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!

Blessed

I don't like asking for help from others--I really hate feeling like I am imposing or making people feel obligated to help me out.  When my children come home with fundraising items to sell, I would rather buy the entire lot of whatever they are selling this year before asking my friends and family if they would like to buy a $2.00 chocolate or sponsor my child in their school dance-a-thon.
But on more occasions than I like,  I have had to swallow my pride and ask for help when I believe it is important, even something as simple as wearing a particular colour on awareness day--like today or hitting "share" on Facebook.

So when I wake up to a co-worker telling me that she and her daughter are wearing blue to support Alexa, when my friends and family start sending their blue and green pics, when my UCD family write in THEIR own blogs about Alexa to support a cause so very near and dear to my heart....
Well, when all those things happen, I am overcome with so much emotion that I can barely choke down and hide those tears that are dying to be let loose...usually while I am at work, I might add....So I do my best to choke them down and get through the day.

When I finally get myself alone in the car, my favourite CD's as loud as the volume will allow...that is when my mind starts to wander and all my thoughts are allowed to surface....that is when I can no longer hold the tears back.  That is when I am overcome with such pride at the AMAZING friends and family I have. That is when I feel overcome with pride at the wonderful girl that I have been blessed with--A little girl who inspires so much good in so many people.  That is when I realize(again) just how blessed we are!

I have friends who I have never "met"; they may not live in the same city or province as us; heck, they may not even live in the same country or continent as us....But sure enough, when Alexa is sick, they stay up waiting until the wee hours of the morning, refusing to sleep until I have posted that dreaded number!  If it is low enough, they can finally rest peacefully for whatever remains of the night....Can you imagine this from people you have never actually met in person?  There is a lot of GOOD out there!

There are days when I have nothing but anger at the circumstances we were forced into, but I have always been grateful for those that are beside us on this journey.  If we have to be here, then I am so glad that they are the group of friends and family that we are here with.

I really have no words to say how touched I am, how very grateful I am, that so many people care so deeply about my little girl.  That so many people worry about her when she is ill and celebrate her triumphs right along side her.
Somehow THANK YOU, just does not seem to cut it, but until I can do better than that, please know how very much it means to me.

Counterbalanced Coquette: National Organ & Tissue Donor Awareness Week: Meet...

Counterbalanced Coquette: National Organ & Tissue Donor Awareness Week: Meet...: April 21- 27, 2013 is Canada's National Organ & Tissue Donor Awareness Week. We have our own experiences with being on the transpl...

NOTDAW

National Organ and Tissue Donation Awareness Week

April 21 - 28, 2013

In Ontario, National Organ and Tissue Donation Awareness Week (NOTDAW) runs from April 21 - 28, 2013.

However,  Trillium Gift of Life Network has designated April as: “BeADonor.ca Month”. 

Now is a great time to help increase awareness for organ and tissue donor registration and help save more lives.

There are hundreds of diseases that could affect an organ to the point that a transplant is required. Here is a list of organs that can be donated:

How did we get here?  Why did we decide to list Alexa?

I had been looking  into liver transplant to cure a Urea Cycle Disorder for some time.  Up until Alexa was about 18 months, she was so stable.  In fact, I secretly would wonder to myself, if Alexa has been misdiagnosed--I know this is ridiculous--Alexa HAD confirmed DNA testing done that showed she had two mutated genes; Chris and I both had DNA testing done and BOTH of us had 1 copy of the mutated gene.  Not to mention that she was slipping into a hyperammonemia coma when she was diagnosed.  But, she had done so well up until that point, that I allowed myself to hope that  maybe she had a "less affected" version of Citrullinemia.  I found out the hard way how untrue this was.

When Alexa had to start transitioning from baby food/finger foods to more toddler food, we encountered many problems.  Food wise--to say she was not interested is an understatement--THIS GIRL HATED EATING--We struggled on a daily basis with getting her to eat her "daily protein and calorie" intake.  The only reason she ate, was to make mommy and daddy happy.....

Even so, her "normal" ammonia numbers were slowly increasing.....It was not uncommon for her "normal" to run in the 60 - 80's...remember now, normal is below 50; some would even say below 35.  So running in the 60, 70 or 80's on a daily basis, when she was healthy and well,  was concerning to me.

Throw in a small illness, even the common cold.and those numbers would skyrocket.  Any stomach flu was a guaranteed admission and one more risk of damage to my baby's brain.

In November 2011, Alexa had a VERY rough go of things.  She was inundated with coughs,colds, ear infection, etc.  In a six week span, we were admitted 3 times.  The first two for 4 days each.  At each of these admissions, her ammonia was between 150 - 180.  Emergency medication intravenously was started each time.  On one of these, the next morning, we were all SHOCKED, that her ammonia went even higher--IT WAS 220!  This was the first time, her ammonia went up after emergency rescue meds were administered--THIS SCARED ME!  SCARED ME SO MUCH!  We did get the ammonia under control quickly--but her body's inability to respond as well to the medication as it used to, is one of the reasons I started to get more opinions on liver transplant.

And then we had to watch Alexa "come down" after an episode of hyperammonemia.  After each crisis, it took my daughter 3 to 4 days to get back to normal.  She could not settle down at all, she could not focus, she was extremely hyper and could not sleep well at all...Clearly these episodes were doing more damage than we realized.

And then there were other factors that came up that we were never concerned with before, like her citrulline levels.  Citrulline is an amino acid, that plays a key role in the Urea Cycle.  It is the marker that alerted the newborn screening department that something was wrong when she was just 9 days old.  At this point, there is NO EVIDENCE TO CONFIRM that elevated citrulline levels cause any damage to UCD children.  However, there is also no evidence to REFUTE it either.  It is my feeling that once a bit more research and progress  in brain imaging investigations(--which is currently underway), we will find out that elevated levels of citrulline does impact the brain.

(AGAIN, this is only MY opinion after doing much research and speaking to many professionals about it, in reference to Alexa ONLY) Citrulline has an upper limit of 80(according to MY laboratory).  Alexa's citrulline runs in the 3200 - 3800 range!  YES, you read that RIGHT..... THREE THOUSAND!!!! And there is absolutely nothing that can be done to lower it!  I have asked a million times--I cringe every time I get her lab results and see her citrulline level.

This was the most excruciatingly painful decision I ever had to make.....I spoke to doctors all over north america, to parents who have had their children transplanted and had great outcomes as well as those who have had the worst imaginable outcome.  It took us 6 months to finally decide. But in April 2012 when the HSC transplant team called us and informed us that after all the testing and after meeting with all interested parties, the team was confident that transplant was the best option for Alexa, Christian and I had no hesitation--we KNEW what we HAD to do.  She was listed and the waiting game began!

It has been about a year, we have had two calls--One in August (as the back up) and one in February which we had to turn down to illness.  Every time my phone rings with a blocked caller, my heart jumps into my chest and I can barely speak.  

Here is hoping that her PERFECT liver arrives during organ donation month!

And just in case, I have not said it enough before.....

You can register to become an organ donor in Ontario here:  https://beadonor.ca/  and help save the life of someone wonderful like Alexa!

Our Dietician!

There is a reason we love Alexa's dietician so much!  She is our main contact at the hospital, the one we go to with any and every concern.  She is really our life-line and I am not sure what we would do without her.

Today we got home, checked the mail and to our surprise there was a package for Alexa!  She was excited to open it and find that she got an early Easter present from our dietician! Low protein chocolate!

Yet one more reason why our dietician is our favourite!

Clinic Day!

Today we had a regular scheduled clinic appointment at the hospital and while it was a LONG day, it was a good one.
But first, a couple of things happened this weekend which made me realize that not only is my baby not so much a baby any longer, she is growing up very quickly and making her mama one proud woman!
The first didn't exactly make me proud but it was a good lesson for us none the less.  We were over at my cousin's house for a get together, and we took Alexa's lunch bag(which holds her medicine case and formula) out of the fridge and put it on the kitchen counter.  Alexa drank her last formula of the day and it was just about time for her last dose of medicine but we wanted to wait a couple of minutes to let formula settle before giving her medicine.  So she went off to play.  Ten minutes later, she called me to tell me she had lost the cap of the medicine syringe and I noticed she had both syringes in her hand--EMPTY!  I guess she wanted to show her friends how she took her meds, and without calling any adults, she took them on her own.  Thank goodness it was at the correct time, but I freaked out none the less, which probably embarrassed her as she started crying, immediately making me feel horrible.  Since then we have had some time to think about it and discuss that she is not old enough to have medicine without adult supervision, but it did make me realize that she probably needs a bit more independence when it comes to her meds.  So I have come up with a game plan--I am going to put her dosages in a colour-code/number-coded something or other (We shall see what Dollarama has that we can modify) and when it is time for her meds, she can go and pick out the correct case and show an adult how she takes her meds.  Key being that an adult MUST always be present to make sure she takes it all!

That same day one of her cousins was eating a piece of meat. I am told the conversation went something like this:
Alexa:  What are you eating?
Cousin: Meat--want some?  Your mom has some, go ask her for some...
Alexa:  No, I can't have meat.
Cousin: Why?
Alexa:  I'm allergic.

Not exactly!  But close enough!  It gives me such piece of mind knowing that Alexa knows EXACTLY what she can and cannot eat!  Not that I will let my guard down, but knowing that when I am not around, she can speak for herself and tell people what will make her tummy hurt, elates me and makes me realize how responsible she is becoming when it comes to her medical condition.

And then, today at phlebotomy she had her blood drawn with almost NO tears.  She sat down on the bed, looked away and counted slowly until it was over.  The technician was so impressed.  Only girl I know who can have 6 BIG vials of blood taken and out and come out looking like this:

So back our appointment:
Today was actually an appointment with our liver transplant team and not metabolics, but we did meet with our metabolic dietician since our last clinic she had not done well with weight or height gain....
A couple of posts ago, I mentioned that this girl was eating a TON! Well, it was super fun while it lasted, but it was not very long..probably about 2 weeks only and it certainly for good reason.  She had quite the  growth spurt!  This girl gained 1 kilo and grew 3 cm since December.  She is still not where we would like on her growth curve and this area continues to be an issue for Alexa, but a definite improvement over her August-December numbers.
We met with our GI nurse and doctor who were both super pleased with how Alexa has been doing over the past year since starting Ravicti.  Her ammonia numbers have been much better than they were in the past and she has remained relatively stable even during viruses and illnesses.
Our GI doctor and I spoke about some new up and coming trials in the metabolic/liver world such as Liver Cell Therapy, explained here:  http://www.cytonetllc.com/?page_id=135  And while I think that the advancement and exploration of different treatment options is wonderful, I think that her doctor and I agree that this procedure is just too new and not enough participants having gone through it, coupled with her general stability to be an option for Alexa at this point. I love that even though her doctor was not really interested in this for Alexa, he took the time to explain the new research initiatives and talked about pros and cons so that I have all the information I need to keep making educated decisions regarding her care. Although, no one is happy about how long it is taking for her to get HER perfect match, he is quite happy that at least we can wait knowing she is stable!
We then spoke about whether transplant is still a good option based on her relative stability over the year. I have been meaning to write a post about how and why we got to be on the waiting list for transplant as it was not an easy decision, but I am just not ready for that yet.  One day, but not yet!  Suffice it to say, that Dr. A said that as more research comes in and as more metabolic patients are transplanted under their care, he is more and more convinced that transplant is the best option for Alexa. And I agree!
So we wait!  And in the meantime, Chris has started the various testing required to see whether he can be a live donor for Alexa.

Oh and Ammonia--She kicked it's butt!  18!!!!!  Go Alexa!