to be sleeping.