Kaylee Chatter Part 2

• "I am so cold right now I wish someone would pour 100 hot chocolates all over my body right now."
• Kaylee still says aminal and knows she says it wrong, but can't figure out how to say it correctly. "Mom, why do I still say aminal wrong. When I was one it wasn't surprising I would say words like aminal wrong, but by three saying aminal instead of the right way is very surprising!" I love how she says aminal and will be sad when she finally figures out the correct way to say it.
• "Mom, before the earth existed where were all the mommies and babies?"
  "That is a great question!"
  "Babies come from mommies bellies, but if there were no mommies, where were the babies? Like, was there just a wood floor with babies rolling around before mommies existed? I just don't get where they came from"
  Stalling to get my thoughts together, "Umm, yeah, well...."
  "Is it hard to explain, mom?"
  "No, it is just that I don't know the answer."
  "Like I thought they might be in the dirt with the worms, but wait, dirt didn't even exist."
  "Baby, that is a great question, but we don't really have the answer to those questions."
  "Maybe somebody else knows... maybe we can look it up on the internet"
  
  Maybe, Google can answer our existential questions... if only it were that easy!

Urgent Care on the Eve of Christmas Eve

My pre-Christmas saga started Thursday morning when I woke up feeling extremely bloated. CF has not been kind to my lungs, but I have been extremely lucky in that it really doesn't effect my digestive system unless I forget my enzymes. So when I woke up super bloated the day of Kaylee's preschool Christmas party (I will do a Christmas post soon) I was less than excited that the one time my digestive system decided to act up was when I had to be out of the home all day.

As the day went on the bloating became more severe and I started to have pain in my lower right side. That evening I started running a low grade fever. I went to bed with a warm rice pack and looked to the worst doctor in history, Doctor Google. Dr Google concluded that I would not be around long enough to see the light of the next day. Figures, that is his answer to everything.

Luckily, the internet doctor was wrong and I did, in fact, wake up the next morning. Unfortunately, the pain seemed worse and started to wrap around to my back. I decided to call my doctors office because I started to suspect this wasn't just bloating pain. I wanted to be seen before the weekend (which just happened to be Christmas weekend) in case things got worse. Unfortunately, everywhere was closed due to it being the Friday before Christmas. So I figured I would call the on-call nurse, which I should know by now, is as bad as Dr. Google. She told me to cease all liquids and food and get to the emergency room as soon as possible since I probably had appendicitis. I tried to inquire about an urgent care instead, but she insisted that I go to the ER because urgent care wouldn't be equipped to handle my appendicitis. So, I did the opposite of her advice and went to urgent care instead (I was pretty positive I did not have appendicitis).

Urgent care doctors know nothing about CF and I was pretty sure they would not diagnosis me correctly, but I did want to confirm I didn't have appendicitis and I was a little concerned I may have a kidney infection (or something similar) that would require antibiotics. The urgent care doc came in with an internet printout of CF digestive issues. She ruled out appendicitis and decided it was either DIOS (a blockage in the intestines which can be a CF problem) or kidney stones. I knew I did not have DIOS (although a partial blockage was a realistic possibility) and I was not convinced of kidney stones. However, I knew I would make it through Christmas and so I went home relieved that whatever was ailing me wouldn't kill me!

The Monday after Christmas I got a phone call from urgent care telling me that my results came back and I had a bladder infection (I assume the bloating may have been a untimely coincidence and had nothing to do with the infection which was causing the pain). I got a prescription for antibiotics and within a few days I felt good as new. Whew, I never realized how fast antibiotics work for non-CF issues. My bladder infection took about 24 hours before my symptoms disappeared which seemed miraculous since I am used to two weeks or longer for my lungs to respond to antibiotics.

4 and a half

Dear Kaylee,
There was a time, not so long ago, when I would lay in bed while rubbing my belly that kept you safe and warm and I would picture being your mother. I could easily picture myself mothering baby Kaylee, but for some reason I could not get my mind to move on past infancy. Rocking, soothing, nursing was something I would imagine daily, but being a mother to a walking, talking, school aged child was beyond my scope at the time. With you still nestled in my belly, it was hard to see so far ahead and in reality, I had no idea what it meant to be a mother at the time. I didn't have the experience to really know what motherhood entailed.

Fast forward 4.5 years later and I have to admit, that each year of mothering I feel my love for you grow (how is that even possible?!) and I love my job as your mother more and more. Now, I can easily picture mothering you all through your school years and even mothering you when you're a mother yourself. Sure, I still have no idea what I am in for when it comes to raising a teenager, but you are no longer this mystery tucked away below my ribcage. You are my daughter and watching you grow is the biggest gift I have ever been given.

I brought you out to dinner last night, just the girls, and when I told you that you were officially 4.5 your eyes grew big, "I am 4.5? I always thought that meant you were a grown up, but I am not even a teenager yet!" You said with some surprise and a lot of enthusiasm. So how can I sum you up as a 4.5 year old? I think the best way to describe you is my mini me. You and I are two peas in a pod! We like to do all the same activities and we absolutely adore spending time together. You share my animated (and dramatic) way of talking, my love for baking, crafting, and being creative. You are sensitive to others (and very sensitive to what you watch on TV/movies. If your anything like me you will have to check ratings for the rest of your life, sorry love) and love to shower the people you care about with love. You take your time making friends and you prefer a few close friends. You are also a home body which you absolutely got from me!

Of course, you are your own person too! You have a love for chocolate that I will never fully understand. You are so inquisitive and smart. You are so observant and take in so much of your surroundings. Your daddy and I worry that this may be the last Christmas you believe in Santa because you are so logical that you already have doubts about a man that can make it all across the world in one night and you don't understand how he can fit in a chimney. You are a touch of a hoarder which makes me cringe! I often find you digging in the recycling bin (and occasionally the garbage) to find things for your art. You find something to bring home everywhere we go to add to your collection of junk that will become art: a receipt from Costco, a leaf, a stick. I on the other hand, despise clutter and have to find times to declutter your art corner for my own sanity!

I know as you get older you will slowly drift away from me as you find yourself and who you want to become. For now, I am basking in the fact that you love spending time with me and you are my buddy wherever we go. Life is such a wild journey, but I know no matter what I am so very blessed to have you as part of my ride!

Love,
Mom

IV Party Tricks

I meant to write this a long time ago, but obviously didn't so here is goes several months later:

I joined a neighborhood book club almost a year ago. At the time I was still in the process of getting to know the ladies in the book club as we only meet once a month and I had only been to a few meetings. When I arrived at the home of a fellow book club member I joined two other ladies at the kitchen table where everyone was chatting and eating appetizers. As we were waiting for the rest of the ladies to arrive we were all talking, but it was clear the hostess could not focus on the conversation and something was distracting her. Her eyes kept darting around the room and she kept standing up as if to go somewhere, but would seem confused and would quickly sit back down. She finally stood up and said, "I am sorry, but I can't figure out what that noise is!" We all fell silent for a second when the other guest confessed she had heard it earlier and thought the noise was just kids playing outside. The hostess seemed to accept this theory and sat down again. As more people arrived the hostess brought up the noise again. I was embarrassed because I could not for the life of me hear this noise she was talking about. I started to wonder if some of my previous IV meds had damaged my hearing. Everyone else, however, could hear this strange noise that some compared to the "sound of a dying battery in a kid's toy" as another chimed in that it sounded like an "electric drill." As the conversation continued I felt more and more uncomfortable. How could I not hear this noise that clearly was distracting to so many others?

Finally, we moved on from the mystery noise and continued on with chatting and snacking. At one point the room fell silent momentarily and as the chatter died down I finally heard the noise that perplexed everyone else. I could feel the blood drain from my face at the exact moment that I realized this unexplained noise was coming from me! I had to sheepishly confess to knowing the source of the mystery noise. The room fell silent and everyone shifted their focus to me. I pulled open my purse and pulled out my CADD pump which made a strange drilling noise each time it dispensed my zosyn which happens about every 90 seconds! I admitted that after hearing my pump go off every few seconds 24 hours a day for 14 days at the point of the party, that my brain had no longer registered the noise in order to keep my sanity.

Needless to say my book club now knows all about my CF and how annoying a CADD pump can be!

Passage Of Time

"Remember when you were in the hospital?" Kaylee says from the backseat of the car.
My grip on the steering wheel tightens and I feel my chest tighten along with it. I take a deep breath, my last hospital stay was a little over a year ago, but the stress and anxiety my absence during the hospital admission caused Kaylee was something that she still brings up from time to time.

"Do you remember how I used to eat all your hospital food?"
I feel relief wash over me. This conversation isn't going to be like so many other conversations she has about the hospital, begging me to promise that I will never go to the hospital again, a promise I can't honestly make.

We share memories about her coming and eating my meals and how I always saved her dessert. We reminisced about how I always ordered an extra dessert so she could have treats when she came to visit. How one time my dinner arrived too late and you had just left when my tray arrived. My floor nurse that day, knowing how much you loved the sorbet, ran down the hall and stopped the elevator door to make sure you got your sorbet before leaving. You mention coloring together in my hospital bed and we bring up all the happy and fun memories we had while I was inpatient.

The car is quiet for a minute when I hear, "I wish daddy would go to the hospital so we could visit him and eat all his food and color." Kaylee is used to daddy working all day so the idea of him being gone felt safer and less scary which is why I am sure she hoped for him to be in the hospital and not me. But the fact that over the last year her memory filtered out the hard parts of the hospital, but retained some of the fun times we had together while I was inpatient, so much so that she wanted to return and relive the good moments made my heart swell.

So often I worry how my disease, especially hospital stays, disrupts Kaylee's happy childhood. I feel an immense amount of guilt over how my life negatively impacts hers. And yet, the very worst part of Cystic Fibrosis to a small child, having it take away your mommy for 8 days, has left her with some happy memories lets me let go of a bit of that mothers guilt that seems to go hand in hand with the disease. I know Cystic Fibrosis will get harder as she gets older and becomes more aware of the long term repercussions of the disease, but for now I am happy to know that together we can make happy memories even in the midst of this hideous disease that is great at leaving destruction and sadness in its wake.

Matching Nightmares

I shuffled to the kitchen, turning on the coffee maker, waiting for my body and mind to catch up to the chatter coming from Kaylee, sitting at the kitchen table. The sun still hadn't made its way to our kitchen window sitting above the sink. A loud yawn enters the kitchen before my sleepy husband makes his way into the room to join us. The coffee grinder fills the cozy room with too much noise for conversation. I wait for the grinding to stop, replaced by the sound of water filtering through the fresh ground beans to say, "I had the worst dream last night." I hand the warm, steaming cup of coffee to my husband, pushing the grind and brew button again.
"What did you dream?" He inquired, sipping his coffee.

I went on to explain the dream I had (some said in code or miming to shield the little ears at the kitchen table). My port was accessed in my dream, but rather than the normal Huber needle and tubing, a tube as thick as my thumb was hanging directly from my port. I went to change the IV bag, but realized when the bag was removed I had nothing to clamp the open ended tubing. In a bizarre turn of events, my port became a vacuum, sucking air from the room into my port. This was a terrifying discovery as too much air pushed (or in this impossible case, sucked) into a port can lead to death. I shouted to my husband to grab some empty syringes and as he quickly unwrapped them I frantically tried to pull air from my port, until I saw it draw back blood, proof the air was removed from the internal tubing of my port. However, every time I removed the needle my port vacuum continued to suck up room air. It was a never ending cycle of pulling the air out of the tube, only to have it suck back in. The dream ends unresolved, as dreams often do.

My husband seemed a little more surprised by my dream than I expected. I have been known to have very vivid and bizarre dreams and this, although not my happiest of dreams, was no more or less bizarre than a pretty typical dream for me.
My husband's eyes grew wide and he told me, "I had almost the same exact dream two nights ago!" His dream differed slightly in that my tubing was cracked which was the cause of air getting into my port, but still the basic theme of a port filling with air and certain death was the exact same.

I guess when you have been married long enough even your nightmares start to match!

Proof Orkambi Works

Several weeks ago I wrote my one year review on Orkambi. I said that I felt it was helping, but did not have the clinical proof to back up the way I felt. My PFTs are unchanged and even though I had a health streak that was unheard of for me following Orkambi, I fell into a typical health slump around the nine month mark. Although Orkambi didn't feel life altering, I also did not feel any worse so I continued to take Orkambi with hope of slowing the decline of the progressive nature of Cystic Fibrosis. Well, I now officially have proof that Orkambi is working for me!

I want to preface this post by saying CF has taught me to be cynical and I am not a blind believer in anything. So many times I have believed that certain procedures or medicine would benefit me only to be left disappointed. Antibiotics that my cultures are sensitive to (meaning they should knock down an infection) have failed me, I have left hospital stays with numbers just as horrible as I entered with, I have gotten worse with no rhyme or reason and I don't fully trust anything medically based until I try it and see it working for me. The medical field isn't fool proof and CF doesn't seem to follow any rules.

To explain my proof I need to back up a few years (this will be short, I promise). In 2014 I was going through an exhausting and frustrating health slump. My doctors, perplexed by my inability to get better despite everyone's best efforts, ordered a CT scan. The CT scan didn't show anything overly telling despite mucus plugging, scar tissue, bronchiectasis, and other typical CF problems in someone with more advanced lung disease. I continued the roller coaster of a CF slump for a few months and eventually stabilized.

Fast forward to summer 2016 (now post Orkambi) and I was experiencing another CF slump. After IVs and months of oral antibiotics we weren't getting anywhere. My doctors were once again stumped, this seems to be a pattern with me, we decided a CT may be needed to see exactly what was going on. I didn't anticipate it to show much and didn't even bother to call for results knowing if something horrible showed up (say a collapsed lung or pneumonia) my doctors would notify me. Honestly, I didn't want to hear about how much more my lungs progressed because there is nothing more depressing than proof your lungs are dying.

When I did get the results they shocked me! Remember, this is a CT scan taken while doctors couldn't get my lungs to stabilize so clearly, I was not in great health. This CT was a comparison to a 2014 CT scan and CF is progressive in nature meaning things usually just get worse and worse. My thirties have been extraordinarily hard on me and good news in regards to my CF just doesn't happen anymore.

When my results were finally sent to me I skimmed through the write up, holding my breath and feeling that uneasiness that comes with impending disappointment. And yet words like stable, significant improvement, markedly improved jumped off that page at me. Wait, what?! There were improvements in my CT over that past two years? And these improvements are showing up despite how sick I was when the CT was taken? My progression of bronchiectasis has stabilized (it is impossible for bronchiectasis to improve so stability is the best case scenario). Nodules in my lungs were shrinking? There was significant interval improvements in my right lung (which has always been my problem lung)?

I had to read through everything several times before it sunk in. My CT scan did not get worse over the past two years and in fact it actually got better! Although I have not seen the miracle results many people have seen on Orkambi including increased lung function, increased energy, decreased exacerbations, I have seen an improvement in my CT scan. When your disease is progressing at a dizzying rate seeing any improvement can feel nothing short of miraculous.

Yes, I wish I had seen an improvement in my lung function especially since I sit so very close to transplant now. Yes, I wish I had increased energy to make each day count just a little bit more. Absolutely, I wish these infuriating slumps in my health that seem to be nonresponsive to antibiotics would disappear forever. However, at this stage in the game I will take any improvement I can get and even if those improvements are only noticeable through an improved CT scan then I can't complain. Without Orkambi I am positive that CT scan would have continued to look worse. Honestly, I don't even want to think about what my life would look like today without Orkambi.