January Clinic Appointment

On Thursday, I went to my regularly scheduled Cystic Fibrosis clinic appointment. I knew that it was time for my annual blood work (plus genetic testing) and a chest x-ray, so I decided not to bring the kids with me. Anna had school and there's no way very-active Calvin would have been happy with all of the sitting/driving.

I decided to head up to Chicago earlier than my scheduled appointment to hopefully avoid the long wait in the lab and knock out any other tests before seeing the doctor. I arrived at the hospital around 9am and was able to get right into the lab to get my annual blood draw (7 tubes) and an extra tube for genetic testing. I believe that last time I had my genetics tested was about 10 years ago, and they were only able to identify one of my mutations - G551D. The other is still unknown. With all of the new medications in the pipeline, it's important to know exactly what my mutations are. As of now, I'm on the best medication for my mutation. Kalydeco is specifically for G551D, so the likelihood of me switching to a different medication any time soon is low, but learning my other mutation is still good information to have.

Jan 2019 - more areas of white
(plugging & mucus), especially
in the upper right lobe. Also a bit
more in my left lung.

April 2017

After my blood draw, I went downstairs for my chest x-ray. I actually requested getting the x-ray because I've been having such a rough time with coughing up blood lately - my latest episode was that morning when I woke up. I was so frustrated after seeing that, I emailed the nurse and asked her to put in the order. I wanted to see how/if my lungs have been affected by the sporadic hemoptysis episodes over the last five months or so.

After the chest x-ray, I waited to get a clinic room forever. As much as I love my doctor, I can't stand how long it takes. I feel like they're always running behind. Anyway, I did my PFTs, met with a new physical therapist (did a 6 minute walk test), saw the pharmacist, social worker and dietician before the doctor finally came in two hours after my scheduled appointment.

Thankfully my PFTs were pretty much unchanged from September's appointment. I was really glad and pleasantly surprised. But, that didn't explain what's going on with my lungs.

My doctor and I had an excellent, over an hour long, conversation. *That's what happens when I don't bring the kids* I showed her the calendar charting all of the dates that I've coughed up blood or had some streaks in my mucus. It seemed to be at least every two weeks, but without any kind of pattern or cause. It's happened when bending over, walking up stairs, just standing there, laying down, sitting up.... no pattern. Twice since August I've been on antibiotics - one round of Cipro in August and one round of Augmentin in November for a sinus infection. Even when on the oral antibiotics, I was having blood/streaking.

Then we finally got to the bottom of it - sort of. It's been months since I've been on inhaled antibiotics. I stopped taking Cayston in the summer or fall because I noticed I was coughing up blood more frequently while on it. I had tried a month of inhaled Tobi, but it made my lungs feel super tight, so I only managed about two weeks on it. So, since the fall, the pseudomonas (bugs in my mucus) in my lungs has been free to do whatever since it wasn't being knocked back by inhaled antibiotics. My doctor thinks this has caused a lot of inflammation in my lungs and irritation of my airways leading to all of the hemoptysis episodes. Thankfully, it hasn't made my lung function drop.

It made sense, and I'm really hoping she's right. So with the mucus sample I provided at the appointment, the doctor requested that the lab test the resistance of my mucus against the inhaled antibiotic called Colistin. As soon as we hear back, and are sure that my bugs are susceptible to Colistin, I'll hopefully be starting it. My doctor also wants me to go back on Azithromycin on Monday/Wednesday/Friday to help decrease the inflammation in my lungs. I originally stopped taking it about 5 years ago when we were trying to get pregnant for the first time. Its kind of a bummer to be put back on a medication that I've been off of for so long, but it's definitely needed right now. I feel like my list of non-IV medications is slowly dwindling, so I am really banking on the Colistin to help give my lungs the boost they desperately need.

I think we have a good plan in place and I'm trying to be optimistic. I know that 80% FEV1 isn't too far from my baseline of 85-88%, but I don't like seeing my numbers there. I hope that the medications will help my lungs get back in shape. I also need to really make an effort to add exercise into my routine to get my lungs working more. This is always the hardest part for me due to time constraints.

Right now my next appointment is scheduled for April, but I wouldn't be surprised if she wants to see me back sooner to see if the meds are working.

Next hurdle.... getting the meds approved my insurance and shipped out in a timely manner...

Calvin, 11 Months

He was not too happy about laying down for his picture this time.

Bubba,
Oh my goodness, how can you be eleven months old today?! This last month we celebrated your first Christmas(es). You were very lucky and received lots of diapers, new outfits, some jammies, and a few new toys and books. You enjoyed ripping the paper and playing with your cousins.

This month (well, actually the day you turned ten months old) you learned how to stand up in your crib. Now pretty much every time I go to get you up, you're standing. Your legs are getting so much stronger. You pull up on everything and cruise around furniture a lot faster than before. Just within the last week or so you've been getting brave and try to stand on your own. You can only make it a few seconds, but it's a start and you are so proud of yourself.

You've learned so much this month, too! You can now say "uh-oh" (learned just a couple days before Christmas), you play peek-a-boo when we say "Where's Calvin?", you give high-fives, and you are starting to understand so much more. You look around when we ask you where Sissy is, or Archie, or your new stuffed animal dog Frankie. You also know what it means when we say, "Go get a book". We're working on a little bit of signing and you inconsistently can sign "more" and "eat" (the most important ones!). It's been so fun this month because you've become so much more interactive.

Your feeding has changed dramatically this month as well. You've learned how to drink from a sippy cup (do the best with the 360). We started with water and you weren't really interested & couldn't figure it out. Then I tried whole milk and shortly after you caught on. While I was on winter break, I was giving you whole milk whenever you'd eat in your high chair and you did great with it. Now that I'm back to work, you drink my milk with your meals, and once that's gone you take whole milk. Thankfully, you don't seem to care about the switch which is good.
Also, we're done nursing overnight. After the holidays calmed down, I made the decision to stop nursing you overnight. With you getting so much food and milk during the day, there really is no need to nurse overnight anymore and you've been doing SO well! You'll sleep all night long, occasionally fussing, but you're able to get yourself back to sleep within about 5 minutes - it's been wonderful!

With all of this food and milk during the day, you're definitely gaining weight. You had your follow-up appointment from your November ear infection the day after Christmas and you weighed 17lbs 7oz then. You're finally in 12 month clothes (some are getting too small already) and I moved you up to size 4 diapers because your belly is growing! Your little chubby fingers are filling out again just like when you were a newborn, along with your belly and your arm & leg rolls... I'm so glad! You also now have 4 teeth - two on top & two on bottom.

You and Anna are just SO cute together. You really love her and she just adores you. She is so good with you (even likes to carry you around which makes me nervous) and I just hope that your relationship continues to grow. It's so fun watching you guys play together.

Oh, and how could I forget - Daddy gave you your first haircut the other day... it was more like a trim, but we had to get your hair out of your eyes. You would rub food in your hair pretty much every time you ate and it was gross. So we put you in your high chair to eat breakfast and Daddy tried his best to trim your hair. Its much shorter in the front (I'm still getting used to it), but I'm glad it's out of your eyes.

Likes: Food, milk, looking at animal books, playing with Anna, people watching, your pacis, Frankie (or any stuffed animal really - you hug them, it's adorable)

Dislikes: When I walk by and don't pick you up, falling down after standing, when there's no more food on your tray and you're still hungry

It's been a really fun month, Bubba. I can't believe we're going to celebrate your first birthday soon! I wish I could have two weeks off every month to spend with you, but hopefully summer break will be here before we know it. 

Love you, Bubba!