Scary January

January is a difficult month for me. Reviewing our "health track record," it's unfortunately our most unlucky month, and for the last few years, I can feel myself get tense the days leading up to the new year, lasting through Scary January. It's a reminder of all the scary times we have lived through in Januarys-past. And by scary, I mean the worst possible scenario that any parent can face. We have come too close to losing Avery more times than should be possible, and many of those times fell during this month. To anyone else, it may seem like time would heal these fears. But as her mother, and at times the only eye-witness to many of these past events, I think I'll be forever haunted during this time. (Not to mention all the times in between.) Three years ago today, on January 16, 2015, I was thanking God for letting me bring Avery home. I found her seizing and unresponsive in her bed, and after a scary ride to the ER, they found that her blood sugar was at a critical 17. You never forget a doctor telling you what would have been if you didn't find her when you did. That scary day also began our journey with epilepsy, which has by far been the most complicated and difficult of Avery's diagnosis. The very next January (2016), we were rushing Avery to the ER yet again for status epilepticus, which as you know would end up to be her longest and most complex hospital stay to date. The effects of that time are with her to this day, and although she has been fighting like a champ, the struggles are constant. And one of those struggles is my own. It involves getting through this time of year without totally losing my mind.

It obviously helps that we have been incredibly fortunate over the past year, as well as now, in the midst of one of the worst reported flu seasons. (I type this as I fervently knock on wood.) But when you live life like we do, you are always waiting for the other shoe to drop. You are peeking your head out from under the covers, waiting for the monster to jump out and grab you. And there are a few monsters. Even though Avery has been doing so well, and we haven't made a trip to the hospital in almost six months, that doesn't mean there is less fear. Because we know that it's inevitable. She is going to catch something again, she is going to have another medical issue. So the question isn't 'if,' it's 'when.' And that has been a heavy burden to bear at any time, but even more so when you are reminiscing this unlucky time in years past. Do not mistake this for negative thinking or hopelessness. This is realistic; albeit, enhanced by anxiety and a little PTSD in January, but realistic. While I am grateful for every single healthy day that Avery lives, and I love nothing more than witnessing her grow, learn and progress as much as she has each day since her last major issue, I know that there will be another monster, and it's impossible to ignore that.

What I am continuing to work on each day is living with these fears, while not letting them take over. I know they do more often than I'd like, but I also know that in many instances, they are warranted. Like the aforementioned flu season. I am terrified to leave the house during seasons like this, because as most of you know, if Avery catches the flu, we are almost certain to be heading to the hospital. But I see how well she does when she spends time at school, and doing the things she loves outside of our bubble at home, so my intuition tells me to let her go. I rely on that intuition, and her support at school to keep her as safe as possible. Oh, and Clorox and Purell... I rely on them A LOT. I should buy stock in those. The flu is a unfortunate part of life that is made scarier by having complex medical issues. We know that it is impossible to completely avoid, but we do everything we can to reduce our risk. So never be offended if I decline an invitation during flu season, or if I'm constantly wiping the kids with anti-bacterial wipes. It's necessary, and ensures our germs stay contained as well. 

Back to Avery doing so well... She really is. I often pinch myself because it's incredible to look back at where she was three years ago and two years ago to where she is now. Talk about proving everyone wrong. I am hopeful for her future, but vigilant and realistic. I always strive to take one day at a time, because we know that looking too far ahead is more overwhelming than beneficial. 
Everyone stay safe, stay healthy, and if you're sick, please try to stay home <3

January 2016, on a rare alert moment between fighting constant seizures

Perspective

After what has seemed like the busiest month ever (although I feel like I say that almost every month), our day today made all the chaos stop for a moment and things just went right.  And that gives me the space to step back, evaluate the path we're on, and regain the perspective that I often lose along the way.  When I compare this year with where we were at this point last year, it's a no-brainer that we're in a completely better place.  I wish I could go back and tell myself back then where we would be today.  I would have probably slept a little more.  But knowing that you're in a better place compared to your last major struggle doesn't mean that you still aren't struggling.  Avery not being in the hospital is our ideal scenario, but even if she's home, her day-to-day life poses a huge list of challenges that often make me feel like I'm drowning.  She has been doing really well and we have mainly been managing the typical responsibilities that come with her medical issues, and some occasional hiccups.  But over the last five weeks, our life has been kicked into high gear once again with back-to-back doctor appointments and new issues to manage.  When you throw this into "regular" life stuff, like work and commitments already made, etc., it's not easy to say the least.  It seems like if we get out of the habit of being on the hamster wheel for even a few months, it's that much harder to get back on it.

Avery is currently being evaluated for some behavioral and development issues that we and her teachers are noticing.  It's nothing that comes as a surprise, but her CP and developmental delays, coupled with the damage that epilepsy - and the drugs she takes - are starting to pull her back farther from her chronological age.  Again, nothing really new, but hearing that your child is severely delayed in several areas is no picnic.  In addition, the physical repercussions of being in a hospital bed for six weeks, including a coma are still ever-present and she has been showing signs of weakness in her legs, and some other issues.  That specialist would like her to get into intensive PT and OT as well as wear some braces on her feet, so she was fitted for those this week.  They will not be the kind that go up her leg, so that's a little easier.  Lastly, her routine bloodwork she gets while on the ketogenic diet came back with some numbers that weren't great.  Expected for a kid on the diet, but still crappy to see on paper.  We had to add a few supplements and adjust her foods a bit, and we are hoping that helps bring the numbers into a normal range again.  The diet is the main reason her epilepsy has been controlled, so we need to stay on it while we attempt to wean at least one (hopefully more) of the medications she is on.  The fact that we give our kid seven drugs and five supplements every day is overwhelming - in terms of principle, time, and money, to be honest.  But at the moment, they are a necessity.  So, while none of these issues comes close to being the worst we've been through, the fact that they all came at once (coupled with parenting a particularly strong-willed three-year-old and a special needs child who is also challenging us behaviorally) has rattled me.

Today, though, was a good day.  Avery had her first session with outpatient PT after being on hiatus for six months.  We were lucky enough that her former school PT now works at a facility that does pediatric PT/OT, and it's FIVE MINUTES from our house.  Major score.  She is amazing and Avery took to her, and her exercises very well today.  Her typical reaction to PT is crying, so anything was progress, but how she did today far exceeded my hopes.  So that boosted my mood ten-fold.

We also got the chance to meet up with a family who we know through one of my vision impairment support groups.  It's an amazing mom who started an awesome resource/support page for parents of VI kiddos, and she's an advocate not only for her daughter, Cailee, who has a rare condition called FEVR, but an advocate for our  community as a whole.  She, her mom, and Cailee came to Michigan to meet with the same retinal specialist we see, because he's one of the top in the world.  She arranged for us to take a tour of Leader Dogs for the Blind (also based near us in Rochester Hills), and meet a few other families with kids who are visually-impaired.  The tour was so informative, but getting the chance to hug a fellow VI mom who has walked in my shoes is so rare, and so valuable to me.  We have spoken for awhile via Facebook, but nothing compares to meeting face to face, talking about your kids, and knowing the other person totally gets it.  She is seriously such a cool person, and Cailee is one of the coolest six-year-olds (besides my own!) I have ever met.  A kid who lives with the burden of deteriorating vision, yet you'd never know it by being with her.  One tough cookie she is.  A group of us met up later with our kids, and Avery had hit her limit by that point, so I brought Lucy in her place (who had the time of her life and fell in love with Cailee) and it was so nice to bond with a group of moms who can understand where I'm coming from.  Makes me even more motivated to make a meeting happen with the rest of my VI mom friends :)

So I regained some perspective today that I had lost over the last month.  Life is hard, and there will be varying degrees of 'hard' with each passing day, but every so often, you get good times too.  These break up the chaos and allow you to recharge your batteries.  Because, boy did I need that.
And one thing that sticks out in my head from the day was something the woman who led our tour at Leader Dogs said.  Her teenage daughter volunteers with blind kids and works some camps that LD puts on.  She was talking with one of the blind teenage girls about the things she wishes she could experience if she had sight.  She explained that she can't just grab her car keys and go wherever, and she had to rely on someone to help her with a lot of simple tasks.  The woman said this really struck a chord in her daughter; the thought of this blind girl desiring something that we take for granted.  She decided to get the word "perspective" tattooed in Braille, on the arm that she leads with (helping along a blind person).  The woman told us this story because she noticed my tattoo in Braille that says "persevere."  I was so touched that kids like mine can affect people so deeply, and how many people exist in the world that have a passion for helping others.  And being one of those people is my goal every day.

Meet Seedlings!

When Avery was about five months old, and we were just dipping our feet into the world of VI parenting, our TVI (teacher of the visually-impaired) suggested we visit the Children's Low Vision Resource Center in our area.  I will never forget that day, because it was one of the first doses of reality I received with regard to having a visually-impaired child and how different parenting would be for us.  I was still getting used to the fact that Avery was "different," and this place was overwhelming, scary and the day was extremely emotional.  However, it was the first time I realized continuing to be afraid of the life we were facing was just going to inhibit us from pushing Avery to her fullest.  We had been slowly learning about VI services up until then, but there were so many more resources, services and tools available to her, even as an infant.  All of these were things that would help her to be safe, functional and independent in this big, sighted world.  My eyes were opened (pun intended) big time that day.  While I was in tears for most of the visit, it definitely hit me that this is reality, and if I want to give Avery every opportunity that I can, this was the first step.

On our way out, the director of the center gave us a care package--some DVDs, a lot of pamphlets, ways to connect with other families, and even a VI/Blind "goody bag," with the phrase, "Hooray for Braille!" on it, and knowing me, I'm sure I sarcastically was thinking, 'yay - welcome to the party!'  The bag was from a place called Seedlings.  To be honest, I didn't pay too much attention to the source of the goodies at the time.  I definitely appreciated it as I went through the items - a baby book in Braille, a Braille alphabet book mark, information about Seedlings.  And my favorite: those alphabet magnets that every kid has on their fridge, only these had Braille on them.  The idea of learning Braille seemed so far off, and one aspect that I could still realistically lock away and not have to think about for awhile.  We were told that the alphabet magnets were hard to come by, so we did put those on our dishwasher, and tucked the rest away for "later."  In the months and year following, I would randomly hear about Seedlings here and there.  Early Intervention would bring new books for Avery and their label was on the back, I heard of their Book Angel Program, and signed Avery up for her free Braille book.  I was looking for Christmas gift ideas for her, and was directed to Seedlings for ideas.  We were urged to start running her fingers across Braille in the few books we had.  But I still kept the idea of Braille at a distance. 

Fast-forward about five years, and my relationship with Seedlings has done a 180.  Once Avery started pre-K, I was doing research on new ways to teach her, and I again stumbled upon Seedlings and learned more about the enormous impact they were making on children like mine.  This small, grassroots organization has an incredible backstory.  The concept was imagined by Debra Bonde in 1984, when she discovered how inaccessible and expensive Braille books were.  She knew the value of literacy and reading and it should start at a young age, so the thought of blind and VI children being denied this was not acceptable to her.  She started Seedlings in her basement, with a mission of making Braille books accessible and affordable for blind and VI children, as well as blind caregivers.  Her first year, she produced 221 books basically by her own hands. Today, Seedlings (a 501(c)3 non-profit) has produced nearly 500,000 Braille books, and gives more than half of them away for free.  Before Deb started, most Braille books cost upwards of $100, but Seedlings is able to sell their books for about $10 on average.  Their mission of increasing the literacy rate among blind and visually impaired children so they can grow up to be independent adults has proved successful year after year.  

I've had the privilege and honor of serving on Seedling's Board for the last year and a half, and it has been one of the most rewarding experiences since having Avery.  I've literally fell in love with this organization and how an idea to give every child the same access to literacy has turned into so much more.  Because when you think about it, I can take my sighted child to the bookstore or library, and she can pick any book she wants, and that is definitely not the case for my VI child.  While Avery still has some sight, that may not always be the case, and Seedlings allows her to have access to the same books her sister does.  It's a little luxury that most people take for granted.  So, looking back to that day when I first heard of Seedlings to now, I hope they know how much I value what they are doing.  Since Avery could turn pages, reading is pretty much her favorite thing to do.  She loves to flip through book after book, whether telling her own version of the story, or having us read to her.  And now that she's learning Braille, everything is coming together for her.  Seedlings has ensured normalcy in a piece of our lives, and for that, I am forever grateful.  And for me, it has turned something scary into something positive.

The purpose of me sharing this is because starting today, Seedlings is holding its first online auction fundraiser.  They've been able to get some pretty awesome items up for bidding, such as four 1-day Hopper passes to Disney World, an Apple iPad with keyboard, Lions, Tigers and Wings game tickets, meet-and-greets, and many different types of gift cards of varying values.  Every $10 raised makes another Braille book possible!  If you have a moment, please browse the online auction, now through November 14th, and hopefully you'll find something you need (i.e. lots of Christmas gift ideas that will also serve a great cause).  You can click here to get there:  Online Auction (www.biddingforgood.com/braillebooks)

Also, we have learned that the Children's Low Vision Resource Center in Royal Oak needs our help to keep its doors open.  This was the place that first gave us our care package, information and resources about the VI world.  These are offered to families at no cost, but the center is in need of help to keep this valuable program going for kids in our area.  I'll be sharing more info on how to help!  

As always, thank you for reading, and following along on our story!

Ann

As of today...

I haven’t posted in a while, so thought I’d give an update on our little peanut and how life has been.  Overall, Avery has been doing very well.  She’s had regular “hiccups” that keep us on our toes, but all-in-all, the keto diet and meds are doing their job in keeping seizures at a minimum.  Over the past month especially, she is progressing behaviorally, her physical energy is improving, and still hasn’t really regressed as far as baseline academics.  Her PT told me today that she has met all the goals they set back in March!  From where we started the year, she's moved some major mountains.  (And I’m superstitiously knocking on wood now.) 

Over the last six months, we have had some scattered ER visits, surprise procedures and too many doctor appointments to count.  Besides the relentless eye issues, our most frequent problem has been whenever she gets sick, and how that tends to open a giant can of worms.  Whether it's a serious cause or a run-of-the-mill bug, it most likely means a trip to the hospital.  Did she have a seizure, is it her shunt, is it her cortisol levels, is it from the diet?  We base our level of panic on how bad she’s acting.  But regardless, if she isn’t keeping her meds down, she can’t miss a dose, so needs to get them via IV.  Being sick affects her adrenal disorder, her hypoglycemia, and lowers her seizure threshold.  So basically, when she is sick, it’s chaotic.

She gave us a big scare in April, when she had a tonic-clonic seizure (this is the type that most people think of when they think of a seizure) the day after her 6^th birthday.  We were definitely caught off guard, so it was a humbling reminder of how unpredictable and relentless epilepsy is.  Thankfully, we caught it relatively quickly and gave her emergency medicine.  This was a major learning experience, as it was our first encounter with a “breakthrough” seizure since being home from her long hospital stay.  When Avery was in the hospital January-February, she had what is called intractable epilepsy, meaning, they couldn’t stop it with any traditional methods.  This can cause irreversible brain damage and even a stroke.  The induced coma plus the ketogenic diet were able to break the continual subclinical seizures, but she is by no means cured.  So, we are to expect that “she will likely have breakthrough seizures a handful of times per year*,” which is what our Neurologist told us to prepare for.  The asterisk being that this is just a minimum expectation – epilepsy follows no straightforward rules.  No two people are alike, and there are caveats to every prognosis.  She may have 3-4 a year, or she could fall back into intractable epilepsy tomorrow.  We have been told to expect anything.  There is scar tissue webbed throughout Avery’s brain (left from the meningitis infection), she has a foreign device implanted, effects from rising and falling pressure on her brain - in general her brain is classified as “abnormal” in terms of structure and activity.  These are all factors that contribute to the misfiring that is epilepsy.  Since most of her seizures were “subclinical,” meaning only visible if connected to EEG, we have had to learn about the other kinds.  Besides that breakthrough seizure in April, we are only aware that she’s had at least one Absence seizure, which are not convulsive, rather staring off and unresponsive, followed by conking-out-sleeping (my technical terms).  These were likely triggered by stress or heat.  At this point, there are not many options for combating her epilepsy.  She is not a candidate for surgery at this time, which is bittersweet when you’re in a refractory situation.  We hope that the medicines she is on continue to work, but the seizures often find ways to get around those over time.  Sounds crazy, and it is.  Same with the diet.  We are often asked how it’s going and how long she’ll be on it.  It’s not a definite, like everything else.  It is working right now, and she could be on it for 2-3 years, in the hopes that her brain will reprogram.  But our expectations are realistic.  We have researched enough and talked to enough people to know that at any given point, it can stop working.  We hope that isn’t the case, but are prepared to shift course.  We pray for the best scenario – she becomes seizure-free (or close to it) for at least a year, and we can wean her off medicine, the diet and maybe on to CBD (cannabis) oil.  The side-effects of the drugs are almost as damaging over time as the actual seizures, so the goal is always to wean off them.  And the diet is not a long term solution either.

She is a total champ with everything, though.  She takes four medicines, multiple times a day, in addition to her many eye drops.  All in pill form, because liquids contain sugar.  We cut them up and she just takes them – still amazes me.  The seizure pills make her really tired, but that seems to be getting better.  And she actually eats these crazy keto meals!  I look back to where we started and am very grateful things got better, because it was a nightmare in the beginning.  We are fortunate that she is the way she is in regard to this diet, because she doesn’t completely understand that everyone else is eating better foods – or maybe she does, and is just quietly cooperating, which is just as likely!  I always say that if Lucy was the one needing to do it, I don’t know how long it would last.  Avery asked for other foods for the first few weeks, but has adjusted.  I did stop buying many of her old favorite foods for “solidarity” purposes, and we try not to talk about bread, cereal, ice cream, etc. in her ear shot.  Prepping meals, weighing everything and following the schedule has just become part of our routine, and we are always figuring out new recipes and ways to make it more palatable.  And, we are learning how to make the most of being on this type of diet.  She is not an ideal age or on the ideal version of keto - especially for a kid with growth issues to begin with - but choosing the cleanest and most healthy fats that we can will hopefully reduce all the downfalls of putting a small child on a restricted diet such as this.  Because we don’t have a choice right now. 

Epilepsy has come with a lot of new baggage that really sucks.  She has additional restrictions on top of an already long list of restrictions.  While it was never recommended she be in high heat for a long period of time due to hydrocephalus, it is much stricter with the risk of seizures.  Her body doesn’t seem to regulate temperature like it should.  And in general, heat and high humidity, for whatever reason, are triggers for seizures, so we are limited on hot days.  Instead, we take her to her favorite indoor places, or go out for short periods of time.  The times we have kept her out longer than usual, we see an obvious difference in her behavior, so it’s a precaution we take very seriously.  Anything we can do to prevent a visit to the ER, we will do.  In addition to heat, she can’t participate in pretty much anything involving food, which breaks our hearts.  Birthday cake, going out for ice cream this summer, eating at a restaurant, etc., are just not options.  And we don’t feel right doing them in front of her, despite the fact that she’d probably not see or notice anyway.  It’s something we are working on making as “fair” as possible.  She isn’t interested in other rewards, such as stickers or getting to pick out new toys.  We have to be creative with her, and follow very unconventional methods of rewarding, entertaining and passing time.  The diet also limits her energy and contributes to her hypoglycemia, so it’s not like she can run around for hours.  Then there’s all the “extras” – the side-effects of her issues as a whole: behavior and sensory issues, and being developmentally delayed.  While they’re secondary to our immediate concerns, they affect daily life for all four of us.

I’m sure it’s noticeable when we come to a barbeque or gathering that things are different or we seem distracted.  Or we just don’t come.  Or half of us come.  And I can see how it can be misunderstood for being antisocial or stand-off-ish, but it's really not.  I am an admitted helicopter mom with Avery, and being that I’m completely not with Lucy, shows that it’s because of the very different experiences I’ve had with my two children.  We have to be mindful of so many things when we’re out of our house and it often contributes to how we’re acting.  With her limited vision, and still learning to properly use her cane, we have to make sure she doesn’t wander off, bump into something, fall down stairs, etc.  With her other health issues, we have to make sure she isn’t grabbing food somewhere, or that another child isn’t giving her food because they don’t know she can’t have it.  If it’s hot, we have to make sure she is staying cool and taking breaks.  And since her diet provides limited energy from food, she can’t burn her “reserves” by over-exerting herself.  It's a lot to keep up with, while still letting her be a kid.  We struggle every single day with finding balance between living life and being safe.  Some days we go overboard.  Some days it’s hard to come to events.  It’s been six months since we took a child home from the hospital that we were told may not ever leave her bed.  It’s scarring and takes time to adjust to.  And each time a hiccup happens, we are a bit more guarded.  We aren’t even at a point now where leaving a 20-mile radius of Children’s Hospital is a smart option.  What seems to be the hardest point to convey is that this lifestyle isn’t changing for us; Avery doesn’t have the types of disorders that go away.  But how we handle it hopefully will get better.  We just need practice and time to loosen up.  She has evolved over the last six months – sometimes taking two steps forward, then one step back.  I know her body language when it’s becoming too overwhelming or too exhausting.  And I know her body language when something is just not right.  So it’s important to me to explain because I want those close to us to understand a little better.  

Our newest concern (as if the kid needed another thing) is her left eye – which is her only working eye – and it is having glaucoma issues again.  To put off surgery, we tried another med, but she had a horrible reaction to it, so that's not an option.  We try to avoid surgery at all cost, but sometimes it’s the only option to save her remaining sight.  She had a procedure last week to determine if the tube in her eye is filtering fluid properly, or if there is a need for a second tube.  It was a good and bad outcome in that they were able to fix the present tube, but the pressure shot way down, which can potentially cause other issues.  We have been in the office many times since to ensure her eye is recovering properly, and during one visit, we learned of an additional issue on the surface of her cornea.  This cornea has been stable for three years, so that’s another devastating kick to the gut.  Keeping this eye functional is Avery’s only shot at sight.  No, it’s not life-threatening, but to take away the limited vision she has would seriously impact her life.  It took her months to slowly regain the lost vision effects of the seizures, so she just doesn’t need another road block.  And, any anesthesia is a bigger pain in the neck these days, so it’s all more complicated.  Your prayers and positive thoughts are much appreciated, as always. 

Despite all the challenges, as of today, we really are doing well.  Or better than expected, not sure what’s more accurate :)  We’ve been able to get into a (new) new normal with daily routines and juggling everything that is our life.  Spending quality time with the girls is a priority, any way we can make that happen.  Lucy is a wonderful little sister, and as of last week, is officially potty-trained!  Such a relief to check that off our list.  She’s also starting to understand more why Avery sometimes gets more attention, or why she has to stay at (insert one of our generous helper’s name)’s house while we go to the doctor with her sister.  She already is and is going to be a great protector.  She and I were able to get away for a few days up to Charlevoix, so that was a nice break for both of us.  John’s turn next J 

If you are one of the people we’ve had to cancel on, reschedule with, or just say “no” to plans with in the last 6 years, know we are sorry and hope you understand.  We make every decision around Avery and her needs, from whether we can go somewhere, how we can both keep up our work schedules, and in making plans for the future.  We appreciate everyone’s patience, understanding and support as we navigate this.  We aren’t as available, reliable or fun as we want to be – but please know that we really do want to be more of those things.  As the kids are saying these days, the struggle is real…with good days and bad days.  We are very thankful for a stretch of good days.   As with most things in life, it could always, always be worse.  Try not to sweat the small stuff or take your health for granted...it is the most important thing.  Thank you for following her story and your support for our family!

-Ann

Keywords: epilepsy, intractable epilepsy, ketogenic diet, Peter's Anomaly, glaucoma, cornea transplant, pediatric seizures, hydrocephalus

Our new normal

It's hard to believe that we've been out of the hospital for over a month.  Readjusting to life at home has been challenging, to say the least.  It probably seems like we would just be elated to be home, and all under one roof - and we are.  However, it's not like flipping a switch and everything is back to normal.  Our life was put on hold for over six weeks - which means we basically abandoned our jobs, house, plans, and even our other child.  Coming back to reality was daunting, and we are just now starting to feel somewhat comfortable with our new routine.  In addition, we truly took home a different child than we brought in, and that has introduced a whole new list of challenges, responsibilities and limitations.  We are trying to "come down" from a constant state of high-alert, and looking at Avery like a ticking time-bomb who will either vomit, fall unconscious, or erupt into seizures at any point.  We were shell-shocked by this experience.  For almost two straight months, we witnessed so many alarming issues on a daily basis, and that's going to traumatize even the toughest person. 


The seizure disorder has caused Avery to regress in many aspects of her life - walking, vision, eating, potty training, behavior, endurance...to name a few.  We added weekly PT and OT to regain her strength, and we are starting to push her more in daily routines and being more independent, so she gets back into doing more things that we've been forced to do for her over the last three months.  Although Avery has always been developmentally delayed, she was able to feed herself, tell us when she had to go potty, etc., and now, we are starting 10 steps back.  To sum it up with a clear reference, Lucy (who is 2 years old), surpassed her almost-6-year-old sister in every developmental milestone long ago.  In addition, she takes three new seizure medications that make her more moody, tired, and impact her cognition.  Avery went into the hospital on January 1st with an already atypical brain, and adding the intractable epilepsy has unfortunately worsened her cognitive impairment.  At this point, between her neurological disorders and vision impairment, the doctors are expecting Avery to be dependent on us for the rest of her life. 


Needless to say, it's been overwhelming, but John and I are tackling everything 50/50.  This is thanks to the flexibility and understanding of our wonderful employers, and help from family and friends.  We had a lot to arrange right away - the new ketogenic diet, homebound schooling, insurance changes, new doctor appointments, therapies and follow-up tests... oh, and catching up on the responsibilities we ignored for two months.  We've made a point to try to keep to a daily schedule, and stay as organized as possible, because it's the only way to keep sane.  The main new fun addition to our lives is Avery's ketogenic diet.  We have had a lot of questions about this and if you have time for some additional reading, it's best explained in this wonderful article (http://nyti.ms/1BVNe9G).  It's not the ketogenic diet that some people go on for weight loss.  It is specifically for seizure control.  Her body needs to be in a state of ketosis (burning fat for energy, rather than glucose) and that suppresses seizures.  In order to do that, she eats a strict ratio of 4:1 fat to carbs/protein.  A typical meal for Avery consists of 90% fat (like butter, heavy cream, mayo, macadamia nuts, or coconut oil) and a teeny bit of protein and carb.  The recipes are quite alarming at first, and everything is carefully weighed on a gram scale, and calorie-restricted so that she doesn't gain or lose too much weight.  She takes vitamins and a supplement to fill in the holes, but goes in for regular blood tests to ensure she isn't deficient in anything.  I can still buy organic, even if it's organic butter and organic heavy whipping cream :)  We prep meals for the week every Sunday, and it takes up most of the day.  Convenience with regard to food is out the window.  We can no longer grab a quick granola bar for the road, or make a simple sandwich for her.  Leaving the house requires a lot of time and planning, and usually a cooler.  Eating at a restaurant (which we won't be attempting for a looong time) means bringing our own food for Avery.  She cannot eat any of her old favorite foods.  (Except peanut butter, which is always mixed with a ton of butter.)  The first month or so on this diet was brutal.  She was constantly asking for Cheerios, oatmeal, "squeezers" (those fruit smoothie pouches) and macaroni and cheese.  We couldn't seem to make anything that was remotely appetizing to her, and so she is typically left unsatisfied and wanting something else immediately after a meal.  To hear your child cry for food all day long is torture.  It brought me back to the time when she was a newborn and I couldn't get her to take a bottle.  To know that your child is hungry is unnerving.  But, as every experienced keto parent assured me, things are getting better.  We are getting the hang of meal prep, what Avery likes (well, tolerates), and how to better manage.  And she isn't constantly begging for food all day anymore.  Although she still would love a big bowl of Cheerios, rather than what she is forced to eat, she's toughing it out, as she does with everything else.  And we have to think of it just as a parent of a child with diabetes, allergies, celiac disease, etc. has to.  It is so hard, but necessary to keep our child healthy.  This diet is keeping Avery's seizures away, and that's what we focus on.


The other big chunk of Avery's issues - her eyes - which had taken up the majority of 2015, were suddenly put on the back burner during the hospital stay.  We knew that the seizures were affecting her vision, but we couldn't do anything about it, so tried not to dwell on it.  It was the least of our problems at that point.  The strong medicine, especially the one that put her in the "coma" affect your senses, so the combination of continual seizures and medicines, on already poor vision, left her with light perception only for several weeks.  When she showed no interest in her iPad, which is normally her best friend, we knew it was bad.  Slowly, her vision started to return, although it's still worse than before.  The seizures were primarily in her right occipital lobe, which affects the left field of both eyes.  Since Avery doesn't have sight in her right eye, she was basically left with limited vision out of the right field of her left eye (how's that for confusing?).  So long as her seizures remain at bay, this should improve over time.  But anytime they flare up, we'll notice a significant change in her vision. 


Before all of this craziness, we had planned to do an eye procedure on her right eye that would hopefully preserve it, in the hopes that future technology would allow us to bring back any sight down the road.  This procedure was obviously put off, and we had a feeling that the window for it being an option was closing.  We were right.  This morning, Avery's retina specialist saw her under anesthesia, and confirmed that it would no longer be of benefit to her.  We now have to meet with an Ocularist, which is someone I hoped to never have to meet.  They make prosthetic shells, which she will wear over her "rested" eye.  Thankfully, we shouldn't need to remove the eye, which was a huge fear of mine.  I'm still processing this, so I will just leave it at that.


The more time that Avery stays healthy, the easier we can breathe and start to relax.  But every day, there is a time when the weight of our reality hits me like a ton of bricks.  We have a medically complex child who has been faced with challenge after challenge in her short 6 years of life.  She is living with very limited sight, which could potentially disappear completely at any moment.  She has a severe form of epilepsy, and depends on a device in her brain, a difficult diet, and many medications to keep her alive.  When we have a good day, I sometimes forget all of this for a second, and suddenly, it jumps back into my memory and scares the crap out of me.  But, we have to do our best to push past these feelings.  After all, there were several times this year that we didn't know if we'd even bring Avery home.  Or if we'd bring her home to be bed-bound.  What we have today is so much better than that prognosis we were given two months ago.  This is amazing, and I'm eternally grateful for it.  There is no choice but to push through each day, allowing ourselves the occasional pity party.  If we can come out of that experience in one piece (pretty much), I think we can handle anything.  We've come out of it a little stronger, a lot more tired, and with more items to add to our honorary medical resume (ha).  This morning, just before her doctor delivered the news about her eye, he said, "She's just the happiest kid, despite all she's been through."  This means so much to me, because all I want for Avery is for her to be happy.  We may not have many other things, but we do have that.


Thank you all for your incredible support through all of this.  We honestly couldn't have made it through without it.  We'd love your continued prayers and positive thoughts for Avery, as she continues to recover.  In addition, John's side of the family could all use prayers now too.  This year is being hard on all of the Devereuxs in different ways.  Thank you, xo

The new year

“The smell of hospitals in winter, and the feeling that it’s all a lot of oysters, but no pearls.” – Adam Duritz, A Long December

 

Silly me.  I thought I would wish away my unlucky, odd-numbered year and 2016 would be magically better.  I said that I couldn’t be shocked by much anymore, but I was wrong.  I haven’t seen the “old” Avery in 24 days.  The smiling, laughing, chatty, jumping-around Avery has been replaced by a different Avery, but I love her just the same and have been sitting at her bedside in the ICU at Children’s Hospital for those 24 days.  To describe this month to anyone who hasn’t lived it would be impossible.  Though we have seen and been through so much with Avery since her birth, these last 24 days have probably been our most challenging to date.  The fear of the unknown and what the next days, hours, minutes will bring have not left us this entire time.  Wondering when and if we will get to bring our girl home, and if she will ever be the same.  If she’ll continue to live the life she was living, or have even more limitations than she already faced.  Wondering how our day-to-day will change, and if I will live in this constant fear for the rest of my life.  I didn’t think I would ever sit down and blog during this hospital stay, but writing things down has always made me feel better, so while this entry may be TMI to some people, it’s helping me get through this day.

 

Avery has been predisposed to seizures her whole life.  We were warned of them along the way, but like everything we’ve been warned about, we just wish they never happen.  She had one seizure last January, but being that it was coupled with extremely low blood sugar, we resolved it quickly and her follow up tests didn’t show a continued need to worry.  But on January 1^st, just as John and I had left to go on a date, we were quickly called back by my dad because Avery had vomited and was seizing.  I was shocked.  We arrived back as she was being put into an ambulance in the driveway at my grandmother’s house in Milford.  She had three more convulsive seizures that evening, and required several doses of strong medications to stop them.  She also needed to be intubated, after her breathing began to suffer.  I kept thinking it was a terrible nightmare that couldn’t possibly be real; and I’ve continued to think that every day since.  The events between January 1^st and now have led us to the realization that Avery now suffers from Refractory Status Epilepticus, and so far, non-convulsive (meaning only detectible on EEG) seizures have been her main enemy.  This is a serious, incurable condition, and so very far from the image we had in our minds of what her seizures could ever amount to.  Although the 4 different seizure meds we’ve tried have kept them from becoming convulsive, she was still having 20-30 non-convulsive ones per day, ranging in length of time from seconds to over an hour.  Between the constant seizing and the strong anti-convulsants required to stop them, she has not been herself.  If she’s awake, she’s in a medicine fog, which limits her motor skills, her sight and her ability to speak and hear.  A good day is constituted by her eating, saying a few words, and answering a question.  The doctors went through every imaginable test and possibility for what set off these seizures – why now?  And unfortunately, no clear cause has been determined.  Her atypical brain structure, and her having meningitis (with permanent damage) as a newborn just left her predisposed to them.  They have also tried every possible treatment that Avery qualifies for.  We’ve even discussed surgically removing part of the brain that causes the seizures – a surgery I had only heard about previously, and was terrified of.  But when you’re desperate to bring your child “back,” you’ll be open to anything.  Unfortunately, the surgeons cannot determine a definitive focal point in her brain to remove.  Which brought us to last Monday: the conference.  Fifteen neurologists from the area, plus her team of neurosurgeons, and image specialists met and reviewed Avery’s case.  They reviewed each test result, piece of her history, MRI, PET scan, CT scan and tried to figure out a treatment that could help her. They came away with one good answer, which was the pentobarbital coma.  Sometimes the brain is like a computer – if it’s acting up or stuck or going haywire, you reboot it.  The coma basically puts her brain on pause, and you slowly reboot it and hope that it breaks the pattern of seizures.  The success rate is not great, but at this point was our best option.  However, there are risks.  She would need to be intubated again, since the medicine inhibits the body from carrying out its jobs – most importantly, breathing.  She would need constant, close monitoring.  We had to sign a form that basically stated this was an “elective,” procedure.  I found this pretty funny – they’re telling us this is the only current treatment possibility for her, but that it’s still considered “elective”…did they mean like a face lift?  

 

We trusted the team, and agreed to try it.  The process has been brutal.  Watching a crowd of doctors put your child in a medicine-induced coma is beyond anything I’ve witnessed before.  Her room has been quite busy ever since that morning last Tuesday, and watching our child lay asleep in a bed, with a machine breathing for her, a tube feeding her, and nurses constantly caring for her, gets harder each day.  And now we are in the “wait and see” phase. She’s been in the process of weaning off the pentobarb since Friday morning, but it’s not as fast as we thought.  She’s taken a few breaths on her own today, and tried to cough.  Her EEG has been clear of seizures so far, but we aren’t anywhere near in the clear.  We’ve become obsessed with watching that EEG, and I think we could say that we have minored in Neurology at this point.  Being that no treatment up to this point has been successful, it’s very hard to be hopeful.  I’m just waiting for them to come read a seizure on the EEG history, and break the news to us.  But, we kind of expect at this point that she will never be “seizure-free” again.  Best-case scenario, she is, but next-best-case (and more realistic) is she has less-frequent seizures, and we can take her home on two medicines, and possibly on a ketogenic diet.  Worst case is they come back in full-force, and then I don’t know what will happen.  I really don’t know if our time here is days away, or weeks away, and the pressure is killing me.  

 

I miss Avery so much.  While she is here physically, she has not been “here.”  I miss her constant stream of questions from the back seat of the car, her whining to get more time on the ipad, her turning on and off every light switch she can reach, or hiding in our shower when we aren’t looking.  I miss hugging her without 20,000 wires getting in my way.  I miss the life I thought was so difficult, but seems like a breeze right now.  I also miss Lucy so much.  She’s been traveling around from person to person these last three weeks, and we get to “visit” with her in between.  She’s starting to get used to it, which I find comforting and sad all at once.  I feel like I’ve been robbed of at least three weeks that I’ll never get back with my girls.

 

I know once we do leave here, that my perspective on life will be so different.  Besides the horrors of our own situation in our own little ICU room, we have witnessed the saddest things I’ve ever witnessed in life, in this unit.  It’s one of the most depressing places to spend time, and lately, just driving into the parking garage here makes me nauseous.  I think we’ll be changed by this forever.  John has, as always, been my steady rock through it all.  I know he’s hurting too, but he always handles the stress so much better than I can.  He keeps me sane by reminding me that we are in it together, and we will come through it together.  While our future might be more difficult, and our new “normal” will be different, we will do it together, as a family.  That is the one thing I can hang onto right now.

 

They say that situations like these will show you who your “real” friends and family are. Again, I say that we must be incredibly lucky, because everyone has been beyond supportive from day one.  I never go a day without feeling the love and support of people in our life.  Both sets of parents have taken the majority of the workload, and we are forever grateful for them, as I know they are suffering each day with worry.  Thank you to everyone for supporting us in so many ways.  All we care about is knowing that you’re behind us, and praying for Avery to get better, and you’ve done so much more than that.  In the past, situations like this have chiseled away at my faith – in God, and in life in general.  I feel like this time, I can get through those dark times more quickly, and my heart hasn’t hardened as it has in the past.  There is always a homeless person standing with a sign at the corner of Mack and Beaubien, just outside the hospital.  In the past, I felt warranted in being cold and ungenerous to that person – after all, they probably put themselves in their bad situation, and my daughter sat in a hospital bed, and was worse off, to no fault of her own.  But this time, I find myself reaching into my wallet, if I have time before the light turns green, and giving them some money.  Because you can let the dark thoughts overtake you and completely harden your heart, or you can give yourself some time to be angry, then decide to move forward.  That is my only option in this, because being constantly angry and depressed does not one thing for Avery.  She deserves better than that, and I am grateful to have learned that this time around.  So while I definitely have my moments, and my bad days, I make the conscious effort to move forward, and only speak positively to her; whether she can hear me or not.

 

Please continue to pray for her – we are far from out of the woods, and we’ll need every positive thought we can get!  Thank you for everything….

There's reason to believe...

As we come to the end of what's been another incredibly challenging year, one that's left us with several new diagnosis, several more wrinkles, and much thicker skin, I'm feeling a little relieved.  Although the transition from December 31st to January 1st isn't some magical shift, we often look at it as wiping the slate clean, and winding the timer back to start.  I have said so many times over the last month that I can't wait for this year to be over.  As a mom, it's terrible to wish time to pass, but I'm looking for some relief, and sometimes a new year brings that.  Although we're not very superstitious, we seem to find very bad luck in odd-numbered years.  2012 and 2014 were our easiest years since having Avery, and although it seems silly, I'm really looking forward to 2016 continuing that pattern.

The craziness that is 2015 ensued right away in January, with the first time I had to call an ambulance for Avery.  It was a combination of extremely low blood sugar and a seizure that threatened to take our baby away from us that day.  As a parent, you never shake the memory of that, and the fact that this wasn't the first time, nor the last, makes it even more traumatizing.  It will stay with me forever, and it adds to our daily vigilance.  From that hospital stay on, it's been a constant ride on a hamster wheel.  We added a neurologist, endocrinologist, retina surgeon and psychologist to her already long list of specialists.  And the appointments!  Oh the appointments... I feel like I have lived inside a doctor's office for most of 2015.  Many months, averaging two and three times a week, all to try to determine what is going on with her.  In late spring, we noticed her right cornea was starting to fail.  And the day it was confirmed she'd need her 8th re-transplant, we also took a fun trip to the dentist, and were shocked to learn she had a malformed molar that needed surgery.  So the chaos continued.

The summer was spent continuing to dive into her endocrine issues, as well as her developmental issues.  We walked away with a confirmation that she does not fall on the Autism spectrum, and a better understanding of how her noggin is functioning.  But the cold, hard reality that she has a diagnosis of diffuse neuronal dysfunction, aka, "slow brain activity," and a global developmental delay.  This is all stemming from the meningitis infection she had as a newborn, and all her subsequent brain surgeries and issues.  This didn't change anything, but to see it written on paper is extremely difficult.  It fuels our motivation to keep working with her on a daily basis, and immerse her in as much learning and therapies as she can take.  And honestly, she's doing better than anyone ever expected.

As I explained in a previous post, August greeted us with one of our worst eye-nightmares: a detached retina.  The first two weeks after learning this news were insane, spent on the phone, begging to get into the best doctor sooner than they had scheduled, and basically keep ourselves from completely losing our minds.  If I only knew that it was just the beginning of an excruciating fall and winter.  The day of the planned retina repair, the doctor found that Avery's cornea (only a few months old) was already failing, and because of which, he couldn't get a proper view of the retina to repair it.  Two days later, we were back, plus one cornea surgeon, for a five-hour surgery to re-transplant the cornea and repair the retina.  He explained that it was now a waiting game, and completely in God's hands.  We love that doctor - he is another really empathetic guy and took a lot of time to explain all scenarios to us.  If you can't feel a sense of comfort with these guys, you're in a bad spot, so I'm grateful for all of our good doctors.  And those who haven't been, have been replaced...

Since that day, the right eye has been on a downward spiral.  We faced a sudden cornea rejection in late October.  One day it was fine; the next day it was not.  And when that seemed to turn around, they found that her pressure was not responding.  You need a certain range of pressure in the eye for it to survive.  Too high is glaucoma, and too low is hypotony, which is what Avery has been experiencing since her retina surgery.  They believe that the membrane that produces fluid may be permanently damaged.  After 5 1/2 years of constant, relentless fighting for this eye; an eye that has seen the most surgeries of anything, we learned a few weeks ago that we reached the end of the road.  We sat across from Avery's Ophthalmologist, after he had just finished a procedure that he didn't feel would work, and he said the words we dreaded since we first sat across from him 5 1/2 years ago: "I'm sorry, but I don't see the eye recovering."  He put his hand on my hand and assured us that we have done all we can, and fought quite a fight.  We are now facing "putting the eye to rest."  It was a slow build-up to this point, with so many obstacles along the way, but we never imagined we'd be facing this this year.  It's been like grieving a loss.  And with her "good" eye being not-so-good, we are closer to the possibility that Avery could one day lose all remaining sight.  But for now, we take one day at a time, and make the most out of the days she does have sight.

During all of this eye drama, Avery was being tested for growth hormone deficiency earlier this fall.  Anyone who knows her, knows she's tiny.  At the time of the testing (at 5), she was standing just under 3 feet tall and only 25 pounds.  That's like a small 3-year-old.  So they were trying to determine if it was an endocrine issue.  The results were not what we expected (shocker).  Avery did have sufficient growth hormone, which meant we didn't need to inject her with it on a daily basis (good news).  However, her blood work showed that she had virtually no cortisol (bad news).  This is the fight-or-flight hormone, and is necessary to deal with physical stress and illness.  Her body wasn't producing it.  At the time, she was not symptomatic, but if she encountered any surgery, illness, car accident, shunt issue, etc., she'd require medication to survive it.  Once again, I felt like the floor was dropping out from under me.  I know many of those scenarios are inevitable for us, so facing another life-threatening, medicine-dependent diagnosis was so overwhelming.  We are so thankful we did that original test, though, because we were able to prepare ourselves over the craziness of this fall and winter for adrenal failure issues.  The likely culprit is her life-long dependency on topical steroids for the cornea transplants.  Steroids can impact the body's creation of cortisol, and not only does Avery take them in her eyes, she had just started on a new one for alopecia (yeah, another new issue).  I always had this nagging suspicion in the back of my mind about her steroids, but was assured that there was not literature to support topical steroids causing any major issues.  But it all started to make sense with the diagnosis of adrenal insufficiency; her small size, her eating problems, etc.  Then a few of my Peter's mom friends were talking about the same issues in their children.  We hope to have her added to a case study so there is literature for future kids with similar symptoms.

When the cornea started to reject in October, the only way to stop it is to do continual steroids.  Before the words were coming out of the cornea doctor's mouth, my brain was going 100 miles an hour trying to figure out how we were going to do this.  With the consultation of many of her doctors, we found a regimen to be able to increase steroids to try and save the cornea, but keep her healthy.  She started taking a pill three times a day to make up for her body's lack of cortisol.  But if she gets sick, seriously hurt, or requires anesthesia, she needs a "stress dose" of the medicine.  And in very serious cases, we now have a shot (kind of like an Epi-pen) that goes in her leg.  We spent several hours at the endo office that week, learning how to see signs of adrenal failure, how to give the shot, and different scenarios that we could face.  "This is all manageable, but when adrenal crisis starts, the child will deteriorate very quickly."  That line sticks with me every day.  And we've already seen it in action. This adrenal issue has caused quite the drama over the past 3 months.  We've made about 50 calls to the endocrinologist, two emergency trips to the ER (one while in Chicago), and we've already had to administer that shot.  Which was another traumatizing day.

The toll that this year has taken on us has been significant, but we have managed it.  With each incident, we learn a little more, and get a little stronger.  John and I were sitting up one night recently, recovering after about 5 days of the girl's having the flu, and "how are we going to finish our to-do list before Christmas?" And as we reminisced the monstrous year we've had, we realized that we've really only become stronger in the process.  Yes, there are days when I feel so tired, emotionally drained and beaten down that I feel like the weakest person ever, but overall, I do think this has thickened my skin.  All I have to do is look back 5 years and how I handled things then.  Very little shocks us these days, and unfortunately, we have been living like something else is lurking just around the corner.  But despite being tougher as parents, it's no easier.  With the new adrenal issue, and the added pressure to keep her only functioning eye healthy, it's hard to see a break.  I think that's one of the hardest things right now - not seeing reprieve.  Our parent's have been amazing at giving us breaks; to go to dinner, go to coffee, have a night with friends - or to work (somehow we both still have jobs after taking so much time off!).  Many others offer to help us all the time, which we really appreciate.  And that is sufficient for now.  Someday we will get a longer break, have fun and relax; but it is hard to see that right now.

The light through all of this continues to be Avery's incredible, sometimes unbelievable resilience.  She's doing so well in school, loves her teachers and friends, and comes home smiling every day.  She's doing more and more "typical" things each day, is learning Braille, and becoming much more independent.  We light up with joy each time she does something new, or shows excitement over something.  Lucy is being a good little sister, and although she wishes she got more attention (ahem, terrible twos...), she's starting to "get" that her sissy isn't like all the other kids, and needs a little more attention sometimes.  I'm sure it's a balance we'll be working on all our lives.

Heading into 2016, I'm focusing on being more mindful.  As cliche as it is, it's imperative we live in the moment of the good times, hug our kids tighter and cherish the healthy days.  Because we know what constitutes a "bad day."  We've experienced our own, as well as others who have it so much worse.  As hard as it can be to focus on the positive some days, I know that it can always be worse.  Avery has also made so.much.progress.  Unbelievable progress since she came into the world.  I remind myself of the mountains she's climbed, and the roadblocks she's overcome to get me through the bad thoughts.  And to try not to dwell on the "what-ifs."

There is reason to believe that this coming year will be a good one, and that's all we need.  Happy, healthy 2016 to you all!  Thank you for your support...xo