From the Archives: The JCFS Interview

Earlier this year, one of the women in the marketing department at one of Zoe's therapy clinic asked me if I'd be interested in talking to them about "The Big Deal" and our experiences with Zoe. I'm not sure I'd ever been interviewed prior to that, but I wasn't going to say no. If there was interest in our story and Rett Syndrome in general, we'd oblige them. What I didn't count on (but should have since I'm a sap), is how hard it would be to get through a phone interview with simple questions about my daughter without getting emotional. The woman on the other end of the phone was incredibly patient, since she couldn't tell why it was taking me so long to fight off tears and answer her question. Luckily, she sent me a draft of the article before they posted it, so I had a chance to make edits and clarify anything that didn't come across clearly. Which was a lot more than I thought. It was like a much nicer version of the end of "Almost Famous". I sounded like a sucker. I am a sucker.

http://www.jcfscommunities.org/blog/2013/7/2/our-story-the-big-deal-documents-family-living-with-rett-syn.html

Better Than a Flying Car

I've been 40 for a few days now and had time to reflect. When I was younger, I was sure that by this time, flying cars and jetpacks would be commonplace (and by younger, I don't mean grade school age, I mean a week or two ago). I still have confidence in science and technology to achieve amazing things, but my own interests have changed...

Ever since Thanksgiving Day, we have waited for daily updates from one of the members of our Rett family. Their daughter has been in a Boston ICU with an infection that came out of nowhere. In addition to the other things she normally couldn't do for herself, she couldn't breathe on her own, and there was no way to know if she'd win her fight. As I write this, they're hopeful but unsure. And all weekend we thought about them, and we thought about their sweet daughter. But we mostly thought about our Zoe.

Given the nature of Rett, we can't predict if the next upsetting personal medical news will be about another girl or about Zoe. We don't know if Zoe's body won't know how to respond to a simple cold, letting it turn into a more dire infection. We just have to be prepared...for things it's not fair to have to prepare for.

I'm 40 years and five days old. I'm a big boy. But Zoe's a little girl and deserves to be one. And to us, her having that chance would be so much better than a flying car. We hope you feel the same way. If so, please consider sharing what you can to help further research (and our eternal thanks to those that already have).

http://www.firstgiving.com/fundraiser/stephanie-prask/prasktoberfest

It Don't Take Much

Zoe's summer school program ended a little while ago. It was a half-day, with her normal preschool teacher and most of the same kids from her class. It was her last chance at familiarity before kindergarten in the fall, and even with just a week between normal school ending and summer starting, we knew she was anxious to go back. For the second summer in a row, the program was at a nearby elementary -- not her normal preschool, but the only school in the district with air conditioning. Because it was within the same district, the same bus company provided her transportation.

This past winter had been a momentous one in terms of Zoe's transportation needs. She graduated to a wheelchair, mostly when my wife got too pregnant to carry her onto the bus every day. When that happened, the bus company started sending a bus with a side door and motorized lift. It introduced a whole new system of harnesses and wheel-locks to learn, but even for those of us without babies in our belly, it made life a lot easier. Zoe also was proud to not be carried on and off the bus in front of her friends by one of her parents (I mean, seriously, Dad).

Once summer school started, we had a new driver, a young woman who couldn't hide that she was a little new to this. We were a little late coming out the first morning, but it didn't matter. She was standing in our driveway, trying to figure out the lift system while maintaining her good nature. If it were me, I'd have been swearing at the lift system and everything to which it was attached. Point of fact: I was still swearing, but with not quite as much frustration as I would have if I were the driver. She called Dispatch, and they figured out the issue. Once Zoe was onboard, the young woman had no idea how to get her chair secured. Luckily, I knew what to do. Just as I was about to get off the bus, something occurred to me. "You're all alone," I said to the driver. "You don't have an aide." She confirmed that there wasn't anyone hiding in the back or any other mysteries at hand. I explained that Zoe's condition carried with it the risk of seizures, and everyone would be better off if she had someone to help monitor (with lift and harness experience being strongly preferred, if not required). A little brusquely, she told me I should call the office to see what they would do about it. I didn't mind her patience level with me, knowing she was late to get the next kid. I asked her name, so we knew it for tomorrow and to thank her. She said her name was Felicia, a little warily, as if I would use it in reprimand when I called the office. I went back inside, worked for a few hours, then called the bus company right before lunch. They told me their computers were down, so they couldn't look up Zoe's route number, but they took our name and said they'd call later to let us know what arrangements we could work out.

An hour later, Zoe's bus pulled up to the house, with an aide onboard. Felicia* hadn't waited for me to call the office. This was very astute of her, given that we'd just met (it takes some people years to fully appreciate my powers of procrastination). She'd taken it on herself to mention it when she finished her route that morning. The next day I was in my actual office, rather than at home, and the bus was a little late. Felicia was very apologetic and assured my wife she was still getting used to the intricacies of the new route. This started a week-long stretch of Felicia reminding us that Zoe was the first girl she'd picked up on her first day, her favorite, and she'd always be her first priority. For the rest of the week, she assured me and my wife that she'd be on time, and she kept her word. Each day, she and the aide asked more questions about Zoe and Rett Syndrome's effects on her. Towards the end of the week, I was wheeling Zoe out. She'd tensed up a bit in our foyer as we'd waited for Felicia, and she had a tight smirk over clenched teeth, while she waited for her muscles to slacken. Felicia noticed Zoe's expression and asked "Is that how she smiles?" I was a little surprised at the question, since I was sure they'd warmed up to each other by now. "Oh, no," I said. "You'll know when she smiles. She has the best smiles. You can't mistake them." By the following week, Felicia and the aide were talking about Zoe's smiles and how much they loved seeing them every day.

* This is not her real name. I know how bureaucratic things work, and it may not help her if anyone in the office knew what she did. She also may not want people to know; she may have just been doing her damn job, which makes us admire her more.

It was that week the weather started getting hotter. That Friday, ever-vigilant, I remarked to Felicia that the bus didn't have air conditioning, as I broke a sweat just getting Zoe strapped into place. It did not, Felicia confirmed. I explained how the heat increased Zoe's risk of seizures, and how sensitive she was to heat in general. Once again, Felicia told me to call the bus company but added that she'd mention it when she got back to the office. That afternoon, a different bus pulled up, and I was greeted by a face full of cold air when the side door opened up. I was also greeted by a different driver and a different aide. The driver was a middle-aged guy, a little scruffy and a little sarcastic. He seemed like he could be funny in certain social situations that might not have involved driving a bus full of children, but he wasn't outwardly friendly, and I was sure Zoe would notice the difference in demeanor. The aide was also a middle-aged man, a little hard to talk to but seemingly harmless. They were not, however, Zoe's newest friends. Furthermore, none of the kids from her class rode this new bus, as had been the case with Felicia. We didn't realize we had to exchange the un-air-conditioned bus for Felicia herself.

We brought Zoe inside, but it was clear she was upset. Her muscles were tense, she was shaking, and she had an intense scowl on her face. Steph started asking her questions, like we normally do when our girl gets home, but we asked some questions we don't normally ask. Was she mad? Yes, she was. Was she mad because she had a different driver? Yes, she was. Was she mad that no one told her? Yes, she was. We let her lay down in bed to calm down, and we lamented the fact that no one thought it was important to tell Zoe about the new arrangements, that something in her life was going to change -- something they would have told any other four- or five-year-old. We hadn't known the change was taking place, but we'd been just as guilty of this with her in the past. During her first year of preschool, we noticed she got very withdrawn over the course of Christmas break. About two weeks into it, it hit us: we didn't tell her she was going to be out of school, even just temporarily. She assumed after a whole week of being home that she wasn't going back, and it made her sad. That was enough of a lesson for me, but we still forget to tell her little shit like that all the time. Luckily, she knows this now and is more patient with us. But it was clear she didn't think losing her friend Felicia qualified as 'little shit'.

My wife sent Felicia a test message that Monday morning after Zoe got picked up, saying how sorry we were to lose her. She wrote back immediately, saying how much they'd missed Zoe that morning, and how sad she'd been that her office had solved the air-conditioning problem the way they had. Once again, she said to call the company to complain, but once again, she took matters into her own hands for her friend Zoe. That afternoon, the new bus pulled up and Felicia got out, walked across our driveway, and over to the lift to reveal our daughter, waiting to be lowered down home, trying hard not to smile too big.

It is strange to admit this, but we, as special needs parents, don't trust other adults, even other parents, to not overlook our children. The instincts, I suppose, are the same for parents of a typical kid (I can vouch for this, as I have a part-time job as the parent of two typical children). You don't want your child excluded, viewed differently, or to be deprived of something simple. It's a normal instinct, but with a child like Zoe, the volume is turned all the way up. Every quizzical look in public is weighted down and we carry it with us the rest of the day. Every quick look away from us is even worse. We wonder, do you pity us? Do you just pity her? Are you judging us for something? Just say what it is. And then when these wheels turn needlessly in our head for an afternoon, and then when we're proven wrong by your kindness, we dissolve emotionally. We cannot discuss it rationally. And we forget that there's work involved from our part to make the connection, to make other people take interest. Felicia had done a lot for Zoe, but she might not have extended herself yet again if my wife hadn't recognized the one thing that would make Zoe the happiest and done something about it. Sure, it was something simple, but it was the right action at the right time. Anyone can be a little girl's new friend, or her favorite friend, but her hero should always be her mommy. And whether it took all the work in the world, or no work at all, all is as it should be here at our house.

Guest Appearance on Rett Girl

The Rett Girl blog gave some fathers a chance to share their experiences with their daughters in honor of Father's Day, and they graciously invited me to be one of those guest posters. I was very happy to contribute and join the ranks of some wonderful, heartfelt thoughts from other Rett fathers. You can read all the posts at the link below.

http://rettgirl.blogspot.com/2013/06/zoe-knows-best.html

Smalltalker

A few weeks ago, I took both girls to the park. Hannah immediately rushed from one piece of equipment to the next, barely playing on anything for more than thirty seconds at a time. I'd warned her that it would be a short visit, given Zoe's fatigue after a long day at school, so her haste was understandable. Zoe's energy level lived up to my expectations, so we hung back a little, and it was clear she wasn't fully enjoying her time on the slide or the swings. There were two other little girls playing, also sisters, close to Hannah's and Zoe's ages. They tried to get Hannah to play with them, but she was too bent on her mission to spend time on all the play equipment before the world ended, so they wandered over to say hi to me and Zoe. They asked my girls' names and ages, and I asked them the same. They seemed intrigued by Zoe but didn't ask many questions about her. I could tell when they heard she was four that something about her lack of mobility and verbal ability struck them as unusual.

When we got ready to leave, our new friends came over to say goodbye to Zoe, and I told Zoe, as I always do, to say goodbye to them. One of the girls waited, cocked her head, and said, "She didn't say goodbye." Immediately I replied, "She can't talk." I am 39 years old. I am married. I dated other women before getting married.  I have said many unwelcome or ill-considered things, given an extra drink at a party or a dinner out. I have caused many awkward cab rides home. I cannot remember, however, a look shot my way quite like the one Zoe gave me when I told this little girl she couldn't talk. She looked embarrassed, she looked frustrated. We were having such a good time, but I had to go and remind her that she couldn't just be a little girl playing in the park with a new friend. That night, when I put her to bed, I promised her I would never do that again. I promised her, I would tell people she couldn't talk yet, and she smiled in relief and went to sleep.

Since Zoe can't speak yet, we have a communications device for her. When she was two-and-a-half, we took her to Easter Seals try out a Tobii. It's essentially a Windows computer that gets input from a device that tracks what part of the monitor Zoe's looking at. She adapted to it almost immediately (as do many girls with Rett, further testament to their intelligence). We were lucky enough that the therapist at Easter Seals helped document this to a degree that our insurance and the Illinois Early Intervention program split the bill for the most expensive thing in our house right now that is not actually our house.

We were lucky to get it. We're lucky to still have it and use it. We know families outside Illinois who haven't gotten the same assistance from their state health programs (including states not subject to the same budget crisis as our own), who've had to rely unsuccessfully on their insurance company for this necessary device. It is incredibly necessary for girls like ours who not only can't talk, but also don't have the motor skills for sign language.

Zoe loves having it and using it, but I'm sure there are things about it that aggravate her. The Tobii's default setting is to speak in a woman's monotone. There are multiple voice options; those options are other unlikely and uninteresting monotones. None of them are her voice, just an artificial substitute. There is very little about it -- its size, weight, or lack of portability -- that make it easy to use spontaneously. Once it's mounted on its tripod and booted up, she has to navigate to the screen she wants. It is probably as deliberate a process for her as if you or I had to communicate every urgent life need through a game of Charades. And to start out, that was all we programmed: urgent life needs. "I'm hungry." "My head hurts." "I want to lay down." There were all useful statements, but what about the times when those options didn't capture how Zoe felt, but she still wanted to talk to us?

About a year ago, my wife and I read a book called Out of My Mind, a young-adult novel about a girl older than Zoe, with a different neurological diagnosis. But like Zoe, and other Rett girls, the main character was non-verbal, and she relied on a hand-activated communications device. I was struck by how valuable it was to her to say the most mundane things with her device. We had completely stifled 80% of what Zoe probably wanted to say, thinking we needed to save the Tobii for the important things. But the overwhelming majority of the entertaining and memorable things Zoe's sister has said to us since she could talk have been the vastly unnecessary, nonsensical, and unimportant things. I finished the book and spent the next week writing out new Tobii pages, grids of commonplace things for our girl to say once we programmed them into her device. Lately, we've tried to capture many of them under a rotating group of buttons called "What's New." Her teachers know to look there before they have Circle Time at school, so Zoe can tell her friends the simple things that are...well, new.

Not all mundane statements are necessarily harmless. In an effort to give her a means to convey how she felt, we programmed a button to say "Go Away." She, like all kids, should be able to say that to us. She mostly saves it for her speech therapist (who she loves, but presumably annoys her every now and then). Sometime soon, I may program a "Shut Up, Hannah" button for her older sister. It's not nice, but what little sister shouldn't have the right to say that (and Zoe, we've all thought it). The least harmless statement was one I grouped under buttons explaining why she might be sad. "I'm sad because I can't do what I want." Her teachers let us know when she says it in class. We've heard her say it once or twice -- too many times. And each of those times, I've made another promise to her I may never be able to keep.

But mostly these buttons allow her to chat, even if she can't talk yet. A new girl joined Zoe's class not long after the New Year. The new girl is undersized. We have no idea what her condition is (this matters more to other special needs parents than we would care to admit). The first day, even though the girl had already been introduced to the kids, Zoe asked her what her name was. Since it was said in the Tobii Monotone, we don't know what sort of mood Zoe was trying to convey. It could have been small talk, asking the girl a question to which she already knew the answer, just to chat. Or she may fancy herself the class bully, unbeknownst to any of us, and she could have been trying to intimidate the girl for fun, in a very "Shawshank, new fish", kind of way. Until we find out differently, we'll assume it was just small talk, and a little small talk is a good start.

Lyrics Reprinted Without Permission

The other night, with both girls in the bath, I started singing something. I don't even remember what it was. As is usually not the case when I sing, someone asked me to sing more. Hannah wanted to hear a special song for her, and also a special song for Zoe.

Hannah actually has a special song, even though I rarely sing it to her anymore. Once, when she was a baby, and she couldn't settle down to sleep, I sang her "The Weight" by The Band. In the moment, I substituted her name for Annie, the woman named throughout the song. The Load was taken off Hannah, it was put Right On Me, and Hannah fell asleep that night. Maybe she enjoyed my rendition, maybe she like hearing her own name over and over again, or maybe she was caught off-guard by the well-meaning atonality that is my singing voice. Whatever it was, it worked.

I've always wanted to have a similarly special and effective song for Zoe. Since I'd wound up with a song for Hannah that I also happen to love, I had the same standards for Zoe. Or to more accurately rephrase that, I wanted a song that she loved that also properly represented my musical tastes. I tried many, many selections --  some Neil Young here, some Wilco there. It was never clear what worked. Most nights, she remained upset, awake, and at the mercy of the flashing synapses in her brain. I even serenaded her with a little "Mr. Brownstone" when I wanted to tease her for getting up Around Whenever, which she did all the time, at different times (it's only recently that she's been able to get up somewhat On Time). But because of Rett, she can't tell me what she likes. She can't make verbal requests; it's guesswork. She can look at me tenderly to say she likes what I'm singing at that particular moment, but she could be silently applauding the vocals, the song selection, or just the effort. The next night, the same song could be met with anguished screams and squirming, all depending on what Rett Syndrome decides to do to my audience's nervous system at the moment. It won't even let us share something so simple as the same favorite song on a nightly basis.

I have one song, of all the ones I've tried over the years, that I return to the most on those nights I need to calm Zoe down. It's not a perfect song for our relationship, but I love it, it makes me think of her, and not many people know it. It's called "Easy Hearts" by Whiskeytown, from the album they never quite finished before Ryan Adams went solo. If you look up the lyrics, you'll see a glimpse here and there of why it makes me think of Zoe, but it's not a perfect, literal translation of our shared experiences. There are really only a few lines that are even a little relevant. It's an imperfect song that, for me, represents an imperfect relationship. And when Hannah asked me the other night in the bath to sing something for Zoe, she assumed Zoe and I didn't have a special song. I had to tell Hannah, we sort of do, and it's called "Easy Hearts". I didn't sing it for Hannah, I didn't play the song for her later, I just told her the name of it.

A few nights after that, Hannah showed me something she'd written in her journal -- her own version of "Easy Hearts", also a representation of her relationship with Zoe, but far more perfect than I could have hoped for.

  Easy Hearts, going to sleep

Must be sure you are the one I love

[Throughout] my heart

I love Easy Hearts

I love you

I don't make you be me

Wait, you have a NOOK...

...I had no idea. That's adorable. I'm assuming it looks sort of like a Kindle, an iPad, or a tiny computer -- I just haven't seen one on the train before. Anyway, you can find the link for it on the right. Please download it, all the while, feeling free to share the link and post reviews.

E-Book is Live!

The essay about our experiences with Zoe is officially published on Amazon and available for purchase. It's our pleasure to share it with everyone who hasn't already read it. More importantly, we want to spread awareness about Rett Syndrome, and if we can raise a little money to help Zoe and the thousands of other girls out there like her, we have to do it. Your interest in this cause, in Zoe, and in her friends can bring the Rett research community ever closer to making this a winnable battle. Click the link just to the right to find the published version of the essay - thanks in advance for your support.

Back Off -- I'm Just Visiting

Zoe was back in the hospital last month, an eventuality we'd managed to avoid this winter. After repeated bouts of pneumonia last year, (including an overnight stay, which happened to be exactly one year earlier) we'd avoided more of the same this winter. Possibly it was the mild weather, possibly it was the vest and nebulizer treatments that have become routine, but regardless, the poor kid who'd been through pneumonia, EEGs, EKGs, sleep studies, and swallow studies had stayed out of the hospital.

Until last month. But this hospital trip wasn't like the ones from last winter, with IV connections and multiple tests and setbacks.This time, she could come and go as she pleased. She wasn't hooked up to any machinery, and she wasn't a tiny island of a kid in a vast hospital bed, way too big for her. This time, she was in the hospital to welcome her little brother to the world.

For the entire week that her mother and brother were in the hospital, Zoe visited them every day. She would sit with the two of them, the baby resting on her lap. The only attention she got from doctors or nurses was the repeated question "Are you a big sister now?", which she answered each time with a contented smile. We have never seen such peaceful expressions on her face, or such pride. Then again, she has never had someone to take care of -- it's always been the other way around.

The rest of us have taken the baby's arrival cavalierly. Granted, our lives have changed a little from before. There's one more person to feed, to hold, to ease into sleep. But for my wife and I, aside from the adjustment of changing diapers for a boy, and the attendant equipment, nothing has really changed for us. His oldest sister is used to helping, given Zoe's condition especially, and she does so in her own way. She didn't get too excited when her sister came along just over four years ago, so why should she start now? The only one who's been completely changed by this experience is Zoe.

To answer the question, from those doctors and nurses in the hospital, yes, she is a big sister now. And while she can't talk to her brother yet and can't do everything she wants, she has an emotional responsibility she didn't have before. Nothing for her will be the same again. And that was what struck me most about her joyous expression as we wheeled in and out of the hospital doors that week. She was finally getting a chance to not just be a big kid, but to be a little closer to having the freedom of being just another kid.

What is Rett Syndrome?

"We were forced to become expert in something that we never knew about, never wanted to know about, and something we will never fully understand. We learned a great deal about genetics very quickly, and each day we dug deeper into Zoe’s cellular mysteries. Rett syndrome is a disorder affecting the brain; it prevents the brains of girls like Zoe from telling their bodies what to do and when to do it, a symptom called apraxia.

The condition is the most physically disabling of all disorders on the autism spectrum. Quite simply, they know what they want to do, be it playing, running, singing, whatever they see kids doing in school, stores, or the neighbors’ yards. Their bodies won’t let them.

This is a result of a random mutation on the MECP2 gene, the Rett syndrome gene. Each cell in a normal female body silences one of its two X chromosomes. Girls with Rett have a healthy MECP2 gene on one of their X chromosomes and a mutated, stubborn version of the gene on their other X chromosome. The variances in girls’ symptoms are a result of the random nature in which the chromosomes are silenced; there is no way to predict which X chromosome, healthy or unhealthy, the body will elect to silence.

One of the hallmark symptoms of Rett is drastic regression in motor skills and abilities. We were lucky to a degree. Unlike many families, we didn’t have to watch Zoe develop on schedule, walking and talking like her sister and other kids, only to have her lose those skills around the age of two. That scenario is sadly far more common.

The affected neurons, don’t die, unlike in other neuro-degenerative disorders, like Parkinson’s. They can be reactivated; in fact, a scientist named Adrian Bird was able to do this in mice. He was able to temporarily disable one of the genes in them, and in that state, they exhibited all the symptoms as a typical girl with Rett. When the gene was reactivated, over the period of weeks, the mice returned to their normal physical state. It is possible to extend that treatment to these girls."

The preceding quote is an excerpt from an essay I wrote about our experiences with our daughter Zoe and adapting our lives to her neurological condition, Rett Syndrome. The entire essay will be available in the Kindle Store in February 2013. Proceeds will be donated to research for a cure for Rett Syndrome.