Living the Unexpected

I've been dragging my feet on writing this blog entry.  If you have spent any time reading posts on Hazel, you'll probably recognize that there aren't many instances where I am posting solely for the purpose of an update on how well everything is going.  Mainly it's because our life is moving at a pretty frantic pace right now and it's easier to cover the things that are going on in chunks instead of short bursts of information.

It's been about a month since Hazel had her most recent eye surgery and to be honest, her eyes are doing pretty well right now.  I mentioned last post that Hazel has her own tiny sunglasses to help with her sensitivity to bright lights and Aubree is taking her in on Friday to get a prescription done for her glasses.  We are still doing steroid drops four times each day in both eyes...but that seems like nothing compared to what she has needed in the past.  Hazel's most recent eye pressure check was on Monday, where both eyes registered pressures in the low-20s (that's a good range for her).  For the first time in her life, Hazel doesn't have an eye appointment for TWO MONTHS!  I wish this meant that Hazel was getting a break from doctor appointments and medical attention....but that's not the case.

The only reference we have for Chromosome 6 deletions is a comprehensive document on all the known active cases in the world -- which is 45...well, 46 counting Hazel.  In this document (linked here) it explains everything we know about C6 deletions and issues it can cause.  One feature of this deletion is that two-thirds of the people with this deletion experience some level of hearing loss.  To this point, the assessment of Hazel's hearing has been a bit of a roller coaster ride.  She failed two hearing screenings when she was born, had an inconclusive hearing test when she was a few weeks old, then had a completely successful detailed hearing screening in November 2013.  On July 14th, Hazel went to the ENT for Children to have tubes placed in her ears to drain excess fluid that was affecting her hearing and to have another detailed hearing test.  The procedure was a quick one and went really well... but the hearing test revealed that Hazel has suffered nerve damage and has "moderate hearing loss".  We are not 100% sure at this time if the hearing loss is permanent or the root cause of the nerve damage.  The top two theories are that the excess fluid in her head has been applying pressure to her nerves for too long or that this is related directly to her chromosome deletion.  Either way, she has been fitted for tiny hearing aids and will need them immediately to help avoid a delay in her speech development.  Aubree and I have also discussed learning sign language to help with her development or if she doesn't have her hearing aids in a noisy environment.

To this point, Hazel has had an MRI on three different occasions and we've determined that she will also need a tube shunt in her head to alleviate the fluid that has built up.  The neurologist and neurosurgeon believe there is a possibility that her hearing could rebound from the nerve damage that has already occurred -- but who knows.  The official date has not been set, but the surgery will be at the end of August and will unfortunately require a short hospital stay.

I'm trying my best to stick to the facts about Hazel's health issues...but it's hard not to feel like our family is being kicked while we're down.  This month was supposed to be the first time in a long time where there weren't any surgeries, we thought there would be less doctor appointments, more positive news instead of getting blind-sided by more health issues.  Guess that is just not in the cards right now.  I guess this is just life.

It would be hard enough to get Hazel to leave glasses on her face without ripping them off....so I'm not sure how we're going to get her cooperation when we also include hearing aids.  Our plan is to start both at the same time so that hopefully she will just see this as a single change instead of adding two different things.  Unfortunately, the hearing aids will need updated ear moldings every month or so to keep up with her growing ear size....which means more scheduling appointments, time, money, etc...

I know everything is going to be fine in the long run, but the relentless nature of Hazel's health issues doesn't seem to be letting up any time soon.