Tuesday, July 15, 2014

TPL Shunt revision (#30-s/t), shorten pleural catheter

Although not bad news at all I really am forcing myself to update this, for whatever reason today I have little energy and typing this update out feels daunting!
I originally wrote out notes yesterday but then must have accidentally thrown that sheet out so am trying to remember details by memory on about 2 hrs of sleep (an hour-ish early this morning and an hour-ish around 12-1 or so. Neredless to say right about not I am feeling INCREDIBLY grouchy and irriated and just want peace with quiet with no one coming in here!
(One of the Pastors from the Hospital stopped by and honestly I asked him if he could come back in a little while, I just needed to be left alone to finish this and something else I was doing + am not really feeling like making small talk. Sometimes I just get soooo tired that I'd just like to be left alone!

Surgery which was scheduled at 1pm yesterday (arrival for labs at 9, check-in to 1st day surgery at 11) started about 1:30 (Dr.Bragg was in clinic and when she came by said she'd had an un-scheduled patient show up, not a big deal).
Really the Head Anesthesiologist and 2 Resident Anesthesiologists working w/her on my case had been standing around just chatting with my Dad and I while we waited.
I liked the whole team and could tell the head Anesthesiologist had done her homework on my past history + was well versed in the cardiac issues and what she would do differently. Her big concerns where to not cause fluid/cardiac overload and keep surgery (which was very short, intubation took much longer than the actual surgery) smooth.

Overall they did the pre-surgery cortisol replacement (there was discussion by the Anesthesia Team if it was really needed given the short length of the actual surgery but per what my Neuro-Endocrine dr has stressed to me numerous times the cortisol is as much for the induction/anesthetic drugs as it is for surgery. Cortisol deficiency with any stress of the body causes stress to the body systems and is why the cortisol has to be given 30-60mins prior to any anesthetic meds are given when possible.
It worked out fine as the Head dr said to me, along the lines of "You've been around the block w these surgeries and you know what your dr has told you regarding your case".

Surgery itself literally took about 15-20mins and if I remember correctly when they where hooking everything up, adding the anesthetic/induction meds it was about 2 hrs after this that I woke up in recovery. I am not exactly sure on any of the total time length though and doesn't much matter given surgery went good!
The Peds General Surgeon w/Dr.Bragg there basically just for "moral support for the Surgeons" (her words, lol) =) just used an endoscopic approach w 2 small incisions in order to view and to move their instruments.
They pulled out the pleural space (tip) of the TPL Shunt catheter, cut off about 1.5-2" and replaced it back in to the lung, which I believe they deflated for this surgery (a big concern the Anesthesiologist had given my airway issues and instruments she had to use).
Apparently per the Team they could visualize where the catheter was in fact sitting up against the chest wall and could see were it was causing irritation, which in turn explains my symptoms of irritation most especially when it seemed like the TPL Shunt would be trying to drain.
With the re-positioning of the catheter Dr.Bragg said it is short enough now where it should not touch any areas in the pleural space and as an added benefit we should get less resistance from the catheter which equals (we hope) more CSF flow through the catheter. That would be a good thing as we know I like to drain a lot and feel better the lower pressure is!
Thankfully the TPL catheter pinching/poking feeling has been pretty non-existent today and any discomfort there is either incisional or b/c Dr.Bragg said even with their draining excess fluid out of the pleural space during surgery there was still some that would absorb (they hope) on it's own.
Other than this positional catheter issue we've definitely had much better luck w the TPL Shunt than we have with VP Shunt functioning fully or well.

Even though the VP works less than optimally and I do have headaches/nausea/some blurred vision (intermittent) it as I've said before is still wayyyy better than when I only had 1 shunt and was endless problems.
I am sure it is at least somewhat improved now b/c the TPL Shunt takes some pressure off of the VP Shunt and they almost seem to balance each other with the TPL Shunt seeming to work more effectively and better, longer.

I feel like if we could figure out what causes the TPL Shunt to work more effectively than the VP Shunt and we could figure out a way to translate that effect to the VP Shunt we'd honestly be so much closer or perhaps as close to perfect as you could get with pressure symptoms.
Our biggest issue is Dr.Bragg is just unsure how to better improve the efficacy of the VP Shunt draining especially when I am asleep. I do sleep semi-propped up on my couch, always starting out sitting up but I think due to positioning/joints/neck I end up only partially upright.
I would LOVE it if we could figure out how to better make the VP Shunt work but hopefully we could keep brainstorming and figure something out in time? In the mean time you just do the best you can, someday's better than others with symptoms.

I will say as 'minor' as this surgery was ie very short and just 2 smaller incisions on my side and back even these smaller surgeries still kicks my butt both discomfort/pain wise and overall! =/
We are in the process of slowly weaning the PCA pump (patient controlled analgesic, I control the short acting pain med dosing based around how often Neurosurgery has it set that I can deliver med.) I am sure the difficulty with surgery recoveries in the first few days pain wise is due to so much scar tissue and so much GAG storage (from the MPS storage) which causes much tougher tissue.
Thankfully Dr.Bragg has a decent system in place for managing post-op pain w the PCA (self administered) pain pump. And she realizes for whatever reason my recoveries are just not a typical (whatever that is!) shunt patients recoveries.

I do know given we had turned up the VP Shunt setting by a notch a few wks ago it's possible if need be Dr.Bragg might also let us re-adjust that setting back down if H/A's don't improve.

Friday once I knew surgery would go ahead I called the Neurosurgery floor Pharmacists and they in-turn ordered the Aldurazyme to be given while here.
My Genzyme Insur. Nurse Case Man. was also watching for the order to make sure it go shipped out on Fri from their end.
This will likely be given on Weds morning now to give us time to fully wean off the PCA pump.
We'll also use the same ramp up rate this time that Stan (Nurse here) calculated last time. This is different than how it is ramped up at CHW, my normal clinic but something is done differently here maybe with how the drug is mixed as we otherwise where running the infusion at double the needed rate, giving it to quickly.
We'll also restart the Coumadin dosing tonight I believe and once released i'll go back to monitoring this via my Cardiology Team at CHW.

Due to my being in-patient the WI Team from Genzyme came up here to visit this morning which was actually really nice!
I'd only met Karen (Patient Care Liaison) before as she lives in the Minneapolis area and we frequently have dinner while I am there from time to time and see each other here and there at meetings.
Amy, my Insur. Case Man. at Genzyme (located Boston) as well as Carol, who is the WI Drug Rep for Genzyme (lives in Madison actually, a little bit newer to the team) where with Karen also and we had many good laughs, funny stories to share. We're all cat lovers so that was like an instant bond between Carol and I especially!
They all really liked the MPS-Aldurazyme bracelet (using the caps from the drug) bracelet I have/made and so my next project is to make one of each of these for them. I told them it would take atleast a few wks but I think it's just cool they liked the bracelet that much! =)
It's amazing the people in the Med world who really like this idea and it is neat b/c 1. they are intimately involved in MPS I pts lives everyday and 2. their company bottles the drug.
Jessi, my Boston Marathon runner also has one of these and had sent a pic of herself wearing it after she got it in the mail. =)

Going to sign off for now but if I've forgotten anything i'll re-update this and otherwise will update sometime again soon.
Hopefully I get out tomorrow or any day now; even if we still are dealing with headaches it's just nicer to be at home at a certain point!
Here are a couple pics from today with Amy, Karen and Carol this morning.

Thanks for stopping by,

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