Kalydeco, Five Years Later

I've been on Kalydeco for five years (August 30th)! I can't believe it's been that long. This "miracle drug" has definitely changed my life for the better, but it hasn't been a cure, especially lately.

I've had a rough month, well, a difficult last 7-8 months, really. Ever since Calvin was born, I've struggled to get my lung function back up to my baseline of mid-80s - it's been hovering in the high 70s the last two times I had my PFTs done (which really isn't that much of a decline, but it is to me). I've been on Cipro twice, May and August, Cayston doesn't seem to be working as well for me (causes hemoptysis), Tobi makes me feel super tight and makes me cough all night long. I'm getting frustrated and I feel like I'm slowly running out of options (that aren't IVs). I've also been battling on & off hemoptysis for the last month which has been really annoying. I don't feel terrible, but I don't feel clear either. I just want to go back to feeling normal (my normal). I'm a little nervous about my appointment next week, especially since our whole family has this disgusting productive cough.

Overall though, I feel like Kalydeco has been amazing for me! Before I started taking Kalydeco, I really struggled to gain and maintain my weight, I was around 108-110 lbs. My lung function fluctuated between high 70s and mid-80s. Today, my weight is steady around 130-135 lbs and my lung function has been in the mid-to high-80s until earlier this year. Considering that the average CF patient typically loses a bit of lung function each year, the fact that I've been stable for the last five years is a huge success!!

I've had two full-term successful pregnancies (and our angel babies), maintained a full-time career of teaching, started my Master's degree and maintained my health.... I'd call that a win! All thanks to two little blue pills per day (plus all of my other treatments). Unfortunately, it seems like CF is starting to show its ugly head while I'm trying to be busy living my life lately. I hope that my doctor and I can come up with a new plan of attack because I have way more living to do and I'm not going to let this hiccup bring me down!

**Oh, and one of the CF nurses just emailed me on Friday asking if I wanted to do another round of genetic testing to see if they can figure out my second mutation (I have G551D & the other is unknown). Of course I enthusiastically replied YES! I'm so curious to know what the other mutation is and if we'll get anymore information when we find out. :)

Calvin, 7 Months

Oops, I just realized I never finished this post.... better late than never, I guess :)

• Finally rolling from back to belly, but only over one arm
• Getting much better at sitting up on his own
• No teeth yet
• Wants to crawl and walk so bad!
• Getting better at eating food - actually opening his mouth for the spoon and says a very enthusiastic "Mmmm" when the food goes in. 
• You've also learned how to spit/blow raspberries.... that's fun while eating
• Would rather feed yourself, so you've tried lots of regular non-baby foods
• Still super distracted when he nurses and/or takes a bottle during the day, which means he's still getting up at night to eat
• I started sleep training (again!) and hope to stick with it this time because I'm exhausted. I'm trying to teach you to self-soothe overnight when you wake and it's not time to eat yet.
• It worked! For the last three nights, you've slept from 7/7:30-4/4:30 without waking! I feed you when you wake around 4 and then you'll typically sleep until 6-7. :) 
• But now your naps are crap!
• Began swim class & love it
• Lots of babbling, yelling, screaming 
• You have a very loud, angry cry when you're mad
• Now that you can roll both directions, sometimes you'll fall asleep on your belly or on your side, it's so cute (except when you face is directly into the mattress!)

4 Years Old!!!!

To my big girl,
This year has been the most challenging so far, but you've also grown so much!

You finally broke the 30-pound mark and are 40 inches all. That means you've gained 4lbs and grown 4 inches this year! You're still very petite, but you've got a feisty personality and have no problems holding your own.

Open House 2017 (left) & Open House 2018 (right)

You've completed one year of preschool and you loved it! You've learned so much, made some friends, and even got invited to a couple birthday parties this year. You know all of your letters and most of their sounds, you know your full address, you can count by tens to 100, you can consistently count to 29, need a reminder of 30 and then can get to 39. You want so bad to be able to count to 100 -- you're getting closer. You can write your first and last name, and even decided to change your name to Elsa at the end of the year. Your teachers are wonderful and I know you're very excited to be going back this fall.

This year you've tried tumbling and t-ball, and continued swimming. You really enjoyed tumbling, but I couldn't stand it because it was so unorganized and you basically ran around and did whatever (not just you, all the kids because there was no structure). After tumbling, you wanted to go back to swim, so we kept that up over the winter. You really have gained a lot of confidence in the pool this year. By the end of this summer, you were going under (shallow) water to grab rings. That's huge progress for you! You're learning how to swim with your face in the water more and you're more comfortable jumping in -finally! This spring you took a break from swim to try t-ball. Your team was made up of older 2 year olds, 3 & 4 year olds. It was like herding cats some days, but you did well. You were a great listener to your coaches, even though I think you enjoyed playing in the dirt more than t-ball itself. It was cute to watch you play!

You love all things Elsa, Frozen, princesses, and dressing up. But you also love bugs, butterflies, playing in the dirt, being barefoot outside, jumping in puddles, playing in the rain, fishing, climbing on everything, playing with your babies, coloring, drawing, taping your creations/art all over the house...

Oh little girl, you are full of sass! Daddy and I are really trying to have you use manners/nice words, be kind to others, and most of the time you are, but sometimes you are just so sassy which drives me crazy. You and I have similar personality traits, in always wanting to be in control, haha, so we often butt-heads. You think you know everything (already) and sometimes I just need to pick my battles because its not worth the fight. I think part of the behavior comes with your age and I think some of it has be learned from being around other kids this year. I hope that this next year you mature and tone down the attitude just a bit (please!). :)

You finally became a big sister this year, and you are very proud of that title. You didn't know what to expect when Calvin was first born, but you've been so good with him. You would get excited over the littlest things (when he opened his eyes or his mouth, haha) and you always want to take pictures of him. Now that he's becoming more interactive and mobile, you're getting more excited too. So far you're pretty good about sharing some of your toys or stuffed animals with him. You always say, "I'm the big sister and the big helper" and you refer to yourself as "sissy" which is pretty cute. You call him Bubba, like I do, when he's upset or when you're encouraging him to do something. You get just as excited as I do when he learns something new like rolling over or sitting up. You're still learning how to share adult attention, but that will come. If someone is playing with Calvin and making him smile/laugh, you like to get all up in there and make him look at you instead. You want to feed him with the spoon (and try all of the baby foods he's having - don't worry, you're not missing out!). I am proud of the way that you behave around him and I love how much you love him! I hope that the two of you can grow up and be close with one another.

4 years old & 6 months old (3.5 years apart, to the day)

Other random things I can think of: you learned how to ride a bike with training wheels this summer; you got your ears pierced the night before you turned 4; you can do hopscotch (not sure where you learned that); we still read a book before bed every night; we've accomplished 1,000 books before Kindergarten this summer; you don't like spiders; you still love the color blue; you now know how to work the TV remote (its terrible because you switch shows every 5 minutes); you hate doing your laundry; you're pretty good at keeping your room clean & organized; you still love Chinese food; you prefer taking showers over baths; you love being outside; you love being silly!

I can't believe you're four years old already! I can remember the day you were born so clearly still. You've grown up so much right before our eyes and we're so very proud of you. Keep being kind, but don't lose your spunk either, big girl! :) We love you!
~Mommy