Wednesday, August 31, 2016

Little Lungs

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

Saturday, August 27, 2016

Mysterious Exacerbation

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

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

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

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

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

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

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

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

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

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

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

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


Wednesday, August 17, 2016

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.

Monday, August 15, 2016

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.

Saturday, August 13, 2016


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.

Friday, August 12, 2016


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.

Tuesday, August 9, 2016

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.

Saturday, August 6, 2016

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.