July Update

I know I've been terrible about posting recently, but it's been difficult with all that's been going on.  Since May, we've had our Great Strides walk, Sienna's first birthday party, a family vacation to the beach, and we are in the process of buying a new home/selling our current home.  It's been busy, and my blog has been neglected because of it.

It feels like it's been a while since we've been to clinic...which I guess is a good thing!  Sienna currently sees her team every three months, unless she's sick and needs to be seen sooner.

We were last at clinic early April during the long stretch of cold and viruses she managed to pick up in daycare this spring.  At that appointment, while sick, her lungs checked out great and we got the OK to scratch our May appointment and push our next visit to July. 

Last week we met with her Pulmonologist and Nutritionist.  I had a long list of questions as always, which I'll share with her Pulm's answers should they be helpful to other CF parents:

1) Are sprinklers safe to use?  Yes, as long as it's hooked up to a clean source of water (such as city water).

2) Are lakes safe for her to go in?  This is a gray area that her doc is not comfortable with.  For now, no.  We should avoid taking her in the lake.  Keeping her healthy during her young years is imperative and lakes are known to harbor bacteria harmful to CFers, such as Pseudomonas.  

3) With how hot it is outside, is there anything we should be doing to ensure she stays hydrated?  Adding salt to her water/bottles/food and making sure she always has access to water is all that we need to do.

And my big question...why don't we have a concrete diagnosis, even with known mutations and a positive sweat test?  His answer, she's symptom-less with only one (known) disease causing mutation.  He refers to her as "the one who has CF but doesn't."  She is followed as a CF patient, exists in their database, follows their preventative care routine, but yet, they won't actually use the medical code for Cystic Fibrosis when billing.  A bit frustrating (or maybe it's infuriating) if you ask me.  You would think we'd be happy with this lack of official diagnosis, but we aren't.  It could stand in the way of potential medicines and medical devices being covered by insurance.  It could stand in the way of her getting a drug like Kalydeco™.  How funny to want to fight for something so negative.  A dreaded diagnosis.  But as a parent, we have to.  Because without it she can miss out on the care she needs or potentially a life saving medicine.  I've posted about the price tag of Kalydeco™ before; it's roughly $300,000/year.  We NEED insurance to cover it should she be a candidate to receive it - which she very likely could be, as it's shown to work on those with residual CFTR function (indicated by a low sweat test number).

For the good news, our munchkin had a growth spurt in height, measuring 29 inches.  She is still weighing in around 20 lbs 5 ozs; which is about the same as her weigh in at her one year wellness appointment.  I think she's somewhere around the 50 percentile for both height and weight.  As long as she continues to grow her team is happy.  How couldn't they be looking at these adorable thighs.

Let's read this one!

Passing the time with some "reading"