Tuesday, August 2, 2016

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 6th 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