Sunday, December 16, 2018

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.

Thursday, November 29, 2018

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.


Monday, November 26, 2018

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.

Saturday, November 24, 2018

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.

Sunday, October 21, 2018

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!

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.

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.

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!