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.

Weekend Recap

What a crazy weekend!  The craziness started on Thursday night/Friday morning thanks to Charlie.  Charlie dog sleeps at the foot of our bed.  In retrospect, this was a bad move on our (my) part because he’s gotten bigger and takes up a lot of space.  But now that he’s gotten used to it, I feel bad making him move.  Anyway, around midnight, he hopped off the bed (which he never does) and started pacing, then scratching at the door.  We knew that meant he needed to go out.  A took him out and we tried to fall back asleep.  Around 1:30am, same thing.  4am, same thing.  It was clear that he was having tummy troubles.  I got up for the day at 5am and was completely exhausted.  I was also worried about Charlie and didn’t want my mom to have to take him out every hour (she was watching S that day).  Luckily, I was able to work from home.  We did make it a 4 hour stretch, but it was pretty clear that Charlie ate something that didn’t agree with him and he needed to go out all throughout the day.  Not at all surprising since he still chews up everything.

Fast forward to 5:45pm.  We had to take the boys to their final tennis lesson.  SO glad this class is over because none of us like it.  I was nervous to leave Charlie, but he had been able to make it 2 hours between trips outside and we weren’t supposed to be gone that long.

In the middle of tennis, A received an email forwarded from a former student.  The original message was from A’s current student.  I won’t get into the details, but it was clear his student was very upset and may need help.  I didn’t realize at the time that she hadn’t been in school for the past two days.  I’ll be honest and say that I initially chalked it up to typical teenage emotions and drama.  But A was worried and we started researching suicide prevention sources and A decided to call the police non-emergency line.  I’m so incredibly proud of A for taking action and this was a good reminder that even if the situation ultimately amounted to nothing, it’s so much better to err on the side of caution.  This meant we had to wait for the police officer to arrive so he and A could discuss the situation.  We waited a longish time - about 30 minutes?  The boys and I had walked the facility, visited the vending machine, perused brochures, and were running out of ideas when the police office finally arrived.  We came to find out that the officer had already been reviewing the girl’s file and would check on her.  If one of my boys was ever in this situation and feeling overwhelmed, I hope they’d talk to me.  But if they didn’t, I would hope someone was looking out for them like A did for this girl.

After all this, we arrived home about 3 hours later, and thankfully, Charlie was ok!  I was so darn tired and couldn’t imagine being up through the night again, but Charlie made it without any problems.  Phew!

Saturday was uneventful.  We decorated for Christmas (everything except the tree) and worked on cleaning up the house, which took forever.  W and I took Charlie for a long walk and that’s all I remember.

Sunday, after church, we headed to the Museum of Science and Industry in Chicago.  Every November, the museum offers a Corporate Family Day and employees of certain companies can get in free of charge.  The boys love going.  This year, I was really excited to see the Christmas Trees Around the World exhibit.  In all of the previous years, we’ve attended prior to the exhibit opening and the trees haven’t been decorated yet.  They were beautiful and viewing them was definitely a highlight for me!  The boys had a blast and we stayed until the museum closed.  It was such a fun day!

Weekend Recap

We have a few inches of snow on the ground and when I left the house this morning, it was 11 degrees.  We set a record for the coldest November 12.  So basically it’s going to be the longest winter EVER (and it’s not even winter yet).  I'm going to lose my mind.

Luckily, it was a bit warmer over the weekend and we took advantage of that.  Saturday, W was supposed to attend a birthday party, but it was canceled.  So A and I took the boys to a bouncy house place where they jumped around for an hour.  S even made me go in one of the bouncy houses – I tried to explain that a different parent had gone in only because his child was little and needed help.  S wasn’t having that explanation.  So I bounced a bit and promptly exited.  LOL.  Afterwards, we stopped at my mom’s house.  The boys had been begging to go there so they could play with my brother’s old Legos.  We ordered tacos and relaxed until it was bed time for the boys.

Sunday morning – chaos as usual.  I’m at my wit’s end.  I don't understand why it's so difficult to get 2 boys dressed and out the door (which they do every day for school), but it is.  We were late to church.  But after that, things got better.  Once we arrived home, I took Charlie to the dog park so he could run and play.  I really enjoy taking him for long walks on the weekend – the exercise is good for both of us and I appreciate the quiet.  While the dog park isn’t quite the same as a long walk, the park is pretty big and I do get some walking in while Charlie plays.  This park just opened and it’s five minutes from our house.  I absolutely love it (and so does Charlie).

After that, we met up with A’s brother and his 4 kids at a local theme park.  All the kids had a great time (I think!) and my nephew even went on his first real roller coaster with his dad and A.  The boys love spending time with their cousins and I’m so glad we could all go.  It helped that the park was relatively empty and we hardly had to wait in line.  Also, rides scare me.  I didn't go on a roller coaster until I was in 8th grade and I loved them for awhile.  But now?  Nope.  Oh, one more thing!  A suggested that we sit in the last row of one of the rides (which happened to be the spot where you felt the ride the most).  Our stomachs were dropping with each swing of the ride.  Poor S and his cousin were nearly in tears and begging to get off.  They'd been totally fine the first time they were on the ride, but sitting in the last row scared them.  It was equal parts sad and hysterical.

Weekend Recap

I know I’m getting behind with weekend recaps, but I love looking back at years past and reading about what we were up to so I’m going to try to keep up with them (no matter how late they are).

The boys were really challenging this past weekend.  I’m not sure why.  Lately, Sunday mornings in particular have been tough.  Mostly because I’m trying to get ready for church and the boys are acting out – trying to get them under control while getting ready is particularly stressful to me.  I don't do well multitasking.  Maybe I need to get up earlier, but after waking up at 5am every day during the week, staying in bed until 6:15am on Sunday feels like a luxury.  So Sundays usually result in timeouts where S is especially challenging and I end up having to sit next to him during the time out or I have to drag him to his bed (because he refuses to walk and he’s too heavy to carry).  And then he’ll loudly tell me in the middle of church that I need to apologize for “sitting on him” or “pushing him over”.  Neither of which actually happened, but oddly enough, I’ve gotten a couple dirty looks from church goers who don’t know any better.  Ugh.  What was the point of this?  Oh yeah… Sunday mornings are not my favorite.

We did have fun Saturday night as we celebrated our niece and nephew’s birthday and a local hibachi restaurant.  There were about 8 drinks spilled and 27 trips to the bathroom, but we had fun nonetheless.

Earlier in the day, A took W and S to Home Dept where there was a little class than involved assembling a wooden eagle and painting it.  I think the boys really enjoyed it and I appreciated a little break.  The weather was decent and I also got to take Charlie on a couple long walks.

In other news, I’ve started Christmas shopping and planning W’s 7^th!! Birthday party.  He really wants a party at home, though, I’ve tried to talk him out of it.

Friday Thoughts

Holy moly, we’re into November and we already had our first snow.  WHAT IS HAPPENING?!  Usually, I’m really excited about the first snow – there’s just something magical about it – maybe a little reminder that Christmas is around the corner.  But I just can’t get on board with snow in October.  Nope.  The boys were in shorts a month ago.  And fall weather lasted all of 3 weeks.  Boo hoo.  Also, someone reminded me that there are only 2 months left in the year… again, WHAT IS HAPPENING?!?

On the bright side, I’m so happy our town’s trick or treating was Sunday because trick or treating in the snow just does not sound fun.  I did forget to mention something that still has me giggling.  I realize that trick or treating prior to Halloween is a bit unique.  Well, the kids knocked on one door in the neighborhood and a very confused guy answered.  The kids all said trick or treat and the poor guy looked baffled, “Trick or treating is today?  We just moved in a few days ago.”  Bahahaha.  Welcome to the neighborhood and by the way, we DON’T trick or treat on Halloween.

W had to dress up as Saint Paul at school in preparation for All Saints Day today.  We all attended a little show where each student recited a line about the saint they were assigned.  It was the cutest.

A attended S’s conference on Monday.  Conferences still give me anxiety so I stayed home and made dinner with the boys.  I’m happy to report that S is doing wonderfully.  Sadly, one of his teachers is leaving and I’m so disappointed.  He had two teachers leave mid year last year and it’s hard on the students.

Happy Friday!!  Let it snow, let it snow…

Weekend Recap

A and I celebrated our 12^th anniversary over the weekend!  I can’t believe how fast 12 years has flown by!!

Nani kindly offered to take the boys on Saturday night.  A and I never get to go out near our actual anniversary.  We usually end up going out to dinner at some point in late November.  So this was a special treat.  While the boys were making pizzas and cookies with Nani and Papa, A and I enjoyed dinner at a nearby restaurant.  We ordered entrees, drinks, desserts, and the waiter even brought us Prosecco and chocolates.  Everything was delicious and we ended up staying for 2 hours.  From there, we headed home and watched a cute movie called Always Be My Maybe on Netflex. 

We met up with the boys at church and soon enough it was time to get ready for our town’s trick or treating.  The weather was perfect and our friends even joined us as we walked the neighborhood.  I’m really glad our trick or treating was on Sunday because we’re supposed to have some rain/snow mix on Halloween Day.  Ugh – so not ready for snow!!

W wanted to be a Starbucks barista and S wanted to be a pilot.  Both costumes were super easy and the boys looked so cute.  

After about two hours of walking around the neighborhood, we called it quits.  

The boys couldn’t wait to examine their candy (though they hardly eat any of it).  It always surprises me that they receive a few full size candy bars.  That didn’t happen when I was a kid!  You should see the amount of sweets in my house right now: A taffy apple from A, truffles from A, cookies from Nani, tons of Halloween candy, apple cinnamon donuts, and chocolate covered Oreos.  So if you see me looking like I gained a few pounds, well, you’ll know why.  I have such a sweet tooth!!

Weekend Recap

I really have no idea where the time goes.  I feel like Christmas will be here before we know it.  Let’s see what I can remember about last weekend…

Friday.  The boys started the next session of their tennis class annnnd I hate it.  Ok, hate is a strong word.  But as soon as we arrived, another mom leaned over and whispered, “I miss Coach Ryan already.”  Not a good sign.  The class is huge – 10 kids for 1 instructor and all the kids are older than the boys.  The instructions were a bit advanced for the boys and I feel like they got nothing out of the lesson.  It was really disappointing since the previous session was through the same company and we all loved it.

Saturday.  Coffee, Juice and breakfast with trivia questions mixed in.  The boys got much needed hair cuts.  And then it was time for Nani and Papa’s surprise 45^th anniversary party!  Great food, great company, and I think everyone had a great time.  I also think Nani and Papa were really surprised. 😊  Also, the boys got to play with their cousins, which always makes for a fun (loud) time.  All the kids behaved really well.  We left with really full bellies and a little too much wine (me).

Sunday.  We began our day with church and Starbucks.  W and I took Charlie to the dog park.  He loves going and the people are always so friendly.  Charlie gets to run until he collapses and then he gets belly rubs from anyone and everyone.  After that, we headed to my mom’s house.  The boys had been asking to go to grandma’s for awhile.  They love playing with my brother’s old Legos and cars and it warms my heart.  We ordered pizza and watched football and after a busy weekend, I was grateful that my mom was willing to entertain the boys for a bit.  It’s funny to me that I was really tired out from the party.  The boys, on the other hand, went to bed way way late and still had tons of energy the next day.  Oh, to be young!  A and I finished the weekend catching up on shows.