Thursday, May 3, 2018

One Week Of Symdeko

I received Symdeko 2 weeks ago, but when I received it I still had some Orkambi left. I was desperate to try Symdeko, but also felt guilty that I was going to waste Orkambi when so many people in the world still aren't able to get Orkambi (or any other meds to restore CFTR function). I also was having flashbacks to the time Acreedo messed up because they couldn't figure out how to bill my insurance (the same one they had been billing of rover a year) and I ended up missing several doses of Orkambi. I decided the responsible decision was to finish out my Orkambi which would allow me to have a few extra Symdeko tucked away in case I ran into a similar issue.

I am about to finish my first week of Symdeko and HOLY CRAP this drug is so much easier to take than Orkambi. You guys, I have continued to live my normal life for the past week! I can walk and jog and be a totally normal human being!! No gasping for air or aches or fevers, no elephant sitting on my chest! I am not terrified that I am digging myself into an early grave by taking this medicine the way I did with Orkambi. No MONTHS of torture before any reprieve. This is AMAZING!

I plan to give a one month update when I get there. I am noticing some things that make me believe that I am doing better on this medication than I was on Orkambi, but we all know how varied each day can be with CF and I want to have a better sense if these symptoms are flukes or actual signs of improvement before I write about them. Overall, I am very happy with Symdeko and I don't think I will miss Orkambi for even a second (although I am forever grateful I was given the opportunity to use orkambi for the past few years).

Friday, April 20, 2018


The last time I updated I was in the middle of a 2 week (turned into 3 week) course of Zosyn. Without too many boring details I went in after 10 days of meds and my lung function only climbed a bit. I went from 26% in clinic (dipped to 22% in the hospital, BUT I hadn't had my bronchodilator and I have horrible asthma and basically can't breathe without it so I totally don't think that one counts) to 28% in the ten days. A 2% increase is absolutely crushing because it isn't clinical significant. I felt totally defeated and super frustrated that the hospital and IVs were all a waste. We agreed to give it 14 days and I also added Bactrum since some of my bugs were sensitive to it. Around day 13 I actually started to feel better and called to extend my IVs to 21 days.

I just went into clinic today (28 days after starting IVs and a full week off of IVs) and my PFTs were 36% which is basically my baseline. Whew! I am so relieved to have improved so much. It just proves to me that my body really does need longer doses of IVs.

Getting back to baseline came at a perfect time because I am getting Symdeko tomorrow!! I wasn't overly optimistic I would have the opportunity to try Symdeko because it is more expensive that orkambi and I didn't have confidence my insurance would approve it. Somehow my insurance did approve it and I am starting soon! I am so curious to see if I feel any different between Orkambi and Symdeko.

Tuesday, April 3, 2018

Drug Drama

Sorry for the delay in updating about the post hospital drama. In order to keep this from turning into a novel I will give a quick and condensed version.

Prior to being discharged I got a phone call from the pharmacy that gives me my home meds (which differs from my usual pharmacies). I was told that the system was down and so they couldn't confirm my insurance. What this meant was the only way I could go home with IVs was to sign a form that stated if my insurance didn't cover my home IVs I would be financially responsible. Since I was 100% confident that my insurance would cover these home IVs I signed the paper. You may have caught on to where this is going...

Two days later (Monday when everything was open again) I got a call from the pharmacy saying that my insurance didn't actually cover medication and that they needed to send the rest of my IVs, but that I needed to pay out of pocket. Now before I go on I must say that I sorta laughed everything off. I know my insurance covers my home IVs and I know that I get medications every.single.month that are covered by my insurance. So I told her she was mistaken and even pointed out I get a medicati0on that cost $250,000/year every single month that my insurance covers so her statement that I have no medication coverage with my insurance was clearly incorrect. I thought she would at least question her ability to understand my insurance if I clearly was getting medication through my insurance, nope! She insisted I was wrong. I then told her exactly how to bill my insurance, but she insisted that I was incorrect and I needed to pay for my supplies. I got frustrated and told her to contact my insurance directly. She hung up.

Basically, to keep from boring you I went back and forth with her, called my insurance and left messages (why do real people never answer), called my CF nurse to help me out (she was on vacation and the on call nurse was completely useless), and spent the first 48 hours home fighting the most ridiculous battle of my life. Finally, I went online and found a form from my insurance about how to bill medications and had my dad (who has a fax machine) fax it to my pharmacy.

The next day the pharmacy called and apologized saying they didn't know the policy. This pissed me off because I was telling them exactly what they needed to do from the beginning. Okay, so I get it. I am just a patient with 33 years of insurance experience and clearly don't know what I am talking about. Fine, but I kept insisting they call my insurance because they would clearly know whether or not they would cover my medication, but that never happened. So rather than do the job they are paid to do I spent the first few days frantically trying to prove something that I knew for certain, but nobody would believe me. Grrr. Can you tell I still get fired up about it?

I am now on day 12 of IVs and thankfully my insurance is paying for them.... just like I knew they would!

Friday, March 30, 2018

Bad Clinic Appointment and ICU

My CF likes to keep me on my toes and for no obvious reason my last clinic visit (one week ago) was an utter disaster. Weight was down (just a bit) and my lung function tanked. I had been very short of breath, but I didn't feel sick. I could manage my day to day life and although I was a bit more tired and very breathless I really didn't feel like I had a major exacerbation. And yet my lung function was a pitiful 26%. Yeah, pitiful! A few clinics ago I was on top of the world at 38% so to drop so significantly without having an obvious infection or any kind of virus was pretty shocking. Needless to say, I wasn't going to mess around and I agreed to IVs and a quick stay in ICU for desensitization.

Just like my last desensitization after several days (3 to be exact) of waiting for a bed to open up I was admitted in the evening, but there were no plans to start the process until the next day when the allergists would be in. After a lot of frustrating issues with meds (I really hate being on any floor that isn't a designated CF floor) and a very late start to treatments I was told I was being moved to isolation. When I inquired about isolation I was told something about a potential issue with  a nontuberculosis mycobacterium. Not the news I wanted to hear!! NTM is one of those bugs (like MRSA) that I just never wanted to deal with. (Later I looked up my new cultures which included 4 new bugs, but none of them seemed to be a NTM. I did have a few that were labeled "concern of infection control" so maybe that was the issue. I am still not completely clear except that when my doctor found out I was in isolation he was kinda upset and said it was absurd that they have to put me in a negative pressure room due to any of my bugs).

The next day I had my desensitization in my isolation room and by the time I completed the process (which was basically the same as last time) I was released from isolation and was told I would be moved once again as soon as a bed on the CF floor opened up. After more issues with meds including the nurses losing my Orkambi and the nurses refusing to give me more enzymes because I had my prescribed "3 times a day" dose of enzymes already I was finally moved to the CF floor. As I was being wheeled into my regular floor I felt so much relief to be around people that actually understood CF.

By Saturday afternoon I was released to finish my course of IVs at home. I was relieved to be out of the hospital and back in my own environment, but of course as luck would have it, the drama was just about to begin!

Thursday, March 29, 2018

CF College Scholarships

We all know that CF can come with an insurmountable amount of challenges. Navigating all of those challenges on your own for the first time is no easy feat. Throw college into the mix and it gets even more challenging. Luckily, AbbVie understands that on top of all those challenges money should not be a factor.

If you have CF and are going to be an undergraduate or graduate student (and have CF, of course) for the 2018-2019 academic school year please check out this link below:

Sunday, March 18, 2018


Spring was starting to appear in all the trees and flowers, and skies filled with birds. But the chill of winter was lingering and as the day started to fade to evening, Kaylee and I snuggled close together under her covers to stay warm.

Kaylee held the book in her hands and she sounded out words and read the pages to me. A new skill learned in kindergarten. As the evening grew later she started to get weary, "Can you read now?"

I took the book from her hands and sleepily started reading where she left off. She listened quietly for a few moments when her little pointer finger came up and traced the inner parts of my arm. Interrupting our bedtime story she asked,
"Are these freckles mommy?"
I looked to where her little finger hovered over the marks inside my arm. Old PICC line scars that were long forgotten.
"No, those are all the spots that the doctors used to give mommy medicine when her lungs needed help."
"Oh, like your port?"
"Yes, similar to my port."
A few moments passed as she touched each scar, looking intently at each one.
"Look, these spots look like Orion's belt. Your spots make a constellation! How lucky!"

And just like that my scar covered arms became beautiful skies sparkling with stars and constellations.

Thursday, March 8, 2018

Our Family Grew

There has been a lot of change going on in our household. I hope to post more about those changes soon, but I wanted to start with one that is coming about 2 weeks late!

Kaylee has spent the last few years begging us for a cat. Two weeks ago we finally gave in and Kaylee's dream came true. We knew going into the search for a cat we didn't want a kitten! After we took home a stray (and quickly adopted her out) when Kaylee was one we swore that if we ever got a pet we would opt for one that was out of that adorable, but oh so naughty kitten/puppy stage. We ended up visiting the SPCA and two local pounds before we found our sweet orange and white tabby. He is 2 years old (they think) and is a ball full of energy! Although he was labeled as a stray at the pound we are certain he was someone's pet before he came to us because he knows not to jump on counters and prefers to be in whichever room is the most occupied.

Kaylee spend a long time thinking about names and finally decided she wanted to name him "polka dot" after the markings on his back. The only problem is that Kaylee thinks "polka dot" is said "coca dot" and so he is now affectionately called, "Kokee" (spelling courtesy of Kaylee and her preference of Ks over Cs).

One last random detail. The first time I did may Vest and nebs he totally freaked out and ran to a bedroom to hide. Now two weeks later he is completely used to it and frequently comes over and sits near (or walks on) me while I do my treatments.