Birthday Festivities Continued....

I have to admit, I was a bit nervous about Alexa's Birthday weekend away.  You see last year we went to Great Wold Lodge and Alexa loved it.  She wanted everything to be the same...EVERYTHING....same people, same place.
However, I had decided to change it up a bit and try Niagara Fallsview Indoor Waterpark, partly because we had seen it last year and thought it looked nice, and partly because we got a much better package than at GWL.  As the time got closer, and Alexa spoke about it more and more, I started worrying that I had made the wrong choice.    I needed this to be a great weekend for Alexa.  Her summer vacation will literally be cut down to about 2 1/2 weeks, so I want to jam pack those days with nothing but fun.   When she found out we were going to "the new place|, she was NOT thrilled.  Oh boy...

Well, turns out I was worried for nothing!  The water park not only met but exceeded our expectations.  We had an amazing, super, wonderful time.  It was all I could ask for and more!  

And someone I know was super excited once she saw the how great the park was!

I love watching Alexa experience something new and watching the joy on her face as she ran up and slid down the water slides for the first time was incredible.  So incredible, that I raced up and down with her as if I was 5 again--she is a lot faster than me and I tired out a lot quicker than she did!  I guess that really isn't surprising though!

We went on those slides A LOT!

 Determined to go down as many times as her little legs would take her up those stairs!

And we played in the wave pool a lot!  I have to say those waves are a lot stronger than I thought!

Loving on her big cousin!

What a great big brother!

 Daddy always makes sure these ones are having fun!

This trio was out cold on Saturday night!  Slept in till 9am on Sunday morning, which was a special treat for mommy and daddy!

Our package also included some non-water related activites.......

We saw Happy Feet and Adventures to the Mysterious Island in 4D

Got to try Bumper cars......

A bit of Mini-Put.....

Rode in  SWAT car trying to catch criminals.....Sorry--we were too busy shooting those criminals to take any photos!

And a ride in the Pink Panther Balloon overlooking Niagara!

We also walked along Clifton Hill to see all the wacky things and for some more photo ops!

It was a great weekend!  I really could not have asked for anything more!  And Alexa is already talking about next time she goes there!

And then she turned 5

It is always important for me to make the most of Alexa's birthdays.  As a parent, I want for her to have the best time imaginable.  I want her to enjoy every second of her day!(or weekend as it seems)  This year, for obvious reasons, it was even more important that her day be as special as can be.


 
Alexa was so excited from the moment she woke up.  She went to her dresser and picked out the princess dress that she bought with the grandmother the night before.  She was so surprised to see the house already decorated.

She had a wonderful day at school.  Since food options are no longer allowed, we bought some stamps and stickers for her to hand out to her classmates.  Her classmates sang to her and she blew out candles, making a wish.  Her teachers reported that it was such a fun time for all.  

I had said that Alexa has a certain  unexplained way of captivating people, and yesterday I saw just how true that was.   Her teachers--all 3 of them gave Alexa a special little present.  Each and every one of the   presents had a significant meaning to their relationship with Alexa.  One of them in particular was quite special as it was from her teacher who will be sharing her knowledge at another school next year. It became apparent to me just how very close the two of them have become and just how greatly she will be missed.  I am hoping we can have Alexa and and her special Ms. D keep in touch--I think Alexa would enjoy that.

I picked her up and she was so excited to get on with the rest of her birthday festivities.

Awhile ago, I asked Alexa what she preferred to do for her birthday.  She could pick a big party with all her family and friends or we could go to Niagara Falls and stay at a hotel with a themed indoor water park.  She chose the later.  She was very adamant--has not wavered at all and could not be more excited to go.  She has been talking about it for months.  She wanted to take her mommy, daddy, big brother Stefano and big cousin Giselle.  So that is what she got. (more about our weekend away on a future post)

However, on Thursday we did have a small celebration to get her to open her presents and blow out a cake.
Here are a couple of my favourite pictures of the night!

Princess Cake

She had been eyeing this dress for weeks

So much concentration!

I think she likes it!

Very excited about her new Ariel dress

She was showered with lots of attention, great presents and good food!  

The night before your 5th

My Darling Little Girl,

It is the night before your 5th Birthday.

It is your last day as a four year old---Tomorrow marks the beginning of 5!

All the presents are wrapped, the decorations are set, there is  anticipation in the air waiting for you to wake up tomorrow and revel in all that is YOUR day.

So I sit here with a moment to look back at the last 5 years.

June is always a VERY emotional month for me. First comes your birthday and I am overjoyed, elated really at having you celebrate yet another year, another milestone.  It reminds me of the happiest day of my life.  

But it also reminds me of  THAT day,  June 29th!  The anniversary of your diagnosis.
The day that brings all those memories to the forefront--All year I try hard to dismiss them--to hide them away in the back of my head. (mind you, with little success) And this one day brings it all right back to the front!  On this day I can remember EVERY SINGLE DETAIL!--All the conversations we had, all the smells of the hospital, the taste of the food we forced ourselves to swallow--EVERYTHING with so much clarity.  It is as if it was yesterday.  The outlook seemed very bad those days.  They told us not to look up citrullinemia on google--we would be afraid of what we read.  True--We did and we were oh so very afraid!

But they also told us, that you were such a strong girl--you fought so hard--just ask those PICU nurses.
You were so brave-- you gave mommy a smile even though you were in so much pain!
You responded so well, amazing all the nurses,  when I sang you our favourite Robert Munsch lullaby.

You amazed them then and continued to amaze us all.

You have become our teacher!  Taught us to be more patient, to appreciate all that life has to offer, to not sweat the small things.  You taught us that life is too short for all the other nonsense.  It is because of you that I am a better person.

You have the sweetest and most loving heart--always trying to make your mommy and papi so proud of you---And we are--Believe me--We could not be more proud of you!

You have an amazing spirit--When you have had the hardest of days, you are still smiling.

You have a way of captivating everyone with your laugh, your giggles, your personality.





So I sit here, the night before your 5th and I want you to know that you have made me the proudest and happiest mother in the world.  I also think I just may be the luckiest to have been blessed watching you grow over the years into this beautiful little girl:

"I'll love your forever, 

I'll like you for always, 

As long as I'm living, 

my baby you'll be"

Love you forever and always, to the moon and back!

Your mommy!

Random Thoughts

If you are not an HGTV nerd like me, you may not love Brian Baeumler (HGTV host) as much as I do.  However, I happen to think he is fabulous and earlier this week I received an email which gave me reason to love him even more!
Bryan Baeumler and his wife Sarah have created the Baeumler Family Foundation (BFF) with the intention of helping children and families in need.  The Baeumler Family Foundation is a charitable organization focused on providing renovations for children and families that need accessibility or improvements to their homes, and could not otherwise afford the necessary modifications.  The BFF strives to help families better the quality of life for their children.
They are encouraging applications from health care workers on behalf of patients, or from the families themselves. More information, including the application form can be found at http://baeumler.ca/family-foundation/.  If you know anyone at all who could benefit from this generosity, please forward to them or better yet, think about applying on behalf of them!  What an amazing gift that would be!

Alexa has had one main EA since the beginning of January. Since being paired up with her, she has done wonderfully at school.  She has made many advancements and has had a great year.  We all (EA, principal and myself) agreed that it would be best if they remained together for Senior Kindergarten as well.
About a month or so ago, I was informed that a new EA had been placed with Alexa for next year.  While we all agree that her current one would be what is best for her, there are union rules and schedules that one must follow.  I get it, I work in unionized environment too--heck I am a union member!!! But when it is YOUR child that gets affected because of these stupid rules......I was upset!  And ready to fight!  As a parent of children with special/medical needs, you NEED to be PREPARED to fight ALWAYS!  Sad that it has to be that way, but it does!  THIS TIME, however was different!  I didn't have to fight (very much! lol)  The EA and principal and superintendent did it for me!  They kept on top of it, emailing the HR dept.; kept in touch with me and then on Friday I got the wonderful news that our EA had been called the night before and was offered (and accepted) next year's placement with Alexa.  Having other people advocate for Alexa certainly was different--I am not used to that--It is nice!  She will have such a difficult adjustment after the transplant; knowing that she has at least one familiar face and one constant person will make it that much easier!  It really does take that one stress off my plate!


I finally started getting things in order for the upcoming transplant--at least at work!  I have put in my leave of absence request and looked into benefit options while I am off without pay.  Last week I started packing up my personal files and other items as someone else is likely to be occupying my desk while I am off.  And then it hit me! The last time I packed up my "stuff" was exactly 5 years ago (almost to the day) while I was preparing for my upcoming maternity leave.  How different it was back then...If I knew then, what I know now! Isn't that the saying?.  It's true! I was young, naive, full of hope, so excited to be off for a year with the new life I was about to meet very soon.
This time if my co-workers had not been in the room with me, I swear I would have cried--I had to hold it in numerous times.  This time is nothing like the last--this time, I am scared, nervous, worried.  This time I know enough to know that we have quite a hard journey ahead of us!  
I gave some co-workers things of mine to "take care" of while I am gone.  Their response was "you are not going to be gone forever".  While it's true, that is exactly what it feels like right now.


Oh yeah--Alexa's 5th Birthday is in 3 days and we couldn't be more excited! We have quite the celebrations prepared.  In the meantime,  she has been scouting the stores for things to add to her "wish list".  This is her latest and I think she is pretty pleased with herself!

News

So I had hinted earlier that I would have some news soon....There are just some things that should be said face to face (or at least over a phone conversation)....Now that we have spoken to our friends and family, I can share it with the online world!

As I mentioned, Christian had started the extensive set of testing to see if he could be a live donor for Alexa. He is almost finished up his testing and so far things look quite promising; so much so that the Living Donor Transplant team have already assigned us a tentative date for surgery.

We got to select from two dates:  June 20th---which is her birthday, so for obvious reasons we declined that date and selected July 18th.
We still remain on the transplant list for now, so if something else comes up in the meantime, we will of course be called through that route.
Should that not pan out and to be honest, after waiting a year and 3 months to get the call (and be able to accept it), I am not holding my breath.  We finally have a plan B and  if all goes according to plan, Alexa and Christian will  undergo surgery at two side by side hospitals on July 18.

We are (or at least I am) nervous, scared, excited and every other emotion under the sun.  There is a ton of stuff that I need to do to prepare and I am not quite sure where to start.....

Living Organ Donation

I sometimes forget that I live in a different world from most people.  My world includes rare diseases and genetic testing.  Trips to the hospital and patient procedures are all in a regular day for us.  Alexa goes to the doctors office and plays with gauze and tourniquets instead of toys.  My knowledge of the medical field and procedures has increased 1000% from 5 little years back.  There are things that I know about that I never would want people to have to learn.  It's a different world that I needed to learn quickly and it is continual adjustment as more information becomes available.

I sometimes forget that not everyone is up to speed on the latest surgical procedures for liver transplant recipients.  And I sometimes throw things out there that not everyone understands.
A bit ago, I mentioned HERE that Christian was starting the process for living organ donation.  I will have some very important updates in respect to this very soon but we need a bit more time)
Immediately after that post, I had friends and family asking what that involved.

Living Donation occurs when a living person donates an organ or part of an organ for transplant to another person in need.
In our case, part of the liver (a lobe) would be removed from the donor and transplanted into Alexa immediately after they remove her liver.
Live donor liver transplantation is possible because the liver, unlike any other organ in the body, has the ability to regenerate, or grow. Both sections of the liver regenerate within a period of 6 to 10 weeks after surgery.  This type of surgery first began more than a decade ago using the left lobe of the liver in adult donors for children who needed transplants.

There is quite an extensive list of tests that are involved:  

• Firstly, blood type must match
• Blood samples are obtained to confirm normal organ function and test for the presence of several viral diseases
• An electrocardiogram and a chest x-ray are obtained to confirm normal heart and lung function. If these studies are normal then CT (computerized tomography), ultrasound, and MRI (magnetic resonance imaging) scans are arranged.
• The surgeons then review these tests to ensure that the liver is healthy and the anatomy is suitable for transplant surgery.  In some cases further studies are required such as a liver biopsy or an x-ray of the bile ducts (CT cholangiogram).  
• If the screening blood work and scans are satisfactory, potential donor is offered a tentative target surgery date based on their availability. Once a target date is defined the potential donor will complete a number of consultations with other health care specialists. The potential donor is seen by a psychiatrist, an independent medical doctor who does not work directly with the transplant team, and one or more of the donor surgeons.

As with any surgery there are very real and scary risks involved (both financial and medical risks).  The recuperation time can be up to 3 months.  Up to 30% of liver donors will experience complications. Most of these complications are mild and temporary; however, some can be very serious and life-threatening complications.   

It is not an easy decision to enter in living donation, most who decide to donate do so as it provides an opportunity to restore health to a loved one.  It also provides the recipient with a better quality organ than from a deceased donor and the transplant can be performed at an optimal time before the recipient's health worsens.

Welcome to a little bit of our world!

It's All About The Numbers

In our world, what it really boils down to are the numbers.  Sometimes these numbers bring cheers, sometimes tears.  Today they just bring frustration.
We had our regularly scheduled metabolic clinic appointment this morning.  We were up quite early and getting blood work done by 8am.
This is how the numbers stack up:
Good news first, cause it is worth celebrating--Ammonia--25!!! Yay!
Weight--has not gained at all in the last month, in fact she went down--only 0.1kg but its not in the direction we want.
Height--has not gained at all in the last month, and due to the fact that she was measured on different scales, it actually looks like she shrunk 1cm.  Impossible, I know but still again, not going in the direction I want.
Calorie Intake--We are about 200 calories/day UNDER our target.....She is at approx. 1100 and ideally should be at 1300!
Arginine--Was super low last month, so waiting to see what it is this time.  If low again, an increase in meds is on the table for Alexa.
The rest of her amino acids are not back yet, so will update tomorrow when they are in.

Updated to include her amino acid and other lab results:

Well not a big surprise here, her amino acids, the important ones at least, the ones needed for growth were low.  I guess we know why she has not gained weight or increased in height.
Her citrulline was in the 3400 range--SUPER HIGH but in HER normal range
Liver Function Tests--high--not a good thing but also seem to be in HER normal range(at least lately)

****SIGH****

I am tired and frustrated....

I am trying so darn hard to get the right amount of protein in her--not too much and not too little--exactly the right amount, and while I have been succeeding lately in that area, it has come at the expense of those so much needed calories.

She has taken a big liking to egg yolks and so I have been letting her eat them more lately, coupled with some other lower protein items.  It seemed perfect, as it was an easy way to get her to reach those measly 8g of protein she requires per day. But I had been warned,  about using lower protein dairy products(like yogurt and egg yolk) as they contain very little calories.  I was hoping that I was coupling it with enough higher calorie items that they could balance each other.  I was wrong!

So now, I am faced with the task of finding higher calorie items that she will like and eat.  Our dietician gave us some good suggestions that I will try out, but I am feeling kind of beat up now.  EVERY TIME, I find a new item that would be perfect(such as gluten free blueberry muffins-which contain 190 calories and only 1g of protein each) she eats one with pleasure and then wants nothing to do with it again.  My pantry is full of items that she promises she will eat and after the first attempt, we are stuck with the rest of it.  And most of these specialty items are SO expensive, so buying a whole package of muffins at $6.49 per package of 6, only to have her eat one and refuse the rest, is not ideal.  I would gladly spend $20.00 per package if she would ACTUALLY eat them!

I feel like I have failed at my job to keep her healthy.  Like her growing is dependent on what I choose to feed her and how much of it I can get her to consume--And although, I do not see it as a burden  it does become exhausting.

My head is filled with all her numbers.  It is full right now and can't take any more in!