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.