Friday, July 19, 2013

More On Bebe Quincy- Devil Is In The Details

After the news from yesterday, I thought we could all benefit from this update today from Daddy Nick. He goes into the details of *exactly* what is going on inside Bebe Quincy, and what it all means. It is incredible information. Modern science is astounding.

I'll turn you over now to hear today's update:

Quincy update for July 19th, second day on transplant list:

Q has stabilized. She's off dopamine and her blood pressure is stable again. She will go up to 1 on Milrinone (highest dose) by the end of the day.

We've been told it is "highly unlikely" that we'll go to step down today...and that's pretty much just fine with us. An extra day lets everything settle a bit more.

A few people have asked why the urgency to list her now instead of trying the medication path longer, so I will try to explain.

Quincy has two issues:

1) Dilated Cardiomyopathy

2) Left-Ventricle Non-Compaction

I've gone over LVNC a couple of times already, but basically it means that her left ventricle (which pumps blood out to the body) doesn't squeeze enough. In a normal individual this chamber compresses about 55-60% to create the pumping action. In a healthy individual this created an ejection fraction (percentage of total heart volume that is pushed to the body) of around 55-70% total. Ejection Fraction is a product of several factors, but left-ventricle compression (notice that's not compaction) is the deciding one.

Non-compaction actually indicates that the muscle fibers in the heart wall are not compacted together and therefore can't create a good squeeze action (compression).

If LVNC was her only issue, medicine would be a path worth considering.

But she also has significant dilated cardiomyopathy. Basically this means her heart is larger than it should be. In Q's case her heart is 2.5 times larger than it should be (.5 larger and we start calling her Grinchy). It takes up a large amount of the chest cavity and has other impacts on the body.

Now, some kids with dilated cardiomyopathy grow until they catch up to their heart. And some kids who have LVNC see their heart muscles compact when the heart starts growing...

...but the combination is fatal.

She will not begin to see her heart muscles compact until her heart has to grow, i.e. once her body gets 2.5 times larger.

But her ejection fraction today is 12 with a heart that is physically 2.5 times larger than her body needs. So her ejection volume is actually the equivalent of a heart with an ejection fraction of 30 (12x2.5=30).

As her body grows to catch up to the size of her heart, her ejection volume will go down. Eventually, before she can grow large enough for the muscles to begin to compact, the ejection volume will be too low to support her body's demands.

And she will again begin to fall into a cycle of congestive heart failure (when the heart can't push out enough blood to move blood into the lungs or supply the muscles and brain).

The danger now is that as her body grows and the demand on the heart increases and the ejection volume falls...medicines will eventually not make up the difference. At that point we will discuss putting her on an EVAD or LVAD device.

Dumb aside: unlike TV *cough*Grey's Anatomy*cough* cutting the LVAD wires will NOT get her a heart faster. Ever.

Anyway, right now we're in the countdown to get a heart. But there's another countdown - a terrible countdown - to the time when her heart can't keep up with her growing body. Which will make her sick. Which makes the transplant more dangerous and her recovery harder and more uncertain.

So we list her for a heart transplant in the hopes that she gets one before time becomes our worst enemy. Right now she often looks healthy; but that is a mirage more deadly than shimmers in the desert. The healthier she is, the better she will do. Time marches on. Every tick of the clock brings her closer to a heart, and closer to the cliff.

So we pray for a heart. We pray it comes soon. We just pray."
Have a good weekend folks, and please, keep Quincy, Nick, Amy, the rest of their family, and all the doctors and staff helping to take care of this precious little bundle in your prayers please. 

1 comment:

Momma Fargo said...

Lots of prayers. I cannot fathom going through this as a parent. (((hugs)))