The Time My Tongue Turned Black

I was sitting in the infusion chair waiting for my port to be accessed by the nurse she asked about any crazy see effects I have ever had from all the medications I have been on. I told her that out of the few medications I am not allergic to, I have been very fortunate to not experience any bad side effects. Sure, I have normal issues like nausea, headaches, diarrhea, but those seem pretty run of the mill. I think the universe overheard me and decided I was needing more antibiotic excitement in my life.


So fast forward a few days and I was well into my third round of Avycaz and my first round ever of Zyvox. My side effects were what I would consider run of the mill. My GI system was way off and my mouth tasted like metal. Nothing too horrible. Food tasted funny and after I ate or drank anything a strange lingering after taste seemed impossible to cover up. I found myself chewing on gum a lot because my own saliva tasted bad. I also noticed a strange fuzzy feeling in my mouth. Nothing overly concerning, but annoying all the same.


And then one day I was brushing my teeth and just so happened to see my tongue in the mirror. It looked.... black... and fuzzy! I stuck my tongue out all the way and sure enough my pink tongue was replaced with a black and somewhat fuzzy looking tongue. I was horrified. It was as disgusting as it sounds. After I showed my husband he cried, "Why did you show that to me before bed? Now I am going to have bad dreams." I still can't figure out why he wouldn't make out with me after that. I tried brushing my tongue to no avail. A quick Google search told me this is a benign side effect from none other than Zyvox. Lovely. It did explain my weird altered sense of taste and the grossness in my mouth, but it wasn't exactly the most attractive explanation.


I have been off Zyvox for three days now and my tongue is slowly transforming back into its old color and texture (although I still have a ways to go). This has been by far the strangest antibiotic side effect I have ever experienced.

CF is Weird

The title say it all: CF is weird! I went to clinic Monday and coming off a pretty horrendous cold I figured my numbers would be low(ish). I was expecting the low to mid 30s. I blew a shitty 25% which is a new low for me. And although that number is super scary low it did not represent how I felt at all. I walked 2 flights to get to clinic...I can't even do that when I am 35%. I don't understand how my numbers are drastically different than the way I feel. I knew I didn't feel like my baseline, but 25% is about as scary as it gets when you aren't on the transplant list.

Considering that number is so very low and extraordinarily scary I just knew I would be going inpatient and felt a bit devastated that I would be miss out on everything holiday related before Christmas. Kaylee's days are numbered where she will still believe in Santa and I didn't want to miss out on perhaps the last year of a magical Christmas.

And then my doctor decided to put me back on Avycaz and an antibiotic for staph which meant I would be doing home IVs. Considering everything I wrote leading up to this sentence I should have been happy with his decision, but mainly I just feel afraid. I have been on IVs 3 times since October (averaging every month!) and I just want to get better so badly. There is so much more pressure to do well when I am at home because everything falls on me. I know that even in the hospital I am the one doing breathing treatments and it is MY body that is trying to heal, but it feels like if I fail...the blame is shared between the hospital staff too. I know that sounds weird and logically doesn't make sense, but I feel like if I don't get better on home IVs I am the only one at fault (well, and CF too!). The wiggle room I used to have in my pfts have slowly disappeared over the years and I know that CF can get deadly so very quickly. I have seen it happen too many times too stay in denial.

Maybe this is a rough patch...I have had them before. Maybe this is a hump to get over to get back to my normal life. But maybe it isn't. Maybe this is a sign my lungs are too tired. I have never been the gambling type and so I won't gamble with this either. I want to be prepared if this bump turns into a downward spiral. And so with lots of tears and a long talk that went well into the night my husband and I decided we needed to start the process for transplant. I never doubted I wanted a transplant. I really do love living and I have so much to live for! How could I leave my daughter motherless?? How could I leave my husband alone on this Earth? I would do anything to live longer than CF will allow and transplant is the only chance at getting extra years.

But I never would have imagined how hard it would be to step over the threshold from "someday..." to "now" when it came to transplant talks. I feel like I failed having to admit that my journey with my CF lungs is over. I know that CF ends the same for every person and so it can't be seen as a failure, but as the inevitable, but that doesn't change the sense of failure I feel turning to the last resort when it comes to CF. I worry I am not strong enough to endure all that entails both physically and emotionally that comes with transplant. I feel sad for my lungs. They worked tirelessly even with all odds stacked against them to let me live these beautiful 34 years and I hate to see them go. I know, I know. It makes no sense to feel attached to an organ, but there is no organ in the world that has worked so hard for me than my lungs and again I feel like I am failing by choosing to replace them. I am so so afraid of what the process will do to my family. Just the thought fills me with so much mama guilt it makes my heart hurt in my chest. And what if they go through all the worry and stress and then it doesn't work?

I know none of these questions can ever be answered and the worry will never fully resolve, but I need to focus on choosing a transplant clinic that I think will suite me best. And in the meantime I need to work as hard as I can to delay the need for transplant as long as possible.

Mr Sharpy

There are certain experiences we have as a kid that through the lens of the parent is so radically different that watching your child live out those same experiences can feel confusing. Early November brought one of those experiences to our household. There was a lot of excitement buzzing in the air because Kaylee, after wishing and hoping since the age of 3, finally had a tooth that was on the cusp of falling out. Being a young first grader and possible a bit late to bloom in the dental arena, Kaylee felt as if she were the very last first grader to have all of her baby teeth. So when that tooth finally started to really wiggle her excitement could not be contained. And one evening as I was tidying up in the kitchen and my husband was working on Spanish with her, she squealed, "My tooth fell out!!"

She was busy jumping around the kitchen when I asked to see her baby tooth. She gave me a wide open mouth grin, bearing a little empty spot in her smile that exposed a little pink gum and she quickly deposited her baby tooth in my hand. Seeing that little strip of gum shot me back to the days when her smile was all gums. And then glancing at the tiny little tooth in my palm I was remembering the day that her very first tooth, lovingly named "Mr. Sharpy" by my husband, burst through. Suddenly, with the emergence of that tiny white fleck in her baby mouth all the fussy nights and cluster nursing made sense and somehow it all felt worth it because my baby had her very first tooth!! And now, that same tooth that brought so much excitement was sitting, useless in my hand. And yes, I was excited. How could I not be with a 6 year old brimming with pride over this tiny tooth and the adult tooth that would arrive a few days later? But I also felt an overwhelming sense of sadness? Or was it nostalgia. I can't be certain, but something was tugging at my heart strings that made me want to pull Kaylee closer and demand her to stop growing older.

These milestones that our children keep hitting bring them so many feeling of joy and excitement and I clearly remember the thrill that came from losing some of my own teeth. But as a mom, these milestones come with a harsh reminder that life keeps marching on and our children keep growing older and more independent of us. And yes, ultimately this is what we want for our children to grow up and apart from us, to be their own independent beings. Oh, but this mama heart wishes time would slow down just a bit so I can enjoy being her mama for as long as possible.

Expectations

It is human nature to try set goals and work towards achieving them. Nobody want to remain stagnant in any aspect of their lives: relationships, career, education. We have a drive that pulses through us so that we can continue to achieve our dreams and goals and pursue a life full of richness and fulfillment. This drive pulses through me just as strongly as it did when I went off to college, when I applied to jobs, when I went up for a promotion and when I wanted to become a mother. But I am struggling so much with taking that drive and that passion and making it a reality as CF presents itself more and more in my daily life.

CF has changed the way I view myself, specifically in the past 5 years. As my health situation has become more complicated I have trouble living the life as the person I think I am. I have always been driven and hard working and prided myself on doing well in school and in my careers. But lately my health seems to get in the way of all of my goals, not just the far reaching career and life purpose goals. Small goals like improving my swim times are constantly set back to zero by an exacerbation which leaves me too short of breath to swim more than a few laps. And even more basic goals like "I am going to catch up on laundry this weekend" get pushed aside when I wake up sick and unable to get out of bed.

In this season f my life I have been trying to focus on letting go of all expectations and just let my life ride out in the way it was meant to be (dictated by my health) and in many practical ways I have learned to adapt. I do everything early when I can because I know when the time comes I may be too sick to fulfill my duties. Like this fall when I bough Kaylee's backpack a month early, but didn't get shoes for her. I ended up sick the week before school started and she didn't get back to school shoes or other nonessential, but fun new items for the new school year. I write things on my calendar knowing there is a good chance that my health won't allow me to follow through. I say no more often than yes when given invitations trying to preserve my energy and minimize my germ exposure. But the practical ways of adapting are easy. It is the emotional adaptation that seems to be a struggle.

For so long Kaylee filled all my desire to feel successful because being a good mother is the most important job in the world and it was a 24/7 job that used up all of my energy and left me happy and full of pride each night. But as she has gotten older and needs me less and less I find more time in my day that I am not needed which makes me want to push myself to take on new responsibilities. With Kaylee in school full time I am finding myself with a lot of free time and that calling to do more, to be more is in full force.

A few weeks ago an opportunity fell in my lap that seemed to be the answer to my need to do more. A mom in Kaylee's class sought me out because she knew my background in education. Her daughter is dyslexic and she wanted my help through a specific program to tutor her daughter. She paid for the training and told me that the demand for these tutors in Kaylee's school alone was high and she had several families that may be interested in the same services. The hourly rate is high and the schedule flexible. I felt like this would be the perfect jumping off point in making money and starting my own business. Finally, I felt a little hope towards a fulfilling career. My husband was on board and we both felt so much excitement for this new endeavor.

The day before my first tutoring session I got sick. I coughed for 24 hours straight. I was able to tutor, but within hours of the student leaving my voice completely disappeared which happens every single time I am sick. The next day I wasn't able to get out of bed. The negative side of me felt so stupid for believing that maybe I could try to spread my wings and be human. I felt dumb for thinking I could push CF aside and try to do something for myself. The hopeful part of my brain that usually cheers me on even when the odds are slim was quiet for the first tie in a very long time. I haven't been well since September. Despite two rounds of IVs and a few rounds of orals, I seem to be in a pattern of sickness that my body can't pull out of. I have been here before and I know I will be here again, but right now it feels like a sinking hole I can't get out of. I also know when I sit here in the bottom of the pit of sickness my hope slowly starts to slip away. With CF the only way to survive is to hold on to hope because we really don't have anything else to grasp for.

For now, I am pausing my plans to build a business. It feels like a failure, but maybe it is just a pause button on a future success. Maybe the timing isn't right. Maybe it never will be, but if the time ever is right I know I have a plan. Maybe when I crawl out of this pattern of sickness I will reassess and try again. I will keep this one student and maybe over the next few years I can add a few more. Maybe I can't, but maybe by then I will have learned to let go of the expectations of what life should be like and just live the life I was given.

Smoke and IVs

If you watched the news last week you may have noticed there were some catastrophic fires on the West Coast that completely devastated communities. We were so fortunate to be far enough away to be safe from any flames and destruction. We did, however, have a much less threatening, but relatively scary side effect from the fires. Our city and surrounding area was completely engulfed in smoke. When the fires first broke out some smoke made its way to our area, but we have experienced smoky days from fires in the past. We usually avoid exercising outdoors for a few days and move on with life so I assumed we would have a few days of avoiding the outdoors and life would go on. Sure enough on Saturday Kaylee's soccer game was canceled and we spent the weekend indoors to prevent breathing in the bad air. We had no idea that within a week the air would go from unhealthy to hazardous which would result in our local schools shutting down. By midweek the air would be so thick that visibility would resemble the foggiest winter morning. It was terrifying that we were breathing such horrible air, but it was even more heartbreaking that the smoke that filled our neighborhoods was carrying small fragments of other people's destroyed homes, and family heirlooms and human lives. 

Several of my friends fled and a few close friends invited me to stay with them in their Airbnb or hotel rooms in the mountains at an elevation that sat above the smoke. I had a strange predicament in that I was on IVS (again!). I guess I should back up and explain the IV situation really quickly...

I went to clinic a few weeks after stopping IVs and blew a 34% on my PFTs. It was up 5% from pre-IVs, but over the summer I blew 40% so we weren't happy to settle for 34 anymore and I went back on IVs for 7 days. I was finishing up IVs when the smoke rolled into town and I called my doctor genuinely terrified of what breathing "hazardous" air and what it would do to my lungs. We decided to extend my IVs just a few more days.

With the extended IVs came drug deliveries and needle changes which didn't really work if I was 2 hours away in the mountains (not to mention that high elevation is pretty hard on me). So we hunkered down with the doors sealed off to prevent smoke from leaking in through any seams and the air purifiers on full blast. My extended family and I eventually left the area to stay in an Airbnb about 50 minutes away so that we would be close enough to return for any CF care issues I might need, but far enough from the smoke that we could go outside.

The rains arrived right before thanksgiving and washed away the smoke that was lingering in the air. I am also officially done with IVs. Unfortunately, Kaylee and I woke up yesterday with pretty bad head colds so we will see in a few weeks if the IVs worked despite the bad air quality and this nasty cold. This Thanksgiving we were reminded to be grateful for all we do have and even when things don't seem to be going well whether it is with health, a career or whatever else seems to be bringing you down, that we are so incredibly lucky to be alive and able to experience this beautiful life.

Skin Sensativities Solved!

My skin has a way of baffling the entire medical community with it's absolute refusal to accept any type of adhesive to touch it for any period of time. And sure, there are people who are sensitive to adhesive and even  people who complain about rashes from Tagaderm or other adhesive bandages, but I have yet to find another human being whose skin is as picky as mine. I have dozens of blog posts on the subject that can be found here, but I must warn you that some of the pictures are rather graphic.

To be fair, my skin is actually pretty easy going in most areas of life. I have zero allergies to any other products I have ever used anywhere on my body. Lotions, creams, sun screen, detergent are all fine by my skin, but for some reason the more PICC lines, IVs and port dressings my skin comes in contact with the more it revolts. And we have tried everything! We have talked to NICU nurses and burn centers and tried dozens of types of coverings to no avail. 

Well, we have finally discovered the perfect solution for all my skin troubles. The only solution we have found is the one and only Prednisone! I know, I know not an ideal solution, but 10 mg of prednisone kept my skin reaction at bay enough that when I got my dressing changed yesterday there was no swelling, no oozing blisters and my skin looked 100% intact.

There is such a huge amount of relief knowing that I can get through IVs without dealing with weeks of itchy, irritated and swollen skin. The only downside is that Prednisone elevates my blood sugar just enough that I have to be careful of what I eat. Now, this wouldn't be too bad except that it seems October is prime IV time for me given my history. This means that not only is Halloween candy off limits, but October is when all of my fall fruit is ripening on the trees. Pineapple guavas, lemon guavas, persimmons, pomegranates all taunt me with their high sugar content. Sigh! But as the saying goes: Beggars can't be choosers!

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.

Summer:
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 that...it 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.

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!

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.

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).

Symdeko

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.

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!

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!

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:


https://news.abbvie.com/news/abbvie-is-now-accepting-cf-scholarship-applications-from-students-with-cystic-fibrosis-for-2018-2019-academic-school-year.htm

Scars

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.

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.

Kaylee Chatter Part 9

I have been so bad at writing down all the funny things Kay says, but here are a few I managed to get on paper.

Kaylee has a boy in her class with Type 1 diabetes. His mom came in to bring awareness to diabetes and explain to the kids what he does to care for his health. A month or so later Kay was asking why people get diabetes. After a quick explanation I then added:
"just like Mason in your class."
Kaylee: "No, Mason doesn't have diabetes he has diabetes awareness!"

After seeing a weathervane on someone's roof,
"Look!! East, South, West, and .....New York!"

"When I grow up I want to have a baby girl. If I have a boys I am going to pass them around to people that want a baby, but don't know how." (yikes!)

A Day In The Life- Kindergarten

I wrote a few blog posts about our daily routine when Kaylee was a newborn, about one year, and again in preschool. I love looking back at the old posts and seeing how much (or little) my day to day life changes as Kaylee gets older. Our life is definitely not very entertaining on paper, but keeping with tradition here is a rough idea of what our day is like this school year:

5:20

• My alarm goes off and I quickly get my bathing suit on, pull on sweats, drink a glass of milk and drive to the gym.
• I swim for about 30 minutes, shower at the gym and come home.

6:30

•  I arrive home from the gym . Sometimes Kay is in bed waiting for me when I get home. Sometimes she crawls in bed with my husband as she waits for me and when I am super lucky she is still asleep!! Regardless, I start my treatments once I get home.

7:00-7:30

• I usually brew a cup of coffee for me and then start breakfast for everyone.
• I also do morning chores like empty the dishwasher, clean the breakfast dishes, and sometimes throw a load of laundry in the wash.
• Kaylee usually likes to draw, color, or read before school. The kid loves lazy mornings,

8:20

• By this point I have usually convinced Kaylee to get dressed, we brush her teeth and I do her hair.

8:40

• Kaylee has to double check her "school list" which is a chart Kaylee made (by drawing rather than writing) in the beginning of the year. It has all the essentials she needs for school including her school folder, water bottle, snack, etc.

9:00

• I drop Kaylee off at school. I always wait in line with her and then walk her to her class. She kisses me goodbye and I leave.

9:10-12:30

• I try to run errands, schedule appointments, and volunteer in her class during this time. When I am sick I try to SLEEP while she is gone which is a huge advantage to Kinder...I can get so much more sleep when sick. A few days a week my husband is home when Kay is at school so we get to spend time together which is really nice.

12:30

• I pick up Kaylee. There is a pretty big group of kids (and parents) that stick around after school to let the kids run around and play in front of the school. We almost always stay for about 30-60 minutes.

1:30-4:30

• We go home and eat lunch, go to the library, go to the park, read books, etc. We really do not have a whole lot of consistency to this part of our day.

4:30-5:30

• I make dinner and Kaylee helps on occasion. Often Kaylee does her homework while I cook so I can help her while she works at the kitchen table.

6:30

• If we eat early then I will do my treatments before Kaylee goes to bed. I much prefer this schedule because sometimes I am so desperate to fall asleep after putting Kay to bed and treatments feel like torture because I am so tired. And if I am not too tired, but my husband is home I prefer spending some alone time with him over spending our alone time doing treatments. However, depending on our afternoon plans I can't always pull this off.

7:30

• Kaylee gets ready for bed. We read every evening. Sometimes she wants to read to me and sometimes we take turns reading to one another.  I tell her a story (which is a tradition we have had for so long now I can't even remember when it started). Finally, I give her a counting challenge. One time when I was in the hospital my husband would count to Kaylee in Spanish until she fell asleep. When I finally got home she wanted me to count to her still so I counted to 20 in Spanish each night for her. Now that she is older I have her count to me (sometimes 1-100, sometimes by 5s or 10s, or I will tell her random numbers like count from 160-190).

8:30

• Kaylee is in bed and I do treatments if I haven't already. Depending on my husband's work schedule I either hang out with him or have a quiet evening to myself.

10:00

• Go to bed!

Unreliable

The first time I was completely blindsided by a bad breakup was in 2010. A few weeks before my planned wedding I got a phone call that I never expected to receive. I was told that my dependable and completely reliable enzymes (Creon) were being removed from the market, never to be used again. This breakup came without warning and I was quickly forced to say goodbye to Creon and hello to Zenpep.

Eventually, I settled in with my new enzymes and soon realized that the new enzymes were an improvement from the old brand. For the past 7 years all has been well.

And then it happened again!

I called to refill my enzymes earlier this week only to hear the pharmacist explain the formulation to Zenpep has changed! What?! Noooo! Seriously, why do they mess with enzymes? I received my new Zenpep today. The Protease was reduced by 5,000 UPS units and Amylase was reduced by 25,000 UPS units! I have no idea what that means for my digestive system, but it isn't really a fun thing to experiment with.  I am hoping this current break up will ultimately be for the better and if not, I hope I find a rebound enzyme soon.

Late Resolutions

There are two things I can always count on when January rolls around. My insurance will suddenly have trouble filling some (or all) of my medicine. And the other is that my gym will suddenly get crowded.

My morning workouts in the winter are ideal. Most of the summer swimmers abandon the pool when the temperatures drop and it is not uncommon for me to have the entire pool to myself. When I am done I have the showers to myself, the changing room to myself and at times it feels like the gym is reserved only for me! Pretty amazing, right?

Everything changes once the new year rolls around and the gym fills with people hoping to fulfill their new years resolutions. The day after New Years (Tuesday) I was bracing myself as I walked into the gym.  I was crossing my fingers there would be a lane open in the pool and a shower available when I was done. The strangest thing happened though, the gym was just as quiet as always. In fact, the rest of the week remained extremely quiet. It was bizarre, but I wasn't complaining because I never had to worry if I would get a lap lane or not.

And then, randomly. on Friday all the people I expected to see right after New Years showed up. The gym was suddenly bustling and every single pool lane filled. It was a complete turn around from the day before. I guess everyone's New Years resolutions started just a little late this year.