Sunday, October 14, 2018

Overdue Update

You guys, I went MIA for so long that now I don't even know how to catch everyone up. (I know I owe some people e-mails and I promise I will get to you if you emailed me during my missing person status) However, I left on such a happy high note praising Symdeko that I feel it is important to give you an update. I am not even sure how to organize this so excuse my format, this is the only way it makes sense in my head.

Following 3 weeks of Zosyn followed by the start of Symdeko my lung function reached new highs and I felt amazing. We did a lot of traveling and Kaylee went on her first international vacation which fulfilled a dream that I thought CF may have already stolen from me because my health continued to decline for soo long. I was really hopeful that maybe Symdeko would give me a few extra weeks or even months between exacerbations and I was hoping to ride that high right into the school year.

The Downslide:
2 weeks before school started (just over 3 months post IVs) I could tell my lungs were slipping.  I was pretty disappointed because 3 months is my usual length of time between exacerbations and so despite feeling so amazing all summer I was still on my normal 3 month cycle. Always an optimist, I thought maybe I would bounce back quickly or my exacerbation would be easier to manage.  Not so. In fact, just last week after trying 2 separate rounds of oral antibiotics my lung function sunk from 40%-29%. 29% is my "oh shit" number that I was hoping to avoid with Symdeko. Am I the only delusional CF patient that keeps expecting miracles only to be disappointed for …oh 20+ years in a row?

Back on IVs:
And so I find myself back on IVs 6 months after my last round. This is actually highly unusual for me because I usually do IVs once a year with orals in between. The biggest difference I noticed is that my lungs seem to be significantly more asthmatic since starting Symdeko. I actually was thinking Orkambi was contributing to my asthma component, but it seems worse on Symdeko. Although if asthma is simply a coincidence or actually a symptom of Symdeko is anyone's guess. (I haven't been on social media outside of blogs in several years and so I have no idea what other people's experiences on Symdeko have been. Is asthma a normal side effect?). In fact, I think I had my first true asthma attack the other night which was extraordinarily frightening, but I will save that for another post.

New Drugs:
The good news is that there are now new IV antibiotics out on the market. They are actually old drugs reformulated (or something like is beyond my scope of knowledge) to become new "old" drugs. I am currently 3 days into Avycaz which is a combo of Ceftazidime and Avibactam. I am hopeful that this new medication will work well because my bugs haven't come across this one yet, but only time will tell. So far so good with the only major side effect being that I got diarrhea almost instantly after starting and my urine wreaks (your welcome for that TMI).

I will be sure to report back how everything goes on this new medication. Hope everyone is staying well.

Saturday, July 7, 2018

Symdeko Update

I have been on Symdeko for 10 weeks as of today!! 10 weeks!! I meant to write an update at 4 weeks, but time got away from me as it often does during the summer months.

The background
I feel like I should give some background information that might be important for someone looking for similar experiences. I should mention I was on Orkambi for a few years prior to making the switch. Starting Orkambi was brutal to say the least and it took about 3 months of Orkambi before I felt human again. After I adjusted I feel like I gained stability, but saw no improvement in my pfts. In fact, I felt like my baseline was a bit lower after starting, but because I was more stable I felt like it was worth the tradeoff. At the very end of my Orkambi experience I was hospitalized with my lowest lung function 29% (although I number I had seen before). 

Two weeks after ending IVs I started Symdeko. This made determining how well Symdeko was working tricky because I wasn't sure how much Symdeko was responsible for and how much was the result of being freshly off IVs. 

Starting Symdeko
The first week I noticed I had a bit of a headache, but nothing unmanageable. I also noticed my digestive system was off. I couldn't really pinpoint what was off except I went from being extremely regular and feeling fine as long as I always took my enzymes to having complete inconsistency with my bowel movements. This lasted maybe a week (and potentially could have been related to being off IVs recently. Although that usually makes me have loose stools which wasn't really the issue here).

The First Month
The first month I recorded my PFTs on a calendar (with a home meter) and I watched them steadily climb. 1.13, 1.19, 1.20, 1.25, 1.28. Part of me felt it was too good to be true and was a fluke with my meter and part of me was so hopeful that this was a sign that Symdeko was working. I did have some streaking of blood in my sputum around week 3 which is really rare for me and was somewhat concerning, but it resolved quickly.

First Clinic
My first clinic since Symdeko was around the 8 week mark. This was 10 weeks after finishing IVs so I was no longer "fresh off IVs" and so I felt at this point my pfts would be a pretty good indication of how well Symdeko was treating me vs a temporary high due to IVs. 

Guess what? My PFTs hit 40% for the first time in YEARS! 40% has been my goal for so many years now and I had 100% given up on every hitting the 40s again. But 2 weeks ago at clinic I totally hit 40%!!! It felt so surreal and was all the proof I needed that this drug was working!!

And that isn't even the best news. I was at the park the other day and for the first time in a very long time I had an overwhelming desire to run. RUN? Yes, run!!! Because when your lungs are functioning at 20 or 30% the idea of running or even speed walking feels so impossible that the thought never even crosses your mind. But when your lungs feel amazing and clear and so much more open things that you never even conceived possible actually seem doable and show up on your radar. And so at my suggestion my daughter and I ran across the field at the park and then we ran all the way back. And you should have seen the look on Kaylee's face. This was something we never experienced together before (or at least in her memory) and she was soaking up every minutes with the biggest smile plastered across her face. That night when I tucked her in bed she said, "mom, it was fun running with you today." And it shattered my heart that I spend 6 years unable to run with her in the way a young mother should with her child and yet it filled me with hope that now I am capable of running along side my 6 year old through the park. It gave me hope that I will be around to run with this precious girl for many more years to come. 

Now I feel even more impatient for the triple combination to come out so that all my fellow CFers can experience better numbers and a run through the park. Because we all deserve that at the very least!

Thursday, June 7, 2018

The End Of Kinder

There are exactly 5 days of kindergarten left. I will spare you all the talk of, "I can't believe how fast..." because if there is anything I have learned about raising kids these past (almost) six years is that everything in life just goes fast and there is no way around it. 

The end of kinder feels like such a significant milestone. Maybe not as big as the end of preschool because in a lot of ways life will be similar now that we are in an elementary school. Kaylee's program is a two year program so she will have the same classroom, half of the same kids and the routine will be very similar. However, her day with me will shorten and her school day will be extended by 2 1/2 hours. We will only have late afternoon and weekends that it is really just the two (or three) of us and a part of me is mourning the loss of our time together. As she gets older and her social life takes precedence over home life I know our time together will get shorter and shorter. Each year she grows closer and closer to growing completely independent of me, which is our ultimate goal for our children, but it doesn't make it any easier. 

I wanted to keep this post light and remember some aspects of kinder that I know I will look back on with fondness as Kaylee gets older. So here are just a few things that I may miss next year in no particular order.
  • A group of 6-8 kids stayed after school everyday to play in the grass at the school entrance. It has become a favorite activity of ours. The kids run around making up games and laughing and playing while the moms stand around and talk. There have been days that we got in our car to go home only to realize we hung out for 2 hours after school just playing. 
  • The teachers walk the kindergarteners out to the front gate to release them to their parents. The majority of kids still run full speed to their parent (or grandparent) and wrap them in a hug when they get out of school. I have a feeling this type of greeting from the kids will quickly disappear as they get older. 
  • Everyday after I hug and kiss Kaylee goodbye she stand in the doorway as I leave and shouts, "bye, love you mom!" at least once, but sometimes up to 3 times. Kaylee is the only kid to do this, but none of the other kids even bat an eye at her proclamations of love for me as I leave. I know that in the near future her displays of love for me won't include shouting down the entire kinder/1st grade wing.
  • The oversharing! One fun part of having a kindergartener is getting to know all the kids in class. Kinders love sharing every detail of their lives including some details that really aren't necessary. One story tyat comes to mind was from a little boy who told me his "dad takes forever when he poops." I hear a lot of odd family info that I would rather not know. It does make me wonder what Kaylee's teachers know about me that they would rather not know about. I am sure with CF they have heard some strange stories.
  • My only name at Kaylee's school is "Kaylee's mom". "Look Kaylee's mom is here!" "Hi, Kaylee's mom" I am pretty sure some of the kids think that was the name I was born with because none of them have any idea what my real name is nor do they care.
  • Kaylee will still hold my hand as we walk to school and while we wait in line for school to start. Although as the year has gone on she has reduced the amount of hand holding. I wonder if she will hold my hand at all next year.

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!