2/365

It can be very easy to feel overwhelmed when I think about everything that I feel I should accomplish in a day. Then I look down and see this.....and suddenly the little things don't seem all that important anymore. Dishes and laundry will always be there. I'm fairly certain they can wait a little bit longer.

1/365

I'm not sure exactly how this project will unfold....but I'm hoping that it will be an interesting journey!

These two have always had a special relationship. Despite his numerous interests and ever-increasing social network, the first thing Keirnan asks when he comes in the door is "How's Tatyanna....is she doing ok?". His concern for his little sister is obvious and I love watching them interact. When it would be easy to just sit and share space while playing the iphone, Keirnan gives a running commentary on what he's doing. He pauses to ask her questions and then carries on as though she answered. Tatyanna adores him. Sometimes all it takes is to say his name and her face will break out into the biggest smile. He's the one that would sit for hours with her, playing computer games that can only appeal to little girls and never fails to make her giggle with his repertoire of zombie noises. While a part of me grieves the loss of the relationship that will never get to exist between them, I can't help but marvel at the awesome bond that they have in spite of it all.

It's definitely been a while since I last updated and for that I apologize. It's funny how 8 months can be both an eternity and not nearly long enough. Since I last updated, there have definitely been many changes. Tatyanna hit a rough patch in the early summer that robbed her of most of her strength and she can no longer sit independently or even hold her head unsupported. Her seizure activity has definitely increased and just before Christmas we made our first trip to the ER in a long time. I don't think watching your child seize is something a parent can ever get used to. Even with 3 years of experience, it still leaves me shaken. A one night stay on the pediatric ward and some med changes and things seem to be more stable. She's still "twitchier" than I would like but the complex partials and tonic clonics seem to be held at bay. But despite all of this, Tatyanna continues to amaze me on a daily basis. Even though her condition has definitely declined, in many ways she is still the same Tatyanna that she has always been. We are no longer sure how much eye sight she has left but she continues to giggle through her favourite shows just as she always has. Books also remain a big hit and she loves nothing more than snuggling up with someone to read through a Jillian Jiggs...or three.

To anyone on the outside, these last 8 months probably seem pretty depressing. But in some ways, it has been the opposite. Despite all that is so obviously wrong I am amazed by all that is right. I am continually blown away by the awesome people that have woven themselves into our life. People who so obviously care for my daughter and want to make a difference. Because of the hard work of a group of dedicated individuals, Tatyanna continues to attend school several days a week. She is currently in a regular grade 2 class with the help of a couple of support aides. Although, at a grade 2 level, the work becomes a little more demanding and Tatyanna and her aides often float around the school looking for where ever the fun is happening. The kids at her school are equally amazing and I often find pictures or notes tucked into her bag, drawn by fellow students.

***

I haven't had nearly enough coffee to come up with a good segue into this next part. Tomorrow marks the start of a new year and with it the requisite resolutions and commitments. Because my journey as an aspiring photographer and a mom to a Battens kiddo have often been so closely intertwined, I am planning to embark on a 365 project. The goal is to take one picture every day for an entire year. Not only will it force encourage me to pick up my camera every day but it will also serve as a way to document our life as a family who is affected but not defined by Batten Disease.

Author -Unknown

If before you were born, I could have gone to Heaven and saw all the beautiful souls, I still would have chosen you.

If God had told me, “this soul will one day need extra care and needs”, I still would have chosen you…

If He had told me, “that one day this soul may make my heart bleed”, I still would have chosen you…

If He had told me, “this soul would make me question the depth of my faith”, I still would have chosen you…

If He had told me, “this soul would make tears flow from my eyes that would overflow a river”, I still would have chosen you…

If He had told me, “our time spent together her on earth could be short”, I still would have chosen you..

If He had told me, “this soul may one day make me witness overbearing suffering”, I still would have chosen you…

If He had told me, “all that you know to be normal would drastically change”, I still would have chosen you…

Of course, even though I would have chosen you, I know it was God who chose me for you…

It's strange, being the parent to a Batten kid -you go along thinking that you're really doing ok and then you get handed a paper at school to fill out and on it is the question "What do I want to be when I grow up" and it's like you've been sucker punched.....so I answered Canadian Olympic show jumping team member - I figure if we're going to dream, it might as well be big.

Just a couple of quick pics to show you how much Tatyanna has improved over the last few days! Although she is definitely no longer a morning person, by noon she is becoming more and more like the happy, perky little girl that we used to see. The school has been awesome in accommodating our new schedule and Tatyanna has really enjoyed her last few afternoons with her class. We are gradually starting to incorporate more PT and stretching into her daily routine and while I can't say that she particularly enjoys it, she is responding. We've been trying to get her up in her walker for short bursts during the day as she seems to really enjoy the freedom of careening around the kitchen. The cats, however, do not share the same appreciation for her newfound locomotion and much preferred the warm lap of the sedate Tatyanna over fleeing the kitchen for the sake of their tails. The increased activity does leave her exhausted and 20 min in the walker usually means and hour in the beanbag chair recovering. But, she's definitely improving. Truthfully, I wasn't sure that she'd ever hold her head up again so anything that we see now is a huge bonus.

love

Definition of sisterly love - taking the time to cover your sister's eyes, because she is no longer able, before your own during the scary part of the movie- Disney's new frog princess. I wish I had my camera for that moment, it was priceless. (I may or may not have had to leave the room because I got all teary eyed.)

two steps forward, three steps back....

Just as we feared the effects of the anaesthetic took their predictable toll on our sweet girl. Every time we have had her put under before we noticed, within the weeks following the procedure, a drastic increase in her seizure activity. This time was no different and we were forced to make some necessary med. changes. Unfortunately these changes aren't without their effects and there are only a few hours in the afternoon where I could call her alert....ish. We are hopeful that she will adjust to the new doseage and perk up a little very soon! We are going to try taking her to school for a couple of hours in the afternoon tomorrow. As much as I would love to keep her all to myself, I know that she really does enjoy the other kids and they have really been noticing her absence in the classroom. So, fingers crossed and praying that tomorrow will be a better day...at least for the afternoon. On the upside though, her seizure activity has returned to its previous level of acceptability.

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.