The Wait

Since Avery was a tiny newborn, and we had our very first exam under anesthesia - the one with two eye surgeons, who we'd come to know as two of the most important people in our lives - we have feared being in this situation.  That day they explained to us the ins and outs of Peter's Anomaly.  As fundamental as what a cornea, retina and iris even are.  How we'd be living on a roller coaster with this disorder all of our lives, and no matter how good things were going, they could change in an instant.  The different scenarios of how things could go.  What constitutes an "eye emergency."  Since that day, Avery has been through the gamut of surgeries - 8 cornea transplants, countless glaucoma and "maintenance" surgeries, and as of two weeks ago, approximately 112 times under anesthesia.  We've seen moments of pure despair and defeat, wondering if she would ever have functional vision.  We watched our community of other families with Peter's Anomaly kids go through the same things - and often, worse things.  We felt their pain, and prayed it would never be us.

In the back of the mind of any parent in our shoes is the possibility of retinal detachment.  It is particularly serious for kids with certain eye abnormalities and disorders, like Peter's Anomaly, because the structure of the eye isn't typical or healthy.  No working retina or optic nerve means no vision.  For the average person, retinal detachment is still a big deal, and should be treated immediately, but for Avery, it is one very small step away from permanent vision loss in that eye.  In our world, it's the most serious thing to happen as far as her eyes go.  Even though it was explained to us from Day One, and even though it's always in the back of our minds - the reason we teach her Braille, and we learn Braille, and we mentally prepare for transitioning to total blindness - it still hits you like a ton of bricks when it happens.  I never thought we'd deal with it so soon.

Three weeks ago, during one of our normal, daily patching sessions (which entails putting a blackout patch over Avery's good eye to make her use and strengthen her weaker (right) eye), I noticed she wasn't using that eye at all.  Since complications from her last cornea failure, and newest cornea defect in that eye, it's gotten extremely lazy, and therefore hard for her to use.  But that is why you patch - to remind the brain that it's there, and hopefully avoid a nasty muscle surgery.  She complains constantly when we patch, but she at least uses the eye.  But this day was worse than normal, so I started doing some of my "tests" to see what was going on.  I bring up pictures on her iPad of things she definitely knows: colors, cartoon characters, etc., to see if she's registering it in that eye.  She didn't get one right.  I tried again, silently panicking inside.  Nothing.  I frantically called John, who was ironically on his way to the Ophthalmologist's office anyway to pick up more patches for Avery.  He spoke to her doctor about what was going on, and they decided she needed to be seen under anesthesia that very week.  Now, I'll admit, I second-guessed myself...selfishly because we had a trip up north with our friends the next day that we'd been looking forward to for months.  But deep down, I knew something was definitely wrong.

It was on my mind all week, and even though we were able to escape for one day to be with our friends, I couldn't stop thinking about her and what could be going on.  The day of the exam, they had to do some other procedures on her defective cornea, but it still felt like it took forever.  In the waiting room there were, as always, plenty of distractions.  Especially the parents who I overheard discussing a port implant surgery for their daughter with cancer.  I tried to focus on that.  No matter what happens, it's not cancer.  But I was still scared as hell, praying that it was nothing and we wouldn't be taken into that "little room" where they take all the parents when something is not appropriate to be discussed in the waiting room.  Sure enough, our doctor came out, looked around, and suggested we go into the "little room."  I've been through six brain surgeries and 18 eye surgeries, and managed to stay out of that damn little room.  This was our more positive-outlook, optimistic, laid-back doctor, so when he told us that Avery's retina had partially detached - the news that we have dreaded like our worst-eye-nightmare for 5 years - it sounded less of a blow than it really was.  But our more negative-outlook, realistic doctor had his own spin on it, that more appropriately matched the panic that was going on in my mind.  Regardless of their outlooks, they both agreed it needed to be taken care of by a retina surgeon (who we have seen before for other posterior eye issues, thank God) IN ONE WEEK OR LESS.

Our sweet doctors were very sympathetic, as they saw me get emotional in public for the first time in almost five years.  They must have given the wake-up nurse a heads up, because she came in ready to comfort me.  This is one of the many nurses that we've known for five years, and the kind that put themselves in your shoes.  She told me how she always keeps her patient's stories in mind when things come up in her own life that seem bad or unfair.  She even cried with me.  Despite everything that went on that day, I knew we were in the best place.

Fast-forward to today, which is 12 days later.  We have not had the surgery yet.  It's tomorrow.  To say that I'm a ball of stress is an understatement.  Since we left the hospital that day, John and I started daily phone calls with all three doctor's offices, pleading to get in immediately for surgery.  But with our great luck, this emergency happened to fall during the most inconvenient week of the year for retina surgeons.  Of course.  The star doctor is one of the best in the country for Pediatric Retinal surgery, but that means not easy to get into.  He couldn't get Avery in until the 2nd, which would be 13 days from the original date we found it.  Unacceptable. Nope, not going to happen.  So, we went to his colleagues next - out of town.  "What about another hospital?" we asked next.  The only other option in Michigan isn't considered the best option for Avery's case, but even so, they too - OUT OF TOWN.  We'll go anywhere - Philadelphia, Duke.... I had my trusty band of Peter's moms online giving me recommendations for surgeons all over the country.  Our doctor understood our concerns, but felt it was the best decision to hang tight and wait for the 2nd.  We were put on a wait list for sooner, but we all know how that goes.  I still held out hope, every single minute that passed, hoping they would call and get her in sooner.  But here I sit, on September 1st, in no better a spot than I was last week.

Fortunately, Avery doesn't show any signs of pain or discomfort.  This helps immensely with our sanity.  If this were a brain issue, we'd be far worse off.  But we know the possibilities and outcomes of this impacting her future.  We know that if it's gotten worse over the last few weeks or they cannot somehow fix it, that Avery's right eye is as good as done.  There's really no coming back from a retinal detachment that isn't fully repaired.  Even if they do repair it, our future is now even more difficult than it already was.  We don't have a "good" eye to rely on as a back-up.  Her left eye is a tad stronger, but it too can go at any minute.  Her vision in that eye is poor - functional, but poor.  Believe me, I've seen it all, and heard all the stories and I know that we should consider ourselves lucky to have any vision at all, but that doesn't change the pain of thinking that your child's vision, which you've worked so, so hard for, is slipping through your fingers.  Even though she hasn't missed a beat these last few weeks, I know that she could suffer for the rest of her life because of this whole nasty complication.

This post has been negative, and although I try to keep a positive perspective, it's not realistic to keep that 24/7.  I thought my next blog post would be about Avery starting Kindergarten next week, because, oh yeah - that is supposed to happen.  I was just starting to wrap my head around that craziness when this retina monster came in and bulldozed over everything.  The best case scenario is that it didn't get worse, and this surgeon can repair it.  We'll still have to add it to our list of things to watch, but hopefully her vision can be restored.  And we can enjoy our fun plans for Labor Day weekend, and send her off on her first day of Kindergarten next week.  Yikes.  As always, we rely on your positive thoughts and prayers, so thank you for those.  We have definitely been through worse, but not with her eyes, so we are sad and scared going into tomorrow.  I don't think we've ever had to wait this long for a surgery that we had so much riding on it.  Please think of Avery tomorrow, and please hope and pray for the best.  Thank you,
Ann