Monday, March 18, 2013

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:  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!