Thursday, May 30, 2013

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!

Thursday, May 2, 2013

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)


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!