Wednesday, August 28, 2013

Plan B

I keep getting asked if Alexa's stomach has gone down any.  Has it gotten any better?
The answer is NO!  
It has not gone down very much.  She is uncomfortable, not eating a great deal and in pain after drinking too much water or milk.  However, it doesn't look like it has gotten any worse either. That is the silver lining that we are holding onto.
Enjoying Starbucks
We let her body "try to work itself out" and it didn't work any so we are onto plan B.
We went for our regularly scheduled appointment yesterday--bloodwork, 1 hour of IV gancyclovir, 4 hour IV of cytogam and a consult with IGT--Image Guided Technology.  You might remember that is where she got her PICC line inserted, that dreaded spot in between the surgical waiting and the PICU--the spot that gives me anxiety just thinking about it--yep right back there!
After a bit of back and forth between the transplant team and IGT team, this is where we are going with the ascites.
They have Alexa booked for an abdomen tap on Friday morning.  A procedure in which they will insert a small needle into her stomach and drain a bit of the fluid to have it sent to the lab for testing.  We should know the results in a couple of days and then we can(well really, the doctors can) decide a course of treatment which can include diet changes (wonderful!--just as I thought we had gotten over the diet restrictions!) and/or IV medications (cause she really isn't on enough right now!)

A lot of things about this procedure was/is causing me some stress--the biggest one being I really do not want her to go under anaesthesia for a third time in 6 weeks.  To do a full drain of her fluid, they would have had to put her under.  It was instead decided that they would take a small sample.  This means that she will get a small sedation and local anaesthesia while they do the procedure.  I am happy about that but conflicted because I do not want her to be further traumatized by witnessing any more procedures.  I asked if I can go in the room with her, and the consult doctor said that if he is on during her procedure, I would be able to go in but it is up to which doctor is on. If they are smart, they will let me into the room with her, because if they don't, they better be prepared for one hell of a fight from Alexa.  She is still not over being taken by the nurse when she got her PICC in, so I don't envision a small sedation being enough to keep her calm during the procedure.

I know it could be a lot worse--It could be SO much worse. She is happy(most of the time) and her liver is healthy!
But right now I am scared. This procedure is bringing up a lot of emotions--probably ones that I have not had time to deal with since July 18.
I do not like not having any answers.  I do not like that the doctors and nurses don't have any answers.  I would like the procedure to go off without a hitch and for us to come back with a plan that works(and quick).  I would like to start preparing Alexa for her first day of Senior Kindergarten next week and the upcoming year, but I feel like we are stuck in some sort of limbo. We are moving one day at a time, so it is hard to get excited about buying a new lunch bag and first day of school outfit when I don't even know if she will even be able to go to school on Tuesday.  

Friday, August 16, 2013

Letting her body try to resolve itself

The truth is that we are not any closer to knowing exactly WHAT is causing all the extra fluid accumulation in Alexa's abdomen and we may not know for a bit longer.  But that is good, if it were really bad, a vessel issue, we would know.  Alexa's surgeon took one look at her ultrasound and is sure that there is not any issues with blood flow or too much pressure in the livers' blood vessels.  There was no need for a CT Scan yesterday.
In addition, Alexa's labs are still perfect--kidney is happy, despite the addition of the diuretics(Lasik) which can be hard on them.  Her liver numbers are great too.  Her prograf level is right in the middle of the range, so no changes to her meds. They are being weaned as scheduled.
The plan is to keep her on the Lasik for 2 weeks to help with the urine output.  Her stomach has gone down about 1 1/2 inches.  With luck, it will continue to go down and we can be done with this issue.
There is another cause which they are keeping in the back of their minds.  It is a bit premature to get into it, so I will leave it be for now.  If we get to that point, I will explain further.  It is not horrible, not nearly as bad as an issue with the vessels but might be a bit of a pain in the a** to fix.  So hoping we do not have to go that route.
So although, yesterday was long, Alexa was very happy and not in too much pain.  She passed the time in the playroom on 6A where all our favourite nurses are.
Alexa's potatoe head, "Ruby"
For the last 5 years, my mind has been conditioned to act FAST!  Living with a urea cycle disorder, we learned quickly that whenever there was anything, even remotely off, with Alexa, it was at minimum a call to the on-call metabolic doctor. It more often than not  resulted in going to the ER for an ammonia level.  It was literally an "act now or risk brain damage" situation. Every test under the sun would be run, until we got to the route cause of the ailment.  Everyone was in super-fast mode.

We are not in that position anymore, and it is hard to untrain my brain.  When minor glitches come up, it is hard for me to sit back and "let her body try to resolve it on it's own". It is not what I am accustomed to by any means.  My mind instantly wants to use Dr. Google to search any other possible causes,  tests, and treatments available.  My mind instantly wants to go into panic mode and find out NOW what is wrong and WHY and FIX it!  Waiting for her body to work it out on it's own, as it was meant to, is not something that we ever had the luxury of doing before.  My mind needs to learn that we have more than 2-3 hours before devastating effects are seen.
We do not have to go back for a check-up until next Wednesday.  This "we have time to wait it out" approach is so new to us, but we are trying.

Wednesday, August 14, 2013

Our Great Camp Adventure for Sick Kids!

It's well know that I have some pretty awesome friends!  I would like you to meet a couple of them:

Cecilia, Carla, Roberta, Marklin (and me)!

And together we will be participating in the Canaccord Genuity Great Camp Adventure on September 28th.
Our fundraising dollars will be donated to The Possibilities Fund at Sick Kids.  This fund is available for urgent needs, including providing the best and most innovative care to patients, whether they are critically ill, injured or living with a chronic condition.  This fund allows Sick Kids to invest in research projects so that scientists can change the future of kids' health, and to train doctors who care for children across Canada and around the world.  The Possibilities Fund supports people, programs, equipment and physical infrastructure, and allows SickKids to continue to be the exceptional place it is. 

Why are we doing this?
Because we LOVE to exercise?  Hahaha!--Clearly NOT!  So why did my friends jump at the chance to walk 20km? I am sure each of them have their own reasons, these are just two of them:

Cecilia's son, Anthony was diagnosed with hodgkin's lymphoma cancer when he was 13 years old.  At the Hospital for Sick Children, he received aggressive treatment and we could not be more thrilled to report that he has been cancer free now for almost 4 years!  Anthony is a Survivor!

As for myself, well many of you know my girl, Alexa was diagnosed at 9 days old with citrullinemia(one of the Urea Cycle Disorders).  For the last 5 years, she has been followed so very closely by the metabolic doctors at HSC.  On July 18, the very best liver transplant doctors gave her another chance at life.  They transplanted a portion of her father's liver into her to cure the effects of living with citrullinemia.  The coordinators, nurses, doctors and surgeons are amazing.  Each and every one of them ensuring that we had (and will continue to have) the VERY BEST CARE during the long road ahead.  Alexa has (and still is) a participant in many trial studies that are available only with these types of funding.

Without the funding, there is no more research, no more hope!

I hate more than anything that we have to rely on Sick Kids Hospital, but I am so very grateful that they are there for us.  Without them, Alexa might not be with us today.

We walk so that one day there is no hodgkins lymphoma.  
So that one day there will be a cure to the genetic mutations that cause urea cycle disorders. 
So that one day in the not so distant future, kids can be kids--happy and healthy! We have hope!

So what exactly is this?
It's an up to 20 km challenge-by-choice adventure walk.  We will go at our own pace and participate in ultimate camp adventures along the way...
I am not going to lie, walking 20km scares me--I have never done it before.  I have trained for 10km runs but walking for 6-8 hours seems so much more daunting to me.  And then I think of Alexa and Anthony, and all that they have been through to overcome their medical conditions.  And well if they can endure all they have and come out smiling, we sure as hell can make the 20km.  Even if I have to crawl across the finish line, I will do it for them!

How can you help?
We would be ever so grateful for any donations.  Small donations add up, and if we can donate enough to get the research coordinator and scientists in the laboratory for even one more hour, then this is all worth it!  If one child is able to get one more hour of physiotherapy or occupational therapy, then this is all worth it! If one more piece of equipment is able to be purchased, well then, this is all worth it!

If you are able to do so, please click on the link below to be directed to my personal page:

Or you can support our team of wonderful ladies (A & A's cabin) by clicking on the following link:

Finally, we would LOVE, LOVE, LOVE, if you would come out and cheer us on!  Smiling faces and familiar voices at the 15th kilometer, may be just the push we need to make the final stretch!

If you would like more information on the event, please click here:

As always, THANK YOU so much for all your continued support!


A fancy medical word for the build up of fluid in the abdomen.

That is what Alexa has developed since Saturday.

When Alexa was first discharged from the hospital, she had it as well.  Apparently, to be expected due to the trauma. Within a couple of days it had come down significantly.
On Saturday we noticed that it was getting bigger again; by Sunday her urine output had significantly decreased from her normal.
We had a regularly scheduled appointment on Tuesday for the 4 hour infusion of Cytogam, so after speaking to the nurse practitioner it was decided it could wait until then.

Tuesday, her stomach was very distended and she was in a lot of pain due to all the stretching of the abdomen region.  One of her eyes was also swollen, I presume due to the water retention. So they took quite a bit of blood.

The good news is that her blood work was perfect--liver function tests are great, kidney function is great. The only thing that is slightly low is a protein called albumin.  This could be causing the water retention. This one is an easy fix.
To be certain they ordered an emergency ultrasound.  Her doctor reviewed it and it was absolutely normal.  *Breathing a sigh of relief*

The not so good news is that her swelling is significant (Dr. A said "impressive"--Never a good thing coming from a doctor) and although expected after surgery, it should be getting better, not worse.
So they have given her Lasik, a diuretic medication to dispel all the water.  We are to return tomorrow to be re-evaluated and if there is no improvement, they will order an emergency CT Scan.  Sometimes the CT Scan can pick up things that an ultrasound cannot but due to the exposure to radiation, they did not want to order it prematurely.  With the CT Scan they are hoping to be able to see if there is any restricted blood flow or high pressure in the blood vessels of the liver.  This one is not such an easy fix and we are really hoping and praying that this is not the case.

So in the meantime, I will be trying to get her to drink and pee as much as possible, consume enough protein to increase her albumin and prealbumin (my how things have changed!), and measuring that belly so I know if in fact it is getting better, worse or staying the same.

I know I have asked for a lot of prayers recently, but with this being our first hiccup with transplant, if you have any more left today, would you please keep us in mind and pray that this issue is not a blood vessel problem?

Tuesday, August 6, 2013

No News Is Good News...

Or at least in our house, that seems to be the case.

In the week that we have been home, we have had two sets of blood work done(Friday and today) to closely monitor all her liver function numbers.  They look great.  On Friday, the AST was 38 (completely normal) and her ALT was 81, almost down in the normal range--I am told this one takes a bit longer to come down.
Both sets resulted in no dosing changes for her medicine.  That is good--that means that they have figured out the exact dosing required for her Tacrolimus(FK/Prograf) which prevents rejection.
And we do not have to go back for blood work until next Tuesday!  Must mean that they think she is pretty stable.
I must say,  I love the ease of blood work when having a PICC Line--Seriously--no tears, no stressing about whether they will find the vein on the first try.  It is so fast and pain free.  If it weren't for the fact that we cannot get it wet at all, I think I would keep it forever!

Alexa is slowly being weaned off of some of the other medications that she gets.  Her Prednisolone dose which is another steroid to prevent rejection is being tapered down every 2 weeks until we have reached 3 months post-transplant.
Ganciclovir, which is the intravenous medication that is administered by our home care nurse has gone from 2 times per day to once per day, again for about 3 months post-transplant.  This medicine helps prevent some viruses that are pretty bad for immunosuppressed people.
I will be so very happy when she no longer needs these medications.  They are harsh on the body and have some pretty serious side effects with higher doses.  I hate that she needs them, but what can I do, she absolutely needs them to remain infection free and prevent rejection all at the same time.
One of the side effects that I knew about but didn't really envision was hair loss.  The medication can temporarily cause small patches of hair loss.  I know it is just cosmetic and I know that it is only temporary and maybe I am being silly about it, but I still feel like crying when I see it. There are just some things, that no matter how much you know in advance, you just can't be prepared to see--This for me is one of them.   In the meantime, there is a lot of ponytails going on in this house to cover it up.

As for food, we are slowly making some progress.  Chocolate milk, although she is not interested in drinking it on her own, she will drink it at breakfast and dinner.  She may not be happy about it but she drinks it.
She loves cheese and everything that cheese is on!
Eating some good old Kraft Dinner--This meal never disappoints
Tim Horton's is quite happy and will sure to make a nice profit off of Alexa's favourite breakfast item--Bagels with cream cheese!  Don't mess with her when she is eating one of those!
She also had a burger for the first time--I wouldn't say she loved it, but she did eat the portion that we set for her--small victories!

Today is Chris' Birthday.  Alexa and I prepared a very rich meat and cheesy lasagna.  She has helped me before, but this time was different.  She was so happy to sneak the shredded cheese and finally I did not have to tell her that she couldn't.  She loved every minute of it!  And I in turn, loved watching how happy she was!

Before I sign off, I would like to wish Christian the very happiest of Birthdays!  This man has shown again and again how much he loves his family and that he would do ANYTHING for his children!  Chris, you are a wonderful father and husband and deserve only the very best to come your way.  Your children lucked out in getting a true hero for a father and no one is more proud of you than I am.