beadonor.ca

 

 

 

Every day, there are two things that I wish for.....

 

The first is that we are one step closer to a finding a cure for citrullinemia.....

 

And if today cannot be that day, the second wish is that those clinging to life; waiting for their perfect organ, receive their call.  That today is THEIR day.....

 

The day when the desperation turns into hope!

 

But that won't happen unless we step up (a little more...)

 

Confession Time:  Prior to Alexa being born, I was not a registered donor.  Don't get me wrong; I have always thought organ donation was a positive thing and wanted nothing more than to donate my organs when I no longer needed them.  I even checked off the option when renewing my driver's license.  But that was not enough.  I never actually sat down and registered online.  It's not that I did not want to; I just never found the time to sit down and do it.

 

And then.....well you all know what happened next.  Life happened and threw me a curve ball...

I became a mother to a child with a serious medical condition and in doing so, I learned amongst many other things, to be a little less self-absorbed.

 

So I sat down and did it--registered online to donate my organs at

beadonor.ca 

 

If you are a reader from United States, you can go to www.organdonor.gov/ to look up your state registry.

 

2 minutes is all it takes! TWO MINUTES!!

 

Seriously, all it takes is your health card and 2 minutes on a computer and you can save up to 8 lives! And enhance the lives of 75 more!  We don't think it is asking too much...do you?

 

 

 





The month of April is dedicated to raising awareness for organ and tissue donation.  Last year, you might recall that some of my family and friends wore green and blue to help raise awareness and encourage people to register consent to become organ donors. 

 

 

 

This year, we thought we would go a step further. 

 

I live in a wonderful community of very supportive neighbours.  Together we have gotten together to paint Cornell Blue&Green on April 11, 2014.  We have full support from our local schools (including Alexa's!!!) and we would love to have your support too!

 

It is super easy; all you have to do is send your kids to school wearing blue and green! Go to work wearing blue and green! Spread some awareness and encourage people to become organ donors! Upload your pictures to facebook, twitter, instagram...And everywhere else! Spread the word and get the message out there. 

 

This is our event page. If you wouldn't mind, please go the event page and JOIN it.  It is a public event, so please feel free to share. 

 

https://www.facebook.com/profile.php?id=462835693816055

 

Bringing up the subject of organ donation with your family may be difficult. But do it now, do it when you can so that if there ever comes a time when you cannot, your wishes are known.

 

 

 

 

 



Packing Up....

After yesterdays all clear, I came home and had to finish packing Alexa's stuff.  I had started but thought that I might be jinxing myself so I only packed the easy stuff like pajamas until we got the go ahead.   

I got stuck on the "other stuff"--

What do I take to comfort a little girl whose world is about to be completely turned upside down--For a second time?  

What do I take to remind her off all her family and friends who love her so much?  

What do I take to remind her of her home, room, bed, all her stuffed animals, and toys that are waiting for her prompt return?

And what do I take to entertain her on those days when she has just about had enough of the medical world?  After all, there is only so much iPad and Disney DVD's a girl can stand.

And have it all fit in one suitcase--I know we are getting a private room and all, but it is not exactly a penthouse suite--Space is at a minimum!

This seemed like an almost daunting task--perhaps that is also why I held off on it for so long(that and my superstitions!). So, I consulted with the Transplant Child Life Specialist, who thought that I should ask Alexa what she would like and have her help in the packing process.....

This is what we came up with....

Alexa's B-Strong Bear was gifted to her by one of her teachers, Ms. G. It has a very special meaning to us and I am sure will give her strength when she is feeling down and courage when she is scared. 

Our "perfect liver" given to Alexa and Chris by one of our dear friends. 

An assortment of sticker and activity books given by Alexa's teachers and her aunt. They know her well and managed to find all her favourites.

Alexa's Ariel dress--cause there hasn't been even one day since she got it that she has not put it on. I am hoping that once she is feeling well enough to start moving, she will put this dress on and it will give her the motivation she needs to take that one extra step. She is the happiest when she is strutting her stuff in this princess dress.  

Her favourite bed time pals--Minnie Mouse, pink teddy, and her new favourite blanket. She sleeps with these every night and always brings them on every trip we go on.

Our Organ Awareness and Rare Disease Awareness collages that I sent to be made into posters. They are pictures of all her friends and family near and far who always support her. My hope is have these up in her room to remind her how loved she is. Seeing all those familiar faces is sure to bring lots of smiles. 

This beautiful rosary is so very special to us. It was given to Alexa by yet another one of her teachers. It was blessed a couple of years ago back him where Mrs. D grew up. We could not go to the hospital without it. I am so happy that I can have it in my hands while I pray for my little girl, my husband and all her doctors. 

So this is it.....all packed to go to our home away from home.  

Anything you can think of that I have missed?  I have one more day, let me know quick....

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!

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!

What a week!

I wanted to write this post a couple days ago; it is probably better that circumstances did not allow me to get to it.  On Wednesday it would have been a VERY negative post.  This way, there is at least a ray of light at the end of the tunnel.  Nothing like all inclusive stay at a hospital to humble you right up!

I am not selfish enough to not realize that there are plenty of others who have it way worse than us, however, there are times when I get just plain angry--Angry at the cards we were dealt, angry that Alexa drew the short end of the stick, angry that every time she should be celebrating with her classmates or family, she ends up in the hospital.  This was one of those weeks.  I was just ANGRY at LIFE--at all of it!

I knew this was not going to be a great week early on.  On Sunday night, Alexa came down with a low grade fever--one dose of Children's Advil later, and she seemed back to self, but on Monday night a nasty little cough started.  With Valentine's Day coming up and knowing that Alexa always misses out on celebrations with family and friends on special occasions, I KNEW this was probably going to end up like the others.  All I want is for Alexa to be able to get the most that life has to offer.  For a 4 year old, that includes being able to go trick-or-treating on Halloween, exchanging valentine cards with her classmates, opening Christmas presents with her family.  However, Alexa has spent the last two Halloweens sick (this past one in the ER). Christmas Eve last year ended with the same fate.  As well as MANY other family celebrations.
So I was pissed off when she got a fever on Tuesday night...I knew that this was going to put her out of commission for school on Thursday.  That all her effort into making valentine cards for her friends was going to have been for nothing.  This was going to be another special day that she was going to miss out on.

On Wednesday we got the call that we have been waiting 10 months for.  As soon as I saw the Blocked Caller ID on my cell phone I knew what it was. The liver transplant nurse called to ask us to bring in Alexa as they had a match for her.  It pained me to no end to have to tell her that Alexa had a fever and was coughing--I won't lie, I even tried to downplay the severity of the cough and fever--Not happy with myself for that, as I would never want to compromise her health or safety, but in my heart, I was hoping that her fever REALLY wasn't that bad!  It didn't matter--There was no way she would be able to take that liver.  To wait 10 months, and get THE call finally--and have to turn it down---Oh boy was I mad!  Immediately tears of frustration rolled down and there was no bringing me back from my despair.

Then I came home, and had to get Alexa to eat as she ate very little during the day.  Immediately tears came to her eyes and she complained that her tummy hurt.  To beg and basically force my girl to eat a bit of soup when her tummy clearly couldn't handle it, well that just put me over the edge...with tears rolling down both our faces, I sat her down and tried to explain to a 4 year old as if I were speaking with an adult, the reasons why she had to eat.  And the fact that she understood and tried, I mean really tried to choke down as much as she could.  I REALLY HATED LIFE AT THAT VERY MOMENT.  Why my daughter was not allowed to just be sick like thousands of other 4 year olds and eat when and if she wanted, irritated me!  And I had to be soooooo careful, she needed to eat enough that she got the calories to keep her metabolically stable but I had to make sure that I didn't push her so much that she would throw up...She got that look in her eyes and I knew one more spoon and it was all coming out--I stopped!  I had gotten enough into her that I was confident we would stay out of the hospital for at least the night.

Managing this illness at home was short lived though.  We did a lot of running back and forth between calls to the metabolic doctors and visits to the pediatrician and laboratories.  Everyone was on the same page--Alexa probably had a viral infection and as long as she was keeping her food, formula and extra calories down, we could stay home--(easier sad than done!).  Well as soon as we got back from the lab, she ate snack as well as some high calorie juice and just could not keep it down.  She had also been way more sleepy than normal.  The on-call had no hesitation in her voice--we had to bring her in.  So it seems that we could add February 14th to a list of dates that Alexa would spend in the hospital.  But you see, yesterday was not only Valentines Day--more importantly it was Alexa's big brother, Stefano's 13th Birthday.  It saddens me to say that Stefano at times gets overshadowed by his little sister and her UCD.  We really try not to let it happen like that, but when she ends up admitted to the hospital on HIS b-day....



That she had to send a video message to him instead of saying happy birthday in person, that Stefano had to worry about his little sister in the hospital instead of basking in the glory of HIS day.....add this to another reason life pissed me off.

Stefano Morales, it has been a pleasure to watch you grow into the wonderful young man you are becoming.  Alexa and I love you very much and hope that you had the most amazing 13th birthday!




And then we spent the night at the hospital--and it seemed that was what was needed for me to be grateful of  a couple of things.
1)Alexa has a pretty bad virus and through it all, her ammonia's came back at 18 and then 9!  Metabolically could not be better--So while we lost out on our liver; at least we can wait in the comfort of our home knowing that my girl is stable!  Our transplant nurse emailed yesterday and let me know that we are VERY high on the list! Our time will come and when it does, it will be the perfect fit for Alexa!
2)I have amazing family and friends.  Friends that call or text me right away to offer support--telling me things that are way too inappropriate for here but exactly what I need to bring a smile to my face while it is covered in tears of frustration.  Friends and family who come to visit and help out while we are in the hospital.  At one point we had about 6 people in our emergency room (other than Alexa).  One of our dear friends was even kicked out (as the IV team was in with Alexa) but that didn't stop her from hanging out to sneak in a visit and offer support when the coast was clear!
3)This UCD life we inherited, has led me to some wonderful friendships with other parents of children with UCD's as well as others who have a UCD themselves.  Thank you Mark Zuckerberg, for this is not a luxury others before us were granted.  We worry when any of our kids are not doing well, stay up to wait for ammonia numbers, offer support and a judgement free zone when we just want to vent.  For their unending support, I will be eternally grateful!  In HSC, I met another UCD mama (in person as opposed to on facebook).  Her son was there for a regular clinic appointment and while there was not much time to talk as we had to be back for rounds, I am so happy to have met another family who we will one day be more than happy to offer the same support I have gotten over the years should they ever need it!

I am not going to lie--I am still not overly happy with the way things worked out this week, but at least I have a bit more of a positive outlook.  Life is full of ups and downs--this one is just a little more down than I would have liked it to be.  Next week will be better--I am sure of it!
And a picture to show how happy we are to be home!