Medical Update

playing and waiting for x-ray apt

As there is so very little known about Gabrielle's condition, I want to document all the information I've received from our doctors visits in the past while.  My hopes are that in documenting any information about I-cell disease, other families and doctors can learn via our experiences.  I hope one day treatments and hopefully a cure will be found.

As I write the medical information down in a very matter of fact way I caught myself many times today choking on my tears as I heard truths about Gabrielle's health that is still so very hard to hear.  I guess you just see your child as perfect and beautiful no matter what health challenges they face.

Today we had our very first appointment with an orthopedic doctor at BCCH.  Up until this point we've been followed very closely by the Oncology/Hemotology clinic and the Respirology team as her transplant and lungs have been our top concerns, but today I have been exposed to another very key team of doctors in helping Gabrielle live her best quality life: the orthopaedic team.

Up until today I had no idea what an orthopedic doctor was or even did.  I was even going to postpone the apt - thank god I didn't!  We had more x-rays done of her hips and spine and this is what we learned about our little girl.  The doctor always spews out so much information and I really need to tape record what she/he says so that I can digest it all.  I'm often lost on the first sentence as my eyes well up with tears and I try my hardest to not cry.   Thankfully, Regan was there and the doctor repeated everything to him so I got to hear the information several times.

* All of my information is in lay men's terms.  I am not a doctor, only a mother trying to advocate and learn as much as I can about Gabrielle's health!  I think I've got the gist and I'm only going to document the 'important' things that I hope will help other families and or medical professionals learn more.  

Gabrielle has scoliosis which is a curvature from side to side of the spine as well as kyphosis.  Kyphosis is a curvature of the back like a humpback caused mostly by the storage aspect of her diagnosis.  Her vertebrae were never properly formed and she has discs that are very tightly packed together.  A brace would be the only way to help her but that also has significant implications on her lungs making breathing more difficult. So no to that.

Gabrielle's bones are very porous, making them fragile and easy to break.  This is mainly because of the storage disorder, and it's made even worse due to the heavy doses of steroids during and after transplant.  Basically, her little bones are missing the 'hard cover' that covers our bones, with only the very porous 'inside' on the outside.

Her hips are doing ok right now but we need to watch that they don't dislocate as she grows.  They are already at a worrisome angle.

They are going to make some splints for her feet as they've turned out significantly. The splints will allow her feet to turn in and bear weight (precursors to walking).  She also has one leg that is longer then the other so her splints will be adjusted accordingly.

Things to work on with Gabrielle:

Head and trunk control and weight bearing activities but not too much as her bones are quite weak and she needs to gradually get stronger.  I wanted to ask the doctor, "How much should I freak out when I look over and see my 40 lb 2 year old lying on top of his little sister?" ... "No, like seriously ... should I be concerned?" ... but I didn't ask.

After our orthopaedic appointment we went over to the clinic and met with the transplant and respirology docs.  Gabrielle is engrafted only 51% and has dropped from 86% engraftment.  Why?  Our response: 51% of 'healthy' cells are better than none.

Exciting news from the respirologist - her lungs sound great, steroids were not needed to help with secretions on her last sickess-related visit to the hospital a few weeks back (YAY!) and he wants to start slowly weaning her off oxygen.  

In our last hospital admission an x-ray revealed a diaphragmatic hernia.  Gabrielle's bowel is up behind her breast bone and is causing her breast bone to push out.  The surgeon is not concerned at this point and the risks of putting her through a surgery outweigh the risks of this hernia.  It seems that this is very common with most children with this diagnosis.  

Now What? 

The most important part of this journey is always focusing on the time spent together.  As a mother of a beautiful boy from up North (with his own very special needs) said to me today,

"Enjoy all the good times that are ahead of you."  

I couldn't agree more.  While difficult, I try not to focus on the details of her diagnosis.

I focus on the amount of smiles and giggles I see on her face in a day.

I focus on the number of kisses and hugs we shower her with.

I focus on time she gets to spend cuddling with her brothers.

I focus on the miracle of her in our lives and what a blessing she's been to us, and what a beautiful world she's opened our eyes to.

When I see Gabrielle, I don't see any of the stuff I just talked about, I see the most beautiful little girl I've ever met.  

Love. Love. Love.

Special Parenting, Special Views.

Parenthood is like going for a hike.

Life is the earth we tread upon.

The views along the trails are the understandings we acquire along the way.

The ascent is the effort parenthood requires you to exert.

Before you start, you know the hike will be worth it in the end.

The view will be incredible. Eye-opening. But you know it will be work getting there.

Very quickly everyone discovers there's no avoiding the work getting there.

Parenting a child with special needs or a terminal illness is different, though.

It's just different than parenting a healthy child.

  *   *   *

Parenting a healthy child is like walking up a lone foothill.

It's work getting to the top and you're stronger for it.

You see things from the top of the hill that you wouldn't have from below, and you're wiser for it.

Parenting a healthy child makes you a better person.

Parenting a child with specials needs or a terminal illness, however, is like walking up the lone foothill only to discover an entire mountain range behind it.

When you first look at it you think there's no way you can do it.

Everyone else is turning back and walking down the foothill back into the village because they can.

But for the most wonderful and daunting reasons, you know you have to journey on.

You have to walk up the next, slightly higher range of foothills that are almost like mountains.

At this early stage, when you look back, the lone foothill appears so much smaller.

You marvel at how much you've grown.

But you look ahead and see how much farther you have to go.

The snowy peaked mountains.

And you march on. 

Because you have to.

Because it's the right thing to do.

Because there's every reason in the world to.

Bluff after bluff, you keep looking back at that first foothill.

You imagine everyone home relaxing.

Talking about what they saw from the top of the lone foothill. About how hard it was.

The reward of parenthood.

And you smile for a million reasons.

Some good, some bad.


   *   *   *

And before you turn your back on that distant scene and continue on with the ascent, maybe never to return, you see a whole world up and above that lone foothill that nobody you knew before has.

It's full of sadness, happiness, wisdom, and beauty.

It's real.

You see it.

You feel it.

It's real. So much more real than what you knew before.

The special reward of special parenthood.

Looking out at the vast expanse of understanding and emotion, you think how impossible it would be to explain this view to people who haven't seen it for themselves.

How a few wonderful souls would try really hard to understand.

How most would not. Could not.

How many would not even want to listen.

But there you stand.

Mostly alone. With a few other brave hikers here and there.

Far beyond and above the heights of that lone, little foothill.

Looking out at things you never knew were there.

Things your former self likely didn't want to know were there ... just like the others.

Tired. Exhausted. Stronger. Wiser.

With so much to offer.

And so much to share.

One happy little girl

We made it home after one week of intense hospital care and no one was happier than Gabrielle.  

Last Monday, Gabrielle was so sick, and after testing found she was infected with the para-flu and a secondary infection in her lung.  It seems that Gabrielle's right lung is quite weak, and any and all infections seem to cause her upper right lobe to collapse making breathing quite laboured and tiring.  Our first night in the hospital was very stressful as we increased her O2 to 2 litres (2 litres of O2 is the most aggressive they can be on the floor before sending you down to ICU where they put you on high flow or bi-pap) and she kept having big desats into the low 60's.  I was one absolutely terrified and heart broken mamma.  Thank goodness for the most wonderful nurse who was very aggressive and pro-active in our first night there not to mention incredibly kind and caring rubbing my back and offering me peppermint tea at 2 in the morning.  You have no idea how grateful I am.

Every day Gabrielle got a wee bit better and was seen and visited by all her doctors and nurses at BCCH and even had some visitors from Canuck Place:)  Gabrielle you are one loved little girl.

We have so much to be grateful for.  We have more time with our little girl.  Thank you to everyone who helped us this week.  Thank you again to the nurses and doctors of 3M and to the team on 3B.   Thank you to our family who helped us as we juggled everything this week, it was busy and with your help we were able to pull it off.

As I write Gabrielle is sound asleep in her bed and was all smiles before drifting off.  Sweet dreams baby girl.  How amazing to have all three of my babies together again.

Here are some memories from the week:

Tired brothers

Tired sister sunbathing in the sun

Thoughts and Prayers to Kason Troehler and Family

Another little I-Cell angel, Kason Troehler from Northern California,  passed away this week.  By all accounts, he touched everyone who met him in the most wonderful way and was fortunate to be surrounded by love in return.

Please take a moment to say a little prayer for Kason, that his journey to the next world is smooth and beautiful, and that his family may find the strength and courage to carry on and share with others the love and wonder that Kason graced them with.

Kason, I wish Gabrielle could have met you.

Much love,

Regan, Amy, James, Michael, and Gabrielle.

Gabrielle Is Improving ... Thanks

Seriously, thanks everyone for your positive thoughts, prayers, and words of encouragement. Again, Gabrielle's fighting spirit nipped a very nasty flu (which has wiped out our 2yr-old boy too) in the bud.

She's still in BC Children's hospital, and given the congestion in her lungs and airways, likely will be for several days ... if not longer. She's regained a lot of her kindred spirit back, which is totally encouraging because we were really worried it could have gone either way two days ago – she was very ill.

Anyway, here are two father-daughter videos I took while with her today. The first one is of me feeding her hospital turkey gravy, and she absolutely LOVED it. I have never heard her coo like this before, and while the moment is always partially lost when the camera/iPhone comes out, you can still see her expressions of gravy satisfaction.



And this one is of her listening to music with the headphones on for the first time. The things you do when you're bored – but she just loved it!



Thanks again everyone ... and here's hoping and praying for a speedy recovery home.

Gabrielle Quite Sick, in BC Children's

A very bad flu has been in and out of our house for the past month or so, and Gabrielle has gotten this new and nasty strain.

After of a night of tough coughing and laboured breathing, Amy took her to BC Children's Hospital for better medical attention than we can provide at home. She was admitted, and we'll be there for the next few nights for sure.

Please say a little thought or prayer for her if you can. She's a little fighter, and always fights a littler harder knowing there's people in the stands cheering her on.

I hope she's home in a few nights time.

Big Milestone for Gabrielle

Gabrielle is now almost 15 months and the other day, I cheered her on as she rolled over for the first time.  

Other parents of children like Gabrielle would understand the monumental moment this was for her and I.  As I cheered her on and talked her through the first time she rolled over, I was so excited for her.  To a little baby girl whose been told that things like walking, talking, crawling will most likely never happen, you can maybe imagine the emotions I felt.    

I am so proud of you Gabrielle.  You rolled over all by yourself and finally figured out what to do with your hand stuck under your stomach.  I laughed and cheered you on with tears rolling down my face;  tears of complete and utter happiness for you.  I love you baby girl - way to go!!

LOOOOOOUUUUUUU...

In October, Gabrielle and the boys were asked to be a part of the PSA (public service announcement) with Roberto Luongo at Canuck Place.  Oh yes, this momma played it cool on the phone hiding my complete and utter excitement!  The day was great fun and long!  We were all crammed in with the whole filming crew into one room for about 4 hours while we did take after take.  I didn't mind:)

James started out super enthusiastic showing off for Luongo but after about the 10th take he quickly lost his interest and was DONE.  I love how kids are just so real.  I had a calm pep talk with him and was very close to secretly offering him anything he wanted if he would just do a couple of more shots.  Thank god he agreed before the heavy duty bribing started!

Michael was not at all interested in playing hockey or in meeting Luongo.  It did have a lasting impression on him however, because he is a huge hockey fan now playing hockey any second he can in the basement pretending to be "Melongo".  I think if the filming were now, Michael would be all over it!  

lunch break

"Gabrielle to the set"

Pep talk with James "come on James just one more take"

I can now say we are big Luongo fans!  When we see him on tv the boys get so excited and we love to cheer for him.  After spending the entire afternoon with him at Canuck Place, a place where life is put into perspective, you realize he's just a guy who plays professional hockey.  He was calm, nice and really easy going.  He laughed with the kids, at himself, and made efforts to connect with everyone.   

The little babies had no idea what was going on, the mom's were excited to meet a handsome professional athlete and praying that their babies/toddlers/preschoolers would behave, the older kids were SO pumped to meet this hockey hero and to get their t-shirts signed, but at the end of the day it was so apparent, even to Roberto, who the real heros in the room were.

Happy New Year! (2012)

I love this time of year. I love how we're all encouraged to take a few moments (or maybe more) to review the highlights and lowlights of the last year.

As we head into 2012, with all its uncertainty, I thought I would would share two things:

ONE ~ It's Worth Doing Poorly

In a conversation I had with a guy who definitely knows what he's talking about, he shared something with me after I explained how impossible it seems to get everything done I want to get done, in the perfect manner I always like to get things done in.

He said:

"One thing I had to learn the hard way was that if something is worth doing, it's worth doing poorly." 

The significance of the statement hit me immediately. Coming from a family whose work-motto was, "If you're going to do a job, do it right," I realized how much grief and stress that motto had been causing me.

Raising Gabrielle has seemed impossible some days and weeks. We've reached lows we never thought we would. Our parenting has been poor and clumsy on many, many days. We've been anything but perfect parents, spouses, and friends.

His advice, though, was so simple and so right: If the job, goal, or task is worth doing, it's more important to do it than worrying about getting it done perfectly.

And there's that word again: perfect. Our culture of perfect. Our collective expectations that things are supposed to be perfect. That there are touchdowns in life.  Well, there's no such thing as perfect, and there are no touchdowns.

The universe keeps on truckin', with or without us, and it doesn't care whether or not we've incarnated our ideals.

So, I share this on the first day of the new year (as we think back on all that's happened in the last 12 months, and think forward to all the things we hope will happen in the next 12 months) because I'm sure we all have a few things in our lives that are vitally important for us to get done, but things that seem utterly impossible. Things that seem like a seemingly impenetrable mountain pass.

If the benefits of searching and scrambling and climbing over these impasses are worth it, then I suppose it's worth climbing up and over them poorly and clumsily. If we do, I think our lives – and everyone's lives who touch ours – will benefit. And we'll all be better for it.

TWO ~ Happy New Year!
A 60 second clip of the Ross kids on the first day of 2012 ... I can't wait to look back a year from now and watch this vid and reminisce on all that's happened in the coming year.

Happy New Year to everyone, and all the best in 2012!