That Doesnt Go There!

"That doesn't go there!"

...not exactly the words you want going through the surgeon's head as he operates on your daughter.

Alexi started off her hospital tour here at CHP (Children's Hospital of Pittsburgh) with the upper GI scan on Friday the 12th that showed a definite obstruction in her duodenum.  The duodenum is the first bit of "plumbing" after the stomach.  They could have checked her in that day, but let her go home over the weekend and attend the Three Rivers Dash for Down Syndrome! 

Monday morning Alexi got some breakfast and a bottle, then off to the hospital we went.  She needed to have a barium enema in the morning and we checked in for our extended visit soon after.  She was on a diet of clear liquids only on Monday and Tuesday morning was banned from anything by mouth.  In went the IV to keep her hydrated and the NG tube to suck everything out of her tummy.

She hated the tubes but eventually learned not to tug at them, allowing her to remain unrestrained.  With no food she was pretty tired anyway so she just wanted held non-stop.  No complaints here, holding my daughter has got to be one of my favorite things to do these days.  Same rules applied for Wednesday before surgery.

Alexi was called down to the OR and we didn't wait long before she was taken in by the anesthesiology team.  I don't think I'll ever forget those eyes looking over her shoulder as mom gave her a hug and kiss.  Then as soon as the nurse picked her up, Alexi was happy playing with her mask and never looked back as they walked away.  I had gone over that moment in my head a hundred times and thought I was prepared.  Man there is no way to prepare for that feeling!


So we had been prepped, this surgery was going to take around 2-3 hours.  We knew that was just an estimate and were prepared for a longer wait.  So after about 2 and a half hours the liaison nurse came in to give us our update, and said the were no longer to continue laparoscopically and would have to proceed with an open surgery.  We had known this was an option if the duodenal web was too difficult.  What she said next is what alarmed us.  (Kerith and I are sort of pro active, and did a bit of research on Alexi's condition prior to surgery. ) The liaison nurse informed us that the game plan had changed due to the surgeon finding that Alexi had Malrotation and some twisting.

That first part is hard enough to hear, it means that when Alexi was developing, her organs never rotated the way the rest of ours did, causing them to be nowhere near where they are supposed to.  In laymen's terms, navigating through Alexi's intestines was like trying to use the new maps for the iPhone 5!  (ok bad joke but I'm tryin to keep it a bit light around here)  This alone can cause lots of problems and made the surgery much more of a procedure than initially planned. 

The second part of the nurse's statement is what floored us.  The word twist is much more serious.  The configuration of Alexi's intestines made them much more vulnerable to twisting around each other.  Wrapping around each other can cause constriction, which can cut the blood supply to organs and cause organ death (gangrene.)  The gravity of the situation went from bad to worse...way worse.

The next hour passed with no update, then another 30 min.  I honestly can not believe it was only an hour and a half, at one point I remembering asking how long it had been since the last update, and my mom informed me about 15 min.  I literally thought over an hour had passed at that point. 

Finally we were updated again that they were almost done repairing her and we going to start closing up soon.  We asked if there were any complications with the twisting and the nurse said he didn't think so because they would have mentioned it.

A bit of relief.

I don't know how much longer it was, but ultimately the surgeon came in and went over the procedure with us.  It turns out there was no twisting at all.  The malrotation required a more in depth procedure, but was completely repaired.  They are unable to put the organs where they are "supposed" to go, but reconfigure them in a much safer way, reducing the risk of twisting drastically.  As a result of reconfiguring her organs, her appendix would have been in the opposite side of the body and up much higher than it would normally be found, so it was removed to prevent misdiagnosis if Alexi ever had an issue with it.  BUT, no twist means no permanent damage to her other organs!

Wow, an organ (however useless) was removed from my daughter and that was the most minor part of her surgery.  They did still find and remove a duodenal web, without needing to perform a bypass.  So that part went very well.  Finally we can breath a bit easier.  Alexi is going to be ok.

Update:  Since surgery Alexi has been in quite a bit of pain but is taking it very well, considering she doesn't know why this is all happening to her.  She now has the NG tube, 2 IVs (one currently active), a pulse ox, some monitor leads, a catheter, and an epidural.  She is seriously not happy with all of these tubes and wires but is doing surprisingly well not removing them.  So far she only needed the NG tube replaced once and the catheter replaced once.   She's behaving mostly because she is asleep 90% of the time and delirious the other 10%.  We'll see how the next few days go, but no matter what, she is recovering.  That is so good to know, she's done, it's time to heal.

No More Juice!

Ok, let's start the the begining, maybe this could help someone else get the right treatment a little sooner.

Alexi was born at 30 weeks, so she had some very close monitoring for the first couple months, even back then we noticed trouble transitioning from IV nutrition, to the feeding tube, then again from the feeding tube, to digesting breast milk.  The doctors watched closely, but since everything eventually passed through they decided there wasn't a concern yet and she got to come home.

Since birth Alexi has been thinner in the mornings and had a big old round belly (like daddy's) by the end of the day.  We've brought it up at every doctor visit and they felt it was a result of her being low tone.  It just seemed odd to us that no matter how much core work we did with her, it didn't go away at all, in fact it got worse the older she got.  She's fought constipation off and on her whole life but in the last 4-5 months is been more often than not.

The constipation seemed to get worse when we started solid foods and the chronic nature of her constipation alarmed us, so we scheduled specific Dr visits around it.  They felt it was a result of low tone and that she needed more juice (we had been told that at every Dr visit for months.)  We did as instructed every time with no result.

One day, while we were visiting her family from across the state, Alexi began vomiting.  Our first fear was that she ate something she was allergic to.  We called the Dr and went over everything she ingested and there was no real concern of allergic reaction.  We continued our stay and Alexi did pretty well at first, but then began throwing up more and more.  Once we returned home and saw the Dr, they decided she was throwing up from being constipated for so long, and put her on mirilax.  This seemed to work for a day or two, then right back to vomiting.  They (Drs) were pretty sure the vomiting now was a result of being on mirilax and being gassy, so anti-gas medicine.  Then after that the vomiting was blamed on indigestion, so on to zantac.

With all 3 of these meds and Alexi still vomiting we saw a nutritionist for Alexi.  This nutritionist immediately knew something was up when we explained Alexi's belly situation to her (6 pack in the morning, looks like she swallowed a beach ball at night.)  The nutritionist did her job and advised us on some foods, but knew she was going to be no help, and recommended that we see and GI immediately.  So we called the pediatrician and had a GI appointment scheduled.  The GI also seemed to lean towards low tone being the main cause, but agreed to order an upper GI scan to be safe.

The upper GI scan showed clear as day that Alexi had a pretty serious blockage/restriction in her duodenum (first section of "plumbing" outside the stomach.)  They were confident that she had what is known as a Duodenal Web.  This caused Alexi's intestines to work as efficiently as a clogged drain pipe.  Sure the sink will drain, but it's gonna be a while, and the bigger particles aren't goin through.

Picture break!

Here's a few shots of Alexi practicing her modeling faces...

Show me Excitied!  Now Anticipation...  Impressed!

Bored...  Now like your thinking.  Now surprised!!

Ok, whew!  Got that out of the way.  Why was I so detailed in explaining what we went through trying to figure this out?  Was I complaining about the Drs?  No, certainly not.  I'm simply trying to help anyone else who may read this and see if we can't help them get the ball rolling on fixing the issue before the situation gets worse (like it did for Alexi.)  We were semi-adamant that there was something serious wrong with Alexi, and the Drs kept denying it.  It's not their fault, but we have learned more than ever that we need to be unafraid of putting our foot down and making sure a situation is checked out, not letting it go and seeing if "more juice" does the trick.

Now onto the hospital stay...  (see next blog entry!)