Saturday, September 24, 2016

Enzyme Trouble

I have been taking enzymes for 18 years now. (I was diagnosed at 14 which means I went 14 looong years without enzymes when I clearly needed them. I will save you the gory details of a CFers life without enzymes. Let's just say certain high fat foods like donuts or anything with cream sauce still makes me queasy because there are some things you can never fully erase from your memory. Gag, just thinking about it... never mind). After living without enzymes I am a master of taking enzymes. But there is one aspect to enzyme taking that I have never fully been able to figure out.... meals with multiple courses!

For our anniversary, my husband and I went out to eat. We had a three course meal (woot!) and I came prepared. I brought my enzyme timer cap which lets me know exactly how long it has been since I ingested my last enzyme. I know enzymes are good for about 30 minutes and so as every course arrived I checked my timer. If it has been 30 min I took a new dose. I adjusted depending on how fatty the course was and how long it had been since the last dose. Basically, I am trying to say, in a way too complicated way, is that I was prepared! And yet, as with every time I eat a multiple course meal the next day I felt like crap... literally!

So after this last experience where I really think I did everything right and yet my stomach still got fussy the next day I decided that I give up. The next multiple course meal I am asking the server to give us an extra big table and to just bring all the courses at once and we will have a feast instead.

Tuesday, September 20, 2016

One Year of Orkambi

This post was incredibly hard for me to write. Like all matters related to CF there was no black and white with Orkambi, for me at least. I wrote extensively on what the first four months were like, which were a rollercoaster to say the least. My purge was intense and utterly miserable if I am perfectly honest. Walking often induced shortness of breath that was so intense I wondered if I would ever walk again at times. In fact, sitting often brought on shortness of breath that left me gasping for air. At the four month mark my negative side effects disappeared and I continued on to have one of the healthiest winters in my recent history which I wrote about at the 24 week mark.

Was orkambi responsible for my winter health streak? I would like to say yes. If you asked me mid winter without a doubt I would have said, "Yes!!" Last winter, my first on Orkambi, was by far my healthiest winter ever. However, somewhere around June sickness struck again and I had three weeks of IVs, followed by weeks and weeks of oral and only now have I seemed to stabilize at a number that is still below my pre-Orkambi baseline. Would this last exacerbation be worse if I wasn't on Orkambi? Possibly. Could Orkambi be causing some inflammation that is keeping me from hitting my pre-Orkambi baseline? Potentially. So many factors come into play with CF that it can be hard to know exactly what is causing problems and what is helping. The progressive nature of the disease can complicate this issue even more. However, outside of several rough months this summer, I do feel that Orkambi has improved the consistency of my mucus and has made my lungs cleaner and clearer. I just can't quantify the way I feel and I have no real clinical data to back this feeling up.

Even though my lung function didn't improve like I had hoped and wished with all my might, my GI system has absolutely improved and that is unwavering to this day. Despite being sick for a couple months without reprieve my weight has remained stable. I no longer have days that I am so bloated my stomach feels stretched and painful. As long as I take my enzymes I really do not notice or think about my GI system at all. Of course, even Orkambi couldn't get rid of my CF belly, but as they say beggars can't be choosers and so I will take what I can get!

Saturday, September 17, 2016


My baby took her first real steps out into the real world this past week. On Tuesday September 13th, Kaylee, my husband and I walked hand in hand into a local preschool, helped Kaylee hang her backpack on a hook, and watched from the sidelines as she explored the school where she would spend two mornings a week.

When the teacher started calling the kids to the carpet to start their very first day I snuck over to say my goodbyes. As I was hugging my baby goodbye, feeling my heart swell with so much pride I could feel the pride forming tiny tears in my eyes, I asked Kaylee if she was ready for mommy and daddy to leave. I wanted to leave her when she felt strong and ready. She looked at me with those big blue eyes, wrapped her arms around me and said, "Mommy, I will let you go." And just like that she smiled, turned to the teacher only to turn back one time with a confident wave goodbye.

Her wording stuck with me as I walked down the hallway lined with miniature sized backpacks. "I will let you go..." I knew in my heart that she was the one ready to adventure out in the real world. Her words rang true that I was the one holding on so tight. I was the one that needed reassurance that it was okay to send the biggest piece of my heart out into the harsh world. I was the one that was having trouble reconciling that each year from here on out she would become more and more independent and I would ever so slightly fade into the background of her life. But I was the one that had trouble letting her go. Kaylee, full of curiosity and wonder and a healthy sense of naivety, was more than ready to find her own path.

To witness the confident, independent, and self assured little girl Kaylee has grown to be was enough to make my mommy heart swell so large it caused physically pain in my chest that morning as I got in my car and drove away. Even after four years of loving this child with every fiber of my being I am still in awe how the human heart can love so immensely!

Monday, September 5, 2016

Six Years Later

My husband and I just celebrated out sixth wedding anniversary this year. I know six years of marriage, living together for seven, does not seem like a significant amount of time. However, the past few years has made me realize how much lives can change in such a short period of time and as a result why so many marriages fizzle out while still in their infancy.

I am thankful every single day to have my husband by my side as my best friend, my biggest supporter, and my strength in hardship. However, delving into our marriage six years ago, I had no idea what marriage was really about. We were in love, we made each other laugh, we loved spending time together, but how could we possibly know what we were really getting into?

When my husband and I first met, like so many other couples, our hobbies were aligned. We loved dancing late into the night, eating exotic foods (the more exotic the better), going to the movies, cooking and making up signature cocktails, and sleeping half the day away on weekends.

And then life happened.

We got jobs that required early mornings and staying up half the night wasn't so easy. We had a baby which meant sleeping in and watching movies all day was out of the question. And eating exotic foods in hip restaurants wasn't the relaxing experience it was in our early marriage with a kid that didn't have the same interest in more intense flavors and textures when it came to dining. And those days caring for a baby meant that things like cooking and making up cocktails (hello breastfeeding) just weren't realistic. Suddenly, our hobbies we enjoyed together we no longer feasible.

And this is where things in our marriage started to shift. We needed to redefine "us" with the demands of jobs and kids and all the intricacies of life.

And somehow we emerged from our young and carefree marriage as a couple that found completely new, but equally (or more exciting) hobbies that we enjoyed together. We found a true love for the outdoors, and playing poker. We started enjoying mornings drinking coffee together and chatting even when it meant chugging it side by side because we were running late. We started discussing the design of our backyard and found joy in landscaping together. We are not at all the same people that got married in 2010. We have different hobbies, different priorities, different lives than that young na├»ve couple.

But we were so very lucky that we find ourselves just as compatible with each other as the day we first met. Our lives look so drastically different, but we are still one another main priority.  There is a deeper sense of togetherness after spending the last six years of marriage supporting each other during our hardest most unlovable moments. There is a stronger sense of unity after changing so very much and watching our lives unfold in ways we never anticipated and through it all we always put one another first.

Saturday, September 3, 2016

Fighting Nausea Naturally

Nausea and CF go hand in hand thanks to all the antibiotics used to treat chronic lung infections. Let's be honest, sometimes our antibiotics cause nausea to such a degree that nothing will help except another prescription, you're welcome pharmaceutical companies. However, sometimes the all day waves of nausea aren't enough to warrant another pill, but are still uncomfortable enough that they can't be ignored. Through lots (and lots) of trial and error I have found a few natural ways to combat nausea.

Ginger/Peppermint tea: Ginger or peppermint tea with the addition of fresh ginger can help ease those waves of nausea relatively well and you get the bonus of hydration (which is important when taking any antibiotics). The only problem with this remedy is that most of my medication induced nausea goes hand in hand with the strange metallic taste that coasts your mouth and for some reason I find tea heightens this effect. So it helps the nausea, but can taste like sipping a hot cup of liquid metal which induces it's own form of nausea. If nausea is an issue without the strange taste buds then using ginger/peppermint tea can be very successful.

Peppermint Oil: Peppermint essential oils helps ease the nausea almost instantly, but seems to only have an impact when I am actively smelling the oil. And subtle whiffs of oil doesn't do anything, it has to be strong. The second I turn my head away from the source, bam, nausea comes rushing back. So unless I am willing to sit around smelling oil all day this really isn't practical for all day use. However, I use this when the waves of nausea are strong, but infrequent. I often use it as I am getting ready in the morning because my nausea seems worse after waking up.

Peppermint Gum: Going with the peppermint theme I find that strong gum actually works really well. It is one of my favorite nausea remedies because it is convenient, long lasting, and can be used over and over (you can only drink so many cups of tea).  The other reason I love using gum is that it helps mask the nasty taste that many meds leave in my mouth so it is a two for one deal.

Snacks: When nausea is constant and severe I find the only thing I want to do is sit by a toilet or garbage can. The very last thing I want to do is put more food in my body. However, I have found that never letting your stomach get too empty makes a big difference. Ugh, I know. But if you can power through the overwhelming desire to vomit and try to put food in your mouth, you will thank yourself later.

If anyone has any other tips I would love to hear them. This round of antibiotics is almost through, but I am sure another nausea inducing medication is just around the corner.

Wednesday, August 31, 2016

Little Lungs

When Kaylee was a baby she was terrified whenever I did PFTs at clinic. I can’t say I blamed her! I would take a deep breath and then blow so hard my face turn bright red and my eyes bug out. Then the machine would beep and I would start the whole process again. My clinic tried everything to ease her fears, but the kid just cried and screamed every time I did PFTs no matter how much prep, letting her try to do them herself, explaining, cheering, etc that happened during my PFTs.
Eventually I got my own PFT machine for home. I am not sure it helped ease the fear of the clinics PFT machine, but my home machine grew on her. Every time I did home PFTs she did too. It was cute to see her scored. Her FEV1 (how much air you can blow in the first second of a forceful exhale) sat around .60 when she started. Her little lungs not having much to blow out at all let alone in one second. We would always look at the machine and comment how our lungs were so happy (even when my scores revealed lungs that were anything, but happy).
Yesterday, I was taking my daily PFT while she was in the room. “I want a turn,” she shouted the second she saw me grab my machine. After taking my three values I handed the PFT machine over to her. It had been a while since she had taken her FEV1 and she was excited to try that PFT machine again. After she blew she handed the machine to me, “How happy are my lungs?” she asked as she always does. I looked at my meter and was shocked! “Try it again,” I said, not believing what I was reading. She blew again producing the same score.
Cystic Fibrosis disease progression has rendered my lungs so damaged that my 4 year old can officially blow more out of her little lungs in one second than I can! Sobering to say the least. On the bright side, my FVC (total lung volume) is still higher than hers so I do have that going for me!

Saturday, August 27, 2016

Mysterious Exacerbation

For my own record keeping and to potentially help others that may find themselves in the same situation (even if it is just for the comfort in knowing others go through long bouts of struggle with CF too) I really wanted to be better about keeping this last (exhausting) exacerbation well documented. However, living with the complications of CF everyday can be mentally and physically exhausting and despite finding this blog as a way to release some of the frustration and thoughts that surround CF I often find that while I am in the thick of sickness taking up mental space to write about it can feel like too much. And so this is a quick breakdown of this last complicated exacerbation, which I might add, is not over.

 June 13th: Despite feeling relatively well most of the summer I blew a disappointing 34% at clinic. Was given 2 weeks of Cipro.

July 5th: Called to ask for stronger meds as Cipro wasn’t enough to make me feel better.

July 13th: Started IV Zosyn in hopes of it doing the trick.

July 18th: After 2 weeks of orals and one week of Zosyn my FEV1 dropped to 28%. Decided on doing 3 weeks of Zosyn total to hopefully kill off more pseudomonas.

July 25th: Feeling better although not great, but my skin started to react to my port dressing. Started Prednisone to contain my severe contact dermatitis due to all adhesives.

July 6th: Went to the ER with a suspected clot. No clot was found!

August 1st: Felt like death (virus? Cold?) and blew 31% in clinic. Decided that pseudo wasn’t the culprit to my sickness (it is sensitive to both Cipro and Zosyn) and tried treating other bugs that may not show up on my culture.

August 3rd: Had an allergic reaction to Zosyn and woke up covered in hives.

August 12th: Started Bactrum and Rifampin for 10 days. No progress

August 27th: Started Levoquin and Minocycline and am currently crossing my fingers this cocktail works.

 After two months of antibiotics I still looking for the right antibiotics for what is causing my lung function decline, fatigue, and afternoon fevers that do seem to disappear while on antibiotics, but instantly come back when antibiotics end. CF can be such a frustrating disease and I hate when these exacerbations seem to turn into months rather than weeks. I am hoping that I can get things under control before cold and flu season begins. I have already started stocking up on hand sanitizer for this winter.