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...

X-Rays. Compliance. Kalydeco.

 

 

On the left: Chest x-ray from January 2013             On the right: Chest x-ray from January 2016

 

What's the first thing you notice? Be honest...

 

Fat! And that's a good thing for a CFer. Let me fill you in on the huge difference in these photos.

 

January 2013 - I was half way through my first year of teaching. I weighed 109lbs, although my lung function was 88% at the time, it wasn't consistently that high. I was showing signs of pre-diabetes, losing weight quickly and not compliant with my treatments. This x-ray is pretty good for a CF adult (24 then). Lungs are pretty clear with mild issues in the upper area of my right lower lobe.

 

January 2016 - I was into my fourth year of teaching, on Kalydeco for a little over two years, a mommy, 124lbs, and a steady lung function of 88%. I had a normal A1C (aka: no diabetes!) and had been compliant with my treatments for a couple years now. The lung damage was very similar, which is key. Typically, adults with CF slowly lose lung function over years, but that fact that my lung function is even better (now) is quite amazing.

 

 

 

There are two giant factors: Kalydeco and compliance.

 

When Tim and I got married and talked about starting a family, I became very serious about my health. I didn't want to sit back and let CF slowly destroy my body as it was. I finally took control and started being complaint with all of my medications and treatments. It was only fair for my husband, and then daughter. To this day, I remain complaint with my treatments because they're what keep me alive.

 

Looking back at blog posts from right before I began taking Kalydeco, I remember that I really didn't want to start it. I didn't want to rely on another drug to keep me healthy. But after realizing, 'what's another pill?!', I figured I'd give it a go. I started Kalydeco August 30, 2013. My lung function the month prior was 77% and I weighed 109lbs. Today, my lung function is 92% and my weight is typically between 124-127lbs. That means, since beginning Kalydeco 2 1/2 years ago my lung function has gone up about 15% and I've gained 15-20lbs! Now if that's not amazing, I don't know what is!! From two little blue pills twice a day!

 

I plan to continue taking those little blue pills and doing ALL of my treatments every single day...well, until there's a cure!