Saturday, October 15, 2016

The Hospital Made Me Lazy

Unlike many people, those with CF do not have the luxury of calling into work and sleeping off an illness. Our illness can't be cured with a few days in bed and extra rest. For me, sickness seems to linger for at least a few weeks until antibiotics kick in and even then I still have another week or two of clearing out extra mucus, recovering from side effects and regaining my regular energy levels. If I were to sleep off my sickness I wouldn't leave my bed for a month! Not to mention all that inactivity would mean my mucus would pool at the bottom of my lungs making it virtually impossible to clear out. So like most people with CF, I do what I can to power through. We can't ignore dishes, and laundry, and cooking meals for a month and so through fevers and violent coughing we find a way to keep life from falling apart.

That is unless we are in the hospital.

Hospital stays, as horrendous as they can be, mean no laundry or cooking or much of anything outside of treatments and interrupted sleep. And somehow as much as I despise the hospital when I start to get sick I crave the lazy days the hospital provides. Let's be real, when sickness finds its way into my home all I want to do is lay on the couch and watch TV while someone brings me food and water on demand, doesn't everyone? And if it isn't already obvious I just want to say, I have no desire to be in the hospital (ever), I just want the lazy perks that come with a hospital stay. I guess I want my cake and I want to eat it too! Sure, most people don't want to do chores while sick, but going to the hospital more often these past few years has started to train my mind that sickness = laying in bed all day reading and watching TV.

Unfortunately, my four year old can't cook and I despise the hospital so I guess for now I need to learn to be productive through sickness and push those lazy desires aside.

*I am currently not sick, but we just got through our first school year colds. The cold made me realize how much I now crave being lazy while sick compared to the past*

Sunday, October 9, 2016

Night Treatments

You know you are doing your treatments too late when you mix your Cayston and pour the contents in your Pari LC plus neb cup. Sigh! I guess I will be one dose short this month!

Friday, September 30, 2016

Kaylee Chatter

Now that Kaylee is four I figured she could graduate from "toddler talk" to "Kaylee Chatter." Same idea, but with the new age comes a new name!

Kaylee came to me pointing to part of her collarbone, "I found my port! I kept looking for it and I finally found it!"

We were driving home and Kaylee was getting antsy with the long ride. I could hear her mumbling and grumbling, but couldn't tell what she actually saying until she shouted,
"It's a catastrophe!!"
Me: "What is a catastrophe?"
Kay: "ME! I AM the catastrophe!!"

"When I was little I used to say, pock-a-sell (popsicle), but now that I am bigger I know it's pos-isil."
I couldn't get myself to tell her it is still wrong!

We went apple picking and Kaylee ran ahead. She shouted, "Come quick! I found a glorious apple!!" Since when did she say glorious??

Kaylee was stepping off a stepstool at her cousin's house, but she did not realize there were two steps rather than one. Basically she didn't realize the drop was as far as it was and she sorta-kinda almost fell, but in reality wasn't even close to falling.
K: "Did you see that?"
Me: "I did. Are you okay?"
K: "I am okay, but it was terrifying!!" (Dramatic much?)

I must have said, I love you to death a few times to either Kay or my husband because Kaylee started saying, "I love you for death" recently.

At different times during the day the ice maker in our fridge can be noisy. No matter what Kaylee is doing if she is within ear shot of the noise maker she will stop and shout,
"The fridge needs to talk to me!"
She then puts her ear against the fridge and will have a quiet conversation with the fridge for several minutes. Some kids have imaginary friends. Kaylee makes friends with the appliances.

Wednesday, September 28, 2016

Preschool and Cystic Fibrosis

When my husband and I toured preschools in the area, like all parents we had a checklist of things we were looking for. For us, the most important aspect was that it was a parent participation preschool. We wanted to be actively involved in our daughter's first school experience. We also liked the community feel of knowing all the children and their parents in addition to the teachers. Some other items on our checklist included a play based school with small class sizes. Since Kaylee is drawn to all things art related we also wanted a lot of access to open ended art to allow her to follow her (current) passion.

Having Cystic Fibrosis, there was another concern that was on my mind that I am sure other parents do not consider. Germs! At the time of school tours my health was doing really well, but having low lung function and knowing how complicated CF and colds can be I worried about the sickness Kaylee and I would be exposed to. We looked for places with several separate rooms that the children could venture in so they would't all be cooped up in the same room, we looked for sick day policies and small classes. We found a school that seemed to fit our criteria, but we knew germs and sickness in the preschool setting was inevitable. Kids that young just don't have the same concerns about sanitation. Coughs go uncovered, fingers find their way into noses and hand washing skills are sub-par.

And then summer came and with it an unusual summer exacerbation that didn't want to leave. As the school year approached my exacerbation was unwavering and my concerns grew. I laid awake at night wondering if sending Kaylee to school while I was having trouble regaining stability in my health was a good idea. I toyed with pulling her from school even as the first day of school was just a few days away. I started bringing up ways to stay healthy with Kay and to avoid germs to the best of her ability (while being careful not to make her paranoid or anxious about they idea of germs).

Finally, I turned to fellow CF moms and got some great advice from women who are in my same situation. I got tips from moms with lower lung function, those on the transplant list and even a few moms post transplant. I thought I would pass along the ideas they shared. Of course air born sickness is impossible to avoid outside of avoiding all public places, but I am hoping these extra precautions will reduce the amount of germs we get exposed to.

  • I asked the teacher to sit Kaylee away from kids that recently came back to school from being out sick or those who have a cough or seem unwell. There is no assigned seating in her class so this is really realistic, but may prove more challenging as she enters grade school with her assigned desk.
  • Removing all school clothes (and shoes) the second we get home. Everything goes in a wash bag in the garage. This one frustrates Kaylee so much because she doesn't want to be bothered with changing again. My husband has done the same thing with his work clothes for years in order to keep us healthier so I am hoping this becomes a family norm rather than a point of frustration.
  • We both sanitize our hands the second we get in the car.
  • The school requires kids to wash their hands before snack, but I also gave Kaylee a small hand sanitizer (with glitter) on her backpack in hopes of encouraging her to use it other times throughout the day. I am pretty sure she only touched it once, but it is better than nothing.
  • I put Kaylee's hair up everyday that she goes to school to cut down on the amount of times she touches her face (she brushes her hair from her face a lot when it is left down). I tell her I am putting her hair up to keep it clean while painting and doing crafts because I really don't want her to become paranoid about germs. She doesn't need the burden of my health on her shoulders. She totally buys it because I can't even begin to tell you how many times at home we have had to clean paint or glue out of her hair while she was busy crafting.
  • We talked about not touching out eyes, nose and mouth. We talked about moving away from kids that are coughing or sneezing a lot and washing our hands.
I am trying very hard not to make her afraid of germ and I do not want to create any anxiety around this topic. It is tricky because for my wellbeing she needs to be conscientious of germs. This will be an issue forever in our family. As long as I have CF I am at risk and if I get a lung transplant someday my weakened immune system will make me even more vulnerable. It is such a delicate balance between not letting CF dictate your life and yet being realistic about the ramifications of "living a normal life" with CF if you are not careful.

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!