and a little about our family

So, many of you are not that familiar with Daniel and I personally, or we've only met recently. I just wanted to say, first and foremost, thank you for loving our family (or our daughter in particular) enough to even read all these ramblings. There is strength in numbers, and your thoughts, prayers and interest are so helpful to us and to Evelyn.

Also, we love our kids. Evelyn and Owen are so important to Daniel and I, and though Evelyn's life looks very different than Owen's, we treasure them both equally. We will always make decisions for Evelyn (particularly medical ones) with the question of 'quality of life' just as high as 'fixing a problem' or 'treating a symptom'. Evelyn has a great spirit, and a true intelligence, and we feel it is important to honor her as a person when we make difficult decisions for her.

Also, we are an open book. We are SO not perfect. We try hard, but sometimes we don't - there are definitely dust bunnies in the corners of my house, and always at least one load of laundry that needs folding. But we, as a family, love each other very much - and we care about and respect each of you enough to want you to ASK any question you might have, without fear of saying the wrong thing or stumbling over a delicate topic. The world of special needs is foreign to some people - it was to us before Evelyn was born - but we really believe that if you have an interest in our family or our daughter, we want to help you feel comfortable around her and us. So please, ask away, come visit, etc - and if something about Evelyn's situation is hard for you, we understand that too.

Also, that's enough blogging for one night. :) If you made through all this, there must not be much on tv tonight. :)

Weary but Working

So that's Rocky, our old, indulgent pound puppy. He's got a little bit of doggy dementia, and his joints are getting creaky, so I guess he's earned a little chair-sitting.

That's how Evelyn and I are feeling today. Tired. Evelyn uses a bi-pap machine at night (google it if needed) to give her nice deep breaths and a restful sleep. Well, she was so lethargic today that we and her pulmonologist felt it'd be best to keep her on it for the majority of the day. So we did. And she appreciated that; though her numbers (meaning oxygen saturation and heart rate) have been consistently good, she just looks worn out. She looks like she's working harder to breathe when she's not on her bi-pap, and I just don't have a great feeling about her sustaining this level of lethargy for much longer. So, first thing this morning I called pulmonology and the ENT to push for an earlier follow up and to get the ball rolling with the trach discussion. Pulmo was very helpful, but unfortunately nothing much happened all afternoon. As I realized we weren't getting the much desired call to schedule an ENT appointment (and they're the ones that lead the way where trachs are concerned) I called them, again, and left a message, again. No response. So, as soon as we got past business hours, I decided to be sneaky and call the on-call pulmonologist to get advice on what more, if anything, we could do for Evelyn. My hope was that whoever was on call would be nice and I'd get the whole story out, and get some action on our behalf.

Well, the doc was awesome. Confirmed that I'm not overreacting with regard to my concern for Evelyn's current health - basically she doesn't have much reserve left, so we DO need to act fast. He said he'd talk with our pulmo first thing in the morning - but 30 minutes later he called back and had already spoken with him. SO, our pulmo will be speaking personally to our ENT (who to this point probably doesn't have a clue about everything that's happening) in the morning, and we will likely have a direct admit to the hospital to place a trach. I expect we'll go in to the hospital this week, possibly as early as tomorrow.

Don't get me wrong, I'm not excited about this. The surgery is, well, surgery, and the recovery stay is a minimum of a week. It is going to be tough - Owen will be with his CC (grandma) who he adores, Daniel will be working days and at the hospital most nights, and I'll split between the hospital with Evs during the day and Owen at night. I'm glad things like this don't happen too often, because it isn't fun. The truth is though, that I'd go through it all twice, and Evelyn would too, if it meant she'd get some relief and energy. And she will, as long as things go smoothly.

I've been a little disappointed these last few days by the lack of perspective or compassion shown by a few people we know, but that's inevitable. We feel so much love, and know so many prayers are being said; and that makes all the difference. It can be lonely and isolating in the hospital, where time stops - yet the world keeps moving - but I am so thankful for all of you - even when it's just a 'like' on facebook - because it means Evelyn was thought of, once again, with love - often by someone she's never met. Some people criticize the digital age because they fear it limits face-to-face contact, and I don't disagree. But when face-to-face isn't practical, or even feasible, it gives us another community of people to walk through life with. Thanks for walking with us.

Ten Thousand Reasons

I would consider today to be our first 'observation day' with respect to Evelyn's breathing issues - in preparation for our follow up with pulmonology in a couple weeks. The last couple Sundays we've been in church have been particularly rough for Evelyn - or maybe just more obvious for Daniel and I, sitting with her in the relative quiet of church, noticing how much she's struggling. While we could see that today her breathing was less noisy, we could also see that it was just as labored as it was a week ago.

It just isn't easy to watch your child struggle, in big ways or small ones, and so I find myself slipping in and out of good and bad moods. For that reason, the sermon today and some of the music was particularly poignant. I don't generally write or talk a ton about my faith or the bible, I guess it just isn't my nature to be overly vocal about it, but I feel like there are so many parallels that they bear mentioning. So often we feel imprisoned - by our desires, by our work, by the demands others make of us - or in Evelyn's case, I imagine she feels somewhat imprisoned by her body and its limitations. We've been studying Philippians and though Paul is literally IN prison, he says that he is free. The interesting point today was that though he wasn't free FROM his jail, he was free FOR others.

If there is one thing Evelyn has shown me over the last (nearly) five years, it is grace. Though she may be weighed down and even imprisoned by her many limitations, she is free for grace - and has been a path to grace for so many. There was a hymn sung today that discusses the 'Ten Thousand Reasons' (and more) that we have to praise the Lord. Well, as we were watching Evelyn closely, and the realization that her breathing really hasn't improved was sinking in, hearing those words was so meaningful. Every single day, no matter what comes, we have countless reasons to be thankful, grateful and joyous.

That doesn't mean we handle all this craziness perfectly; it just means that sometimes the right words (or sermon) come at the right time. We all face struggles in our lives. I can honestly say that there is no one I know who hasn't faced or is facing challenges in their lives. Because a lot of our challenges center around Aicardi Syndrome they may look different than the struggles you face - but we all have a choice about how to handle them. We can become worn down and embittered by the 'cruel hand life has dealt' or we can look for the ten thousand reasons we have to be grateful. Sometimes we have to make that choice over and over again, moment by moment - but it is definitely a choice worth making.

Hello again!

Well, it has been nearly two years since our last blog post, but for many reasons it seems high-time to begin posting again on a regular basis.

For starters, Evelyn's little brother Owen is now 2 1/2, and very good at it. :) Evelyn will be FIVE in a couple months - which is just crazy - and Daniel and I will be another year older soon as well. In most respects, Evelyn has had a great couple of years. She has been in school, wowing her teachers and therapists with her knowledge (and fashion sense!) and continues to be the such a sweet, happy little girl. Since the winter of 2010 we've managed to avoid any hospital-worthy respiratory issues, though her seizures have caused some trouble from time to time.

Which brings us to now, March 2012. Over the last few months we've noticed on occasion that Evelyn will slip into a very labored breathing pattern - lots of noisy snorting, chest heaving, etc. She then resolves the issue on her own and carries on with her day. Typically these episodes don't impact her oxygen saturation, but since they don't look or sound like much fun, we've been watchful of them. About three weeks ago Evelyn got a cold. It was just your average, run of the mill cold, and overall she handled herself well (no scary moments with oxygen dropping, no fevers, etc) but the cold just wouldn't go away. We started her on an antibiotic just in case, and as we were finishing it we came to realize that though the cold symptoms were gone, she was really struggling with her breathing. Anytime she got 'up' to do anything more than lounge and watch movies she would slip into the obstructive breathing pattern - and wasn't able to really recover from it without a breathing treatment or time on the bi-pap.

Well, that's no good. So, we took some video and went to see her pulmonologist. Though I had a litany of potential causes to discuss, as expected it comes down to muscle tone. Evelyn's muscle tone is very low (weak) in general, and that affects the muscles in her mouth, throat and airway as well. This means that the breathing difficulty is occuring because her tongue is ever so slightly slipping back and blocking her airway. Because of her weakness she has trouble pulling her tongue back 'up', which leads to saliva pooling in her mouth. When she finally recovers from the obstruction she then has to deal with swallowing an overload of saliva. Basically this issue is only resolved by placing a trach. We thought in general that Evs might need one someday, but now it seems fairly clear that that day will be sooner than later.

We will have a follow up appointment in two weeks to discuss how she's doing and what the plan will be going forward. We need to spend the next two weeks evaluating her breathing - how frequently is she obstructing? for how long? does it affect her o2 or heart rate? how is it resolved - position change, self correct, etc? The answers to these questions will give us a good idea of when the surgery will need to occur. My expectation is that it will be needed in the next couple of months, as I'm just not seeing her improve after this illness the way I'd expect.

In general we are so thankful that a trach is an option for Evelyn, and we're glad we've caught these issues before they became worse. Of course surgery is scary, and we worry for Evelyn - both for her health and that she will feel 'okay' about going through all of this. We want her to have a happy life, and to be as healthy as she can be. It does make us sad, because the need for a trach is yet another reminder of how medically fragile Evelyn is, and of how fleeting life is for us all. Having a trach is highly likely to greatly improve her quality of life, and will likely extend her life significantly as well - so that's why we feel its the right choice.

We hope the few people that still read this - or that start reading it - we be praying for our family and sending good thoughts our way.

Solo con un click

"Only with one click"

Along the lines of Aug Comm - I thought I'd share this video, of a Spanish(?) girl that appears to have some type of movement disorder (perhaps some type of Cerebral Palsy - however, I do not know her specific challenges) - but still manages to make incredible use of augmentive & assistive communication devices.  Evelyn uses some of the same tools (notably the hand-activated switches seen in the video), and some we haven't used yet.  According to the blog where I came across this video, some of the devices are older - with the very key point that "it is the communication that matters not the tool being used to communicate".

Evelyn's "talker"!

(As originally published via Facebook Note by Kim, 6-Apr-2010)

As requested, here is the story of how Evelyn found her voice.

Evelyn's diagnosis comes with all kinds of negatives: seizures, health challenges, developmental delays, medications, blah blah blah. But Evelyn herself comes with all kinds of positives. We always felt that she "had a lot going on in that little head of hers", but were hesitant to say or do much about it in a public manner. Our reticence was largely due to the fact that Aicardi Syndrome is associated with significant cognitive delays (aka mental retardation). So it seemed presumptuous to think that our little toddler who can't walk or talk (or sit or move very much at all independently) could think and understand.

Well, if you know Daniel and I, we didn't waste much time wringing our hands and wondering - we started testing her. Every month we go to Lekotek, a special needs toy lending library, and have been since Evelyn was about 7 months old. This was her first introduction to switches and computers. We would hold a big button in front of Evelyn, support her arm, and show her how to press the button. A wire would connect the button (switch) to a toy, and when she pushed the button the toy would dance, or sing, or make loud annoying noises. She figured that out pretty quickly, and would bang away - she became really good at this around her first birthday. Lekotek also had very simplistic computer games - push the switch and a song plays, push it again and it skips to the next song and plays it. She also became really good at that - and would even skip through four or five songs to play the same one over and over again.

We continued to use games and toys like that for a while, until we felt more of an urge for specific communication. She would cry, and I would blame it on teething, but not know for sure. Or, just to see what she wanted to do, instead of always choosing for her. That led us to the Augmented Communication Department at Children's Healthcare of Atlanta at Scottish Rite. In July of 2009 with met with a communication specialist who spent about three hours evaluating Evelyn (along with an occupational therapist) and talking with us about motivates her and what strategies we could start to use at home to elicit more communication. We came away from that meeting with a loaner 'device' and some great tips to use.

From July 2009 - October 2009 we practiced 'scanning' with Evelyn. We gave her three choices (three foods, or three toys, or three activities) and she picked. We would record "yes, that's what I want" on a switch, say each choice and she would hit the switch when she heard the one she wanted. Often we would rearrange the choices and ask her two or three times to make sure she was really understanding the concept. She understood. for real. and it was awesome. :) She loved to choose strawberry sauce or yogurt to eat, to play in her little room, and to read stories. We still weren't totally convinced, but it seemed to work, and several times temper tantrums were avoided by using the button to discover she wanted a bath (and to go to bed, not that she'd admit it) or needed orajel for a cutting tooth.

So, we went back to the communication specialist, and we ordered her device. She has a Dynavox VMax - google it to see more details (hers is pink). Basically it is a fully functioning computer, with a touch screen, and some super fancy software (though Daniel says he could totally make it himself). We plug her switch into the device, much like you might plug a mouse into your computer. We start the software, and it begins scanning through her menus: Want, Play, Go, Talking with People, Feelings, Time to Learn, About Me, My Words. It announces each category out loud, and when she hears the one she wants, she pushes her switch. Then it opens up the menu for that category and starts scanning all over again. When she hits her button, it basically talks for her. So, if she hits the switch when she hears "play" the talked will say: "I want to play".

Here's an example:

• Evelyn hits play, so it says "I want to play"
• then she hits "Computer" so it says "I want to play on the computer"
• then she hits "Read a book" so it says "I want to read a book on the computer"
• so then we load one of her books on cd (living books - google them, they're awesome)

From those main categories there are layers and layers of menus, so under "I want something" she can tell us she wants her hair done, or lip gloss, or a massage. Under feelings she can tell us if something hurts, and which body part. This device has the capacity to function at the highest level (so someone with quadriplegia could run their business with it, by using a switch or eye gaze, etc) - so basically we can program it to enable Evelyn to do just about anything. Currently she can skype people, load websites to play games, and talk to us about lots of different things.

The response time is very different than having a "normal" conversation with someone, but since getting the device in December she's done remarkably well. We love our daughter, and seeing her gain some level of independence is just amazing. It was so encouraging to set up her talker in the hospital and give her the chance to tell us how she felt. She wanted to get in her stroller and go home. Her head hurt and she was tired. She wanted to watch Tinkerbell. It's hard to hear that your child doesn't feel good, but it feels SO good to know she's able to tell us.

This has given her wings, and it is probably the greatest blessing she has received thus far.

VNS

Well, Evs is now the proud owner of a Cyberonics Demipulse Model 103 VNS (Vagus Nerve Stimulator) implant. In very short, it's essentially a pacemaker for the brain.

Her surgery was Monday morning (19-Jul) - technically neurosurgery, and thus performed by a neurosurgeon, but they never had to touch her cute little head. ;-) It's 2 small incisions; one in her neck, and the other in her chest (see illustration).

More information can be found on Cyberonics' website - but very basically, the device sends electrical pulses to the brain via the left vagus nerve.  As with most seizure treatments (anti-convulsant drugs, ketogenic diet - etc) - "they don't quite know exactly how it works", but it can both help prevent seizures, by sending electrical pulses through the vagus nerve to her brain, as well as when she has a seizure - it can be used to "interrupt" it, by a magnet-activated switch.

At any rate, the surgery was relatively easy, as far as surgeries go - normally, it's actually an outpatient procedure, or doesn't typically require a stay overnight.  However, due to Evelyn's recent respiratory issues, we had already planned with her doctors to keep her a night.  After the procedure, she did have a hard time coming off the ventilator - The anesthesiologist & recovery team brought me back to help try her on her Bi-PAP for a little bit (fitting the mask is a bit of a learned art), but between irritation, agitation, and leftover anesthesia keeping her airway a little floppy, she had to be re-intubated for the next day.

She (and Kim & I) spent the rest of Monday in the PICU.  Around 10AM the following day (Tue 20-Jul), Evs was extubated without much issue, and had a pretty relaxed day (as relaxing as the PICU can be) - mostly involving sleeping to Tinkerbell.  ;-)  We were discharged Wednesday morning, also without much event.

The rest of the week was a relaxing one for Evs.  As far as the VNS goes, we'll have a post-op follow-up late this coming week, and about 2 weeks later, we'll go in to actually have the device turned on.  Then it will be kind of a like starting a new medication - we'll slowly ramp-up the settings, tweaking both intensity and frequency until we find the right settings for Evs.  It will be a process over several weeks (or even months), but we're hopeful this will be another helpful addition towards helping keeping seizures a smaller & smaller part of Evs' day.  :-)