Saturday, September 10, 2016

#40, Shunt revision.. relief at last..

Post op, #40
Sorry it's a few days our from surgery and I've not updated... I did get out after just 2 days as the surgery itself was pretty minimal (changing out part of the catheter that was abnormally scarred to an area) and I didn't have a lot of pain. If anything I've had far more discomfort since getting home but not actual incision pain.
For whatever reason despite the headaches being a lot better (not perfect, not as good as say when entire new shunt system is put  in but the headaches are better) - BUT side pain, HOLY SHIITTEEE, it's like it won't let up , hurts something fierce when I cough, or stand up or walk or try to sleep on that side (which stinks actually b/c my hips do not take well to being laid on all night on just 1 side. EEkk!!! I try to hold my side when I cough which helps a little but not enough.
My stomach is swollen.. It's like, seriously body, enough already... Couldn't we just enjoy a little time with something feeling some better!?!?!?! Is enough to drive the Pope crazy!
I've only ever experience it like this one other time and I absolutely pray this time is not like that time.. I may cry if it is!
I guess if it's still like this on Monday, basically unrelenting i'll call, though who exactly I'm not to sure. Seems likely shunt related but I really don't know given swollen stomach and headaches aren't bad... 

Why it matters to mean to someone... why it matters that Providers care, are willing to try (and listen) and work together..
I've thought a lot about this very thing this past week. I was pretttyyyyy nervous going in to Tues surgery, while I knew there was something wrong I didn't know what and I didn't know if it would be found easily. Thankfully it was or seemed to be but still with Dr.Bragg I knew what to expect and I just didn't this time.
Post op blood thinner mngmt was a bit of a mess. Despite Cardiology Team having sent over their specific recommendations that wasn't followed and the wrong (prior) recommendations where going to be implemented. After 2 days essentially of this I talked to Dr.Iskandar and asked if he would himself reach out to my Cardiology Team and talk to them himself + sort out a future surgery plan. He was agreeable to this and actually called them the same day + he sent me the recommendations typed up andasked for my opinion by Sat. morning.
After this he revised/edited a bit and was planning to send to the rest of my Core Providers and  run it past Cardiology Team before having it entered in my Med record. I am thankful he asked my opinion and listened when I asked him to reach out to the Team!  Seems positive initial steps...

Do I think we'll still have times we don't see eye to eye? Probably bc we're 2 distinctly different personalities with different views on my MPS and mngmt BUT if we both try and both listen to each other then I think this may work... I am cautiously hopeful....

Related but unrelated I will say I've always been curious with the manometer 'test' (what they use to test the shunt function in surgery)  if they are holding this in a vertical position wouldn't gravity cause the spinal fluid (CSF) to drain better even if say there where a partial issue further down? Especially if say that issue where at the distal (bottom) portion of a shunt?
I suppose another reason I often wonder this (not really related to this surgery but just in general curious question/thought) is the fall after I started seeing Dr.Bragg we where having 1 shunt issue after another so she (Dr.Bragg) had 1 of their Peds General Surgeons help her on a surgery of mine where she/they went directly to the distal (end) of the then LP Shunt and despite it looking like it flowed with the manometer test when they had a direct view they could see where the shunt catheter  was stuck/scarred to intestine or something in that area and likely having an affect on drainage without it being obvious.

Like I said I think we found the issue in this revision but makes ya wonder about that portion of shunts in a general way.

It's not about replacing..
As any rare disease family and especially those of us with ultra orphan (very) rare disorders know it's important our docs talk, are familiar with our whole history and they have the 'Team player' ability (vs I can figure this out on my own, I don't need to know what that Pts other Providers feel/think).
On fb someone commented on a post I wrote about getting discharged today and saying they felt the whole situation sounded more hopeful and where praying things do stay good between us, in working together.
I after thinking about this some commented back that while Dr.Iskandar is never going to be Dr.Bragg bc she just has a uniquely caring personality he and I will find our own normal if we keep working at it and each can adapt a little to the other (that sounds weird to say perhaps).
Really it's not even about replacing her (Dr.Bragg)   b/c she's still   a part of my Team and well some people (Providers in this case) just aren't meant to be replaced...

For him and I it's just finding what works for the 2 of us, to make our Provider/Patient relationship a good one where I trust him and he trusts me..  and we con't to make decisions together. Sharing with each other related to the issue at hand (for instance he gave me a few things to think about and ponder and decide if I was willing to try them and as I mentioned I asked of him a few things (such as talking to Cardiology) and I thanked him for con'ting to work with Dr.Bragg bc I know she knows my history inside out and she can help him and I as we look at possible options.

He did use a dressing over the incision which has been annoying and which I still wash my hair but thankfully quite a few of the staples seem to have fallen out, yay!  Otherwise I get the rest removed next Thurs. I LOVED that Dr.Bragg used no dressings the last yr or so after VP Shunt surgeries! =) Not the worse issue just more of an annoyance really. =)
   AZ Bound....
On a side note I am going out to AZ to a Hydro mtng in early Oct. - I have friends out there and Dr.Bragg is speaking at the mtng so though why not. All arragements are made so am kind of looking fwd to this. =)
Will update sometimes soon. Say a prayer this side pain either resolves on it's own or is something SIMPLE!!


                                       I chose this pic because I had prayed so often leading up to surgery that if it be God's will an answer could be found and atleast some relief  be given. I do think energy wise it may (I hope!!) help once we get the heart (rate) stuff under control but I so needed atleast some relief of these headaches to. Not perfect but better than it was!
                              Just have been thinking a lot this wk about 'Zebra docs' and how I am

                                           blessed to    have so many on my Team and a new 1 that I can wear in.

Monday, September 5, 2016

Tues (9/6) Shunt Surgery (3pm surgery)- getting things in place, new but not new Team

   Sorry I wrote this last wk but forgot to come back and publish it..
Tues surgery is scheduled for 3pm with arrival at 1pm.  If lucky surgery will be on time?
I am bummed to say the least about this - mostly just b/c the ridiculous 'no eating after midnight' no matter that actual surgery isn't until 3:00 (1:00PM arrival) which if it runs as afternoon cases most generally do at UW means 3pm will likely be several hrs later. I hope not but realistically this is more often the case. I NEVER understand why if surgery is at 7am 1 can eat till midnight but if surgery is at 3pm one is still supposed to stop eating at midningt? I'm just venting, please don't take this as my being pissed off, I really just need to vent. There is just a lot that frustrates me and writing is my outlet.
People say 'well if you get called in earlier' but the thing is I'm not in-pt so wouldn't be at UW anyways... ARGGHHH!!!! Hard candy it will be.

It's definitely been a lot of sorting and coordinating this wk for this surgery to make sure Neurosurgery had what they needed from Cardiology (Fri must have talked to/emailed with or left voice mails for my Cardiology Team and the Neurosurgery Nurses no less than 4 or so times each)! - Was crazy (!!) though I also have to say each office and the NPs I was working with where AWESOME about helping to make sure each side (Cardiology/Neurosurgery) had what they needed for Tues surgery. For that I am grateful and really appreciate both Team care and for that matter each NPs that I talked to would ask how things where going (not necessary but nice).

Definitely seemed ALOT messier this time then most other recent surgeries but by the end of today, I think (hope, lol) everything is in place.. There are a few things I wonder about but can only pray, right?... I think it will be a very, very, VERY long time till I will stop wishing Dr.Bragg where just here and am sure next wk, in pre-op, in the OR (especially as being sedated/anesthesia) and then post op in-pt i'll wish she where there bc she listened, she knew and she made things run smooth. I'm sure some think 'she's not coming back so get over it' which fair enough if they do but as any rare disease Pt who handles their own care and who has a good Team knows when we have a good Provider(s) ir is genuinely hard to lose them!
 She just knew the little things she did to make various people (Residents?) back off, or not mess with things (Cardiology meds? Stress dose steroid for surgeries?)  or make sure post op was managed well pain wise (often more about joints than is about actual incisional stuff or as much about body as it is about incisional stuff from positioning and my body just not doing to many positions to well).

I can't change it but sure doesn't mean I wont miss having her who just knew so much about making things go well AND she listened + didn't try to pretend she knew what she didn't.
Non-the-less, everything happens for a reason (I guess) and I guess I just have to trust? Is hard but trying. Sometimes easier than others.

Phoenix -
- On a side note, arrangements are made to fly out to Phoenix next mo (Oct) for 3 days (Fri-Sun) (fly in Fri, mtng Sat., fly home Sun afternoon). Not exactly the best timing with Sun School just starting and potentially missing the 1st day (hoping not) next Sun, 2 wks later we're supposed to go camping (not entirely my favorite thing, lol) and then this in early Oct. I do have the person who subs for me lined up for the late Sept and Oct dates. Playing it by ear for next wk and just hoping i'll be there! I have my lesson written up (worked on it at ERT last wk, to get it done ahead of time). So we'll see?

Anesthesia - Airway -
As far as anesthesia (airway) goes (and fully hoping it IS someone familiar with my airway!) i'll Print the Anesthesia information from Dr.Taylor from this past Heart procedure (Heart Cath / Heart Ablation) to give to the Anesthesia dr. assigned to my case. At UW thankfully it is usually someone assigned that has had me before but since she (Dr.Taylor) had increased difficulty this last time with airway (she sent what she found, and what equipment she specifically ended up using which i'll share with the Team next wk.)  I figure it makes sense to share that information with the Team that will have me, to try to make it perhaps easier for them and perhaps they can use the same things Dr.Taylor did to make intubation/airway easier. Hopefully it's someone that is amenable to just following another drs. suggestions! Most of the Anesthsia drs that have been on my Team at UW have been pretty good about this sort of thing (following what's worked easiest in the past).

  Cardiology - Blood Thinners / Surgery Plans
As far as pre/post surgery and what Cardiology told my Primary dr., the plan is to stop the Lovenox (Enoxperin) injections the night before surgery (tonight) and skip the morning dose then either this injection is to be restarted Tues night or the Neurosurgery Team will have to start a heparin drip until they are comfortable restarting the injections.
This is the case bc we have the mitral valve and aortic valves now so with mitral valve requires more careful monitoring and (if I remember right) due to higher blood flow blood has to be thinner so thus no window like there could be when I had just the aortic valve. Before we could be off the Coumadin for upwards of (I believe it was, right off hand) 3 days before and up to 5days after).
The Neurosurgery Team has these instructions and when the NP messaged me the other day to ask about getting INR checked the day before surgery (I'm guessing she hadn't yet seen these instructions/plan) I let her know this info as well so that part should be set. Lol, I am not a worrying type but have to admit I think I am going to worry about this blood thinner-post surgery aspect till it is set in stone the day of surgery and I am probably more stressing about Port access and what to do with this.
  Aldurazyme (ERT Infusion) In-Pt.
I talked to the Neurosurgery NP (she's kind of amazing, she's already talked to the Neurosurgery (D6-4) Pharmacy and gotten the Aldurazyme ordered for next wk + that was shipped earlier last wk per my my Case Manager at Genzyme. When the floor Pharmacists called Fri they confirmed they had the Aldurazyme at UW.

 Port Access - Access Team (Timing w/Holiday wknd)
The thing I am stressing a bit about is this same NP talked to the Access Team at UW who told her they can be paged the morning of surgery to come down and access the Port but they can't set up a time ahead of time. This simply (hopefully simple!) means as soon as I got to UW and checked in I'd have to have my pre-op Nurse page the Access Team to come down which would be fine in and of itself and then I'd just have to hope they could come down before and access it (the Port) atleast an hour before the actual surgery as the steroid has to be given in a specific time frame.

I talked to my Primary dr and she's going to put in an order with the Home Health Company so in case of future surgeries where it falls on a weird day I can just get the flush and heparin for the Port from home health but this time I am just going to have the Port accessed the day of surgery. Makes me a tad nervous but hoping it works out ok!?!

Stress dose Steroids:
 In any case these are the stress dose steroid instructions, I am sharing here as much so I have a simple place to access them (i'll print a copy to but in case I don't have that on hand) to look and comfirm what post op dosing is. I admittedly even after all these yrs still have to look and see the dosing schedule when there are questions. That's a little bit sad actually, probably given in just the past 5 yrs I've been through more than 45 surgeries and tmrw's surgery is #40 (overall) for the shunts!

In any case will update sometime this wk after Tues surgery. If you believe in prayer please pray there is an easy answer and an easy fix with the VP Shunt!
 Thanks for stopping by,


PS This is completely (sort of) unrelated but I feel like people judge a person for trying to stay busy and sharing what they are experiencing and almost especially for not whining constantly about what they are feeling. Why is this? Bc I share what I'm feeling but not every other word is a bitch about what isn't being done or whose not doing enough or how bad I'm feeling (I feel like I vent enough but I also try to apologize for doing this very thing, I don't want to be insufferable?!?) Yet I feel like at times people expect bc 1 goes about their life, trying to stay busy, being involved in things that 'she must not feel that bad' and why is that? How can we possibly judge what another person feels? Why do we care what another person shares? In reality shouldn't/isn't it about trying to help each other?                                          

Stress Dose Steroid Scheduling:
Due to patient's adrenal insufficiency, stress dose steroids will be required for her surgical procedure.

Day of surgery ---Hydrocortisone 100 mg IV Q 8 hours. The first dose should be one hour prior to the surgery.
If hemodynamically stable then:
POD #1---Hydrocortisone 50 mg IV BID or 50 mg PO BID
POD #2---Hydrocortisone 30 mg PO BID
POD #3---Hydrocortisone---20 mg PO in AM and 10 mg PO in PM
POD #4---Hydrocortisone---resume regular daily dosing of 10 mg in the AM and 5 mg in the PM.