Sunday, April 30, 2017

Pharmacy Overload

Can we just talk about pharmacies for a minute? Now you all know how much I hate Accredo, but this is not that kind of complaint. This is a vent about how calling pharmacies seems to be like a mini part time job.

My insurance allows me to get most of my antibiotics through Walgreens Specialty Pharmacy. I have had really positive experiences with them and have absolutely nothing to complain about as far as the pharmacy itself. My only complaint is that I get 6 meds one time of month and then a few weeks later I get hypertonic saline from them because they are on different schedules of when I can refill. This means I am in contact with the pharmacy twice a month. Haingto make two calls a month to get all the medication we need to survive isn't really worthy of complaint except I don't get all of my meds through Walgreens.

My insurance requires me to use Accredo to fill Orkambi. It infuriates me because in the past I was allowed to use Walgreens (when I had different insurance) so I know they CAN provide it, but due to rules with my insurance I can't use anyone, but Accredo. So once a month I am calling them for Orkambi. Which as many of you know can turn into 12 calls a month to get my Orkambi, but that I another story entirely!

If having two pharmacies wasn't enough, I have to use a completely different pharmacy, Foundation Care, for Cayston which I take every other month! So every month I have two different pharmacies to call with a minimum of three calls. And every other month I have another pharmacy to call in order to get my inhaled antibiotics. It can all get very confusing and I have to keep schedules in my phone to know when to call which pharmacy for which medication! And that, my friends, is what I am complaining about. Is seems a little too complicated for a group of people that already have very complicated lives!

Now that I have vented about my pharmacy issue, I have to admit that despite how many pharmacies I am required to use I am grateful that all my meds are delivered to my door. I do not miss the days of going into the pharmacy and walking out with a grocery cart worth of medication! I would rather people not know I need 9 medications to keep me alive (including half a dozen bottles of enzymes) every month!

Thursday, April 27, 2017

Duck Days

Our little home sits a few blocks from a canal that comes off of a major river. During the blistering hot summer months, you will find us splashing on the beaches of this river almost weekly, but as summer turns into it's cooler cousin fall, the river starts to fade from our thoughts. And there the river lies dormant from our minds, until early spring.

Late February the geese signal the turning of the seasons with their return home. They fill their skies with their loud welcome home calls and their majestic formations. Around this same time the canal and river ducks (mallards, mostly) can be seen wandering around he neighborhood. The reason for this behavior is unknown to me, except that I have heard rumors the females often look for safe havens in people's yards to lay their eggs. And these ducks, who have lived their whole lives in a quiet part of the city, are very much aware of the food that is stored inside the homes in the neighborhood.

Around March we often can hear small groups of mallards quacking at our front door. Upon hearing the quacking, Kaylee fills with delight while she shouts, "Get the bread!" I, just as excited as she is, dutifully follow orders. We crack open the door to find a small gathering of ducks waiting on our front stoop. They continue to come several times throughout the month, happily gobbling up bits of bread before moving along to find bugs in our bushes.

And then as suddenly as they show up, our porch is quiet. We no longer find ducks following us to the mailbox or waiting outside our door, calling out to us to see if we are home. The absence of ducks lets us know, that the next phase of spring is upon us.

Last week, Kaylee and I were riding our bikes along the canal when she let out a gasp that was so loud, I was certain something horrible had happened. She instantly jumped off her bike and ran towards the canal, "Look mom!! Babies!!" Sure enough there were 13(!!) ducklings swimming along the canal behind their mother. Our first duckling sighting of the year. For the next few months we will walk along the canal to find it spotted with fuzzy little ducklings following their mothers along the water.

Next March I am sure some of these ducklings, all grown up, will be quacking at our front door looking for some scraps of bread and we will be eagerly awaiting their visit.

Thursday, April 20, 2017

Weeks Or 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


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...