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.

"Pizza"

What kid's favorite food isn't pizza?!? 

Well it's been Carson's for as long as I can remember.  That's right...this kid who eats pretty much no solid foods will eat pizza.  Pretty crazy!  I've been looking and looking online and in grocery stores for fat free foods for Carson.  My quick trip into the store on my way home from work now last an hour of looking at labels.  Talking to Mama Con the other day about what Carson could eat she found a website with recipes (mostly vegan) fatfree.com.  Trying to find Fat & Lactose free things to eat is not an easy task and then to actually get Carson to eat them is even more trying.  I've almost given up because I can't tell you the number of things we bought just to have Carson turn his nose up at them.  So an idea mom got off the website was pita bread and to me pita bread looks like pizza crust!  Then it was on to pizza sauce.  Most pizza/spaghetti sauce in the stores had fat in it so I was going to attempt to make it, but I found one fat free sauce and bought it. 

And this is what Carson has looked like at dinner the last two night.  This little guy LOVES his pizza and I don't even have to cook it so that's a huge plus for me.

I think he really just likes tomato sauce, but it's calories so I'm ok with that :)

July 5th At the University

Lets start with the fun stuff...We had surgery follow up part two yesterday with Dr P.  This time he didn't find another reason to cut Carson open again :)  (Unlike last time with the hernia's that magically appeared)  We got to chat with Dr P and as he said to the Med Student this appointment was more of a social visit or us than medical.  He is such a great guy!  An no more appointments with him unless/until we need him again.  If Carson has any complications from his PVT Dr P said he would be our go to guy for any surgery that's needed.  Makes me a little more comfortable to know a surgeon if we ever need one.   
And now the not so fun stuff...Carson's tummy has always been a little tricky ever since he had that virus in January 2010.  It took us until about May of that year to finally figure out a formula that worked for him that didn't give his diarrhea.  We were nervous when we switched to pediasure, but when we did all was well.  So we didn’t think much of it when we made this switch.  According to everyone we talked to Portagen is pretty much the same as Pediasure just a different type of fat.  Well Carson had diarrhea from the day we switched him.  I didn’t want to overreact so we kept going with it with hope that his body was just taking time to adjust, but on Sunday Carson’s gut told us “NO MORE” loud and clear.  His intestines kicked it up a notch and pretty much demanded we stop that formula!  I switched him over to pedilite to get him hydrated and to give him a break.  His poor little red bottom couldn’t take anymore! 

I called Dr R’s office 1^st thing on Tuesday morning and they got us an appointment for that afternoon, we were already going to be there for our meeting with Dr P.  Met with Dr B, Dr R is in Lebanon for three weeks.  After he talked to the nutritionist they decided to try mixing Carson’s formula a little “weaker” to see if that helps out.  It was 30 cal/oz and now it’ll be 24/oz.  Now we pray that Carson can tolerate this.  They said Portagen is pretty much the end all and be all of treating Ascited.  He said that while there are other formulas that would be ok for C to be on they aren’t as good, this is the best.  If this doesn’t work then we will be forced to switch to something else, but they wanted to give his a shot before we change anything.  We are to call back in a few days and let them know how it’s working. 

With fewer calories I will be interested to see what this does to Carson’s weight.  We are still down about 2 pounds from his pre-surgery weight.  Which doesn’t seem like much, but when it’s 10% of your total body weight that puts things into perspective.  If I was down 10% of my weight in a month that’d either be a very good diet or a problem.  We are hoping that once we get things in the bottom area leveled out and get thing absorbing a little better he will once again start packing on the pounds!

We also asked Dr P to order a CDC and Auto Diff yesterday to see what that would tell us.  His CBC’s have looked so good since surgery (minus right after surgery when they took a nose dive for no apparent reason) there was some talk (very quietly as to not give us any undo hope) that things might actually be working in the graft, but just not able to be seen on ultra sound.  Well those hopes were pretty much completely struck down this morning.  With the new “My Chart” website that the U has you can get test results back online which is good and bad for me.  Good because I don’t worry about them and get them quickly and bad because I’m a little crazy and Google every little thing!  But Knowledge is Power right?  His WBC that had been at a 4.8 (5.0 is “normal”) is back down to a 2.7.  His platelets are still “normal” at 155, but that’s on the very low side of “normal” and they had been up near 300 two weeks ago.  So all in all his CBC was not the good news we had hoped for.  Makes me sad.  There is now pretty much no hope that by some miracle his surgery actually worked.  And now we move on.

Sunday Funday

We decided Sunday we were just going to have fun as a family.  It's been quite a while since we had a carefree day and I think we all needed it. 

We started off with 11am church service for the first time since Carson's surgery.  The 1st weekend in June kids move rooms so both kids finally got to go to their new rooms and meet their new teachers.  It was hard to leave Carson with someone we don't know with the situation the way it is right now, but it's only an hour and we both needed it!  After church we did Carson's first "on the go" feeding, aka tube fed him in the Car while we were driving. 

After a quick lunch and stop at Target we went Miniature Golfing.  It was more of a chase the kids around while they hit or tossed balls toward a hole, but they had fun and so did we.

After a great 18ish holes we decided to check out the spray park I'd read about in Rock Island.  It was so much fun!  It's right along the river and it just opened last year.  Lots of green grass and nice play ground equipment...add a bunch of water and it's every kids dream. 

Had so much fun yesterday we went back today (with swim suites this time).
 

Chylous Ascites

...Bet you don't know what the heck that is!

I didn't either until about five days ago because that's Carson's newest diagnosis.  The definition is "an abnormal condition characterized by an accumulation of chyle in the peritoneal cavity. Chylous ascites results from an obstruction in the thoracic duct that may be caused by a tumor or by a destructive lesion, resulting in rupture of a lymph vessel.". 

What all that actually means is that the fat that is normally absorbed into your body by the lymphatic system is leaking into his abdomen instead of being used as energy.  This caused increased pressure in his abdominal cavity causing bilateral hernias (they noticed the condition because of the hernia repair surgery). 

So now Carson gets to be on a NO FAT diet for the next 4-6 weeks.  That's right NO FAT!  My little kid that we've tried to "fatten up" for the last two years is not on a no fat diet and special formula, portagen.  The Portagen is apparently really gross tasting so he won't drink it and therefore he is back to being NG Tube Fed :(  and not just overnight feeds like we've done in the past.  He gets 2 feeds during the day and then over night also.

So this is what dinner time looked like the other night...notice the IV pole in the background with Carson's "Dinner" on it.  I've already come up with ways to work around the feeds including feeding in the Car.  Called our home health company today and they're sending us a backpack to try out.  Hopefully Carson will be able to carry it around so that'll make things a little easier.  Getting him to sit in one place for 30 minutes for a feed isn't the funnest thing I've ever done!

So that's pretty much where we are right now.  Carson had Bilateral Hernia Repair last Wednesday morning.  Thank the LORD he had no problems with anaesthesia this time.  We were in and out in about 5 hours.  He has recovered nicely from the procedure and now has two more scares to add to his collection.  If it's not one thing it's another with my kid!

Terrific Two's

Two years ago today we completed our little family. 

Can't believe it's been two whole years since I had Carson.  Wow what a ride it's been!  I thought the hard part was over when I delivered him that day...I was oh so wrong.  I would have never thought I could know the in's and out's of early intervention in two states or what Carson's white blood count usually is.  This isn't the life I would have chosen for our little man, but it is what it is and we will make the best of. 

The events of the last week make me thankful for my son.  I can't imagine our family without him and I look forward to lots of great memories in the future. 

Because of Carson's surgery we celebrated his birthday on Memorial Day and here are some pictures of our little party.      

I'm VERY Happy to be TWO!

Just because she's Cute :)

The Eeeeelmo Cake

They both played in the water/sand table for long time and LOVED it!

Then we played with Bubbles for a while

Cake

That about sums up his interest in the cake...not so much!

And we opened presents.



It was a great day with our family and I'm glad we did it early so Carson could enjoy it and we weren't worried all day about him hurting himself.

As for his recovery he is doing really well.  He seems like he's more and more back to normal each day.  We go back to see the surgeon on Tuesday for a check up.

Finally some good sleep!

Poat surgery day two was no fun at all. Carson was very agitated all day long and only slept for sort 15 minute naps every once in a while and in between he screamed and thrashed. It was really no fun at all. Finally we moved the bed out of the room and the chair over and I held him and he slept for 5 hours! And since then minus a few hours here and there Carson has been calm and very sleepy. He is still on the morphine iv, but know we also have an awesome little button to push to give him extra. His personality is showing through here and there too so it's good to see him coming back to us.

Here ar some tid bits of what's been going on
-Repeat CBC after blood transfusion was much better. WBC was up from .7 to 1.8.
-two ultrasounds were completed (6/1 & 6/2) both showed no flow in graft
-repeat dray yesterday was improved. Guy still sounds "junky"
-yesterday morning arterial line removed and dex stopped
-moved out of PICU about noon yesterday. Have I said how great the nurses are up there? They are!!

We were told last night by the nurse that they changed Carsons orders and he was getting no more blood thinners. I knew this meant that the graft had officially failed, but I was also pissed that the Resident just changed the orders and didn't even tell us. We pretty much new this was going to be the outcome since right after surgery, but still. If the graft had worked they'd have come in patting themselves on the back all day, but since it failed they can't even tell us that. I had the nurse get the Resident on the phone since he wasnt at the hospital so he could tell me. Would have liked to make him tell me face to face just to watch him squirm....I'm such a nice person. Dr Pitcher is great! We absolutely love him, but clearly he needs to teach his students some bedside manner...don't worry I'll be sure to let him know.

We got moved down to 3rd floor yesterday morning. To a double room :(. Not too happy about this but we are making it work. Our "room mates" from last night are ver nice people with a tiny 6 month old baby. They got good news this morning and will be leaving this afternoon.

Besides tha AJ and Emily brought Kay up to see us yesterday which was nice and mom sat with Guy last night so Dave and I could step out for a minute. We got dinner and picked up a DVD of Mickey mouse clubhouse for Carson. It's been emotional and we are tired, but only a couple/few more days and we should be out of here.

Thanks for all the toughts and prayers over the last week. This proceedure was the only one that will "fix" Carson that we know of so it looks like we will be on to managing the symptoms of this blood clot/portal vein hypertension. There are lots of things that can be done, but none of them will take the blood from his portal vein to his liver so they aren't fixes. So basically now we wait until we have a problem and go from there. Not exactly what we wanted for Carson, but as Dr P said, lots of people with PVT lead long normal lives. I just pray that this condition doesn't define who Carson is.

Today our goals are to get out of bed and to get him to drink some clear liquids!