Wednesday, February 27, 2013

Then and Now...

Phuoc Nov 2012

Binh Nov 2012

Phuoc and Binh Feb 2013

Alagille, on a Day to Day Basis

I will be visiting my oldest daughter's class tomorrow to introduce the twins.  The grade 7 class has been welcoming a mommy and her baby on a regular basis for some time now.  The program is called ''Roots of Empathy''.  The little boy they have been observing was lucky at the lottery of life, being born in a rich country, healthy, and loved.  This is not the case for everyone, as we all know.  I am proud to bring my daughters to school to show them around.  It will also be an amazing eye opening opportunity for the students to see with their own eyes how sickness can affect the life of children, and above all, that not everyone gets the same start in life.

My intention with the students is to talk about how the girls came along in our lives and tell them a little bit about where they are from.  But I have a feeling most of the time will be spent on the girls' condition, Alagille syndrome.  What is it and how does it affect life on a day to day basis.

This is how I got the idea of writing this post.  Michael and I have talked about Alagille on numerous occasions, but how did it affect our life on a day to day basis?  Many people wonder how we do it with so many children (by the way, I still refuse to think that we have a large family), including 2 heavily special needs wonders.  The house is not chaotic, everyone is clean and dressed, no one hides in corners to cry, kids still go to school, Michael and I are keeping our running up.  We are organized and I guess we have an attitude that allows us to accept it easily.  When we adopted Toan 2 years ago, who was believed to have cerebral palsy, we thought that worse comes to worse we would have to move in a bungalow to accomodate a wheelchair.  And that was the worst outcome possible in our opinion.  Would that have been so bad?  Not at all.  We still would have loved him just as much.  Toan was lucky and suffers from nothing else but from a 2 year old attitude...!

Before adopting the twins, we read about the possible conditions they might suffer from, to a point of feeling dizzy. Don't get me wrong here, we never ever considered not taking them: from the moment we heard about them, they were part of our family.  Then we resigned ourselves with the ''we'll cross the bridge when we get there''.  If we have no control over life, let's just go with the flow.  Hmmm...  Again, we would do the same thing all over again, because we were meant to be their parents and they were meant to be our daughters.  Plus, they are so adorable!!!

So we flew to Vietnam to meet them: our lives changed forever the minute we saw them.  Yes, it was WAY worse than we thought, let's not kid ourselves here.  Did we panic?  Yepper.  We let our guards down and allowed ourselves to panic live on Skype with our trusted friends D&M and D&B.  Did we think we were nuts?  Absolutely.  Only stupid people do that.  But again, we are proud to be this kind of stupid.  With the aid of angels from Halifax, Edmonton, Newfoundland, Ottawa, Quebec city, Montreal, Kingston, we shook ourselves up and moved on.

Alagille is the third triplet with Binh and Phuoc.  Twins are a fair amount of work, twins with special needs are a whole lot more work.  Alagille means on average 5 to 6 medical appointments per month: GI, pediatrician, hepathologist, ophthalmologist, endocrinologist, cardiologist, surgeon.  We also get weekly home visits from a nurse and a dietician.  We needed to find a specialized pharmacy who can prepare all of their meds.  Talking about meds, I prepare between 15 and 20 serynges each morning, depending on the day, and probably another 10 later on in the day.  Alagille also came with feeding tubes that need to be changed on a weekly basis, if we are lucky.  Sometimes, the girls pull their tubes out and you have to reinsert when it's the least convenient.  Alagille also came with feeding pumps.  This required from us to adapt very quickly to our new routine because the girls' survival depends on those machines to feed.  They are tube fed 3 meals per day and overnight for 10 hours.  We installed a camera above their bed to monitor them and make sure they don't get tangled in their lines, or pull their tube only halfway (which would make them drown), choke or simply beat each other with bottles..!

We were naive a bit before the adoption, thinking once they ate well they would get better.  Alagille brought Peptamen in the house, which they consume in grand quantity.  Peptamen contains MCT oil, the only fat absorbed by their system.  We are lucky to get funding, because with what they consume, we would be bankrupt by now.  And for those who think the girls cost too much to the Canadian gvt, you might as well leave this page and never come back.  Because yes, I have heard that comment from people in regards to internationally adopted special needs children.

But overall, Alagille has brought with it a new appreciation for the real people around us who chose to help.  It has strengthened our family and our convictions in regards to adoption, in particular the adoption of special needs.  And Alagille has given us 2 wonderful little girls who are loved beyond words. 

Sunday, February 24, 2013

On the Road to Transplant?

We went to Ottawa to meet with the hepathologist at CHEO on Thursday.  One word to sum it up: wow.  What an amazing doctor, what an amazing liver team with the dietician and the contact nurse.  We feel so well taken care of, once again.  Those who complain about the health system in Ontario must not need it as fast as they think they need it.  The girls are really sick and we feel we are getting the care they need in a very good time frame.

We sat with the hepathologist before she examined the girls and the biggest issue is the itchiness (caused by the bile acids), which is the most annoying symptom of Alagille syndrome.  No word of a lie, the degree of itchiness seems to be going up by the day.  The doctor examined the girls and the first thing she noticed is their spider veins on their abdomen, a sign of cirrhosis.  Also, the girls' livers are very big and hard, and so are their spleens.  She is worried about internal bleeding and wants to make sure the girls are placed on the transplant list earlier than later.  In other words, she wants to make sure they are on the list before it becomes an emergency.  Blood tests were done on Phuoc.  They tried on Binh but were not able to draw any blood.  We received a phone call on Friday with Phuoc's preliminary results showing high levels of AST, ALT, Bilirubin and Conjugated Bilirubin.  Binh will get another shot at blood tests tomorrow Monday when we see our pediatrician here in Kingston.  The doctor also wants a scope done as soon as possible, here in Kingston or at our next visit in Ottawa.

Once we have all of our numbers and data, it looks like we would then be referred to the liver transplant assessment team at Sick Kids in Toronto.  The assessment takes 3 days of multiple testings and you end up with a score to place you on the list.  If everything stays the same, no complication, it could take 2 years to get an organ.  If things get worse and the girls end up in the hospital, their score changes and they go up higher on the priority list.  Anyway, this is the way we understood it works.

For sure, a liver transplant will not cure Alagille in the girls.  Alagille is there to stay.  However, we can hope that with a new and healthy liver, they would do better, particularly with absorption of nutrients.  With a transplant starts a whole new game, the rejection issue.

Tuesday, February 19, 2013

G-Tubes, Here We Are!

Good news, we finally got a surgery date for the girls' g-tubes: March 18 it will be.  We are happy since it seems like it will be easier on everyone, starting with the girls.  No more tube in the nose, no more tape to hold everything in place, no more checking placement for mom and dad.  Each girl has an enlarged liver and spleen which complicates the procedure a wee bit, but we are confident that they will do fine because they are little fighters.  They will have a piece of tube hanging out for about 6 weeks until the surgeon installs the Mic-Key button to cover.  Something else to learn about.  They both gained a lot of weight, each having finally reached 7kg.

We are heading to Ottawa on Thursday to meet with the hepathologist.  Michael and I are hoping that the girls will be referred to the transplant team in Toronto for assessment.  The itching has increased considerably and it is hard as parents to see them distressed and dependent on meds in order to get a bit of relief.  I have heard of biliary diversion (internal and external) to treat pruritus and I would like to hear what the specialist has to say.  Also, both Michael and I are willing to see if we could be living liver donors.  It will be a very interesting appointment and we are looking forward to it.

The last month has been busy for the Wagner family.  We celebrated Tet (Vietnamese New Year), eating yummy food and decorating our home with red lanterns, and everyone has gone back to their usual activities after the Christmas break.  Fiona enjoys her tap and karate, Grace her ballet, Rose her Pathfinders and karate, Noah his karate, and the last 3 little boys are busy with just about anything.  Liam and Logan go to school half time, and I started to take Toan to a little kids club 2 mornings a week.  I officially started my marathon training a month ago and Michael does a lot of his runs with me: it is our little special time.  My marathon is scheduled for the end of May, providing that I can remain injury free until then.  Since this will be my first one, I don't feel any pressure to beat a personal best, which makes the whole thing fun.

We are already starting to think about what next summer will bring and we have pretty fun ideas for the kids in mind.  All in simplicity.  Isn't it the best way to go???

I will write another post later on this week after the specialist in Ottawa.

Monday, February 18, 2013

Je n'ai pas besoin d'amour / I don't need love

Beautiful song from Celine Dion  - If I had talent, that's how I would describe my life.  For my English readers, here is the translation.  Enjoy, as much as I did when I first heard the song.

No, I don't need love
I've someone everyday
In my arms
No, I don't need love
I don't need moon
I've some kisses that light up better than all stars

No, I don't need flowers
Words sprout in my heart
No, I don't need dreams
I don't need lovers
Wind blows out
Night goes to sleep, singing
As I'm rocking my family

A little little great happiness
A beautiful gift from life

I don't need love
I've got friends that surround me
I've got my arms full of happiness
No, I don't need love
I don't need moon
I've some kisses that light up better than all stars

A little little great happiness
A beautiful gift from life

You're not the love of my life
You're the life of my love
My little little great happiness

No, I don't need flowers
Words sprout in my heart
No, I don't need dreams
I don't need lovers
Just one day without embracing you
Leaves fall out
Help Help
I need love..

(Jean-Pierre Ferland / Daniel Mercure)