A Story for Sisters

I love being able to recommend movies and music to friends, especially when it's something they wouldn't have had exposure to otherwise. I'd love, right now, to be able to say that one of the movies that our girls have gravitated to is this little unheard-of gem from Disney with a great story about two sisters, and a few great songs. I'd love to tell you how much you'd like it, if only you knew about it. If only people had talked about it more. How if you searched hard enough on YouTube, maybe you would find one or two of the songs. But I can't, because I'm talking about Frozen.

Of course you've heard of Frozen. Everyone has heard of Frozen, as this great article in the Times illustrates. I've been thinking about the movie and a few of the songs this week, even before reading that piece, not just because they have been sung non-stop since January by the women who live in my house. Our older daughter Hannah turned 7 then, and chose, as her party, to have her girl classmates over to watch the movie and have ice cream in their pajamas (a sleepover with a more humane endpoint for us parents). We even had a special surprise mailed to us from my wife's cousin, who works for Disney.

All the girls, including Hannah had seen the movie already, so they didn't need to pay full attention throughout. They would mess with each other's hair, run around the room, or bounce around on our mini-trampoline --  basically fool around like seven-year-olds. Until the songs played. Every song commanded complete silence, which amazed me, but it didn't prepare me for the near-religious trance that descended on the room once "Let it Go" came on.

Hannah has one friend in particular who doesn't fit in with the rest of the girls. She gets picked on by a few of the other girls, and we're very happy that Hannah tries to stick up for her and be her friend. My wife and I  had to take care that night to make sure this girl was having fun; she'd even gotten teased at the beginning of the party for her choice of footie pajamas, and there had been tears. This girl was completely transfixed and transported when "Let it Go" started. Unblinking, her mouth moved silently along with the words. I didn't have, at that moment, a full command of the lyrics (at least compared to after the weekend's many repeat viewings). Once I knew them, I could see how the song spoke to this girl, who clearly felt like an outsider, at too young an age.

And as we watched the movie again and again that weekend, when the song came on, I would look over at Zoe, my own unusual treasure of a daughter, who at this point is old enough to wonder just how she fits in, here at home, at school, out in the world.  I could see her, too, absorbing the words and music. We played her the song more and more on her sister's MP3 player, or with the YouTube clip of the movie scene. Even her teacher caught on, replacing the Wiggles clips she used to play for Zoe as a reward for, or break from, hard work, a reflection of Zoe's tastes growing up along with her. And it is a great song, and a great scene (although it is not my personal favorite rendition of it).

But that is not the song from Frozen that resonates most with me. There is a different song that destroys me. Every day of my life I see two sisters with barriers put between them by Rett Syndrome. Some of those barriers may be working in our favor; a younger sibling who can't talk or use her hands effectively is also a younger sibling who can't tell on you, take your Barbies, or tell you to shut up. But most of the time, we see the girls' disappointment that they can't do all the same things, together, whenever they want. Riding bikes. Playing dress-up. Building snowmen. We see and feel that disappointment ourselves. It goes beyond disappointment.

We are raising two separate girls but trying with all our power to not have them have two separate lives. Granted, no one is wearing gloves to save the rest of us from her icy touch or quarantining themselves in their room, but there is still a division on which we never planned (especially for two girls we were so excited would be just 18 months apart forever). This spring we rearranged furniture in the house to make space in our son's room. As part of the moves, Hannah got upgraded from a twin- to a queen-size bed. Immediately, she started talking about having Zoe sleep over in her room. At first, I wasn't sure if it would work, but we rigged up pillows and cushions to help keep Zoe stable and in place. They've had a few sleepovers, but it's not the same as what, I'm sure, Hannah envisioned. They may giggle, Hannah may whisper to Zoe while they're still awake, but they can't pretend they're sharing secrets or arguing or anything that should come with a sisters relationship.

It is a beautiful spring day here, and even though we had snow flurries the other morning (a reminder that this winter refuses to give us up), no one is building a snowman. Zoe is napping, but we will try to do something with her outside later. I'm watching out the window as her older sister walks around the yard, talks to herself, and picks dandelions. It doesn't take much imagination on my part to know exactly what she wants.

The Promise of Ongoing Trials

Today was International Clinical Trials Day (not that I needed to remind anyone -- our Target started decorating for it after Valentine's Day). Our family obviously has a vested interest in the different clinical trials for Rett Syndrome treatments going on around the country, as each of them present an exciting research and testing avenue for Zoe and girls like her. However, when we hear that phrase, we think of one specific trial and one specific team, first and foremost.

Last month, we took Zoe down to Rush for her semi-annual visit to the regional Natural History Study. We've been making this trip with her twice a year, dating back to just after we got her diagnosis. It's provided an introduction to many other Rett families, whose friendships we've come to treasure. The study has also helped us form a wonderful relationship with a research team from Children's Hospital in Boston. Every six months for the last four years, we've gone down to Rush to meet with the same team, who are dedicated to finding a cure for Rett Syndrome. They've measured Zoe, taken her temperature, annoyed her on some days, but most importantly, gotten to know her and us. We hope through this whole process (which includes meeting with a hundred or so girls and their families in the span of a weekend), they've learned a lot about this awful condition. We know that we, in turn, have gotten invaluable insight into the work they're doing, as are their colleagues from Baylor University, Alabama-Birmingham, and Greenwood Research, who join them for the trip.

Sadly, this was the last time the teams will travel here for the foreseeable future. The study is being rearchitected to shift the data collection responsibilities to a local research team at Rush. This will save the research study funds in the short term and allow these teams to focus on their work in their home facilities. Each team is incredible, but we are partial to the Boston team that has been our constant, Dr. Kaufman and Kate Barnes, who are managing a very promising clinical trial, yet in its early phases. They are studying the impact that insulin-like growth factor (IGF-1) can have in strengthening the nerve synapses that have never had the chance to function in girls with Rett Syndrome, as well as people with numerous other neurological conditions like it.

Since this was our last trip to meet with them, we brought our older daughter Hannah with us for the first time in these four years. She had always been curious what these visits entailed (and why she'd been excluded from them; she didn't realize it was to shield her from the monotony of a four-hour hospital visit). About a week after our visit, she told me she wanted to invent a kind of medicine that Zoe and her Rett friends can drink to make the condition go away. I told her that was a wonderful idea. My wife and I had always told Hannah she can do whatever she wants in life. I didn't have the heart to tell her that this was probably the one thing in life she wouldn't be able to do, as much as it would mean to her. I know that Miss Kate and Dr. Kaufman, Zoe's great team of friends in Boston, are going to beat Hannah to the punch.

Good luck to them and to all the other research teams. We'll miss you, and we'll see you soon. We hope we helped.

Guess Who IRSF Let Be Dad of the Month?

Once again, I welcomed the chance to write about our experiences with Zoe in a forum different from this one. Sad to see my month in the spotlight come to an end...

You can find what I wrote, along with great contributions from even greater Rett dads, right here.

Missing Grammy

My girls have lost their Grammy. My mother-in-law was an incredible woman, as all who knew her would attest, and over the last few years, she was unfairly robbed of the version of herself that we loved. Originally, doctors thought her slow-building dementia was the result of Parkinson's or Alzheimer's, but repeated tests ruled those out a year or two ago. Instead, she had a condition called Lewy Body disorder. The person who suffers from it actually retains more of their mental faculties than is apparent. Their memory may suffer, but not to the same extent as with Alzheimer's. Their body just deteriorates, to the point that it requires an incredible amount of effort to perform simple motor tasks or express the most mundane thought. Once we learned more about the symptoms, my wife and I found them chillingly familiar. We already had someone in our lives, someone we love dearly, who is trapped in a body they cannot control. As Elise's condition worsened, I think our experiences with our daughter Zoe gave us better insight into what she was going through. The connection they already shared was bolstered by the common enemy they had to fight: a neurological impediment that waylaid the connection between their minds and their bodies. This bond of theirs made us more sympathetic to Elise's suffering. I hope, at least, that she would say the same, now that she's gone.

This was the first person close to our girls who passed away. Knowing that Elise's health was in decline, I was hoping that the girls' goldfish would die first, so we had an easy way to introduce this intimidating concept. It wasn't an unreasonable expectation -- we'd had the fish since August, and the family shared an amiable negligence for it. He didn't eat every day, and his water went way too long before it got changed. Still he stubbornly hung on, and he refused to die early enough to be an object lesson, which is always the way it seems to work out on TV. Instead, we had to explain death to the girls in the context of someone whose value to them was beyond measure.

Hannah got to see her Grammy in the last few days before she passed, which may have helped her to process things. Due to different factors in our schedules, we never were able to get Zoe over to see Grammy in time. I don't know if it would have helped or not. She may have had a hard time recognizing Grammy if she had. Elise had become a withered version of the vital person we all remembered. She spent the last week of her life in bed. Like all of us, Zoe would have too many memories of Grammy, even in the last few years, just doing something -- being active, playing with her and the other grandkids, cooking, singing. The Grammy at the end was not that Grammy, and it may have confused Zoe even more to see her like that. Instead, I had to explain to the girls, much more abruptly in Zoe's case, that Grammy was too sick, and her body was too tired. She was going to go to sleep soon, and she wouldn't wake up, which is what happens when someone dies. I was proven right within days.

It is hard enough to explain death to any child that age. There are just too many elements of it -- the finality, the overwhelming absence, for which they have no frame of reference. Hannah asked fewer questions than we expected; the questions have filtered in over the last month, but we aren't sure how well her mind has processed all this. We are even less sure with Zoe. We don't know what questions she has, because none of our means of communicating with her are sophisticated enough. She has an eyegaze computer that she uses to tell us simple phrases, ask questions, or answer them. The phrases and questions that would provide us an entryway into the topic are even too overwhelming to try to broach with her. It's even more difficult to try to guess what questions she has and put thoughts in her mind, worries or confusion that weren't there. Still, Zoe has always been Daddy's Little Stoic, so she might be coping just fine.

While Zoe and her Grammy loved each other immensely, I will always suspect their relationship was not as deep as her older sister's relationship with Grammy. The girls are only eighteen months apart, but much of the time Hannah spent with Grammy was time that Zoe couldn't share, by definition. In the time around Zoe's Rett diagnosis, and the year or so that followed, Hannah would stay with Grammy while my wife and I accompanied Zoe on hospital visits: EEGs and sleep studies, overnight stays to battle pneumonia, or biannual trips to Rush to participate in a research study. While we were off watching Zoe get measured, or have her temperature taken, or get hooked to an IV, Hannah was at Grammy's house, playing the piano with her, or baking cupcakes, or setting up toy trains. It's safe to say one of the girls may have been having a better time in those instances than the other.

But that's not to say that Zoe and her Grammy did not have plenty of special time together. It was just quieter and different. Grammy was always content to sit and cuddle with Zoe and watch the same Wiggles video over and over, or sit in a rocking chair and sing with her, as she'd done with all the grandkids. As Zoe got bigger, but her body and its abilities didn't catch up to her, Grammy may have had a hard time thinking of different things to do with Zoe, but those old favorites always worked. And cruelly, as Zoe started school, and we got welcome assurances about her normal mental abilities, her sense of humor, and her increased interest in age-appropriate activities (all things that Grammy would have appreciated and tried to cultivate), Grammy's own abilities began to deteriorate. They dovetailed. I will never forget one of my mother-in-law's last incisive thoughts that she could fully express. She, like us, suspected something was wrong with Zoe prior to her diagnosis. She passed along information to us about Angelman's well before we had any testing done on Zoe, well before we had resigned ourselves to the fact that she needed to be tested for anything. I read the information and dismissed it immediately. I was angry with my mother-in-law for putting this information in front of us, not-so-distant relative to the facts that it was. It was one of the few times I can remember being angry with her for anything. But she was right, she got across her point, and it helped us ultimately be open to accepting Zoe's condition. It was one of Elise's final, appropriate gifts to us and a reminder that she knew children better than anyone.

Prior to her funeral, the younger grandkids wrote letters to put into Grammy's coffin, and Hannah wrote letters on behalf of Zoe and her baby brother. Hannah didn't know what Zoe was thinking any more than we did, so many of the memories she wrote about were her own (and many of those things Zoe didn't participate in the same way). I made a point of asking Zoe some specific things that were important to her about Grammy, and she looked at me intently and smiled. I had Hannah add them to Zoe's letter, acting as her sister's hands as she so often does. It was one of the many times that I remind myself to trust my instincts with Zoe and know that she can, despite the many obstacles, clearly communicate to us that we are on the right track to understanding her.

At the actual funeral, Zoe fell asleep in church, possibly because she was legitimately tired and possibly as a defense mechanism to the physical and emotional strain. She missed her mother's beautiful eulogy about Grammy, but even in her sleep, she might have heard it, observing the words that any mother would love to hear from her daughter. Zoe is a daughter who loves her mother; she knows the message my wife conveyed whether she was awake to hear it or not.

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.