Everyone stay safe, stay healthy, and if you're sick, please try to stay home <3
Tuesday, January 16, 2018
Everyone stay safe, stay healthy, and if you're sick, please try to stay home <3
Thursday, March 23, 2017
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.
Sunday, October 30, 2016
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.
Tuesday, August 2, 2016
Wednesday, March 23, 2016
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
Sunday, January 24, 2016
“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 1st, 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 1st 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….
Thursday, December 31, 2015
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.