Thursday, April 20, 2017

Weeks to Months

So remember my last post when I was worried about swimming after being sick and taking a break? Yeah, well I shouldn't have worried one bit. And it is not because I am some superstar that can be sick for 2.5 weeks and magically jump back in the pool and do as many laps as I did before getting sick. Nope, it is more like I showed up to find that the gym has a major plumbing issue (or at least that is what I thought was going on) and not only is the pool closed, but the spa, all the bathrooms, and showers are closed! The worst part is they won't re-open for another 3 weeks to 6 months!!

I can either drive 20 minutes to the next closest gym that is within the chain of my original gym to swim or I can try to do something that doesn't require the pool. Considering how early I need to be home from the gym to do my mom duties commuting 20 minutes one way is out of the question. I am so comfortable swimming and I love swimming in a way that I have never liked any other exercise. I feel like when I swim I am in shape and healthy because I am good at it. However, when I try to run on the treadmill or do other equipment I feel old and sick and broken. But I know my lungs need exercise to function well and at this point I need to do something, anything. So tomorrow morning I will hang up my bathing suit, lace up my sneakers and give the treadmill a shot. Eek, lets hope the plumbing is fixed in the next few weeks and not the next few months!!

Tuesday, April 18, 2017

Tomorrow Morning

You know when you get sick and you don't have enough energy to fit everything you need to into the day? And you end up skipping the gym to conserve what little energy you do have? And you end up missing a full week of working out? And then that week turns into 2 1/2? And then you feel better, but your scared to go to the gym because now you are out of shape? You know that little post sick dilemma?

No? Maybe that is just me.

Well, after a full two and a half weeks of sleeping in and not working out I promised myself I would go to the gym tomorrow morning no matter what! I am now sorta freaking out about waking up early, and if my favorite lane will be open, and if I will successfully be able to swim an actual lap, and if I will be able to survive the day with less sleep or more exertion than I have had to cope with in several weeks.

The longer I wait to start up again the harder it will be so tomorrow I will suck it up and start my day at the gym again.

Thursday, April 13, 2017

Snap Crackle Pop

I made it through almost the entire preschool year and through the whole holiday season without getting CF sick. April came along and somehow I caught a cold that went to my lungs. I was so close to making it to spring. Oh well, such is life with CF. I haven't been CF sick since summer which is pretty amazing and so I really can't complain.


There are a lot of aspects of being CF sick that are annoying, but the snap crackle pop sound coming from my lungs all day and night is driving me insane! Does it drive anyone else crazy? Like I can't sleep because the buzzing and whooshing and crackling coming from inside my body is so noisy I can't block it out. I feel like no matter how often my husband uses the precursor on my back or how often I vest or how many times I huff cough I have a constant symphony going on in my lungs that just won't quiet down. 


I am really looking forward to when this infection clears up and I have more energy, less coughing, less mucus and I feel better overall. But of all the aspects of getting well I am looking forward to the one I am looking forward to the most is the silence of my "healthy" lungs.

Tuesday, April 11, 2017

Tezacaftor/Ivacaftor

I am sure most of the CF community has heard by now, but Vertex's latest medication to treat the underlying cause of Cystic Fibrosis has completed two phase 3 studies and have met their primary endpoints! First and foremost, I am so excited to see the development of new medications and I am especially excited to see that it benefits my mutation (DF508) as well as other mutations. It gives me so much hope to think in the next year (or so) a newer and better drug than Orkambi, that has helped me with stability, will be available. I am especially hopeful this medication will help me raise my lung function, even if only a little.


The other part of me remembers how I felt when Kalydeco became available. I was excited to see advances in CF care. At the same time I felt heartbroken that Kalydeco was not available to me. It came out at an absolutely horrendous time in my health and hearing story after story of this miracle drug that was changing people's lives was painful when I was feeling as if my life was slipping away. I know so many people with CF are feeling those same mixed feelings with this newest Vertex medication. I also know there are many in the world tonight that have the right mutations, but are still fighting for Orkambi so this medication must also feel out of reach.


I do have hope that all of us with CF, regardless of mutation, will have access to drugs that will completely change the course of this disease. I really hope to see this in my own lifetime.


If you want more details you can find the press release here.



Saturday, April 8, 2017

The Complexity of Birthdays

I turned 33 earlier this month. I don't often mention my birthday on my blog, but I thought I may share how I feel about my birthday in case others may have the same confusing birthday feelings.


Birthday are complex for me, wrought with so many emotions and many of them unpleasant.



There is a the childlike excitement that springs up around my birthday. And even though as an adult the actual birthday isn't usually all that exciting and I haven't had an actual birthday party for myself in years, I still get that gity excitement that it is MY day!





Another part of me is relieved to see another birthday and to realize I am not dead yet. Pessimistic? Yes, but it is true. A part of myself is still so tied to that 14 year old who searched "Cystic Fibrosis" online and realized that although I never knew the consequences of my disease, I was in fact more than half way through my life according to statistics. That 14 year old me never thought I would be alive to see my 33rd birthday and yet, here I am.





And just as morbid I can't help, but still feel the stress and fear of the life expectancy hanging over my head as each year passes. And considering where my health is, it is not an easy thing to ignore. Maybe if I had great lung function, or even average lung function for a CFer I could ignore it more easily. I know this disease has been rapidly destroying my lungs and I know that without better medications and treatments (that aren't even available yet) I do not have a sprawling life ahead of me. The life expectancy of someone with CF is around 37, but for people born in my birth year the life expectancy is still only around 28 (according to Vertex). And to make matters worse I have officially outlived all of my closest CF friends. The friends that I could relate to the most as our diseases presented themselves similarly have all passed away. It is a lonely feeling and not one that makes birthdays seem overly exciting.





I guess being 33 with CF is a bit like being in your early 80s (mid 70s for a man). You know you are old and you are lucky to be that old. You know you could live to see 90 or potentially 100 and you live each day as if you still have a good amount of them left. And yet in the back of your mind you also know you are old and there is just no getting around that!




And mixed in with all those emotions I also feel a little sense of hope on my birthday that maybe, just maybe I will be one of those people that lives to "100" (in CF years, of course!).

Friday, April 7, 2017

The Waiting Game

I have so many posts to put up, but for now I am just posting a super quick update. I had a head cold that traveled to my chest. We all know the pattern. Currently, I am trying to Vest as much and as long as possible (with a four year old who isn't a fan of mommy being tied to a Vest for too long) and hoping to avoid antibiotics. Pre-Orkambi I wouldn't even attempt to get through a cold that hit my lungs without calling for meds, but the last few colds resolved on their own. I may have run out of luck though... we will see.


As for Kindergarten, we were waitlisted! Ugh! As of now, everyone who got into their schools will have to confirm spots and there will be a lot of movement. So in 3 weeks when the movement will have stopped we will find out if we made it off the waitlist and onto the class roster. Class sizes have gone down recently which is great, but makes getting into schools harder. Our favorite school said they had so many kids waitlisted that they are going to try to open another kinder class which would mean all the kids would get in. I am keeping in close contact with their office to try to get in if/when the new class opens which could be anytime between now and after the first day of school. One year the school added a class three weeks into the school year! Yikes. I never realized how much work it would be to get my kid into a good school!


We do have a neighborhood school that we could default on. The problem is our education system is so broken and there is such a huge discrepancy between schools even within the same neighborhood. One reason we were drawn to the school we chose was their incorporation of the arts. Our favorite school has art docents that go to each classroom, they have a music teacher, a PE teacher, class gardens, they teach to the social and emotional wellbeing of the child in addition to the academic. Our neighborhood school (as most schools these days) is void of any arts. Yes, a teacher can add a lesson here or there, but that requires a teacher who enjoys the arts/music and then has time and the desire to incorporate it to the classroom. Again, there lies another discrepancy within the school. The kids do not have equal exposure to the arts through people who studied their art and are true masters of their specific art. Kaylee is very art minded. In preschool she spends almost the entire day of free choice in the art room.  It is where she finds here joy and I want her to have art incorporated into her elementary education.


Oops, I meant this to be a quick update and I got carried away! Anyway, this kindergarten update is to be continued... again...

Friday, March 31, 2017

Waiting On The Mail

School tours, applications, rejection and acceptance letters. College applications can be rough, but kindergarten applications don't fall far behind. No, I am not kidding. I am not sure if it is because we live in a city or if schools are like this everywhere, but there are a million choices when it comes to public education (even more options if you also want to explore private schools, which we are not). Waldorf inspired, Montessori inspired, art focused, science focused, parent required participation, bilingual schools, schools that meet 4 days a week for longer hours, year round schools. The list goes on and on.

After touring eight schools we narrowed our search down to three schools we really liked near our home. Of course, you are only allowed to pick two schools. They also have this absurd rule that if you put down two choices and don't get into either of them, you can only be waitlisted at your second choice. I guess it is punishment for having two choices, who knows. So after painstakingly eliminating one of the schools (based on the fact they only have a 20% admission rate) we submitted our schools request and then waited. And waited and waited.

We were told the end of March we would receive a letter in the mail with our school placement. And March dragged on and on because of it. All week it took everything in me not to sit in front of the mail box waiting for that letter. A letter that never came.

And then on the very last day of March the texts started rolling in:
"We got our placement"
"Check your mailbox."
"My daughter got her first choice."

Which should be exciting (well, it IS exciting) except our mailbox is one of the last on our mail carrier's route. And so while all of my friends know where their kids are going to school next year (including one that lives two streets away) I have to wait until the very end of the day for our letter to arrive. And while Kaylee is oblivious to all of this excitement, I am a total ball of nerves!! This day can't be over soon enough!!

To be continued...

Friday, March 24, 2017

Just 20 More

"Don't go anywhere because I love you too much..."
My husband whispered into my ear as he was climbing into bed. I had been asleep for a few hours at this point and was woken by his words just enough to process, mumble a quick response, "where would I go because I love you too?" and start to drift back to sleep. However, just before I slipped back into slumber a feeling of dread and sadness filled my heart. Before I could figure out why those words made my heart break, my thoughts were lost to sleep.

A few days later my husband and I found ourselves sitting on the front stoop with glasses of wine. The night air carried the perfect feeling of spring, the neighborhood was quiet with slumber, and we were discussing the events of the day gone by. There was a quiet lull in conversation and I tipped my head back to see thousands of stars dancing above us. Even after all these years the stars seem to surprise me with their glaring presence in the city. Just as I was about to comment on the beauty of the night sky my husband uttered those same words he did the other night,

"Please don't go anywhere. I love you so much."
My heart filled with that same sinking dread it had the other night as I responded. "where would I go? My life is here with you and Kaylee." But even as I was said the words I knew what he meant. I knew it all too well.
"But you are going to die and I don't want you to leave me."

And there is was, the reason for my dread and his desire to ask me to stay despite knowing that it was a promise I was sure to break.

"I am doing everything in my power to stay here as long as possible"
"Just give me 20 more years."

And the thought of twenty more years stretched ahead of me felt overwhelming and impossible. Look how much my lungs have been destroyed by this disease in the past 30 years, how would I ever survive another 20? 52 years old, still so young to the average person, and yet I couldn't imagine how this broken body could make it to such an age. But when you love someone so much that hurting them hurts you even more, you sometimes are willing to tell small lies.

"I think I can do twenty." The problem when you love someone so much and that love is reciprocated they know when you lie. He knew. I turned to him, thinking of my mother in her in 50s and how youthful she still was,
"You will only be 50, still so young."
"But I won't age well," he said with a smirk, "so everyone will think I am 70, at least!" It is a running joke we have, that we will age so very horribly. And I guess when I put it in writing it isn't funny at all and yet it always leaves us with tears in our eyes from laughter.

And somehow all that sadness that enveloped us under that night sky seemed to be washed away in laughter and talking about how horribly wrinkly and old my poor husband will be at the age of 50. Through all our years together sadness and fear often weaves itself into the lives were are creating together and yet it has made our bond stronger knowing our days may be limited and that the future is always uncertain.

Saturday, March 11, 2017

My Fitbit Is A Liar

The other day I wrote about how my fitbit proved I was a liar. Apparently, my fitbit isn't so honest either.

I was getting ready for bed the other evening and had just finished my treatments. I was going into the kitchen to take all my evening pills when suddenly my fitbit vibrated and I got a notification on my phone that I had completed my workout goal for the week. Perplexed as to how I reached my 5 day goal when I hadn't even worked out that day, I opened my app to find I had apparently rode my bike for 20 minutes that evening. My fitbit mistook the vibrations from my Vest as the movements used while biking.

Although I found this mixup humorous, it did make me think how nice it would be to have an exercise tracker that could also keep track of treatments and medications!

Wednesday, March 8, 2017

Kaylee Chatter Part 4

Kaylee woke me up one morning and I was having such a hard time getting out of bed. Kaylee was standing next to me scolding, "Don't be dramatic, just get up!"

She sometimes confuses or combines words. My current favorite is "disastrophe".

"Was everything, like pictures, only in black and white when you were a kid?" Hey, I am only 32!!

"When the very first baby dinosaur was born who too care of it because it didn't have a mommy?"

Kaylee loves wearing my clothes, my jewelry, my shoes, basically anything that belongs to me she wants to wear. One day she came out in a dress she owns that looks just like one I own plus some of my jewelry, "Abra Cadabra, I am mommy!"

Monday, March 6, 2017

These moments in time

Dear Kaylee,
There are some moments in motherhood that I wish I could freeze time and relive over and over. At this age right now, motherhood is so much less grueling than when you were a baby. Sure, there are still bad moods, and the ever growing "attitude" and moments that can be tough, but the day to day workload of diapering and feeding and rocking and calming two year old tantrums are all a distant memory. I find my moments with you, as a kid, rather than a baby so much more relaxed. I held on to your babyhood so tightly because my love for little baby Kaylee was so strong I couldn't imagine that I could continue to love motherhood as much as I did when you were an infant snuggled in my arms. Oh, but a mothers love doesn't know how to regress, it only grows and grows.

One of my absolute favorite activities to do with you these days is read to you while you keep busy doing art work. When you were three I started reading kid chapter books (Ramona!), but as you got older you became more and more interested in the chapter books I was reading for myself. I joined a book club about a year ago and you often asked for the details of the books we were reading. Your interest in my books continued to grow and I found myself reading excerpts of the book club books I was reading. As long as you found an activity to do while I read, you wanted longer and longer excerpts. Now, you beg me to read my books to you while you busy yourself with crafts. Last Saturday I read almost 100 pages to you in the course of a day. Every time I tried to take a break you would beg me to continue.

Of course, my sweet sensitive child, I need to edit or omit parts because you are only four! You pay attention at times and at other times I know your mind wanders which makes it easier for me to skip over parts, improvise, or "lose" my spot only to pick up again a few pages later. You have rejected bedtime stories in exchange for listening to me read whatever I am currently reading.

This, my dear girl, is the parts of motherhood that make me thankful for the here and now. It is easy to look at old pictures of you when your cheeks were chubby and your hair still held tight curls around the nape of your neck and miss those sweet and tiring baby days. These magical moments are especially amazing because I know we will never have to leave them in the past. These traditions we can continue forever. Eventually, we will swaps roles, you will read chapter books to me! And even when you grow up and move from my home, we can read the same book no matter how close or far you live and we can discuss the books over coffee or, if distance requires, over the phone.

My sweet girl, I feel so very lucky to have you in my life and as each day passes my love for you just grows and grows. Thank you for going on this adventure called life with me and daddy. You bring so much joy to our lives and we can't wait to continue to watch you grow!

Love,
Mom

Thursday, March 2, 2017

You Know You Have A Chronic Disease When

A few days ago I went to the eye doctor to get a new prescription for contacts. I was filling out the forms they give you to fill out while you are waiting for your appointment. Easy enough. And then I got to the line that asks if you are on any medication. IF?? Wait, if? I sometimes forget everyone's life doesn't revolve around their medication schedules.

I quickly checked the yes box and went to the next line to fill in my medication list. The line they provided was 4 inches long. 4 inches!!  How do I fit 12 medications on a 4 inch line? Oh right, most people don't take 12 medications and in fact a lot of people can skip that line entirely!

You know you have a chronic illness when you need extra sheets of paper to staple to the back of your intake paperwork to fit your medication list.

Wednesday, February 22, 2017

Guest Blogger- Kristen

I am so excited to introduce to you another CF mother, Kristen! Kristen has a unique CF story in that she was diagnosed with Cystic Fibrosis at the age of 31. She was already a mother of two children at the time of diagnosis. Six months after her own diagnosis her children were tested positive for Cystic Fibrosis as well. Below is her journey with CF and motherhood.

Like me, you had a late diagnosis compared to most people with CF. Can you explain how you were finally diagnosed with Cystic Fibrosis?

For many years I struggled with GI symptoms, chronic sinus infections and low weight. Doctors were unable to connect pieces until I was 31 and diagnosed with pancreatic insufficiency. After the pancreatic insufficiency results and a borderline sweat test, the pieces were put together.

I am sure getting a CF diagnosis as an adult was life altering. Your children were also diagnosed with CF which I am sure added to the shock. Do you mind sharing a little bit about what it was like receiving three CF diagnoses in one family over a short period of time.  

I was told the chances my children had CF were very slim, due to the genetics of myself and my husband. However, both my children were in fact diagnosed with the disease. It was shocking to say the least, but yet I felt that it immediately encouraged me to stay as positive about my disease because it was my children’s disease as well. And I did not want them to have any negativity associated with CF.

Cystic Fibrosis presents itself so differently in different patients. Can you share your biggest hurtle with CF?

My digestive system. Even with clean eating and digestive enzymes, I struggle with DIOS episodes, lack of absorption, and low body weight. And because of that, I can be weak at times.  I also have chronic pancreatitis which can cause pain many days and my blood sugar to be erratic

What are your children's biggest hurtles when it comes to Cystic Fibrosis?

We are very blessed that at the moment my children are only exhibiting mild CF symptoms. My son is very strong. He runs almost daily to keep lung function up. However, he does struggle with intestinal issues as well. He has had several “back-ups” as they are referred to and needs to be diligent with laxatives and enzymes.

My daughter is very tiny, like myself and struggles with her pancreatic function as well. She is prone to upper respiratory infections. We have had a good 6 months, but the 6 months prior to that, she had several cases of pneumonia.

Being a mother, regardless of health status, can be challenging at times. We all know CF can be extremely demanding and challenging as well. Does CF ever get in the way of the mother you want to be?

YES! There are times I am too sick to go into the schools for this or that for my children. There are times I am too sick to get out of bed and I hate not being present for my children. My own care takes a lot of time and that is time I have to schedule in my day and away from other things.

What advice would you give and adult that is diagnosed with Cystic Fibrosis.

To not let this disease define them. CF will become part of your life, but it does not need to be your whole life or define you negatively.

What advice would you give to a parent whose child recently received a CF diagnosis?

I would tell them to raise their child to be responsible for their disease, but to raise them with HOPE. Allow them to be who they want to be. CF is only one fact about them, it is NOT who they are!

You and I have very similar philosophies when it comes to food: whole foods, healthy sources of fat, and limited junk or processed foods. This kind of eating can be unusual among CF most CF patients who are told to get as much fat as possible regardless of the source. What made you chose this route when deciding what foods to put into your body?

It was as simple as trial and error. The bottom line is that I feel better when I eat clean foods. My weight stays more stable when my fat and calories come from natural sources. When I stray from clean eating, I suffer with pain and weight loss. I also believe that people with CF need to plan to live a long life (we are making strides that is a reality now) and I believe you should protect your heart, etc when caring for your body and meeting your CF needs

Please tell us about your website, http://amindfullmom.com/, where you share tons of amazing recipes.

A Mind “Full” Mom is a place where I share real recipes made from real foods that meet the needs of real families on real budgets. That breaks down to me sharing recipes that are family-approved, easy to pull off and won’t break the bank. I also share ways to be intentional with life and to not let the craziness of this world impact the memories you make with your family. And of course I share encouragement for staying positive when living with a chronic disease.





To read the stories of ten 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 have CF and are pregnant and want to share your story e-mail me at inhalinghopecf(at)gmail.com

Monday, February 20, 2017

Polar Bear Plunge

I missed a few days of workouts because the whole fiasco with missing some Orkambi which made me too short of breath to workout. Friday I decided my lungs felt well enough to get back into my usually workout routine. I set my alarm and found myself checking into the gym early morning. To my dismay the girl working the front desk informed me that the pool was closed for maintenance and apologized for any inconvenience. Sigh, I wished I had decided to sleep in that day instead.

Over the weekend, I called to ensure the pool would be ready for Monday morning as I didn't want another early morning without a workout. The gym staff assured me the pool was up and running. So this morning, I found myself back at the gym and more than ready to get back into my workout routine. As hard as it is to get myself out of bed to go to the pool I never feel myself when I skip and so I really do enjoy my morning workouts. After checking in (and yes the pool was open!) I ran through the heavily falling rain to the pool, sat on the edge to adjust my goggles and dipped my feet into the water. Eeek! The water felt like ice! I went to the heat pump to check the water flow to see if the water being pumped into the pool was warm (it always is at this time in the morning) and it was also coming out as ice.

I wasn't about to miss another workout and so in I went for my very own polar bear plunge (although the mornings have been around 50 so I guess I really can't pretend I am as crazy... err... brave as the people that really do the plunge). I will say that being forced to swim in cold water sure encouraged me to swim a lot faster though!

Here is to hoping tomorrow morning that the pool will be open and the water will actually be warm!

Thursday, February 16, 2017

Restarting Orkambi

Due to issues with my pharmacy I did not receive my January shipment of Orkambi on time. Of course, I was upset that due to issues with my pharmacy and their lack of organization and communication was causing me to miss medicine that is beneficial to my body. But the reason I was most upset is that when I started Orkambi I had a lot of issues with side effects specifically chest tightness and shortness of breath. I was very concerned that restarting my Orkambi would bring back some or all of these side effects.

When I finally received my Orkambi I was so relieved that I wouldn't have to fight Accredo anymore. I momentarily forgot my concern with side effects. I was abruptly reminded of this concern a few hours after taking my morning dose. Although the side effects weren't nearly as intense as when I first started Orkambi I did notice a huge difference in my breathing pretty immediately. I often felt as if someone was sitting on my chest. I also had random sporadic bouts of extreme shortness of breath that seemed to come out of nowhere and disappear just as mysteriously. The biggest issue I had was I was too short of breath to exercise efficiently so I felt like my lungs weren't getting cleared properly by swimming which seems to be my best defense against mucus build up.

The side effects only lasted about fours days and yet after they subsided my lungs felt congested and heavy. I currently feel like I am on the brink of an infection. I will never know 100% if stopping and restarting Orkambi caused this infection, but I do think adjusting off and back on was really stressful to my body. I also think missing/more lax workouts didn't help the mucus build up nor did the tightness caused by restarting Orkambi. I seriously hope that I am never forced to go off of Orkambi again unless a new and improved drug comes out.

Monday, February 13, 2017

I Hate Accredo

I am not even sure how to explain how impossibly hard it was dealing with Accredo these past few weeks. I think instead of writing a post that could easily go on for hours I will give you the quick and dirty run down. I think it is important to note that a rep from Vertex as well as my clinic coordinator was also helping me deal with Accredo and I have no doubt it would have taken significant longer to get my meds if I was doing it alone. Which is so beyond ridiculous because it took over two weeks for me to get the medicine as it was.
  • At one time my clinic nurse called and explained in great detail what she discussed with Accredo. I called Accredo and when I told the rep what my clinic nurse just explained the rep told me there is no record of such conversation in their computer system. Huh? What? How do you even respond to that?
  • Every time I called the rep would flip flop what they were saying. Sometimes they would say it wasn't covered by my primary which meant my secondary wouldn't cover it either. Other times my primary would cover it, but my secondary didn't leaving me with a co-pay of $5,300.
  • Two times my Orkambi was scheduled to ship out the next day only to be cancelled at the last minute due to "issues."
  • Between Thursday and Friday I spent FOUR hours on the phone! About 90% of this time was spent on hold with Accredo.
We eventually found out a lot of these issues were because someone opened two accounts under my name and neither had both my insurance plans. So depending on which account the rep opened there was differing insurance information. Hence the reason some reps said that my nurse or Vertex never called in. They had the wrong account open. After this issue was resolved I assumed we would be good to go. Oh, but no that would be much to easy!

  • Accredo kept insisting over and over again that my secondary insurance didn't cover Orkambi although it absolutely DOES cover Orkambi and I was told over and over again that the only way to get my medicine is to fork over $5,300. (The issue was that they were using the wrong authorization number)
  • My case had been escalated a few times to people "higher up" in order to try to expedite (HA!) my shipment. However, there was complete lack of communication between the higher up "center of excellence" and the reps. The center of excellence (I use the term excellence very very loosely) made a plan and was told to me by my Vertex Rep. When I called Accrued explaining the plan I was told by both the rep and her supervisor that, "Nope, it doesn't work that way!" I ended up hanging up and calling back until I found a rep that would do what I said.
  • I was told by a supervisor and someone in center of excellence that they would call me back within a specific time frame. Did it happen ever? Nope. And when I called them, the rep said the person I needed to speak to was nowhere to be found.
  • After about two weeks I talked to a rep that said that maybe I should let me doctors office know I was having trouble getting my medication. Uhhh, if you look at my log they have been calling in for almost two weeks now...I think they know! This seemed to be another common theme... reps that didn't bother to read the notes in my file and wanted to d the bare minimum as far as work goes.
So we finally figured everything out and I was starting to believe I would finally receive my Orkambi. But wait, that would be too easy right?

  • I was scheduled to get my Orkambi and although I had already ran out of my supply and it was coming super late I was beyond relieved to know it was on its way. Just when I thought I was safe my phone rang.... it was Accredo... nothing good comes from those calls...
  • They were calling to say that their inventory was low and it would take 7 days for me to receive my shipment. SEVEN DAYS!!!
  • At this point, I will not mention the things running through my head. However, the rep that delivered the news was one of the few reps that knew how to do her job. She had talked to me several times and I kept telling her if there is ANY inventory ANYWHERE they needed to send it to my pharmacy to get it to me. She told me to hold.... came up with a potential plan and told me she would call back.
  • And guess what? Whoever is in charge of updating their inventory electronically didn't do their job (notice a theme??) and they actually did have some to send me. So I was going to miss another entire week of medicine because someone "forgot" to update their inventory list.
And then I got Orkambi and we celebrated. The whole family. Kaylee wouldn't be neglected anymore while I spent all day on hold. My husband didn't have to put up with my mental breakdowns anymore. And best of all I had my medicine. Except, restarting also meant my body had to readjust to Orkambi which my body doesn't do well...

To be continued...

Saturday, February 11, 2017

A Day In The Life- Preschool

I wrote a blog about our daily routine when  Kaylee was a newborn and again when she was about one. I loved looking back at it and was shocked at how long it took to do my treatments! I forgot that I had to break them up throughout the day because a baby's attention span is so short. I decided I would do "A Day In The Life Preschool" version since I know someday these moments will seem like such a distant memory. So like any "Day In The Life" series this is a rough idea of out day because as we all know children often have their own agenda.

5:20
  • My alarm goes off and I quickly get my bathing suit on, pull on sweats drink a glass of milk and drive to the gym.
  • I swim for about 30 minutes, shower at the gym and come home.
6:30
  •  I arrive home from the gym and my family is still sleeping. I start my Vest and all my nebs.
7:00-7:30
  • A very sleepy Kay comes to find me in the living room. (Why are kids so darn cute when they first wake up?) I am usually almost done with treatments, if not completely done. If she gets up before I am done I let her watch Daniel Tiger until I am done.
  • I usually brew a cup of coffee for me and start breakfast. I always let her choose between eggs and oatmeal. I do not have CFRD, but I don't do well with sugar in the morning so breakfast is never something convenient like cereal. Even my (and Kaylee's by default) oatmeal has to be a mix of oats, chia, ground almonds and flax meal because straight oats cause issues in the morning too. The older I get the more complicated my body gets!  
  • I also do morning chores like empty the dishwasher, clean the breakfast dishes, and sometimes throw a load of laundry in the wash. I am so bad at letting laundry pile up.
8:30

  • Kaylee gets dressed, we brush teeth, and I always do her hair (having her hair off her face means she touches her face less often which is better for controlling germs even though she has no idea that is why I do it. I am trying to be germ cautious without making her realize it so that she doesn't become a germaphobe). Then we hop in the car and go to preschool.
8:45

  • Kaylee likes to be the first person in line so we are usually the first people there and I let her play on the playground until school starts. There are a couple other early birds so I get to chat with some of the other parents before school.
9:00-11:30

  • Preschool! Kay goes to a parent participation preschool so I am there working twice a month. I am also the class mom so I go to all holiday parties and any big events so I feel like I am there a lot, which I love!
  • If I don't work at the school I usually try to make phone calls or other boring errands that aren't fun for Kaylee. On days my husband is home we sit around and drink coffee.
11:30-12:30
  • Pick up and come home for lunch.
12:30-2:30
  • We either go to the park, ride bikes, take a walk or run some errands. I usually do some chores (aren't house chores endless?). Really, we have no consistency during this time. On my Cayston months I do my afternoon nebs.
2:30-3:30
  • Quiet time! Kaylee plays independently and I get to rest as well. We both really need quiet time or we both get grumpy!
3:30-5:50
  • Kaylee has a snack, plays and then we get dinner ready (Kaylee usually helps).
  • My husband usually gets home right before we have dinner although his schedule can be all over the place so a few days a week he misses dinner.
6:00-7:30
  • Kaylee and my husband play and I clean up dinner. I often do treatments during this time or if I join in I will do treatments after she goes to bed. If my husband works late I usually do treatments after Kaylee is asleep so then the two of us play together after I clean up.
  • Kaylee picks out her clothes for the next day and I hang them on her doorknob so she can get herself dressed in the morning.
  • We do a five minute clean to make sure all her toys are cleaned up before bedtime.
  • Kaylee has a small snack before bed.
7:30
  • Kaylee gets in her pajamas and brushes teeth.
  • My husband and I read books (mine are in English and his are in Spanish) and then we each tell her a story (also in English and Spanish). Mine usually incorporate something we did that day, after telling stories every.single.night for years it is hard to be creative. Then Kaylee stalls as much as humanly possible.
  • Finally, Kaylee goes to bed!
8:30
  • I do treatments (if I hadn't already done them)
  • I set out my gym bag and everything I need in the morning.
  • I take all my pills
  • I eat a snack so I can take Orkambi (my morning dose is with breakfast)
  • I also do the evening chores like run the dishwasher.
10:00(ish!)
  • Bed!!!

Thursday, February 9, 2017

Bliss In Pictures

This past week has tested my patience in more ways than one. I found myself grumpy and irritable and in a funk. I decided to take some time to remember the simple things that bring me joy. I needed to find moments of bliss between making phone calls, sitting on hold and fighting with my pharmacy over and over again. So here are a few pictures of the moments of bliss that saved my sanity this week.




Fresh brewed coffee on a rainy morning


A brand new jar of raw local wildflower honey. The plastic bear has nothing on the real deal!


The first sign of spring on my blueberry bush, no less!
 
Asparagus season has officially begun. It takes three years after planting before you can harvest asparagus and this year finally marks year number three!



Monday, February 6, 2017

Kaylee Chatter Part 3

Kaylee dumped out a box of small wooden whales and shouted, "Woah, there are whales ga-lord!" (galore)

My husband was doing something that Kaylee was not a fan of (I can't remember what it was) and she was giving him the evil eye and said, "I am judging my father right now!"

Kaylee's grandma on my husband's side always tells her, "suena con los angelitos" meaning "dream with the angels" or basically, sweet dreams. So the other day before bed Kaylee shouted, "Suena
con los burros!" to my husband meaning "dream with the donkeys."

"Mommy, I love you more than anyone in the world. I want to marry you because I couldn't love any wife more than you." I have to write these things down so when she is 16 and finds me the most annoying/humiliating person alive and I can fondly remember the days she was obsessed with me!

Thursday, February 2, 2017

Missed Lessons

I will be posting later about my ongoing struggle with Accredo, but honestly, I am so beyond pissed right now at them that I just can't let myself think about it or I may seriously lose my mind. Can you tell I still haven't had my medication situation figured out yet? Despite endless calls from Vertex, my clinic coordinator and myself we are still in the never ending hell that is called Accredo. Okay, I can feel myself getting fired up again so I am moving on to the real reason I am posting.

After dealing with Accredo for two weeks trying to get Orkambi and still not making much progress (it is one step forward, ten steps back and then an oops we, as in Accredo, messed up and we are now nowhere even close to where we need to be) I was pretty much at my breaking point for the tenth time today. I was venting to my husband that I feel there is a reoccurring theme in my life. I feel like the Universe is trying to teach me to be patient. Over and over I am thrown in these sitatuation that extreme amounts of patience is required and I can't figure out why because I already am a very patient person. Trust me I have a lot to work on in life, but patience is hardly one of them.

My husband looked at me and said, "Maybe the point was not that you needed to learn patience, but that you needed to learn to be more assertive."

Bingo!

Yes, I have patience for days and I also am super laid back so it takes a lot to get me upset. Patience is something I am not lacking. Being assertive on the other hand is not something that comes naturally to me. I always envied people that could be very assertive (while respectful of course) and got their point across firmly and without hesitation. That is not me, but I wish it was. Sure, I was assertive today, but while I was fighting for what I deserve and need I felt like a fish out of water, like I was trying too hard to be something I am not. It did not come naturally. I eventually lost my cool (oops) and I know that is not productive either.

From now on, when my patience is pushed, I am going to be more assertive and COMFORTABLE while doing it. I know the more I practice the less uncomfortable I will be because like anything else it is a skill that can be learned. So in 2017 I am going to work on being assertive and saying exactly what I mean without an ounce of hesitation!

Monday, January 30, 2017

The January Curse

Anyone else with chronic illness (or not) feel like every January you spend the entire month making insurance phone calls because nobody can seamlessly transition into the new year? Seriously, it is the very end of January and I can't even tell you how many hours I have spent on hold trying to fix insurance glitches. Somehow I had problems with my regular insurance coverage accidentally switching my info with my daughters and then disenrolling us because of it. This took maybe 4 hours total to straighten out via phone... seriously I wish I was joking. Thank goodness for cell phones and speaker phone so I could do chores while waiting! I can't imagine being tied to a corded phone in the kitchen and being on hold that long.

I also spent several hours on the phone to my insurance company that covers Orkambi. I am sure the fact that I have 3 different pharmacies doesn't help because one is bound to mess everything up. And no, I have no idea why I need three different pharmacies!! I used to get everything from one pharmacy, Walgreens, but then my insurance told me I needed to get Orkambi from Accredo. I am assuming they got a better deal on the cost of Orkambi? And then somehow I needed to go through Foundation Care to get Cayston and so now, yes I deal with three completely different pharmacies every month. As you can imagine it makes for plenty of month phone calls. Of course, Accredo (the supplier of my Orkambi) had to be the one that gave me problems. Despite refilling my Orkambi two weeks before I ran out I am now down to 4 days of the medication and still no word on when they will get their act together. My clinic even called a few times to try to help me out, but everything is moving so slow. Since the drug cost $21,583 a month (yes, you read that right, it cost $258,000 a year) I am probably not going to be able to pay out of pocket. Ha!

The most frustrating aspect of this whole ordeal is that I have the exact same insurance as last year! So you wouldn't thin it would be that difficult, but apparently it is. And the even more frustrating issue is that every single year for the past three years I have had some issue that took the entire month to resolve.

I have no idea what I am going to do with all my free time in February when I don't have to spend all day every day on the phone!

Wednesday, January 25, 2017

Slow Motion

There was a time in my life that I would start a new workout routine and instantly my lungs would respond. I remember being so amazed when I started swimming again after a long break and my lung function jumped to some of the highest levels I had seen in a long time. I naïvely assumed that this meant that if people started exercising they would absolutely see positive changes in their lung function. See, the problem with CF is sweeping generalizations just don't hold up. What works for you doesn't mean it will work for someone else and even more mystifying is what works for you now may not work for you later.

About two and a half years ago. I had just gone through a horrible time in my health and very slowly started working out again because it was the first time in a long time my body would even allow me to work out. I started with water aerobics and had trouble keeping upon with the 60+ year old women in my class. Seriously, I was half their age and most of them smoked me in the pool with workouts. Humbling to say the least.

I eventually worked up to swimming laps. I did about 15 laps and took minute long breaks between each laps to catch my breath enough to try another lap. I was humiliated and ashamed that I was the youngest person in the pool and yet everyone's beautiful continuous laps that started long before I arrived at the pool and continues far beyond the time I left made me feel weak and broken. I would repeated excuses in my head as I did laps, "I have 30% lung function, but when I was healthier I could swim like you too," as if the other swimmers could read my mind and would be less judging when they heard my silent explanation.

I made progress and started swimming for about 15 minutes with fewer breaks although I only managed to do breaststroke (the easiest stroke for me) and hadn't built up the lung power to try front crawl. I was feeling less ashamed and humiliated until and older woman made a comment, "You are like my husband. You work out for about 15 minutes...I guess that is all you need." She was more talking out loud than saying it in judgment, but I remember feeling like I never wanted to show my face there again. I wasn't being lazy and working out as little as possible, it was all I could manage!

Eventually, swimming got easier. I started swimming 40 laps (front crawl!) and often only stopped because my oxygen sats dropped too low and forced me to stop. Over time, I slowly worked up to 50 laps, then 60 laps, and currently I do 75 laps before I call it quits. I don't stop between laps and no longer need to pull myself from the pool because of oxygen issues.

And as for my naïve assumption all those years ago? Over two years my lung function hasn't budged even 1% point. Not yet, at least. I think about how hard it was to do two consecutive laps two years ago and now I can do 75 in a row and it is hard to believe my lung function hasn't changed. However, I know my lungs have changed! They are in much better condition than they were two years ago. I know my oxygen saturation is better and overall my lungs are healthier and clearer. I may not have been rewarded with higher pfts, but I do believe exercise is allowing me to have a good quality of life despite my low lung function. Also, when I am in the pool I don't feel sick, I feel strong and healthy and powerful now. That is something, as someone with CF, I don't often feel out in my regular life. So emotionally and mentally it helps me too. I guess when I think about all the benefits exercise has given me, I can't be too disappointed that it hasn't helped my lung function (at least not yet...).

Monday, January 16, 2017

My Fitbit Proved I Was a Lair

I think of myself as an exercise conscious person who prioritizes my health. So when I got a fitbit I was curious as to my results, but assumed I would be in pretty good active standing. My fitbit proved I was a lair! After a few weeks of using the fitbit (omitting the week we got the stomach flu) here is the honest truth of my exercise\self care.

The Good:
Sadly, this is the only good news my fitbit had for me! I have the fitbit flex 2 which means I can swim with my fitbit and it keeps the stats of my swimming workout which I love! The fitbit told me I actually swim much further than I was telling myself. When I changed gyms years ago I went from a gym that had an Olympic sized pool to a gym that does not. The drastic size different always made me downplay how much I swim. Not only do I swim a further distance than I thought, but I exercise longer than I thought. I always said I swam for 20 minutes (I never time it and just try to get to the gym, swim, shower, and dress within an hour), but my fitbit let me know I swim continuously for 30 minutes.

The Bad:
I am much less active than I thought! To be fair, it is winter and it has been cold and very rainy. Although we usually still get outside to play and walk despite the rain we just don't last nearly as long as when the weather is nice. Also, my winter garden this year is pretty basic and filled with food that needs almost no care (garlic, lettuce, peas, and perennials) so I am not spending much time out there. However, I was shocked that most days I struggled to get 10,000 steps. And without making too many excuses, swimming doesn't give me step credits (obviously) so despite working out every morning when I get home from the gym I have only logged ~100 steps from the parking lot and changing room.

The Ugly:
I was also curious about my sleep because I know when I wake up early to swim I am probably not getting as much sleep as I should. I had no idea how bad my sleep stats really are. I average around 6 hours a night. I also have a few wakeups (which I knew). I also have a lot of restless sleep, which I can't really help and assume everyone does. I was just shocked at how few hours a night I get. No wonder I feel so tired all the time! Lack of sleep and CF really aren't a great combination so I am now consciously trying to put myself to bed at a more reasonable time for my early wake up time.

Now that I know where I lack when it comes to leading a healthy lifestyle (SLEEP and walking) I am hoping to make changes to lead a healthier and more active lifestyle!

Friday, January 13, 2017

Altitude Testing

It has been five years since I have been on an airplane. How sad it that? Well, that is about to change in a big kind of way. However, my lungs have significantly declined over the past five years and so I was concerned that if I try to fly, my oxygen saturation may tank. I really don't want to be responsible for grounding a plane so I decided to voice my concern with my doctor. We decided that altitude testing would be appropriate before I step foot on a plane.

I wanted to share what altitude testing consists of for anyone that may find themselves in a similar situation or if you are just curious. Altitude testing is supposed to simulate what breathing feels like at 8,000 feet which is how high most commercial planes go. I was super nervous for the testing, not because I was worried it would be a difficult test, but because I really wasn't sure I could pass which would mean I would require oxygen to fly. My oxygen saturation on room air is still really good, 98%, but my lung function being around 32% makes me more likely than someone with good lung function to struggle with any changes in altitude. I also had a quick panic while waiting to be called back for the test because my legs were a bit (lot?) prickly as I get lazy with shaving in the winter and I panicked I may be asked to change into a gown!

When I finally got called back I quickly relaxed realizing there was no need to change from my street clothes. Whew! There were two RTs waiting for me, but they were waiting on the doctor who had to be present during the test in case something went wrong. After signing all the consent forms I was hooked up to several monitors. There were 5 heart monitors, a pulse ox (on my forehead! Apparently my hands were too cold and not reading well so they wrapped a pulse ox reader to my forehead), a nose cannula, a blood pressure cuff and finally a mask that looks exactly like a bipap mask (or so I am told because I have never used a bipap). The oxygen cannula was used so that if any point during the test my oxygen SATs fell below 89% they would slowly administer oxygen to see what amount of oxygen I would require while traveling via plane.

After everything was set up they turned on the mask. Apparently, air with a lower amount of oxygen (to simulate the air on a commercial plane) was being blown into the mask. I couldn't feel any air being pushed through the mask, but they made me sit there for a few seconds to watch my oxygen saturation. They asked how short of breath I was using a scale from 1-10.

They then asked me to talk because they want to simulate what a plane ride would be like and likely I would be talking at some point. Although I am not a shy person randomly chatting to three men while having wires protruding from your head, all sides of your body, and while wearing a huge mask was not totally in my comfort zone. Luckily, everyone was nice and I got in a flow of telling them about our future vacation. Although about every 60 seconds one of them had to interrupt to ask me about shortness of breath and they often looked away to check the monitors. So basically, I was chatting, but wasn't really being listened to which made talking feel somewhat pointless (even though I know I was doing it in the name of science!).

My oxygen was fine so they made me stand up and march in place (while talking) which again was somewhat humorous considering the monitors. Every one minute I was asked about my breathing and every two minutes I had to quickly sit down when the arm cuff started reading. Oh, to be a fly on that wall, I am sure the whole thing looked so absurd!

After a few minutes my oxygen was still fine so they had me sit and stand (like squats) over and over. The told me that if this didn't drop my sats nothing would. My oxygen saturation hung on through the squats although they did hit 89% which means my oxygen was only 1% away from being considered too low. Luckily, I likely will not be working out on the plane. At the end of the test they congratulated me because I passed and could fly without oxygen!! It was such a huge relief!! They did say that if I were to get sick before the flight it would be best to postpone because a respiratory infection may bring my oxygen saturation down. They did, however, feel that even if my oxygen dropped a little I would probably be okay to fly, but should bring a pulse ox reader and limit my activity. Of course, on the way to a trip it is much easier to cancel than on a flight home especially since I couldn't extend my stay longer than I had medication supply for so hopefully I will stay healthy on my next vacation.

Sunday, January 8, 2017

Silver Lining

A few days after New Years our family came down with a stomach bug. I will spare you the details as I am many of you are much more familiar with the stomach "flu" than you would like to be. However, despite the horror, there was a silver lining I thought I would share.

Kaylee caught the bug first. I ended up as Kaylee's head nurse because my husband was at work for most of the day. She was very sick for about two days. Of course, as it often happens, as Kaylee started to improve I started to feel a little off. And one evening I passed on an old fashion and a movie to lie in bed (or roll around in bed trying to find a comfortable position) while gripping my stomach. Needless to say I was hit hard and as with everything, I got a weird complication in that my fevers got out of control and I slept about 20 of the 24 hours that made up Thursday.

Kaylee, who was on the mend decided to become my head nurse. She had slept in our bed the previous two nights (running a sick child to a toilet is much easier when you are next to them) so when she woke up and found out I was sick she announced she would be right back and ran from our room. A few moments later she walked in and presented me breakfast in bed. Of course the tray was a baking sheet, the plate was a coffee filter (she can't reach the plates), and the breakfast of choice for a person with the stomach flu was a easy to digest pile of mixed nuts. And she presented me a plastic disposable water bottle which gave me a moment of pause because we don't buy disposable water bottles, but she assured me she poured out the old water and put in fresh water.

After breakfast Kaylee informed me that if I got sick she would hold my hand so I wouldn't be scared. As her mother I never left her side when she was sick and miserable and it was heart whelming that she wanted to be there for me as well. Of course, when I did get sick she dutifully came with me to the bathroom. I took one look at her face and told her she could leave if she wanted to and she ran out of that bathroom as fast as her four year old legs could carry her. It is the thought that counts, right? By the time my husband got sick Kaylee had lost some of her empathy because with both parents sick, being a kid just isn't fun. However, I loved seeing the thoughtful side of my daughter who wanted to take care of her mama (even if she needs to work on her follow through).

Sunday, January 1, 2017

The New Year

2016, as with every year, was a mixed bag of good and bad. However, overall I have to say that 2016 has been kind to us. My health was the best it has been in several years (thanks, Orkambi) and outside of a health blip in the summer I was pretty stable. Kaylee and my husband are healthy and we are all happy, what else could we ask for?

There are lots of changes coming our way in 2017. A major life change is in the works and I am sure a dozen more changes that we are completely unaware of will be hurled our way as life seems to be unpredictable and full of surprises (both good and bad). I am hoping for continued stability with my health and lots of love and laughter with my family in 2017. I hope everyone reading this blog finds peace, love, and health this coming year.