How is she doing?

This is long overdue…and I apologize for that… but I find it hard to write about this stuff…. so I decided that tonight a few nights ago would be my first attempt… and after a glass of wine (or three), there went nothing… (and after 4 days, this is still a work in progress – the first edits got deleted TWICE and I’m back to recreating brilliance readable stuff).

One of the most difficult questions ever posed to me is “How is Ally doing?” The easy, short answer is to say “she’s doing okay…” I mean… the reality is how long do people want stand there and listen?  The dichotomous bigger picture is one of the hardest things to reconcile.  She’s running around, playing with American Girl dolls and begging me to iron her latest perler bead creation while looking up Grumpy Cat memes on Pinterest and trying to talk like a minion.  She’s crazy and fun and one of the shyest kids you’ll ever meet.  But for 8 weeks this summer, she was also sporting a massive IV line in her upper right arm (called a PICC line – Peripherally Inserted Central Catheter) that we used to deliver high doses of antibiotics designed to kill the bacteria that live and thrive in her lungs.  Now, “normally”… this process is a 2 week stint in the hospital and then life goes back to status quo…. but we all know this is Ally, right???

Over the last several years, Ally has been maintaining her own level of status quo.  She does several rounds of oral antibiotics until we move on to the more intense, IV delivered ones; she’s no longer a 2-week and done sort of gal…. she’s a high achiever needing at least 3 or 4 weeks, usually (although this current round was excessively longer).  We do several (3-4 breathing and vest) treatments a day on the regular.  She’s still getting 17 medications a day, on average.  She remains entirely dependent on g-tube feedings; some because of her hesitation to eat orally and some because of the stability offered by delivering a specially formulated diet that has the right ratio of protein to fat to help control the level of ammonia in her bloodstream that her liver is no longer able to process (from the shunt we had placed several years ago).  Even with the special liquid diet, she struggles to gain and maintain weight (especially while sick) and remains on the short vertically challenged side.

We had her PICC line pulled just yesterday, as 8 weeks is a long time to have one and it made her infectious disease docs very nervous.  Also, trying to avoid forming a blood clot…. so while she’s not 100% better in terms of her lung health, the risks were starting to far outweigh the benefits.  We switched her over to some oral antibiotics; the caveat being that they make her far more sensitive to the sun.  Always seem to be trading one issue for another.  But on the upside… she can now get in the pool (and avoid the sun?!??!?!).

There has been a lot of buzz about the latest and greatest CF drug – Orkambi.  Orkambi is the 2nd in a class of drugs designed to help control CF at the cellular level.  Kalydeco was the first, and has shown tremendous results in those with a particular mutation of the disease (about 4% of the CF population).  Orkambi is a combination of Kalydeco with another drug that works on the most common mutations of the disease (of which Allison has), and while a very far cry from a cure, it’s the best thing we have to help control the hallmark symptoms of the disease. (Orkambi has not been shown to be as impressive as Kalydeco with symptom reduction, but has demonstrated a significant decrease in the number of pulmonary exacerbations – and that’s awesome).  That said, there are a few parameters that qualify you to be able to take this “miracle” pill – you must be 12 years old.  For Ally, that point is basically moot as she’s less than 3 months away from turning 12.  You also should have good liver health.  Well…. her docs are concerned, but still willing to forge ahead and try it, with lots of monitoring.  Finally, it’s contraindicated with a few other drugs she’s currently taking to battle the war being fought in her lungs.  So… we have months before we are even able to contemplate her taking Orkambi.

Well… and there’s that small matter of cost.  Orkambi will cost $259,000… a YEAR!  Yes…. it’s math time!  Get out your calculators, folks… a typical dose is 4 pills a day – which comes out to $21,583 a month, approx $719 a day… and about $180 a pill (likely the most expensive breakfast you’ll ever have – $360 – before you’ve even gotten to the smoked salmon eggs benedict topped with caviar).  Yes, insurance and patient assistance programs will cover the vast majority of this, but for those working in small business, the hit on pharma costs could be devastating.

This fall, Allison will start the 6th grade… I have two middle schoolers!!!!  As unfathomable as this may be, we are so proud of her scholastic ability and not using her CF as an excuse to slack off.  She’s a stellar student and a great friend.

It’s hard to decide what to share, or how specific on the details to be… there’s a fine line between providing information and sounding like we are complaining about the demands that CF has put on our lives, not to mention exposing the vulnerable parts of her/our life.  There is no room for defeat, nor do we ask for pity.  We simply do what we need to do to get done what we need to get done while we continue to live life to the best of our ability.  There are molehills and there are mountains; we keep our hiking gear handy.  Ally’s just like every other kid out there… with a few extras.  So when you ask how Ally is doing… be prepared to pull up a chair – this is going to take a while.

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