Same couch....different day....and looking so much perkier! :)

(pic taken in the first few days home from the hospital)

It's now been 2 weeks since Tatyanna's surgery and although the recovery has been slow in some areas, we are noticing consistent improvements. She is back to enjoying her Nick Jr. and even spent the morning at school yesterday. Her strength is slowly returning and she is able to sit up for longer periods without much support. She does, however, tire very quickly and still spends a large portion of her day snuggling with family on the couch or in her bed. Something surprising that we have noticed is that Tatyanna seems to still have some eyesight remaining. Before the surgery she no longer looked at books or t.v. and did not seem to focus on those around her. We had assumed that it was the disease taking its inevitable toll and were saddened to think that our little girl was now totally blind. In the days following the surgery we started noticing that Tatyanna seemed more alert and was actually focusing on faces and tracking the movement of the nurses in the room. She began to look at the pictures in books as she was being read to and would turn her head to look as the pages were turned. Our assumption is that she simply felt so lousy before the surgery that she had lost all will to do anything that she once enjoyed and it only looked like her eyesight was completely gone but we aren't sure. It doesn't really make any sense.....but I'm certainly not complaining!

The rest of us are adjusting to having a family member who is gt feed and all it entails. The older kids are good at helping me "watch the drips" and are good at sitting with Tatyanna if she is being fed and I needed elsewhere. Lexi is particularly enjoying the influx of fun, new medical equiptment and has been known to careen around the kitchen, using the IV pole as her personal scooter. Old syringes make handy water guns and the cans of formula make a pretty spectacular tower when she stacks the up. :)

Thanks so much for the prayers and encouragement that we have received over the last few weeks, we truly appreciate them and don't want to imagine where we'd be without them! I feel truly blessed to have so many amazing people in my life!

Tatyanna's g-tube surgery went really well on Monday and they were able to perform the operation laproscopically. This means that there is only the tiniest incision and no stitches. Unfortunately, tiny incisions are still painful and 5 days later she requires regular pain medication to stay comfortable. We did have a few stressful hours at the hospital while we waited for our sleeping beauty to awaken in recovery. After 5 hours of coaxing from mommy and daddy, consults with anaesthesiologists, she remained difficult to arouse. Eventually we had an idea and someone brought in a Dora dvd. Apparently Dora and friends were worth waking up for and she opened her eyes the moment the intro started. Go figure.

Tatyanna adjusted smoothly to the g-tube and was at her target intake by Wednesday evening. By Thursday afternoon we were on our way home!! She still has a long way to go but I can already see a vast difference in her appearance. Her colour is better and she is so much more alert....this morning she was laying in bed, happily yelling at dora and trying to yell "Map!". Truthfully, I never thought I would see her that alert again. Physically the surgery took a significant toll on her body and she has lost a great deal of strength. Sitting up, supported, in a chair for only a few minutes leaves her exhausted. Even holding her head up requires supreme effort on her part. Over the next few weeks we will work on gradually increasing her strength and are hopeful that she will be able to return to school very soon!

Unfortunately it has been a relatively rough week for our Tatyanna. Despite our attempts to keep her eating over the last several weeks it has become obvious that intervention will be required. After much discussion we made the decision that it is time to have a g-tube placed in order to give Tatyanna the calories that she needs to function. Over the last few weeks she has been drastically losing weight and has lost any desire to eat....despite my spiking everything with whipping cream and butter. :) Her energy levels are almost non-existant and there are times when she has difficulty holding her head up or even staying awake. We are hoping that once she recovers from the surgery that she will regain some of the ground that she has recently lost and that this recent regression in her condition is not due to disease progression. Please keep our sweet girl in your prayers. She is a fighter but I know that her body is tired and that the effects of the general anaesthetic can cause significant regression in some Batten's kids. I will do my best to post some sort of update from the hospital post-surgery.

Two years ago today we received Tatyanna's diagnosis of LINCL. In the time since, I have learned to become a researcher and advocate, I have become stronger than I ever thought possible. I have learned that it's ok to live in the moment, to let go of what or who is not important and to celebrate the small things. I've seen that there is light and hope and beauty in the darkest places and that sometimes the miracle you pray for is not the miracle that you receive but that they are all around you .....you just have to look -sometimes really really hard. :)

As put, rather insightfully, by one of the other Batten parents, "our children aren't dying from Batten Disease, they are living with it." (thanks for the awesome quote Nikki!) I love this quote because it eloquently sums up how we as a family strive to live. I believe that if we focus on the negative then the disease wins and our losses will be far greater than the obvious. That isn't to say that there aren't bad days, that there aren't times when the pain threatens to overwhelm and the very act of getting out of bed is a supreme effort of will. I'm human, after all, there are days when my strength falters and my spirit is tired. But, I have to remember, this isn't about me....it's about a little girl with more courage and heart than I have ever seen in another human. It's about a little girl who once radiated enough energy that she almost seemed to sparkle. It's about my sweet daughter who at the end of being poked and prodded for the millionth time still had it in her to smile and want to give out hugs to anyone around. Because of her, at this moment, I choose to aknowledge what is good. Even when things look their bleakest, there is still love and laughter and life.

Carter's Valentine's Day Miracle Campaign

One of the first Batten parents that I "met" in the days following Tatyanna's diagnosis was Miranda. Over the months that followed we talked on the phone, exchanged emails and supported each other through what can only be called a parent's worst nightmare. We evolved from recruits, drafted by force but eager to fight, into seasoned veterans with more knowledge about "trench warfare" than either of us could have anticipated. Optimism gave way to realism and although hope prevails, neither of us is naive any longer. We know the costs, we've watched the ranks swell and mourned the losses of so many courageous kids. Kids who had to fight so hard and ultimately lost their battle. On January 17th, Miranda and her husband Neil lost their beloved Hailey to Batten Disease. She was only 7.

Although their hearts are breaking the Goranflos are committed to the fight. Their son Carter also has Battens and unless a cure is found soon, he will join his big sister long before he's ever had a chance to live. The attitude of many Batten parents is very much one of "We may lose this battle, but we will NOT lose the war" Our kids can't die in vain. A cure must be found. Please, this Valentine's consider helping in the fight. Miranda and Neil have organized a fundraising campaign. It's simple, doesn't require a huge committment and has the potential to be very effective. For the price of a box of chocolates this Valentine's Day, you could make a difference. You could be part of a miracle. Donate $10 (or whatever) and then tell 4 people to do the same. Ask that they tell four more. And on it goes! See, I told you it was simple! For more information, please go to www.haileyandcartersdream.com Donations can be made through the paypal link on their website.

I apologize for the lack of updates on here as of late but as they say, "No news is....." Truthfully, it's just no news this time. Tatyanna isn't doing too bad, but she's not nearly as good as we would like. We have noticed some regression but so far, it has been fairly slow. The biggest area of decline is in her strength and stamina and we find that she gets tired much quicker than she did in the fall. There have been times where she will doze off in the afternoon for a quick catnap and this is extremely uncharacteristic for her. Other than that, she is doing fairly well. She had a really good Christmas break and thoroughly enjoyed the mass quantities of books and movies that she received for Christmas. She is still extremely vocal and although she doesn't necessarily have the words, she can still get her point across. She even surprised us several times by yelling actual words at Dora. It's funny how excited the word "Map!" can make a person. :)

Now that the holidays are over, Tatyanna started back at grade 1 today and seemed quite happy to be there. We are really hoping that this part of the school year goes as well as the beginning.

With the new year also comes our next fundraising effort! I hope to be able to fill you all in very soon but want to make sure that we have all the details down first. Keep checking back as I hope to have things ready to go before the end of January. All money collected will be going directly to the fight against Batten Disease on behalf of Tatyanna's Hope. We do not have any trips to China planned at this time and would like to focus our efforts on finding a cure.

In the meantime.....

On January 22 a movie entitled "Extraordinary Measures" will hit the theaters. Starring Harrison Ford and Brendon Fraser, it is a story of a father's fight to save his 2 children from Pompei Disease. His fight so closely mirrors our own struggle in the Batten community and we are all hoping that it will help create awareness of the plight of the many children who suffer from rare conditions. To help give back to the community, Extraordinary Measures has a contest up on their website. The winner will receive a donation of $10,000 to put towards the charity of their choice. My plea to you all is to please take a moment to go to the website: http://extraordinarymeasuresthemovie.com/ and click on the link "Inspiration Quilt" On page two of the quilt is a video entitled "Noah's Hope:Fix You", you can also find it by typing Noah's Hope into the search feature. Noah and his little sister Laine, like Tatyanna, suffer from late infantile Batten Disease. Please take a moment to watch the video and then vote. Then, you can do the same thing tomorrow and the day after. In fact, you can vote every day! The 2 seconds that it takes may be all that's needed to make a difference. Noah's Hope Foundation will match the dollar amount if the video wins and all proceeds go directly to the fight against Batten Disease. That's $20,000!!! Our amazing kids fight so hard but we need your help. Please vote and encourage your family and friends to vote as well!

I just wanted to give you a heads up that the email account info@tatyannashope.ca has not been working for the last several months. If you sent an email to that account and have been wondering why no one responded, I truly apologize. Somehow the email settings got changed and so although it appeared to be working, nothing was actually getting through. Once my primary computer decided that it was calling it quits and I had to reset my email accounts on an old laptop, a tonne of emails from the last 6 months have come flooding in. Wow, I feel really bad! I am in the process of responding to as many as I can but it may take a while! I truly apologize for any inconvenience that it may have caused! Now that everything seems to be sorted out, please feel free to try sending an email again and I will respond much quicker this time!

Tatyanna's Hope email can also be reached at tatyannashope@live.ca .

Tatyanna is still doing really well! She seems to be avoiding any of the major illnesses that are going around her school and has been having a great year so far. She is not so thrilled with the coming of winter however and has perfected a look that can only be described as utter disgust the moment she hits the cold air. Poor kid.....and this is only the beginning!

For all appearances it looks like the picture of sibling devotion, no? A big brother sitting next to his little sister, patiently enduing websites that are filled with an assortment of cartoon animals. What the picture doesn't show is that on this particular occasion, the awesome big brother happens to be playing on the Webkinz site. It also doesn't show that he has just entered the code from a brand new Webkinz toy belonging to an absent party and has named the resulting cyber kitty "Sticky-Head". Because this is only a photo on a blog you don't hear the resulting screaming, yelling and general sense of woe coming from the owner of the Webkinz account. Apparently the creators of Webkinz did not take into account things like the devious nature of older brothers and once named these pets cannot be re-named (unlike real life kittens who can go from Stripey to Alienfishcat to Olivia). I'd love to tell you that I did my job as a good mother, reprimanded the offending party and fixed everything for the individual who has been so profoundly wronged but for some as soon as I start talking, it all seems kind of funny and by the time I get to the word "Sticky-Head" I'm laughing too hard. Word of advise to those considering becoming parents, laughing is NOT the appropriate action in this instance and will only makes things worse. It will make you seem just as guilty as the perpetrator of the injustice and you will immediately get the title of "worst mom ever". But, by this time, it won't be the first time that you've worn that particular title and you know that it is fleeting and so you will probably continue to giggle and plan to make amends once the dust settles a little. On that note, I guess I'd better go and drop another dollar in the kids' future therapy fund.

Kid of the day

Every so often Tatyanna's grade 1 class has what they call the Kid of the Day. It's a moment where they can celebrate the individuality of their peer and practice their drawing and printing skills. Recently, it was Tatyanna's turn and before we could even get her stuff in her locker there were kids running out of the class excitedly informing us that Tatyanna was going to be that day's chosen child. Truthfully, I had no idea what exactly it meant so I smiled, responded with an enthusiastic "wow! that's awesome!" and went on my way. When I returned to the school to pick her up, I was given a package containing all the drawings that the kids had made. As I began to flip through them, smiling at the kids' burgeoning art skills, I began to notice something that truly made my day. Some kids had chosen to draw the gold ring on her finger, some gave her ponytails, one even attempted to draw her bright orange wheel chair, but almost everyone had drawn her with a huge smile. As a parent to a "sick kid" I always worry about how her condition will affect those around her. Batten Disease is complicated and we have really struggled with how much information to share with her class. I know that eventually there will come a time when we will have to withdraw for the sake of the other kids but for now, I feel good about our decision to have Tatyanna attend a regular grade 1 class. At this moment, she is an important part of their class, and although she has some obvious issues, they choose to celebrate what is good.

I can't believe that we're already half way through October! Tatyanna has now been back at school for over a month and continues to do really well. She actually really enjoys going to school this year and we have been adding in a few full days every week instead of all half days. This is something that we had serious reservation about at the beginning of the year. The kids in her class are truly an amazing bunch and really try to make sure that she's included in what's going on in the class. So far her favourite parts of the day are definitely gym and music. :)

Physically, Tatyanna continues to be relatively stable. We do, however, have some concerns that her eyesight is starting to fail. While she still enjoys watching T.V. and looking at books, we aren't certain how much longer this will last. We have decided against having any sort of evaluation done, as for us, it's not important. With Batten Disease, loss of eyesight is inevitable and we feel that we function better not knowing exactly how much our little girl does or doesn't see. Despite this, she is still very much a happy little girl and blesses us with her smiles and giggles every day.

I could feel my heart sink and anxiety start to prickle at the edges of my consciousness. Of course that particular store in this mall had to be right next to the only place that I buy jeans. For a moment I debated going home, destined to spend an eternity borrowing clothes from my older daughter....or at least until she outgrows me (which could be tomorrow at the rate she's going). But, that would be foolish. Living in a relatively rural part of the country means that when you're in the city, you have to shop and normally, that's not such a bad thing. I gave myself a brief pep talk and began pushing Tatyanna's wheel chair with determined steps, hoping beyond anything that she wouldn't notice the sunny, yellow glow emanating from the source of my unease . It used to be her favourite place in the world. When we lived in Edmonton she would spy it from the upper concourse of the mall and make a mad dash for the nearest escalator yelling "I'm going to Build-a-bear....to make-a-bear!!!" She didn't care if she wasn't getting a bear, she just loved the process. The picking of the outer layer, the fluffing machine that stuffed the chosen companion to the perfect level of huggability and the kissing of the heart and making a wish before the new friend was sewn up, ready for dressing. She kissed a lot of hearts. I think most of our menagerie of build-a-bears contains more than one. Her eyes would sparkle as she danced through the store eagerly helping to chose the perfect outfit and accessories. The shoes were her favourite. A girl after my own heart, it didn't matter if they didn't have underwear...the outfit wasn't complete without an awesome (or is it pawsome?) pair of shoes.

I wasn't sure which would be worse, if she saw the store and didn't react at all, or if she did. At first I thought we were going to make it by without incident. Tatyanna's attention was caught by something on the opposing side and it wasn't until we were almost clear that her head turned. A veiled look of recognition crossed her face as she peered hard into the interior of the shop.

"Look! It's Build-a-bear! Wanna go in?" I asked, trying desperately to keep my voice cheery. Drawing on some apparently vast internal reserve of strength we made it past the helpful store staff and down the row of bins full of waiting animals. They finally have the pony. It was only out in select stores during the "old days", and we kept waiting for it's arrival, knowing that it would have been Tatyanna's new favourite. But the release date kept getting pushed back, and then it just didn't matter anymore.

It wasn't until we hit the fluffing machine, with the bin of hearts waiting to be kissed that the sense of loss came crashing in around me. Apparently, while vast, my reserves are also shallow. Tatyanna stared hard at the swirling fluff and I could feel my heart shatter once again. It was as if she was remembering. Remembering a time when things were better, when she could dance and sing and kiss hearts and make wishes. But with the expression of nostalgia there was also confusion. As if those past times were maybe a dream and perhaps never really happened at all. As if she couldn't comprehend how she had gotten from there to here.

It was all I could take, I think I fled the store fast enough that the employees checked for fires and counted their inventory after I left.

I hate Batten Disease. I hate that it is stealing our little girl before our eyes. I hate that remembering the fun times of days gone by is now done with a smile on my lips and tears running down my cheeks. I hate that every day, I have to pick up the pieces of my heart over and over again. But, I do. I hold my head up and keep going. I pick up the pieces and try to make our life the best it can be. Sometimes it works, sometimes I just really want to go back to a time when my little girl could kiss a heart or two.

Pimpin her ride

It was probably one of the more difficult transitions on our journey as parents to a child with a progressive condition. By switching out Tatyanna's stroller for a full fledged wheel chair it seemed that we were finally publicly acknowledging that this was, in fact, a battle we were losing. For a long time I would simply carry Tatyanna wherever we went. The Lightening kids stroller that we were using was uncomfortable for her and had a distinct prima dona preference for only the smoothest of paved surfaces making its usage cumbersome. But, one can only carry their growing 6 year old for so long before you realize the complete ridiculousness of the situation. Although she has yet to weigh anything more than 42 lbs soaking wet, Tatyanna's legs now dangle down to my knees and the once comfortable hip carry, is now anything but. The health of my back also seemed a high price to pay for my own personal sense of pseudo-denial. And so, with much reluctance, we came to the decision that it was definitely time to look into a more conventional method of getting our non mobile child from point a to point b and took Tatyanna's occupational therapist up on her offer to make the referral for wheel chair funding. Once at our local Medichair we were quickly given the specs on a couple of the pint sized models and took them for a "test drive" to determine their suitability for our needs. Once a decision was reached we were asked to chose a colour. I'm not sure why this somehow makes the whole ordeal verge on fun, but it did. After looking at a rainbow of hues and being assured that the chosen chair could be power coated with an entirely different colour if we wished, we decided to go with the stock orange. It seemed bright and funky - a perfect way to honour our daughter's spunky spirit. We also came to the realization that just because it's a wheel chair, doesn't mean that it has to be boring and thus we have started on "operation pimp her ride" in an effort to make it the coolest damn wheelchair a kid could ever have. Kind of a lemons to lemonade thing.

My apologies for the lack of updates on here as of late. The summer went by far to quickly and there weren't nearly enough hours in the day! Tatyanna had a really good summer and will be starting her second week of grade 1 tomorrow. She has an awesome full time aide and an equally amazing teacher who both report that she is doing really well and having a good time in school. She seems to enjoy the interaction with the other kids and especially likes gym class. We have an excellent team of individuals at her school and everyone seems to be on the same wavelength with how her year should go. The main idea is that Tatyanna will have the best year possible and will be included as much as much as she can be with what is going on in her classroom. Gr. 1 is off to a superb start!

For the moment, her condition is relatively stable and we haven't noticed any significant changes since our last trip to China. While she can no longer walk independently and is mostly non-verbal, she doesn't let these things stop her from getting where she wants to go, or from getting her point across!

One of the nice things about moving her updates over to the blogger site is that it makes it so much easier to add pics to the entries. The following photos were taken over the last couple of weeks.

Welcome

Greetings and thanks for coming! This is now the new site for all of Tatyanna's updates etc. Over the last few days the old Tatyanna's Hope website has been completely redone. I hope that you like the changes and new format. There are still a few bugs to work out but for the most part, it is up and running, business as usual. We made the decision to move the regular (ok, semi-regular) updates over to a blogger site so that it is easier to navigate for all of you, easier to update for me...and should allow me to actually post a photo or two every so often. It will also allow people to leave comments, ask questions or easily locate a previously made entry. Please bear with me as there will undoubtedly be a few growing pains as everything gets transferred over.

Tatyanna has been having a good summer. The record-cold temperatures have put a damper on our summer fun but we have still found opportunities for riding her pony and trips to the spray park and playground. Although she is no longer able to climb, Tatyanna still LOVES going down the slides! We are also currently looking into some various physical therapy options as we found that she really benefited from the concentrated PT that she was receiving in China.

going home

As I type this entry, the suitcases are stacked against the wall, waiting for the last of the laundry before they can finally be closed. The dr. has been in with the meds that Tatyanna will need to continue and has authorized the removal of her IV. It's official, we are going home tomorrow. Truthfully, this time I don't have the eager sense of anticipation of last year's departure. While I miss my family and home, the break from reality has been a much needed reprieve. For a few moments I am allowed to have hope, to believe that we can fight the demon that is Batten's. At Tiantan Puhua, we are surrounded by people who want to try, who work incredibly hard and believe that they can make a difference. The results of the last few weeks have far exceeded our expectations, I saw my daughter walk across the room, unsteady, but completely unaided, I heard her say “love you” as she gave me a hug. Those moments alone made the entire trip worth it. To all who have helped make this trip possible and supported us on our journey, we thank you from the bottom of our heart.

In these tough economic times it's important to follow the "3 r's" a little more closely. Buying products that allow for versatility, and are easily re-used can easily help to keep a little more change in your pocket.... and no one seems to have mastered the art of that 2nd "r" quite like the local silk store. Finally, a shirt that the whole family can enjoy:



from the very smallest member

to the largest

Easter eggs.....

Every day we order pretty much the same thing for lunch...some sort of meat, some sort of vegetable and jidan chao fan (egg fried rice). Not exactly adventurous but with 2 little kids, one of whom has certain dietary requirements, playing it safe is sometimes the way to go....at least at the beginning of our trip. Everyday at some random time before lunch, the specified food arrives. We generally request 1200 for delivery but in reality it may appear anywhere from as early as 1045 to 1215. See, there is an element of adventure after all!
On this particular day though, things got a little mixed up....slightly lost in translation and our staple jidan chao fan turned into this:

Truthfully, I have no idea what exactly it is, how the eggs were turned a purplish brown colour or what turned them into a jelly like consistency. Trent and I thought that it seemed a rather fitting mistake given the nature of the day, but the kids? They failed to see the humour.

We are finally here in Beijing! I'm not sure whether I was more excited that the temperature was 27 C when we landed with not a snowbank to be seen or the fact that we survived another landing at the Beijing airport. I have no idea what it is about that landing but it always seems precarious at best. There were actually audible gasps from a majority of the passengers during a couple of moments. Fortunately the landing was even worse on our first trip so I think that I managed to pull it off looking calm and collected.....white knuckles aside.
Once we arrived back at the hospital we were greeted with some excitement from the staff that remembered us from our first trip. I've got to say that it was really nice to come back to a familiar place and within the first hour we were off to the corner store to pick up the kid's favourite snacks and drinks. It's funny, it's been an entire year since we were last here but it doesn't feel like more than a week or two. Here's hoping that the girls will settle in and adjust to a 13 hour difference quickly!

Beijing bound

It's funny how you can be both excited about a trip and dreading all that it entails. In early April we will once again be making the trip to Beijing for a second round of treatment for Tatyanna. Initially our plan was to follow her original doctors to the new hospital that they have recently established. Lured in by the idea of a potential new treatment involving a gene therapy component as well as the shiny newness of the facility, it was an easy sell to a set of desperate parents. Then, as it so often seems to, the proverbial rug was yanked from beneath our feet and we were left wondering if we had made the correct decision after all. The lack of information regarding the new therapy was disconcerting and logically it didn't add up. My slightly unused scientific mind finally started thinking of the right questions and when the answers didn't match up to our satisfaction we really started to wonder if we had made the right choice. After several conversations with respected people in the Batten's community we decided to completely alter our course of action and will be taking Tatyanna back to Tian Tan Puhua. It was not an easy decision but for the first time in the last month, I feel good. All of a sudden there is a sense of peace that things are the way they should be. We know the treatment protocol at Tian Tan and we know the outcome. It's not a cure but the changes that we noticed in Tatyanna were substantial and it went a long way to increase her quality of life over the last year. We are familiar with the staff and the surrounding area. While I agree that there is a time when you should push the envelope, I don't think that for us, this is that time. There's a huge difference between a viable working therapy and something that is still highly experimental. I'm all for trying something new if it makes scientific sense but the new therapy just didn't fall into that category. I do however strongly believe that gene therapy will ultimately be the cure for Batten Disease, I just don't believe that anyone has that cure yet.

Heres lookin' at you kid

This is one of those moments where I, thinking that I had taken a nice "memory photo", put the camera away a few minutes too soon. Right after this shot was taken, Tatyanna, in a moment of unbridled Bovidae adoration, leaned forward, placed both hands on either side of the goat's face and planted a big wet kiss on his lips! Comical does not even begin to describe it. I "kid" you not. Umm...I'll stop now....