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.

Unusual Therapy

The other day my husband, Kaylee and I found ourselves wandering around an arcade. Kaylee was having fun playing all the arcade games geared towards little kids, you know the bowling, wack a mole, carousel, etc. All of the sudden she stopped dead in her tracks, pointed her little finger and shouted with the gusto only little kids seem to posses, "I want to play THAT one!"

The game that caught her eye had three shelves with pins (that looked like clowns) and a reservoir of balls. My husband and I knew this game was way out of her league. First off, she couldn't even reach the reservoir of balls let alone throw them the distance that required to knock down pins. But life is short and really besides 50 cents we didn't have anything to lose so we agreed. My husband held her so she was able to see what she was doing and I passed her the balls she couldn't reach herself. With all her might she chucked those balls at each of those pins and when the buzzer sounded to let us know that the game had finished she had hit a total of zero pins.

Kaylee, realizing that maybe she was being overly ambitious, decided that once was enough. My husband on the other hand wanted his chance. And before long a friendly competitive game of "who can score higher" broke out between the two of us. (Not that it matters, but for the record I totally won). However, we kept falling short of the coveted high score and accompanying jackpot. And just like you teach little kids when trying to solve a problem, we decided teamwork was our best strategy. Within two more attempts the machines lights and buzzers were going crazy, we won the jackpot!

At this point Kaylee, who wasn't really all that impressed with out success, was ready to move on. I waited while the machine kept spitting out tickets while my husband and Kaylee went on to other games. After a moment, I could hear my husband laughing. I looked up to find he was laughing at me, "All that money on a vest and a 50 cent game works so much better." I realized that I was coughing...a lot! Who would have thought that an arcade game could be such amazing airway clearance!

Cool Days and Morning Laps

"I am just a head!" my daughter can be heard shouting, her entire torso and limbs submerged, when the bath has turned lukewarm and the chilly air in the bathroom is too cold for comfort. This is my cue to grab the towel off the towel rack, hover it just inches from the bathwater and wrap her the second she emerges from the chilly bath. She is then whisked into the living room where I wrap her in a blanket so she can warm up. Oddly, this routine is the same whether it is the dead of winter or a scorching 100 degree day.

Lately, when I finish my morning laps, I feel like shouting, "I am just a head" because I too find the idea of letting even an inch of my wet body be exposed to the morning air chill inducing. The warmth from heated pool can feel like a safe cocoon from the cold morning air. I find myself in a daily battle between pulling my body from the warm water to briskly walk the 20 ft to the changing room and doing just a few more laps just to stay warm for a few moments longer. And for those lazy days? I fight with myself trying to convince myself to stop huddling in the corner of the pool staring at the clock wondering how late I can push my shower.

How many more months until spring?

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*

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!

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.

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.

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.

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!

Preschool

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!

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 naïve 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.

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.

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!

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

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

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

July 18^th: 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 25^th: 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 1^st: 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 3^rd: Had an allergic reaction to Zosyn and woke up covered in hives.

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

August 27^th: 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.

 

Missing Butterflies

I have been posting so many boring health updates so I wanted to share a cute story for a change.

My four year old hates when I get IVs. She calls my accessed port "the tube" and there seems to be nothing she hates more than when I get "the tube." The last few times I needed IVs I was in the hospital so she equates IVs with mommy leaving.



Here is a picture of the butterfly after we de-accessed.
No matter how many times I took this photo
it came out blurry. Sorry!  

My doctors agreed I could do home IVs this round of IVs (before we knew how disastrous it would all turn out) and I was hoping that would change Kaylee's feelings about "the tube." When my port is accessed the safety on the needle (the part that helps slip the needle into a sleeve when it is pulled from the port so that nurses don't stab themselves with my used needle) resembles a butterfly. So I tried to rename "the tube" to "my butterfly" to take away some of the fear she has put around my IVs.

Unfortunately, she still hated my butterfly and asked me daily when it would go away. After 21 days my husband pulled off my dressing and Kaylee watched as he pulled my port needle out of my port. We all cheered because it signaled IVs were done (even though it was bittersweet for me because I didn't feel any better). As I turned to look at Kaylee I saw her eyes full of tears, "Can you just wipe my blubbly eyes, mommy" (blubbly is what she calls her eyes when they are full of tears. It makes no sense, but she has been doing it since she was really little so it has become a real word in our household). My heart sank. I thought she would be overjoyed that my IVs were officially done and yet her eyes were full of tears.

"Can I ask why you are crying?"
The tears started flowing the second I said those words.
"I am going to miss your butterfly!"

I guess the end of IVs was bittersweet for Kaylee too. Sure, the dreaded tube was gone which meant fewer doctor visits, less restricted play with mommy, and no crazy IV schedules, but it also meant her beloved butterfly would be gone.

Goodbye Spots, Hello Fevers

*warning: TMI info about sinus issues below*

After 8 days my hives officially disappeared! In true CF fashion I guess I picked up a virus because I started getting tons of sinus drainage, a very congested wet cough, and the feeling of drowning came back.  The constant Benadryl may have had something to with the sinus issues because it can dry out secretions. I had almost constant hard sinus crud " draining" (more like tumbling in slow motion and frequently getting stuck) down the back of my sinuses. It was bad! The kind you can feel wedged in your sinus and can smell the bacteria before it dislodged (tmi, I know).

And then the fevers started. I already had meds at home that I was supposed to start right after IVs because Zosyn didn't work. However, I had to wait for my allergic reaction to Zosyn to wear off before starting new meds. I am so relieved that the hives are finally gone so that I can start taking my new med combo. I am so hoping that these meds work because we are approaching two months of dealing with this exacerbation that just doesn't want to leave. I feel like I lost the bulk if my summer to being sick or trying to recover. Our family loves summer because we love hiking and going to local lakes and rivers, but this year my health has kept us home and I can feel summer already slipping into fall. Mainly, I am just ready to have a clinic appointment that actually goes well (or at least decent) especially as we approach the winter season.

Olympics

The summer Olympics arrive during a season when I am usually too busy enjoying life to be glued to the television. This year, however, I was in a middle of a bad few months due to CF and found the summer Olympics a nice distraction for both myself and for Kay. It eased a bit of the mom guilt that surrounds not feeling well and being unable to go out and be as active as my four year old would probably prefer. I convinced Kaylee (and almost convinced myself) that watching the Olympics was more exciting than riding bikes because it only happens once every four years!

 I haven’t actually watched the Olympics at all during my adulthood. Most of my Olympic memories are from childhood and even then I am not sure how much of them I actually watched outside of gymnastics. My fondest memory around the Olympics was the few weeks I spent watching gymnastics and then pretending to emulate them in my own, completely void of any skill, childlike way.

And as parenthood usually allows, I was able to relive that magic by watching my daughter pretend, in the same unskilled childlike way, to be an Olympian herself. I turned a blind eye to the wild spinning tumbles she took off the couch with each Olympic diver and the crazy obstacle courses she set up across the living room to run alongside the hurdlers.  I realized once again how she really is my clone when we both witnessed the false start of a French hurdler who was immediately disqualified. Kaylee continued to bring up the “man that started before they said go” with great concern throughout the day and what I didn’t tell her, but she must have known, my heart felt heavy that day for him as well. Her bleeding heart she inherited from me.

 The Olympics provided a nice ending to a summer that did not allow me to do the typical summer activities. I hope the next summer Olympics 2020 finds the same not so little girl tumbling around the living room next to her hopefully healthy(ish) mama watching by her side.

Spots

I am trying to catch up on updating everyone on my summer health saga, but a lot has happened so I am back tracking a little. This is an update from about a week ago, right when I finished three weeks on Zosyn after two weeks of Levaquin.

During this time I started to improve a little and was feeling okay. I was not back to baseline, but decent. And then the day of clinic I woke up feeling so exhausted that I felt like I was sleep walking my way over to clinic. The second my doctor walked in the room he took one look at me and said, “You look tired.” My PFTs weren’t exactly what you would expect after 3 weeks of IVs following 2 weeks of orals. I was up 3% from my last appointment, but still 5% below my “minimum” baseline and 9% from my ideal baseline. We made a new and somewhat unusual plan, but more on that later.

The next day I was so excited to get my port de-accessed. After 24 hour infusions for 3 weeks I was so excited to get rid of the pump that had followed me around all day, and was my snuggle buddy at night. I had an itchy spot on my arm that morning, but didn’t think much of it. This seems like a random detail, but I am sure some of you know why this comes into play later. I pulled the needle a few hours early (naughty, naughty) and spent the rest of the day out with friends.

The next morning I woke up to find my entire torso, arms and legs covered in hives. Naturally, I was concerned, but I had a non CF related procedure that morning and was reassured by many people that my hives may be related to nerves. I popped Benadryl and went about my day hoping the hives would fade as time went on.

The next morning I woke up only to realize my eyes wouldn’t fully open. The hives had spread to my face and my eyelids were swollen shut! At that point I knew what I had suspected all along, it was an allergy to the medication I had already finished. I called my doc, who agreed that my symptoms (fevers and hives) were likely an allergy to the penicillin antibiotic, Zosyn. It seems strange to have an allergy after completing an antibiotic because often allergies to antibiotics occur within hours of the first dose. However, allergies to antibiotics can happen at any time and do occur after the completion of the drug at times, like in my case.  

 

So after 5 weeks of antibitotics including 504 hours of continuous infusions my lungs feel no different (if not slightly worse bcause of the round the clock use of Benadryl to keep my hives under control) and I have spent the last several days covered head to toe in hives with fevers.

Hotel ER

As a person with CF I avoid the emergency room at all cost. I don’t want the germ exposure and maybe even more importantly I don’t really trust the emergency room staff with my CF. I have found most people with chronic and complex diseases are weary of general doctors because of the complexity of our conditions that are not always understood by those in the medical field that are not specialized in our disease. A perfect example of this is the time I went to urgent care because I thought I had a kidney stone. I did not have a kidney stone, but they wanted to find the source of the extreme pain I was in and decided to do an x-ray of my lungs just to be sure. The doctor came in after a very long wait (especially for a very slow urgent care) and admitted he had never seen an x-ray of lungs with Cystic Fibrosis so he wasn’t exactly sure what looked normal for my already severely damaged lungs. I appreciated his honesty, but it highlighted exactly why I do not bother with non CF specialists.

As much as I avoid the ER, unfortunately is isn’t always avoidable. The other day I woke up in the morning and my arm had the sensation of being asleep (pins and needles). I assumed I slept wrong so I shook my arm to try to wake it as I got out of bed. After getting breakfast ready I realized that my arm was still tingling from my elbow to my fingertips. The pins and needles feeling seemed to come and go as the day went on. Like all medical problems as the evening went on the tingling got more severe. Around 10pm a blue vein started to bulge from my upper arm and the veins below the bulge were all much more pronounced than my other arm. I have a port which puts me at higher risk of blood clots. This knowledge in combination to losing two friends with CF to blood clots I decided to call the on call doc just to get his opinion.

The doctor confirmed that these symptoms may be due to a blood clot and I should go to the emergency room right away. I had to call my husband who was at work. He had to quickly find someone to cover his shift while I called my sleeping dad to come and stay with our daughter. I didn’t get to the ER until almost midnight. I had a quick assessment by a nurse at intake who said he could see swelling in my arm, although I wasn’t sure I agreed.

I knew going in that the wait would be long. However because the doctor was adamant that I needed to get to the ER right away and the fact that he said he “would let them know I was coming” I assumed the wait wouldn’t be obscenely long. Oh, how I was wrong. The hours ticked by and we weren’t actually seen until 5am which made me wish I had just waited until morning. Clearly the ER staff wasn’t worried about the clot breaking loose and wreaking havoc in my body and I wish the on call doc hadn’t made such a fuss. When they finally called me back they threw me in a bed in the hall where an ER doc told me that the ultrasound tech would not be available until morning and so there was nothing they could do at the moment. I was so irritated that I spent the entire night awake in the ER when there was absolutely nothing they could do for me. However, at this point they told me to stay because I was only a few hours away from morning. I sent my husband home to relieve my dad, especially since my daughter would be waking soon. And eventually I was taken out of the hall and put behind a curtain where I tried to sleep.

A few hours later, 7:30 am, I woken for my ultrasound which turned out to show absolutely no clot. I was not given a theory as to why my arm was numb and bulging and was released. I was so exhausted at that point I wasn’t able to think clearly and was just so grateful to get home. So I basically sat in the ER for 12 hours only to find out there was absolutely nothing wrong with me! I am eternally grateful that I did not have a clot which meant I got out of the hospital faster, did not need blood thinners, and was absolutely fine. The results could have been much worse leading to more worry and treatment. I do not want to sound ungrateful because I really am. However, I was a little annoyed that I( spent 12 hours through the night in the emergency room when there was absolutely nothing wrong with me! I had numbing for a few more days and as quickly as it started, it stopped and I will probably never know exactly what happened.

My CF Summer

This past month and a half has been a blur of chaos and I feel mentally fatigued from all the craziness CF brought into my life. It feels so strange to think just a few weeks ago I was waking every morning and swimming, spending my days in the garden and with my daughter, my house was clean, and I was using produce to make homemade spaghetti and pizza sauce. And today, my life revolves around sleeping, treatments, and IVs and feeling sick. It is amazing how quickly life changes week to week with CF. There are times that life feels exactly as I picture my life should look and despite the pills, treatments, and extra doctor appointments I sometimes forget that my life isn’t “normal.” And then there are times that life feels so uncertain and scary and even the most simple of tasks seem completely unattainable. These past few weeks my health has deteriorated. My low pfts from last month seemed high in comparison to my numbers after two weeks of Levaquin and a week of IV Zosyn, where they sat at a mere 28%. Two weeks ago I spent 12 hours in an emergency room which left me completely exhausted an overwhelmed. Then my contact dermatitis from all port dressings which has been a chronic struggle flared up in a matter of 24 hours to a point that I wasn’t sure I could continue IVs. And then the blows kept coming.  

I miss the days that CF exacerbations were “simple.” Where I would feel sicker than normal, call the doc, start IVs or orals, continue life as normal, complete IVs and feel 100% myself. It is amazing to think that all through my 20s tune ups hardly changed my normal routine. I would’t even take time off work or school when I had IVs and somehow I would bounce back to baseline without much extra effort. I know that I am incredibly lucky to have been so very healthy for so long. I know so many people with CF would think getting through your 20s with such ease seems almost too good to be true. I am grateful every day I had such a long easy run with CF. And yet, I can’t help but feel sadness and frustration at how utterly impossible CF tune ups have been the past few years. How my lungs just don’t respond the way they used to and despite more aggressive therapy, exercise, and medications my lungs just never seem to cooperate. I guess no matter how old you are nobody can ever be prepared for the intense progression of CF.

Shame

This past May I attended our annual Great Strides event. It was the best event we have had to date, but something happened while I was there that I haven't really spoken about to anyone outside of my husband. It was one of those experiences that seems to shake you to the core and as much as you try to brush it off, you just can't fully let it go.

After the walk there was a carnival area set up for kids to play games while they waited for the lunch to arrive for all the walkers. I wandered over with Kaylee to one of the carnival games, the kind with the big wheel that spins until it lands on a prize which is one of my daughter's favorite games, and waited in line. While waiting a young girl around my own daughter's age, maybe 3 or 4, wearing a purple "fighter" shirt, which told me she too had Cystic Fibrosis, wandered through our line to get to another activity. She was young, distracted by all the excitement and was walking with her neck craned to the side watching kids across the grass cheering at their victory.

Suddenly, I was startled by a woman sharp voice screaming someone's name. The voice was urgent, panicked, and much louder than seemed necessary given the small area we were in. My eyes darted around looking for who could possibly be in danger. Everyone seemed safe and everyone my eyes met seemed to be having fun. I could not figure out what was causing this woman to panic. I looked back at the woman to see where her eyes were staring only to realize the panic, the shouting, the big scene was directed towards the little girl only now becoming aware of her mothers shrieks. As I looked back at the mother I saw her pointing a finger shouting, "SEE!?? LOOK!?! DO YOU SEE THAT LADY?? DO YOU SEE HER SHIRT?? GET AWAY FROM HER NOW!!!"

My heart caught in my throat as I realized the accusing finger was pointing at..me! She was screaming and shrieking and in a complete panic because of me?? I was the danger that this women felt was worth creating such a big scene that people stopped what they were doing to see what had happened? She quickly grabbed her daughter and loudly scolded her, "Do you see that lady's shirt? You need to stay away from people wearing those shirts! It isn't safe to be near people like her!"

I wasn't safe to be around? My eyes immediately went to my own daughter. How would she feel hearing someone scream that a young girl her own age would not be safe near her mother? Relief filled my entire body to realize my daughter was busy looking at a woman who was making balloon animals and had somehow missed the scene. Once I knew my daughter was unscathed from this woman's fit over my proximity to her daughter, I suddenly felt shame fill my body. I had no real reason to feel shame, I had done nothing wrong except to be born with a genetic defect (which meant my lungs may harbor bacteria that her daughter could catch and get sick from), the same as this woman's daughter and yet her screaming and deeming me unsafe around her child made me feel as if I were a monster.  I wasn't coughing and in fact, having seen her sweet girl I had turned slightly away from her as a way to subtly protect both of us. Somehow the interaction brought back all the feeling of shame I carried as a newly diagnosed teenager for having this broken body and being so very different from everyone else. I knew in my mind I should not take this women's overreaction and her accusing words personally and yet I have never had a complete stranger publicly point me out of a crowd and shout that I was unsafe around a child! Those are not words that are easily forgotten.

I also felt heartbroken for the small child who is still completely naïve to the idea of bacteria and cross infection. She was taught to be afraid of people just like her and in such a irrational way that I wondered how that effected the way she felt about her own disease. Rather than feel inspiration or understanding in people just like her, she was told she have a deep sense of fear and doom in our presence.

Finally, I felt sadness for the mother, so consumed by fear that she had to not only make her daughter feel bad, but to shame a person who knew exactly what her daughter dealt with every single day. As a mother, I understood the need to keep your child safe, but it was clear her fear of cross infection was beyond rational. We were outside, neither of us was coughing, neither of us was even talking, I was turned slightly away, and we were several feet from one another. I hate that any mother feels that much fear for their child's wellbeing, rational or not.

It was all a reminder how isolating this disease is and that even at an event meant to support, encourage, and build up those that suffer from CF we are still so very alone because we don't have the freedom to sit down with a child and commiserate with how boring treatments are, or hug a person with an accessed port and say, "I get it," or pat the back of someone doing the walk with an oxygen tank and tell them that they inspire us. That in a group of people that desperately care about the CF community we can be made to feel like a monster for simply existing.

Accountability

Most mornings I start my day at 5:20am. I crawl out of bed, pull on a bathing suit, puff my bronchodilator, grab a glass of milk and drive to the gym. I come home, showered, dressed, and having my workout done to a sleeping household. I quickly start my treatments so I don't waste any precious time before my daughter wakes up. She comes wandering into the living room sometime around 7-7:30am. By this point, I am sipping my morning coffee and somewhat impatiently waiting for her to wake up. My favorite part of this routine is that when Kaylee wakes up I am all hers. I can make her a healthy breakfast and sit and chat about our plans for the day. She gets a mommy that is totally present.

While I was feverish and sick I took a week off from the gym. My body was exhausted and I was able to sleep an extra 2 hours everyday which I felt I desperately needed. However, this meant she wandered into my room to wake me up when she was ready to start her day. I was groggy and tired and had no desire to get out of bed while she was bouncing with morning energy that only small children seem to possess. I threw fruit at her and snapped on the TV so I could start my 45 minutes of treatments. I was interrupted because she was still hungry, needed water, was bored watching TV, etc. She would try to tell me something, but I would be coughing so violently I couldn't respond which left her repeating herself over and over. In turn, it made me frustrated that she kept repeating things while I was coughing so hard I couldn't breathe and she got frustrated that I wasn't responding. My 45 minute treatments turned into an hour because of all the times I had to stop. By 8:30 I still wasn't showered, neither of us had breakfast and we were both a little grouchy. One morning after I strapped on my vest to start my treatments Kaylee asked me teary eyed, "Mommy, when are you going to do shaky (vest) in the middle of the night again? I like when you do that better."

Those two sentences were all the motivation and reassurance that I needed to know that my workout routine was something I needed to keep up for a very long time. It is better for my health and my lungs. It is better for me because I can do my treatments uninterrupted and in peace (the way I like it), and just in case that wasn't enough motivation, it made may daughter's morning experience so much better. Trying to fit in treatments and an exercise routine while parenting young children can be a challenge at best, but this time having a small child request that I workout again with tears in her eyes (although for selfish reasons on her part) was the very best motivation I could have asked for.

Monday Surprises

Two weeks ago I had my CF clinic. I felt pretty confident going in because I had been swimming 5 days a week for 9 weeks at that point. I felt good, energetic, and was still compliant with my treatments. I was excited to tell my doctor I was swimming and because summer is usually my healthy season I was more than ready for a quick and easy clinic visit.

Soon after arrival the RT brought in the pft machine and I did my first blow. My eyes scanned the computer screen waiting for the results. The numbers that flashed on the screen shocked me. My numbers were lower than I had seen in a very long time. I sorta laughed telling my RT that I must have made a mistake because those numbers were ridiculously sad. However, there was a sinking feeling in my heart because I have never actually messed up a pft, after doing them for so many years it is a hard thing to do wrong. Just as I had secretly expected, but hoped to no end was not true, the next two blows were consistently low. I was left dumbfounded. Everyone seemed a little baffled that I was working out, felt great, and had no clue that my numbers had plummeted. My doctor ended up deciding to put me on oral because of a unusual (but not concerning) new bacteria in my culture. I agreed, happy to do something, anything to improve my horrible numbers, but was utterly confused as to how I could have an infection and feel... well perfectly normal.

Fast forward three few days, I was waiting for my meds after a problem with my first pharmacy, when I started to feel the telltale signs of an infection: fevers. The next several days my fevers flared, despite my lungs feeling okay. If it weren't for my low pfts I would have easily blamed the fevers on a different infection because my lungs actually felt fine, but I kept reassuring myself that my low numbers confirm that my lungs were the ones wreaking havoc on my body.

And then one week after my clinic appointment, an entire week after my low numbers I sat up in bed after a good night sleep to start my day only to feel the weight of my lungs, brimming with infection causing them to feel monstrously heavy. So heavy in fact, that I felt they may just fall from my body to the mattress below. My husband saw my grimace, heard the sound that puffed from my chest, and watched my body move in the way it does when your lungs are completely infested with millions of bacteria that are quickly taking over and said, "there it is...". He was right...there is was. The infection had finally reared it's ugly head a week after it tried to warn me.

Four Years Old

Little Kaylee,
You are four years old today. My first real memories are from when I was four and for some reason I have always held being 4 near and dear to my heart. Because all of my four year old memories seem so carefree and so full of joy I feel so excited for you to embark on the next year. As excited as I am for you to experience being four years old I am finding it hard to sum up who you are as a three year old in one little blog post!

You have decided that you are wise beyond your years and that unfortunately my 32 years on this Earth did not provide me with the same depth of knowledge you have obtained in your short 4 years. You ask a lot of questions about the world, but you often disagree with my answers. After disagreeing with me you are sure to back up your facts that are pointless to dispute.

Kaylee: Are there blue carrots?
Me: No. There are yellow, red, orange, purple, and white carrots, but there are no blue carrots.
Kaylee: Yes, there are! When I was living in China, blue carrots were everyone's favorite. You just don't have them here.

You can be so delusional that you once argued that daddy didn't know his own mother's name. You claimed that "back when you were a teenager, your abula told you here real name and daddy was wrong.

Clearly, your confidence is not lacking.

You still love anything creative. You draw, color, create for large portions of the day. A few months ago you found my adult coloring book and now it is officially yours. Another creative outlet you seem to enjoy is helping me cook or bake. Whenever I enter the kitchen you are quick to grab your little chair and pull up next to me at the counter. For the first time, your assistance in the kitchen is actually helpful. For breakfast, you know how to get the eggs out of the fridge, crack them, whisk them and then put the bread in the toaster all by yourself. Sure, sometimes our eggs have a little boost of calcium and some crunch for texture from those eggshells that found their way into the pan. Baking still makes a giant mess as your aim with flour and sugar seems to be a little off. You do know how to level off the measuring cups which always makes me smile! You are becoming a Foodie like your father and like to talk about "textures" (which you use to mean flavors) whenever we make something new. "This has a lemony texture and is a little bit sweet"

You have decided, once again, that sleep is pointless. You often assure me as I tuck you into bed that you will not sleep under any circumstances. Many mornings you insist that you were laying in bed the entire night wide awake. The few times I try to tell you I checked on you and you looked as if you were sleeping you assure me that you were in fact not sleeping, but rather just staying very still. Although you apparently haven't slept in months you do find your way to our bed in the mornings. Most weekday mornings I am at the gym, but I come home to find you snuggled up to your daddy sound asleep... err laying very still. There is nothing more precious than seeing the two people I love most in this world snuggled together.

As you get older I find it harder and harder to sum up your likes and dislikes, your personality, and your current stages in one little post. You are affectionate and love to snuggle, you are in tune to other's emotions especially mine. As you get older our bond changes, but it seems to grow stronger with time. You talk all day long and surprise me with the vocabulary you pick up. You have come out of your shell so much the past few months. You are no longer shy or unsure around people you don't know. You are gaining confidence in your Spanish as well.  You are stubborn and independent. You are beautiful inside and out.

I love you in a way that I could never put into words! Happy birthday,
Mom

7 Weeks

7 weeks ago my head was foggy, my eyes blurred as my alarm rang out in the darkness that accompanied 5:20 in the morning. 7 weeks ago dragging my sleepy body out of bed, in what felt like the middle of the night, felt like torture at best. 7 weeks ago I left my house into the dark and frigid night air. 7 weeks ago I pulled over to admire the moon laying full and heavy in the sky. 7 weeks ago my teeth chattered as I walked the long outdoor hallway to the pool all while trying to convince myself not to just turn around and go home. 7 weeks ago I struggled while I swam my first few laps since last fall, huffing and puffing and sputtering. 7 weeks ago I lived for the weekend when the gym wouldn’t see my face.

A lot can change in 7 weeks.

 

7 weeks later, my body is already waking before my alarm rings out. 7 weeks later by 5:20 the sun has already made an appearance and the birds are cheerfully singing. 7 weeks later the walk to the pool, still cold, no longer makes me shiver right to the bone. 7 weeks later, 40 laps comes relatively easy, my arms and legs accustomed to the motions required for front crawl and breast stroke. 7 weeks later my lungs have adjusted and my oxygen levels stay 90 or above (most of the time). 7 weeks later, my arms show clear signs of definition and toning. 7 weeks later my hair is dry as straw despite wearing a swim camp. 7 weeks later I weigh 3lbs more than when I started, which I am convinced is muscle mass. 7 weeks later my body craves my morning swim. 7 weeks later I dread the weekend when I don’t swim because my cough will be more prevalent all day long. 7 weeks later I am proud of myself for fitting this missing puzzle piece back into my self care plan.

Toddler Talk Part 10

Kaylee loves rhyming. Sometimes she rhymes real words and other times made up words or a combination of the two. In the morning she sniffed my coffee and quickly pulled away saying, "Yuck-o fuck-o!" (it took everything for my husband not to laugh).

I was telling Kaylee how much I loved that her eyes get lighter blue as they approach her pupils. She gazed into my eyes and said, "I love how the white of your eyes have red cracks in them"

Kaylee and I were getting the mail when we noticed a neighbor left their keys in the keyhole of their box. So Kaylee and I walked over to their house to return the keys. As we were leaving Kaylee turned to me and said, "You are a wonderful mommy to those people."

A few days ago I was in a really bad mood. I wasn't even sure why I was so grumpy, but I could not shake my foul mood. I told Kaylee that I was sorry I was so grumpy that evening, but that I was sure I would wake up in the morning in a much better mood. She reassured me by saying, "Even when you are grumpy, you are still the best mom ever."

I have at least a dozen nick names I give to Kaylee, often they are names I just make up on a whim. The other day my husband and I were snuggling on the couch and Kaylee started running at us full blast while shouting, "Here comes love muffin!"

Three and three quarters

I am a little late on this update, but you have hit two huge milestones (?) since you were three and a half and thought it would be appropriate to have it in writing somewhere.

You officially have your first real crush. You have been going to story time at our local library for years, but about a year ago the story time teacher left and a new young male, Mr. Nate, took her place. For about a year you would participate in class, but whenever Mr Nate came your way you would clam up and refuse to look at him directly. This was especially true during arts and crafts immediately following story time. You would flat out ignore his presence if he came over to look at your art or try to make any conversation with you. You still wanted to attend every week so I brushed off your behavior as being shy.

The past few months however, you seem to have grown more than a little fond of Mr. Nate. When we visit the library for any reason you demand on knowing his where abouts. A few weeks ago we went to "art hour" at the library where Mr. Nate often helps out and you spent most of the hour keeping tabs on your beloved, "Mr. Nate is going in the back room, Mr. Nate is talking to that boy, WHERE is Mr Nate??" This was a turning point and after this day you started actually acknowledging Mr Nate to his face. Eventually you built up to initiating conversation and will even give him a high five.

You recently decided you wanted mommy and daddy to go out of town so Mr. Nate could babysit and you were confident that after spending the day with you he would love you too much and would have to move in. The love affair has grown and the other night when I went to kiss you goodnight you were pretending to sleep. With your eyes sealed shut you whispered, "Shh, I am with Mr Nate." You may be delusional when it comes to love, but at least you think of yourself as a great catch!

The other huge milestone was one mommy wasn't totally ready for. Last week you went to your parent and child Spanish class (after taking several months off) and during class you were sitting still, watching the teacher, answering all her questions in clear Spanish. It was clear all the content she was covering you already understood and that colors, numbers, and the alphabet in Spanish was just too easy for you. It wasn't just that the content was easy, but you seemed like a real student rather than a little kid in a toddler class. You were just so much older than you were just a few months ago. At the end of class your teacher said she thought you were ready to join the 4-6 year old class. The big difference between the 4-6 class was that you would be attending alone, as in without me! I agreed with the teacher and said it was up to you. I explained that you would be in class with other kids and that I would be waiting outside for you. Your teacher even said you could try the 4-6 year old class that day (as in 15 minutes from when she told me about the level change). You were so excited you could hardly stand it and eagerly agreed.

I was excited and proud and felt like I wanted to cry all at once. This was your first time at "school" and I was not completely mentally prepared (you are starting preschool in the fall) for this major step. Sure, we had been apart from one another. You have had many sleepovers with your grandparents and my last hospital stay was 8 days. This hour and a half was nothing in comparison. However, it still felt monumental in your growth and development. You were so ready to take this next step in a school setting and I was overwhelmingly proud of your confidence in yourself. I was also feeling a slight loss of the baby that used to need me so much while navigating through this world. Needless to say, you absolutely loved class and you ask daily if it is the day you go to Spanish class.

Guest Blogger- Sara

I am thrilled introduce to everyone to another CF mommy! Sara is 35 years old and a mother to Wiley and Gunnar, ages 8 and 6. Sara, her husband Bill and kids are an outdoorsy family (just like mine!!) and they enjoy hiking, skiing, and going to the ocean! She is a fellow double delta F508 with a baseline of 75%. She stays healthy by doing the Vest and hypersonic saline 4x a day as well as running up to 4 times a week.

Everyone that has CF seems to have a very different experience. Share a little bit about your CF story or how CF effects you.

When I was born in 1980, I had a meconium ileus. I was being prepped for surgery when I passed it on my own, luckily! The doctors suspected I had CF. But didn't test until I was a month old. With a positive sweat test at that point, my parents were told that my health was in their hands. They began doing manual CPT twice a day and giving me enzymes. I was followed at a CF center that was 3.5 hours from my home, so that I could receive the best care possible. My FEV1 remained well above 100% until after I graduated college at 23 years. CF was more of a nuisance, requiring CPT, nebulizers, pills, visits to the doctor, and occasional 2 week stays at the hospital.  I was always full of energy and never felt sick. My parents and doctors were very proactive and aggressive with treatments. Unfortunately, during the transitional years where my care was turned over to me, it became  reactive and lazy. And my lungs paid the price. Although I had played  division III lacrosse all through college, I did not do much else to maintain my lung health during those 4 years or the 2years after. , and as a result I had my first case of pneumonia upon graduation and in the months after that I lost a lot of lung function. CF had little impact on my health or life until that time. I spent minimal time doing treatments and only thought about CF on the 2 days a year that I went to clinic. Now it seems I think about it everyday, and I spend hours doing treatments and trying to stay ahead of the germs my two sons are constantly bringing home to me. Although I have maintained my lung function at 75% for the past 10 years, I have had to work harder everyday for that 75% than I ever did for my 120%.

 
Deciding to have children when you have CF is not an easy decision. How did you and your husband come to the decision that having children was right for you?
 

We didn't really give it much thought. I always wanted to have kids, and I was going to do that. My husband had a genetic test done, and he was not a CF carrier, so we went for it.

 

How did CF play a role in your pregnancies with your sons? Were both pregnancies similar or were they different?

My first pregnancy had a rough start. I had a miscarriage just 2 weeks before I conceived my first son. When I was first pregnant I didn't know it. I suspected I might be, but didn't want to get my hopes up. I wasn't going to test until after I had gone past the point where  I miscarried my previous  pregnancy. However, I caught a nasty virus that had me coughing so hard I was vomiting, and delirious with a fever. I ended up in the hospital for an urgent clean out. At the time of admission, they did a pregnancy test and confirmed I was pregnant. I had lost a good amount of weight from the virus, so I had to play catch up all through the pregnancy, but I managed to gain about 40 lbs by the end. I did not need IVs again for 7 years.

Life is harder with CF than without. It was a drag  going to so many extra doctor’s visits. And the tone of the doctors detracted from the joy of being pregnant, particularly in my first pregnancy when I was a first time mom and was unsure of what it would be like. Rather than reassure me, they fed into my insecurities. The second time around, after having a successful  and uneventful first pregnancy, I had confidence and enjoyed the experience 100% more. Both of my babies were full term, big (8lb1oz, 8lb 2oz) and healthy, and I gained weight easily with both. The best part about my pregnancies was that my lung function actually improved throughout them. In the 8^th month of my second pregnancy I hit an FEV1 of 88% !

While CF did not have much impact on my pregnancies, I would say pregnancy had a huge impact on my CF, in a good way. I think the combination of wanting my babies to be as healthy as possible as well as sharing life with a healthy being in my body are the two things that helped me gain health throughout both pregnancies. I controlled my blood sugars so tightly that the doctors told me to loosen up a bit. Or maybe I was just so full of happiness it literally brought me health.

Please share a little bit about the birth of both your children. Did CF play a role in the delivery or recovery?

Both of my babies were full term. They were both delivered vaginally. But other than that, I had two very different birth experiences. The first was induced, I had an epideral, and I had a lot of tearing requiring stitches. I pushed for a few hours on my back, at the doctors command. Recovery was hard because of the tearing and because I got very engorged when my milk came in. It took a few weeks to get everything under control.  The second time I mostly labored at home, and delivered him 16 minutes after arriving at the hospital. I pushed on my hands and knees for 30 seconds.  I  had no IV, no epideral, not even a heart monitor on my belly, and very minimal tearing, if at all.  Recovery was much easier the second time as well. I didn't have the tearing, and my milk came in just right, I never got engorged. Although learning to be a mom of 2 was challenging.

I didn't feel that I was limited at all from CF. But I did have the usual extra stuff to do to care for myself in addition to laboring, recovering, and caring for a newborn. I did my vest and nebs during labor both times. And continued to check my sugar levels, give insulin, eat and so on. Doing the vest when I was engorged actually didn't hurt because it promoted the milk to let down, and afterwards it was like I  had just pumped (but it was messy). I nursed my first son until he was 17 months old, and I was 3 months into my second pregnancy. I nursed my second son until he was 2.5 years old. And I still haven't lost all of my “baby weight”.

Caring for another person day in and out regardless of health challenges can be extraordinarily challenging at times. What aspect of CF and motherhood do you find the most challenging?

 I feel like I just don't have enough time or energy to be the kind of mom I thought I would be. So the hard part is being the best mom I can be, and trusting that is enough. I'm tired all the time, but I am very careful not to let myself get so run down that I get sick. Because being sick makes everything harder. So it seems like I am constantly choosing what is most important to do each day with the limited energy I have. I can't do everything that other moms do, and I feel like I am always letting someone down. Also, when I am feeling extra run down from fighting off a cold, or taking home IVs the whole family’s day needs to revolve around me. That makes me feel guilty, and inadequate as a wife and a mother. Thankfully my mom has stepped in whenever I need help. She will stay with us and do everything I normally do with housework, and watching the kids, as well as take care of me. But it is hard knowing I need help like that. Also, having my mom live with us for 2 weeks at a time has it's own set of challenges!

Kaylee is only 3 1/2 and is already aware that I do things that most moms do not have to do such as treatments, taking enzymes, etch. How do you explain your CF or tackle health questions your boys may ask?

From day one, my goal has been to include my boys in my CF care and decisions as much as possible. My goal was to normalize CF. And I realized that CF would be to them, whatever it is to me. So I had to decide what it means to me, then make sure my actions and words back it up. I brought them to all of my clinic appointments until they started school. They would sit at the doctors office with me for 2-3 hours 4 times a year. When they were small enough I would roll them in in the stroller and there they would sit, and take it all in. When they got older, they would walk in and sit in the chairs or my lap, and continued to be a part of my appointments. Sometimes they would ask what we were doing, or what something was. They asked small, simple questions, and I gave small, simple answers.

At some point, I brought up the fact that other people’s moms don't do the vest or have a port, or drink “milkshakes” (boost plus). We have participated in a large fundraiser for CF for as long as they can remember, and that has been a way to bring up CF as well. Through the event we have met other healthy Cfers who are thriving as well. We’ve had conversations about why it is important for me to do all my treatments everyday. And why I decided to have sinus surgery and go in the hospital for a clean out. The answer is simply because I have CF my body needs different things to keep it as healthy as possible. My sinuses and lungs were getting too filled up with germs, and I'm  going to clean them out so I feel better. To them, CF is just another part of who I am. I'm different from Dad because I have long hair, CF and a vagina. They ask all kinds of questions about all kinds of things, and CF is no different.

What advice would you give to other mothers that are juggling both CF and motherhood?

I think that growing up with a mom who has CF can be a great thing for a child. They get an excellent role model for overcoming adversity, demonstrating work ethic, and living a life with intention. Everyday I work for my health. Sometimes I feel lazy and start wishing I didn't have to do so much, but I never give in. They see that.  Sometimes I'm sick and I have to take IVs at home. I schedule my days in a way to get all my treatments in, get enough rest and still be emotionally present for them. I struggle, and I mess up sometimes, but I try again the next day. They learn that. They love me unconditionally, and I didn't even know what that meant before I had them. It’s their love that led me to love myself just as I am. I have a peace in my soul I didn't have before. I'm so grateful to have them on this journey with me. At the end of the day, life is the connections we have with those we love. Everything else is just trivial distractions.

It is our job as parents to teach our kids about life, and how to live. I like to include death in those discussions as well, because it is a universal truth that everyone is going to die. Death is not something to fight or avoid, but to embrace. No one knows when they will die, but when it is time, it happens. Until then, we live! We take care of the bodies we live in, we connect with our world, we laugh, we cry, we love, and we talk about death.

There is a parenting style that embodies the quote “never do for a child that which he can do for himself”. As well as “if he can walk, he can work”. I don't have the energy to parent any other way, but even if I did, I'd still parent this way. My kids are confident and capable little people. They are tuned in to what is happening around them, and excited to do their part for our family. You can learn more about it from Vicki Hoefle, and Parenting on Track. As well as her two books “ duct Tape Parenting” and “Growing a Grown up, the Straight Talk on Parenting”

 

 

To read the stories of other women with CF and their journeys with CF and pregnancy/motherhood please click here.

If you are a CF mother (though adoption, step children, surrogacy, fostering) or are CF and pregnant and want to share your story e-mail me at inhalinghopecf(at)gmail.com