Rarely Defined: Pre-op Appt (check), checking with Cardiology for 'ok'..

One more step checked off towards having the VP Shunt re-replaced Nov 12th. Today was the pre-op Appt with my Primary dr. She has cleared all aspects other than she wants to talk to my Cardiology Team and run a couple things by them make sure they are ok to sign off and do not want to see me before hand or have any concerns with symptoms. To use Dr.Simpson's words indirectly "I'll clear you for surgery if your Cardiology Team will but if they have any hesitations we'll have to put the shunt replacement on the backburner till these heart issues are sorted out". Yay?
I said to Dr.Simpson re this that I hope my Team will either say we can go ahead with surgery and keep the f/up appts I already have scheduled in the wks after surgery (both w/Dr.Kovach and with Dr.Earing + 6mo Echo) or if anything, if they have any concerns maybe request closer monitoring by Anesthesia but give us the go ahead for surgery. I am hoping we can get this surgery done, it seems like it's the 'surgery that's never gonna happen in some ways'!?
I swear if this surgery gets cancelled I am going to lose my mind!? I CANNOT even explain how bad I have felt lately, during nights/mornings to mid-afternoon and then I finally start to feel some better. - It is both shunt/headache pressure and heart symptoms BUT I absolutely know from a headache standpt we're doing the right thing putting a 2nd shunt back in. Mornings, nights and some parts of the day are beyond ridiculously aweful - sure I keep functioning but literally I need that 2nd shunt to take some of the pressure of the LP Shunt, my abdomen, very low back and neck are so uncomfortable (in 3 different ways). I'm gonna for real cry if this surgery where to be rescheduled!!!!
I do see Dr.Bragg on Thurs so will talk to her about all of this, it is really, really bad often lately so if you believe in it say a prayer! I want to say my Cardiology Team will clear me but who even knows, I don't know why they wouldn't b/c I think they realize it's not as if we're just doing this surgery for the sake of doing surgery but in fact to help symptoms overall.
Between the heart and shunt stuff Dr.Simpson and I where talking today, she (as do all of my Providers besides Dr.Bragg) was asking about the recent shunt surgeries we'd done, trying to sort what shunt we'd just did revisions on. Honestly the conversation was kind of funny, I think we where both laughing by the end and my teasing her (PCP) a little, she couldn't keep it all straight (teasing)? ;)
I don't fault Dr.Simpson or my Providers at all but today she was asking me about what was done in the 1st of those 2 last shunt revisions (about a month ago, a couple days apart) and what was done in the 2nd revision + what is it exactly we are doing in this next surgery.
Honestly I have to laugh a little b/c who could keep all these shunt revision/surgeries straight much less the overall issues? I am not sure there is any one of my Specialists/drs who can really keep straight what shunt I have vs what I've had in the past and what each of these surgeries are we do. =) It is a lot for sure!
My Primary dr. does a really great job managing everything and though she's still relatively new to my Team (3yrs I think) and had never heard of MPS prior to that (she told me as much) but she's learned a lot, really tried to work with my Providers and to stay on top of what is recommended for testing, even testing that others of my Team (Genetics) usually does as is the case now. She also never hesitates if she thinks it's needed to reach out to any 1 of my Providers such as Cardiology this time and last month she contacted my Genetics dr. in Boston to get better details re the gene mapping study.
She ordered a chest Xray today, is calling my Cardiology Team and is going to try to order the urine/blood test that is done for MPS. (all came back normal, we're sorting out how to go about ordering the urine/blood MPS test as it's pretty specialized and just a few labs our E. that run the testing.
We where talking about her caring for me and learning about my MPS, about how ideally a Patient with a complex diagnosis like my MPS would be followed by a Provider in a newer specialty like Med/Peds. This is what Dr.Earing / Adult Congenital Cardiologists train in before doing Cardiology residency/fellowship as these Providers train in both Pediatric Medicine and in Adult-Internal Med. so would see Patients from Pediatrics on through Adult.
In any case my Primary dr. now is Internal Medicine trained and has been really good for my Team, she may not know everything about MPS but she learns, reads and talks to my Providers and she isn't afraid to make suggestions where she thinks something might be better.
As we where talking about everything going on I made the comment to her 'wouldn't dull and boring just be good' (her MA comments pretty much every time I am there how frequent they see me for pre-ops and as she's leaving each time she'll say 'I hope this is the time we don't see you for a surgery visit for a good long time'. Funny, funny sometimes these visits. We definitely laugh a little at the insanity of it all.
After I made the 'dull and boring' comment, Dr.Simpson said to me something like 'In healthcare that is often the case but that just wouldn't be you". LOL, sadly so true?
As far as knowing if surgery is a go from Dr.Simpson and Dr.Earing/Dr.Kovach's perspective Dr.Simpson was going to reach out to them and thought she'd know by Fri. I am praying for a 'YES, She can go ahead' (with surgery)!
Dr.Simpson said something along the lines 'I know your not going to be excited if the surgery is postponed (an slight understatement, I don't want any surgery but I do want these headaches/symptoms better controlled) but technically your heart comes before anything else'. I think she also know I wouldn't be wanting this surgery or going for it though if we didn't think it would really help so I think she'll do her best to get surgery approved.
Sometimes I think it is just it sucks how tied in each issue is to 1 another and for that matter I commented to her these days, sometimes I feel like we'd be better off just to do the 3rd OHS and manage the risks, get it over with so I'd feel atleast some better on the heart front!? Ugh!! As I've written about before my Cardiology Team has tossed around doing the 3rd OHS at just about every visit so it's something we've all considered and just a matter of when is the right (best/better?) time I guess.

I think in some ways it's just hit me the past few wks how truly terrible I feel many days, it kind of stinks actually. My Mom, Sister, nephew and I went bumming this past Sat and the 1 before that which is something I'd have normally looked fwd to but instead I sort of just 1/2 dreaded it (it was fun, I just stay in the car or wait at the front of stores when done) b/c even little amounts of going from 1 store to the next or garage saling flat our wears me out which I might add stinks!.
On a fun note Zan spent the past 2 Sat nights, he's easy enough so that was fun. =)
Often 1/4-1/2 way through anything we do I end up staying in the van which Zan often then will chose to hang out w me. I don't mind just waiting but I truly look fwd to the day, perhaps post a 3rd Heart surgery as kind of daunting as that tends to seem (b/c of the unknown, knowing it's never been done before in an MPS pt. 3x's) I hope I get more energy again after!
These days some days are definitely better than others and maybe it will be a little better after we put the 2nd shunt back in.

I have 2 more appts set up w Dr.Bragg, for her to take off fluid before the surgery the 12th as long as it goes ahead. The 1st is this Thurs, the 29th and Mon the 2nd I believe which is this Thurs and next Mon, (ERT switched to Tues). Otherwise I did the Stealth CT Scan last Tues, 20th which is what she uses to place the ventricular (brain) portion of the VP Shunt catheter.
As long as no issues come up with the surgery i'll test INR per usual a day or so before the surgery. Given surgery is now on a Thurs, I may have to make a separate trip to Milw. but have to think about this some.
Lastly I did the Holter (Zio) monitor last wk for just under 2 days which turned out to be a tad on the interesting side as I kept bleeding under the monitor on/off and making a huge mess especially at night. My Team will have the results in 1.5wks or so.
Un-related to surgery I talked to the Genetics Counselor-Research person at my Genetics drs. ofc. in Boston and they mailed out the consent form for the gene mapping study. This in turn she said can take a while to get the test results, up to 6mo once I sign and mail back the consent form and complete the actual test which they use blood or saliva.
This literally maps the entire body-looking at any and all abnormalities in all genes and chromosomes both to see if there is any other underlying, un-diagnosed issue to explain the (especially) unusual heart issues my Team has dealt with.
Per the UofMn drs and my Genetics dr., both of whom see a lot of MPS pts they have never seen the issues apparently my Team dealt w in surgery and the issues we deal with now/the unusual progression I guess.
In addition to the moderate-Hurler Scheie like mutations but very mild outer features. My Cardiology Team here and Genetics Team in Boston wonder if a separate/secondary issue could potential explain the issues we have/or help them when we do go forth w the 3rd OHS planning.
In any case will update here when I know anything,
Thanks for stopping by,
Erica Pre-op, pre-surgery check list