How is Carson?

Never thought that'd be such a loaded question after this surgery.  According to the surgeon the surgery he had was "like running a marathon".  It took a lot out of him.  Plus...apparently he was getting a cold when he went in for surgery.  Wasn't a huge surprise.  I had a cold for a couple of days so it's probably my fault.  He didn't show any signs of a cold...he seemed fine by all accounts.

Surgery was Monday.  Went beautifully...flow is GREAT in his shunt.  As far as actual surgery goes Carson was a Rock Star. 

Monday night I stayed with Carson while Dave and Mom went to get some sleep.  There was suctioning...LOTS AND LOTS of suctioning...deep suctioning.  Carson wasn't a fan!  His blood gases and O2 kept getting worse.  At about 1am the doctor explained that they would probably need to help Carson breath...they'd do "high flow" and pi-pap and if that still didn't work they might possibly have to reintubate, but he'd have to get "MUCH worse" then he was right then.  A little later they started "high flow" oxygen.  Stayed on that for about an hour and it didn't get the numbers where they wanted them.  Then they started bi-pap, its the machine people with sleep apnea use.  It's a big mask that pushes the air into the lungs.  That still didn't work so they decided intubation was our only option.  He is sedated pretty heavily and only tries to move every once in a while.  We are now on almost 48 hours of intibation and they haven't really been able to wean him off at all yet.  Every time they turn a setting down his Co2 goes up and they end up putting it back up. 

He's had a fever, his pulse has been high, his blood counts are low so they just hung red blood cells and he is +2300 on fluid so they've given his two doses of diuretics to try to get that going.  He is puffy!  Hardly looks like himself.  He pretty much has a terrible cold and just had a very invasive surgery...pretty sure anyone would feel pretty terrible with all that.  Doctor said today viruses take 7 to 10 days (not to say he will be intubated for that long).  So we're here and we will be here until he's better...It might be a while, but he will get better and that's all that matters.

What exactly is wrong with Carson?

I get this question lots.  I'm not sure I've ever really been able to answer it until recently.  There has always been LOTS, but his most of his issues (probably) it seems stem from a blood clot in his portal vein. 

What does that mean you ask...their are two veins that take the majority of blood to your liver one of those is the portal vein.  Here is the best diagram I could find that has the "portal system".

As you can see the Portal Vein takes all the nutrient rich blood from you intestines, spleen, etc to the liver.  Carson's portal vein is clogged with a blood clot.  They have done a full thrombosis work up on him and there is no reason for the clot.  He has no underlying clotting condition except a recessive gene that doesn't increase his clotting factors enough to make them think that's what made it. 

The clot will not move, it will not dissolve.  It's going to be there forever.  They call it idiopathic, which means there is no reason for it. 

The biggest issue with the PVT is that it causes portal hypertension.  Basically because the blood has to go somewhere and it can't go through the portal vein it backs up into smaller veins.  The pressure in those smaller veins around the intestines, stomach, esophagus etc is much greater than it should be (portal hypertension).  Also, Carson's Spleen is a minimum of three times larger than it should be because of the backed up blood and is also overactive.  This is the reason his blood counts are always low and the reason he get sick so easily.     

"Hypersplenism is a type of anemia that may develop when there is a reduction of blood cells as a result of splenomegaly. The lower number of blood cells because the bone marrow is unable to keep up and replace the blood cells that the spleen is filtering too quickly."

What can happen?...the biggest risk with portal vein hypertension is internal bleeding.  The veins that have lots of blood in them aren't meant to have that much pressure on then and they can pretty much burst at anytime.  If this happens it will be a very emergent situation.  We have talked to the doctors and the University and we have a plan.  We will immediately go to the closest hospital and be life flighted to the University.  Do not pass go, do not collect 200 dollars...that's what we are to do.

Sounds like a fun waiting game doesn't it?

Well the surgery Carson has last June was to take part of his jugular vein and make a bypass around the blood clot...as we all know this was not successful.  The veins inside Carson's liver are too damaged so there was nowhere for the blood to flow into.  We were disappointed.  It's the only known "restorative" surgery...only one that will take blood to the liver.  We went to Chicago right after the 1st of the year to see the first doctor to preform this procedure in North America, Dr Riccardo Superina...He's the best. Dr S agreed...the Rex Shunt is still not an option, it will not work. 

What we did learn in Chicago...Dr. D told us the Carson's in a bad situation.  His portal hypertension is high.  It's not "if" he has a bleed, but "when" he has a bleed.  And it will probably be sooner than later.  He has seen MANY, MANY, MANY kids with this condition and by his estimation there is over a 50% chance that Carson has a bleed within the year and the odds go up from there.  Ugh!  That's NO FUN to hear. 

We've known it was a possibility, but no one has done the tests that Dr S. did to know exactly how bad his hypertension was.  We knew he had it, but not to what degree. 

Dr S said in his opinion waiting around wasn't the best option given his degree of portal hypertension.  He wants to do the surgery that Carson would have to have once he has bleeds now.  It's called a Splenorenal Shunt.



Basically they unattach the splenic vein from the portal vein and hook it up to the left kidney.  This diverts a portion of the blood away from the area intern decreasing the pressure.  It is the hope that this will also decrease the size and activity of Carson Spleen. 

Most doctors across the county have maybe seen a kid or two with this condition.  This guy has seen hundreds.  He knows what he is talking about.  He's the best...We're going to trust him.  Carson can not live the way he has been.  We can not live the way we have been.  Always in fear of what's to come.  Sick every other week.  It sucks for us, but it sucks for Carson more.  He is always happy because he doesn't know what it's like to not feel like crap all the time.  In the years to come it will only be more of an issue.  He will miss school because of illness and because of bleeds.  His quality of life will not be what we want for him.

So we're doing it.  Scheduling surgery.  We hope to get it done in the next month or two since we don't know how much longer we will be here in Iowa.  The recovery for Guy will be much like the last surgery.  I'm not looking forward to it, but this is our plan. 

I think it's the best plan for Carson.