The club we don't want to join

I feel like a broken record saying this but it looks like Avery's going to have to face yet another surgery sooner than we thought.  We had a visit with Dr. Sood, her neurosurgeon, this morning, and due to the size of her ventricles, and the fact that she's been getting randomly sick from pressure in her brain since January, he feels a shunt revision is necessary.  Even though this issue has kept us up at night (literally) for months, we both had hoped he'd say, "just keep doing what you're doing - everything is functioning fine."  Instead, he showed us the most recent scan of her little brain, pointing out that her ventricles (which hold the fluid in her brain) are larger than he'd like.  He explained that the shunt is draining too slowly and a clog is most likely the problem.  The clog is probably tissue forming around the catheter in the shunt.  Tissue forming around important objects in Avery's body is something we're super familiar with... it happens with the little shunts in her eyes, and around her cornea grafts.  It's never good and it's never preventable.  Gravity has been helping her thus far (being elevated when she sleeps), as well as us manually pumping the valve every night and early morning.  However, he believes a serious clog is inevitable, and we're worried we may end up in the ER if we let it go.

A few months ago, while in the waiting room at Children's, we heard a mom referring to her daughter, who was in surgery for a shunt revision, say to Dr. Sood, "we don't want to become members of the 'Shunt Revision Club.'  Basically, some kids get shunts and go years and years without needing a revision, and their revisions are easy and totally successful.  There are other cases, though, where revisions happen multiple times a year, because doing one causes problems that result in needing another.  Each time they do a revision, it requires cutting into her head and a minimum one night stay in the hospital.  After a revision is done, for one year following, there is a 30-40% chance that something will go wrong with the shunt.  These don't seem like horrible odds, but if you know our history with odds, we have reason to worry.  Like always, I hope for the best, but expect the worst.  The Shunt Revision Club seems like the worst.

So, again, I have that numb feeling that is so familiar after receiving information like this.  We are faced with putting Avery through more pain, more anesthesia, more time off school, and more risks.  We have to face a very real possibility that while we are bringing home our second child this fall, we may be dealing with Avery's health issues at the same time.  Two or so years ago, this is news that I'd flip out about.  I'd be inconsolable and hide under the covers for a few hours, then be angry with God for awhile.  Today, I am sad, I am disappointed and scared, and I am angry at God, but I'm handing it.  I'm in control of my emotions more these days because I've had so many experiences under my belt to know that losing my mind will get me nowhere.  I can worry all I want that this surgery will cause more problems than we already have, or that we'll be carting our newborn to the hospital to visit Avery.  But worrying about things we cannot control is useless.  We can be proactive and bring up concerns, which is how we got in his office this morning in the first place, but we can't dwell on the unknown.  I am typing this to also try and convince myself, honestly.  It's hard to believe these words.  I am 110% worn out, and tired of bad news every other week.

The good this week was the fact that Avery started communicating the words, "on" and "off."  She's tried these words in the past, but now she's using them correctly and consistently.  She is so proud of herself and this morning, the first thing she did when she got up was point to my lamp and say "ah," (which is "on").  It's such a small little word, but means so much progress.  And "progress" is a word that Dr. Sood brought up this morning.  He asked us, as he always does, about her development.  We explained that it's still slow, but it is not regressing.  He did confirm something that he has only alluded to in the past.  Something that John and I both figured she had, but never heard him say for sure.  She has a mild case of Cerebral Palsy, or CP.  In some way, it's a bit of a relief to put a reason with the problems she has, but it's never fun or easy to hear your child has a diagnosis like this.  It is mild, and we know that it could be so much worse, but it's hard to swallow.

Please continue to think positively for her ... days like this, I wonder if praying and positive thinking even work, but I suppose that things may be much worse without it!

Three Years Old...Already

Happy 3rd Birthday, my sunshine! I know every mom says this, but cannot believe three years have passed so quickly.  I am sad that you are not feeling your best this week – I wanted so badly for you to enjoy your birthday totally healthy, and even though you won’t remember (which everyone keeps telling me like that makes me feel better), I am sad for you that you have to go through this stuff.  You have had a lot going on with your brain and your eyes this year (and it’s only April).  As always in your little life, when it rains, it pours.  But, also as always, you’ve handled it like a champ – never knowing what you’re missing or what you should be feeling like.  You feel better for one minute and you smile as if it’s been okay the whole day.  And that’s what I wish someone had told me three years ago, when I was trying to cope with your diagnosis and imagine how your life would be.  I wish someone told me that you would handle it, smiling like a trooper.  I am so proud of you and so proud to be your mom.  Now let’s go have a fun day…and some cake and ice cream :)

Avery’s Light

I started this blog in November 2011, but like most moms, time is a shortage, and more important things came up.  I thought what better a time to restart it than her third birthday, especially since she has so much going on.  This will be used to update interested friends and family on her health and progress, as well as to explain various things about Avery’s conditions that people don’t understand.  And if nothing else, and no one reads this, it will still serve as a place for me to vent.  I apologize ahead of time for rambling (since I’ve waited 3 years to start, there is a lot to catch up on), and for the next 5.5 months, I apologize for overly-emotional entries, as I’m dealing with pregnancy hormones J  Thanks for reading!

Reflection and a quick recap of her journey so far…

As we sat in that hospital room yesterday, waiting to go back for what felt like the thousandth exam under anesthesia since Avery were born, I couldn’t help but think about the last three years and how we got here.

We were awaiting the status of Avery’s 6^th and 7^th donor corneas, and I felt like I was hanging by a thread – emotionally, mentally, physically.  We had started our day on such a bad note – waking to the sound of her getting sick from the pressure in her head, and feeling that helpless feeling because there’s nothing we can do to prepare for it or stop it.  In addition, the three of us haven’t slept a good night’s sleep in months (due to middle-of-the-night drops and repositioning her sleep position), making us edgy and exhausted.  Oh yeah, and I’m 15 weeks pregnant, sometimes wondering, “what were we thinking?!”  To say the least, we are tired.  A person can only take so much, and it’s been a lot.  But, just when I think I can’t take any more stress or bad news, more comes our way and we handle it.  Or we get a piece of good news, and use that to get through the bad stuff.

Like so many times before, our concern yesterday was not only the status of her eyes, but also the status of her brain.  Days like this, I am so angry.  I want to blame someone for making my daughter go through all this pain and discomfort, especially the day before her birthday!  We were in the hospital on her 2^nd birthday – why again?  I probably am grumpy and short with the nurses and 20 people that come through our room asking the same questions over and over…”when’s the last time she had something to eat or drink?, “How does she usually do with the anesthesia? “do you want to carry her back to the OR?”  But, I soon realize that I have to snap out of it, and carry on with the tasks at hand because this is our situation and there is no running from it.  Thankfully, John was able to take off work and come with me.  When she woke up vomiting and lethargic two days in a row, and my pregnancy hormones and nerves were fried, I didn’t think I could handle taking her alone, which is what I’d normally do. 

We talked to Dr. Sood’s (Avery’s neurosurgeon) assistant, and he recommended we start manually pumping the valve in her head – which done by compressing a little “button” just under the skin, behind her right ear – consistently, every night before she goes to bed, and right before she wakes up.  If this doesn’t do the trick, we need to replace the valve in her head.  A surgery that opens the door for other issues.  But again, there are little options, and this is the life of a patient with hydrocephalus.  Again, anger overcomes me as I think back to the reason why she has this condition in the first place.  The result of poor care from her first neurosurgeon when she was a newborn.  This is a pain and an anger that I will never get rid of.  I don’t think I’ll ever get over this incident, as it caused my child a lifetime of health issues, surgeries (four so far on her brain) and developmental delays.  When she’s already got enough on her plate.  More on that another time. 

So, check off “brain” on our list, now onto the eyes, as Dr. Dunn (her cornea surgeon) comes into the room.  At these exams, although I’ve become somewhat jaded throughout these last three years (I don’t freak out anymore about anesthesia, IVs or saying goodbye in the OR anymore), I still get that familiar pit in my stomach waiting to hear if everything looks okay.  I have heard from Dr. Dunn and Dr. Roarty many times at EUAs and surgeries that “things are looking great,” only to hear a month, three months, eight months later that her glaucoma has drastically spiked, or her valves are clogged, or her corneas have failed.  It is hard to get excited when you hear “things look great” when you know that can change tomorrow.  But I remain hopeful.  I have to – if I look at our history, there is no other option in our situation than to remain hopeful.  And yesterday was one of those times – kinda.  Things looked “great” in the left eye, but the right eye has a small defect so he needed to once again stitch her lids partially closed.  This is called a tarsorophy, and it’s an evil thing in my book.  It makes putting drops in a nightmare, it makes her sore to the touch for several days, and it narrows her line of sight.  But, he insists it’s for the good of healing her grafts, and again, we have no choice. 

I can still clearly remember waiting to hear the status of her very first cornea, almost three years ago.  At that time, she was 7 weeks old, lying in a hospital bed of the Pediatric Intensive Care Unit, fighting a life-threatening meningitis infection in her brain (the aforementioned “incident” I’ll never get over).  To this day, saying those words is still unbelievable.  We had been through so much already, and sat there about to hear that not only was that graft not doing well, it was probably toast.  At the time, we thought this was the end of the world – “what?! It failed??  This was going to last her forever!”  We had to get through this naïve period as every parent with a Peter’s Anomaly child does.  The day the doctors formerly diagnosed Avery (at just over a week old), they told us that Peter’s would be a roller-coaster ride – lots of ups and downs, unpredictable turns and twists.  She was a severe case.  And oh are they right. 

Since that first failed graft, we’ve seen Avery through 13 eye surgeries, ranging from more cornea grafts, implanted valves to control her fluid, cauterizing her fluid membranes, sucking out the excess vitreous fluid in her eye, removing both her natural lenses… and more cornea grafts.  The domino effect of glaucoma caused most of her problems, as well as her body attacking the foreign grafts.  And the worst part is, there is little we can do to prevent any of the bad stuff from happening.  We are vigilant about drops – we’ve put multiple ones in, everyday (often every couple hours) since the day she was born.  We go to great lengths to make sure she doesn’t rub her eyes, scratch her eyes, get an infection, etc., we listen to the doctors, cart her back and forth to appointments and exams, living at the mercy of this disorder, but still, many issues have arisen.  And will continue to arise, for the rest of her life (or until they find a better treatment).

This is the hardest part of Peter’s to explain to friends, family, and that curious stranger in the grocery store.  She will never be “fixed.”  Yes, it’s great that this surgery is over and went well, but that is just the beginning of a very long road.  Why so many of the same surgeries?  Are you sure you don’t want a different doctor?  It’s the nature of the disorder, and our doctors are doing an excellent job.  Avery is lucky to still have sight in both of her eyes, which is more than many Peter's cases have.  We never know what’s around the corner for Avery and she can loose her sight very easily, no matter how well she appears to be doing today.  Her vision will most likely never improve from where it is today either, which is estimated at 20:400 with her glasses on, and 20:1000 without them on.  The average person can easily achieve 20:20 vision (or close to it) with glasses or contacts, if not naturally. If you or I had Avery’s vision, we couldn’t function, but it’s all Avery knows.  Her view of the world is extremely blurry, distorted with almost no peripheral vision and almost no depth perception.  She bumps into a lot of stuff, needs us to put toys, spoons, etc. right in front of her, and often times, she holds things right up to her eye to see.  She doesn’t see farther than about 6 feet, so she won’t spot you across a room if you don’t make noise.  She is learning to walk with a cane so she doesn’t trip over everything in strange places, and she will likely never drive a car.  And developmentally, well, that’s a whole separate but very large challenge for us. 

Her vision impairment, the amount of surgery and hospital time, and her neurological issues from meningitis have made a major impact on her development.  She has been delayed on every “milestone” from day one.  She cannot sit in a crowd of her peers, i.e. “average” three year olds, and even compare.  She is chronologically 3 years old, but much younger in terms of communication (she doesn’t talk), interaction with others, some physical movements and completing daily tasks.  So not only do I stress about her health issues, I stress that she’s so behind, and how that’s going to continue and affect her as she grows each year.  i.e. How do you potty train when your kid can’t tell you she has to go potty?  Or, will Avery even realize she has a baby sibling next fall, since she doesn’t seem to interact with other people besides her close inner circle?  These are things I was terrified to hear when she was born, but I’ve learned to accept.  It’s very sad and unfair, but we will deal with it and she will have a very happy and normal life.  She was enrolled at four months of age into a program that helps us with these things, and today, she is in a wonderful special needs pre-K where she receives therapies for each of her needs.  This program and the therapists and teachers have saved me from going completely crazy on many occasions, and they assure me that she will be okay.  They have taught us how to teach Avery simple tasks, in the early months things such as rolling over, sitting up, grasping things, turning toward light, responding to music, and now - communicating that she wants more of something, walking, going down steps, getting around new places, etc.  Most kids pick these things up much more easily.  When we used to take Avery to another child’s birthday party, the park, or any place with other children for that matter, I would get so depressed, comparing them to Avery and how much more advanced they were.  I still watch other toddlers, who are six months to a year younger than Avery, run circles around her and talk in sentences when I can barely get my kid to say “mama.”  I won’t lie; it SUCKS.  It’s like a knife to my heart.  But, I’ve learned, I cannot compare Avery to those kids.  It’s like comparing apples to… monkeys.  Avery is Avery.  She faces her own challenges and goes at her own pace.  She will do so for the rest of her life, and all we can do is our best to teach her, help her progress, and love her for who she is.  And like I said, the good times and small successes get us through all the bad crap.  Like a few weeks ago, when her new clear corneas allowed her to see bubbles for the first time, or when her teachers tell me how well she’s doing at school.  When she makes a sound that kind of resembles the word, “on,” when she hands me a toy.  When her doctors are amazed how well she gets around and sees knowing what she has physically.  She has overcome an immense amount of challenges, considering all she’s dealing with.  And she smiles all the time, which heals any bad day.

Ok, I’ve rambled on enough for this entry.  I could go on forever but I’ve probably lost most readers by now.  There’s more to the story for sure, but I’ll save that for other entries.  Thank you for listening, for your love and support these last three years and for your continued love and support.

Have a great 4-13-13!