Four Years of Ezzy: Year 3

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Year three of Ezzy’s life was a lesson in making peace. All of her first year we thought “if only we could get her GI system working better.” And then during her second year we thought “if only these seizures would stop” …and “if only we had a diagnosis” and “if only her GI system was working better.” Early in year three we had all of these things. The ONFI was controlling Esmé’s seizures fairly well–she was clustering once every one to three months instead of every week. This felt like a vacation. We had a diagnosis, we had a “community” that we fit into. And in March Ezzy had surgery to fix her original anti-reflux surgery from 2011 (fundoplication)–which had become increasingly problematic, creating a giant hernia from about of a 1/4 of her stomach which had moved into her chest cavity.

But then she was hit by the year of infections…it was a constant barrage of sickness–often unspecified sickness–with constant high white blood cell counts. At the same time, in typical Esmé fashion, despite these infections, she was making gains in mobility and communication–seeming to understand much much more of what we said to her…and her gains inspired a few gains in the lives of the adults around her.

Here is Ezzy in her Tiny Superheroes cape!

Over this year she used a number of mobility devices. First was her Pony Gait Trainer (I wrote a whole post on the Pony that year):

Then, later on in the year, her Rifton Pacer (the link to the post about the rifton):

She also got her amazing adaptive stroller/wheelchair, which made being out and about much more fun for her, since it better supported her in a good posture. (By the way, she still fits in every single article of clothing you see in this picture a whole 18 months later.)

In March Ez had her fundoplication revision. This surgery was done laproscopically over 8 hours, in order to save the abdominal muscles she’d been only recently gaining. And although our insurance thought this surgery should be ambulatory (LOL), she spent a week in the surgical ICU courtesy of a number of alarming (get it??) desaturations, which we eventually determined were a more pronounced version of the breath-holds we had been seeing at home.

Not too long after Esmé’s recovery from surgery, we met a new friend, Paola, whose niece has PCDH19 Epilepsy and who is involved in the oldest PCDH19 Epilepsy foundation, Together for PCDH19 Research, located in Italy. Soon after this meeting, we launched our foundation for PCDH19 research: The Cute Syndrome Foundation, in Esmé’s honor.

Although we’d been able to manage most of Esmé’s infections at home with the help of her nurses (and that wonderful feeding tube!). In May she wound up in the hospital with a nasty UTI…But, still with the smiles…

In September Esmé met two other little girls with PCDH19 in NYC for a photo shoot with Rick Guidotti from Positive Exposure. Check out the TCS Foundation video that we put together from those photos. It was such a lovely experience.

Ez spent a lot of the year sleeping off sickness:

And exploring new things–since she was better and better able to concentrate:

We helped establish and celebrate the first annual PCDH19 Epilepsy Awareness Day on November 9th, 2013. Ez really enjoyed the purple balloons we used to celebrate!

Soon after I completed my dissertation after six and a half years–two of which were spent on some sort of modified leave. Ezzy and I celebrated my defence with a trip to Boston for more doctors visits!

In December Esmé was placed with her I Run 4 runner, Lori. Lori runs and exercises “for” Ezzy…it is a kind of mutual support and inspiration system. Lori sent Ezzy a 5k medal…which she was pretty excited about.

The biggest news of 2013 was, in my humble opinion, Esmé being able to sit herself up. This ability changed her entire view of the world…and inspired her to move more, to command her environment more, and to focus in new and very exciting ways…ways that lead to the amazing year that followed!