Emergency shunt revision

So Riley has reached an even dozen number of surgeries.  

Last Tuesday morning started like any other day.  I got up and got ready for work my mom came to watch Riley. I was 3 hours into my day when I got a call from the hubby stating that they (he and my mom) thought that we might have a problem.  The area over Riley's VP shunt was puffy.  I told him to pack her up an head to the ER.  He told me that she seemed okay and that they would wait for me to come home (I only work 15 minutes away). I told him that I would call the neurosurgeon and let them know that we were going and that I would be leaving as soon as I told my boss.  So I packed up from work and headed home.

On the way home I called the neurosurgeon team and told them that we should be at the ER within 45 minutes.  When i got home I changed my clothes and packed a bag quickly.  I know that at the very least we would be staying overnight, but I was pretty sure that we were in for a surgery.  So then we packed up and headed to the ER.

Now before I go any further with this I thought I would explain a VP shunt.  VP shunt is short for Ventriculoperitoneal shunt. This type of shunt is a device which drains the extra fluid in the brain into the peritoneal cavity where the fluid can be absorbed.  The peritoneal cavity is the are between the two membranes that separate the organs in the abdominal cavity from the abdominal wall.  The way it placed in her body is the ventricular catheter is placed in the ventricle in her brain that is enlarged then the shunt valve is located behind her right ear (you can feel this under her skin as a hard bump).  The Distal catheter runs from the valve down her neck and over top of her clavicle (collarbone) then down into her peritoneal cavity, there is a coil of extra tubing that allows for her growth without having the have an additional surgery to add tubing.

Below is a picture of what a VP shunt looks like not in the body: 

 

And here is one that shows you what in looks like in the body.

So back to our story.  We get the ER at about 11 and check in.  There was no one there it was kinda amazing to be the only people in an ER.  We get called back to the triage area within 5 minutes of checking in.  The nurse asked us what is going on and I explain that we think that Ri's shunt has stopped working.  The nurse wants to know what symptoms she is having, so I tell her that we can feel a build of fluid over the shunt and that Ri seem a bit more sedate then she normally is.  I also told her that it seemed that the swelling was getting bigger.  The most common symptoms for a shunt malfunction are headaches, lethargy, Irritability and/or sleepiness, Problems with balance or gait, nausea, and vomiting. Less common symptoms of shunt malfunction can include seizures, A loss of previous abilities (sensory or motor function), Difficulty breathing. Now Riley was not showing any of these.  She had eaten breakfast that morning with no problems.  She was not cranky indicating that she was in any pain.  She woke up smiling that morning. The only indication we had was the puffiness over the shunt, which my mom had found while putting her hair in pigtails. 

So after taking her vitals, which were all fine, we were taking back to a bed in the ER.  The doctor came in and said that they were going to get a couple of X-rays and a CT scan to see if there was a problem with the tubing. They also let us know that they had gotten in touch with neuro and that they were in a procedure and that as soon as they were done they would be down to check us out. They took us off to do a CT scan.  That was "fun."  You have to be still in order for them to get there scan.  Have you ever tried to get a two year old to sit still for any length of time it is not easy and it is even more difficult when they don't understand what you are saying to them.  So they wrapped her tightly in a blanket, like a burrito. She was still able to move her head so they had me hold it.  This still didn't work so then they added pillows.  They finally got what they needed.  We were able to get the X-rays much easier.  

The doctor came in when they got the results of the scans and said that they could not fine anything wrong with the tubing and that all they could see was the fluid build up around the shunt valve.  When I took a look at for shunt site I realized that it had doubled in size since we had been there and the Riley was becoming more and more lethargic as we were sitting there.  Neuro came down shortly after I informed the doctor of this change.  The Neuro Fellow (who is a full doctor but is learning a specific specialty  ie Pediatric Neurology) came in and check the setting on Ri's shunt.  She has a programmable shunt meaning that by using a strong magnet the doctor can change the setting of the shunt as need be without having to do surgery to do so.  

Ri's shunt has been set to 2.0 for the last 2 years.  The way her shunt works is the lower the set number the more fluid/faster it is draining and the higher the number the less fluid/slower it is draining.  The Fellow came in and put the device (which I don't know what it is called) that checks the setting and Ri's shunt was set at 1.5.  This is the opposite of what they were thinking it was going to be.  The fellow stepped out to checking with his Attending and lets him know what he found.  After finding out the the shunt was a faster setting and fluid was still building up the Attending decided that a revision was necessary.  They were not sure what part of the shunt was the problem so they need to go in and look at what they were dealing with.  

We were rolling to surgery pretty quickly after that, so quickly in fact we did not wait for transport, the Fellow and hubby rolled Ri's bed to surgery.  And we were on our way to surgery 12.  The very best, and I mean the very doctor was the attending on Riley's surgery.  This doctor is was one the of first doctors to every use shunts, so we knew she was in very "gifted hands."  Surgery took about hour and half.  When the Attending came out he told us that the ventricular catheter had been completely blocked.  It is unfortunately a risk of shunts.  Riley came out of surgery really well, she was able to have some dinner when we got up to our room.  

When the Neuro team came in to check on us we asked about this blockage because it was not something that just happens over night and she was not showing any symptoms before that morning.  He explained that what probable happened is that the ventricular catheter has pin prick opening on the end and that it has probable been clogging for awhile and that one more hole clog and it stopped working completely.  He also said that so people will go their whole lives with a shunt and not have it clog and others will have it clog every couple of years and need revisions.  Why do I see us falling in the second category?

We were discharged the next day and Riley was back to her normal self.  We had a follow-up appointment with Riley's neurosurgeon (who had been out of town while all the drama happened) this past Tuesday.  He said that she looked great and was healing nicely.  He also thinks that the worsening of Ri's apnea might have been because of the shunt.  He went on to say that since she does not show normal symptoms to a shunt malfunction that we need to keep an eye (which we already do) on the apnea and if it comes back again that we should look at the shunt function.

He also showed us some results on Ri's MRI (which we had done at the end of August) that he could not really explain.  Riley has a Arnold Chiari Malformation.  And in her MRI in January 2011 there was a small pocket of fluid at the base of her skull, this is not there any more.  He is not sure if this is because the shunt was not working, or if her brain has just grown that way, or if it is a worsening of the Chiari.  So he wants to wait 6 months and do another sleep study.  If the apnea has not gotten better or has gotten worse then we will need to start thinking about doing something about the Chiari which would be major surgery called for a stay in the hospital of about a week.  We are hoping that this is not need and he is hopeful that it won't either.  

So this was a super long post.  I am going to sign off and head to bed.