Neurosurgery revision update: 12th or 15th (since May) and 6th or 7th for VP shunt (since Oct)

Hi All, Just updating for yesterdays surgery. I drove while  my Dad came along (I like driving I guess bc I drive so much the rest of the weekI otherwse dont know what to do and even if it's jsut 45mins get bored just sitting there! Dad will drive when I get dsharged. Pre-op went well and suprisingly on-time for the surgery being at 2:00. Dr.Bragg had other surgeries at her Peds hospital next door and mus have finished in good time as she was over by 1:30. I had 1 of the same anesthesiologists ive had in the past (not surprising I usppose given how many revisions/shunt surgeries we've had.) - this was one of the anesthesiologists that did realy well and I liked so that was good. I like the ones that have no problem with letting me stay completely awake right up until they are ready to intubate and start surgery. (versus given midazolam in pre-op before heading over to surgery). They as per usual used the fiberoptic scope due to airway issues and anesthesiologist was really happy afterward when he stopped by. I had my Port accessed since this past tues from my infusion nurse and so we used that initialy heading in to surgery and then anesthesia placed a 2nd peripheral IV once I was asleep. (my neurosurgeon is such a god send in this respect as when any anesthesia folk give a hard time about starting a 2nd peripheral IV she tells them to do it after I am asleep. She has walked in, in the past when I was being accessed and even the access team took 4 or 5times before they went to using the Port so since then she has been a champion for me for using the Port. Both the Port and peripheral IV where used im sure for the clindamycin and cortisol stress dose (solucortef) and then for the anesthesia drugs and both where running when I woke up. After about a 30min period anesthesia gave the ok to remove the peripheral IV. All I have now running is fluids and the colucortef by IV tapering every 8 hours.

To borrow my neurosurgeons analogies (she always apologies for using these but in all reality they make it so easy to explain what she finds and what causes the problem! Im unsure where she comes up with these but they are actually kind of funny to. So when she got in to the top incision she initially apparently didnt see anything material wise (brain material from choroid plexus and another area that frequently apparently sheds) but then when she looked further she said the valve and catheter where more than 3/4's occluded (in a 3 weeks period - yikes) and then using the endocscope she saw another area further down that had material scattered further around. Her way of explaining this was much like a toilet; in my case with the fixed pressure valve I essentialy was like a flushing toilet - you flush and all the water is dumped and then you are unable to flush the toilet again until it completely refills. Instead she wants the shunt to work the opposite like a broken, running toilet where the CSF continually is draining so that there isnt the build-up, drain, collpase, build-up, drain, collapse which causes the ventricles as described to collapse in on the shunt (she thinks because my ventricles do not expand normally like many non-MPS hydro pts I may already have the ventricle and shunt catheter resting on each other which isnt what they'd normally like.)  Because of this on-going collapsing it acts like a straw and wet bread where if you where to suck through a straw and apply that back force on the wet bread it would cause dimples in the bread and small pieces to break away and clog the straw. The shunt essentialy in other words acts like a straw.  With this new programmable valve they have 5 settings they can choose from and this is done by a small device resting on the shunt valve (on the head) and a 2nd device checks the setting and this then is removed and a 3rd device - the magnet acts to reset the dial on the valve. The valve is longer and a bit taller so there has been more discomfort with this incision and surgery with many movements but my headaches seem to be doing well and I havent had much nausea and no needed zofran. I am taking oral and IV pain med which helps the discomfort and Dr.Bragg thought i'd be able to get out tomorrow.  Right now she has the shunt set at 2 which is the resistance setting and i the best setting for preventing the dump, drain, collapse problem. If needed we can adjust the shunt down to 1.5 or lower though and will just monitor to see how it does.    Being the so down to earth and definite Peds dr she is instead of stapling telfa to my head over the stitches (1 big incision and 1 smaller incision at the end of the valve) and  she said they combed out my hair at the beginning of surgery and then when it was finished she tied that side of my hair (see below) back in a ribbon to keep the telfa dressing in place. - Very cute and I might always do this just because at home as I do think it is cute. She said she thought the Adult hospital OR staff probably thinks she's silly for the care she takes with peoples hair but I completely appreciate that she doesnt shave all of the hair on that side of head and then tries to make it look cute to boot! :)

I am still on Isolation pre-cautions which means Dr.Bragg and the nurses have to gown up before they come in to my room and for pre-op everyone had to as well as in surgery before they scrubbed. She is going to try to get this removed through Infectious Disease who placed it on my file after the injfectious UTI bug came back after the last surgery (XDR bug - antibiotic resistant). Since ive finished the course of both antibiotics (initially cipro then nitrofurontoin)  she thinks we'll probably just have to get 1 or 2 more clean cultures. My PCP's office should have faxed over their result but Dr.Bragg hadnt seen it so was going to look for that. It isnt a big deal but I tihnk everyone would love to have the isolation measures taken down! I wouldnt mind either. :)


                                                            Dr.Bragg's post-op ribbon

                                               My silly Dad taking pics in pre-op

Because I now have the programmable valve Dr. Kakkis (dr who created aldurazyme) and DrDickson really think I should consider the IT Study as they think we could use the VP shunt for the injections (given my lumbar laminectomy/nerve root surgery and nerve injury Dr.D wasnt sure it would be an option to do it via LP). Because I have a reservoir directly within the shunt they feel the programmable valve could probably be turned off for a few hrs for the IT injection (using aldurazyme) and then re-programmed after a couple hrs to the original setting. The IT injection would be given via the reservoir. Dr.Dickson didnt say but I assume maybe she would have to get IRB approval for this as when I did the decompression initial phase IT study 3+ years ago we had to do the injections at the base of my neck vs giving via an LP and she got approval for that. Im unsure if the study would be done at her hospital or at Oakland. I need to gather much more info and weight it against my other on-going medical MPS issues but their thought it maybe tbat could aid in preventing these on-going occlusions or help minimize the occlusios as they feel it is happening so much because MPS storage is likely causing the choroid plexus and ependymal tissue from the top of the brain to be thicker and thus harder to get through the shunt without clogging. A big downfall for me would be 1. flying every month for 4 1/2 hrs (lay overs help but still a long time) and I need to talk to Dr.Bragg about this and get her feeings. Since she is acting as both my neurosurgeon and my neurologist essentially I feel it wouldnt be right to make a decision like this without talking to her and getting her opinion. She has atleast a little MPS experence so I would trust whatever she said. We will see - at this point I am not leaning yes and I am not leaning no, I am just squarely indifferent I guess till I talk to Dr.Bragg and weight options. In the mean time since this would take some time and there isnt even a guarantee id get in to the study (or could be randomized for first year to placebo which is essentially 'wait and see' but you still have to go every month for other testing. I also would need to find out if id still be eligible for Paige Kaplan's  chaperone drug study (ataluran for use in people with atleast one non-sense mutation, they expect this may be in trials by the end of the year and may cross BBB and is an oral medication.)  Ive spoken with Elsa Shapiro at UofMn about this and her feeling was if we could do it she also thought I should really consider giving the IT another try for teh shunt occlusins specificaly.  So we will see.

Thanks for all the kind messages and prayers on my fb page yesterday - this means alot and I know means jsut as much to my Mom.

Thanks - go in peace and may you be blessed with good health but if not then appreciate the little things and find light where sometimes it might seem light doesnt shine.

Erica