Wednesday, January 27, 2016

3rd Heart surgery, 1 week post op! Went so well!!

"We all went in to this a little worried but you've exceeded expectations" - Best words ever from a Provider!

I am 1 wk post the 3rd OHS and potentially going home today!  (updating thos I got out!!! Labs Mon., fup in 10days as long as all stays good!!
My entire team has said how good this went and better than most expected!  It pays to be stubborn! I seriously,  truly think 'stubborn, going to prove them wrong' has helped me do so well in all these surgeries!
I can't tell you how many Anesthesia, drs, Techs and Nurses have said to me "how many surgeries have you had now??" (both asking heart surgeries and surgeries overall)  and then comment about my functioning so well. =)
I think my being showered, hair washed twice and in regular clothes amazes some of them but that's  a good thing, I am glad I did so good so far!!
I have showered, dressed in my own clothes, functioning despite the fractured big toe and I rarely complain. Even the toe, I didn't say much about it in last wk and Nurse only asked Cardiology Team for a Xray bc so swollen and black and blue a wk out. - Dr.Earing (and so many others, lol, they apparently don't know my clumsiness) said to me (indirect quote) 'Only you Erica would do something like that before a big surgery.  - takes talent to be me!? I have a walking boot I'm supposed to wear and fun w Orthopedic Dr at FMLH  in 10days. Why be dull? Thankfully other than an incessant feel like I need to flex that toe it's not to painful. =)

- Asked if I needed addtl pain meds, the  NP was amazed it seems that I declined, =) Aamazed I'm happy to just go home on what I take regularly! (Fairly low doses). :)

- I really, really can't  say enough how important it is to trust ones Team, have Faith, walk with God and stay positive even when ya might not feel like it! - To quote the NP indirectly "You have way to much perspective " - :)  Perhaps why I've gotten that perspective is stinky but overall  I'll admit I am glad my Team sees these things vs honestly what I feel like is whining sometimes!  Besides who wants to make someone else miserable and thus they (Providers in this case) dread seeing you? - I like to be liked =)

I feel like there needs to be a way to raise awareness that as pts with a really good Team we can do great and surgery - even a high risk, risky surgery especially  repeat can be done. I'll say it a hundred times, good Teams matter!
So worth it to feel better which hopefully keeps coming! Pain wise isn't bad at all, 5 at most but honestly the past few days (minus headaches) are 1-2!
The worst symptoms where extreme itchiness till I could shower finally Weds and the awesome headaches which had been soooo much better after we put the VPL Shunt in but that to perhaps just fluid around and body in general affecting their function!? Hope so!!
My getting to go home today depends on CXR, if it's clear I guess due to cough but ooh soooo close!!!
Lol, my Cardiologist said to me (after they did rounds ) where talking about how up and down INR will likely cont to be for awhile.  Dr.E says to me (maybe tongue in cheek) but like anyone we always hope for no surgeries for awhile. He says (indirect quote) " this is it for surgeries, right?' LOL, I never promise anything  and said as much to him or anytime I am asked that ques.when anyone says that as I expect nothing and thus lose nothing if it does occur.
That said - INR range is 2.5-3.5 now  but when I asked the Team if say INR where low would anything  change w what we do now? They feel if INR is close to 2.5-3.5 they'd  just adjust dose.
If it where below 2.0 they may bridge but not certain yet. My overall picture which is complicated  makes decisions they make sometimes not as clear cut as would be in most people. They'll figure it out though!
I'll have to bridge w Heparin or Lovenox before/after any surgeries or procedures requiring I go off Coumadin but that to doesn't seem bad. Their main comment was to the effect, given my underlying issues and how Rollercoaster INR was before this 3rd OHS (again not direct quote) 'we'll likely have some adjusting to do over the next wks/mo's' but that to not to hard and we'll all figure out what the best mngnt plan is to get INR preferably more stable.

Other good news is I get to stop the Digoxin and Corlanor (lol also known as 'the  med that no one can say'.
Lasix we're backing down dose from 60mgs 2x day to 40mgs, 2x day. It's a start!! Anything else here on out ie lasix, new beta blocker (but low dose, Lisinopril)
or other changes depends some on how Heart rate (HR) does after this surgery and symptoms overall. Even a little positive news on meds is better than nothing a wk out!

The rest they'll  watch, monitor and we'll see how things go! The NP said there was some off # with the new (mitral) valve gradients but that to she said they didn't know what it would mean and Dr.E didn't seem concerned so seems no sense worrying when it's likely nothing.
I'll  do INR Tues (going to ask if I can do Mon., before infusion)  and then fup w my Cardiology Team around the 9th-10days as long as things stay good!

On a totally different note, I had so many thoughts on my mind going in to this 3rd OHS. The knowledge my Team (all of them in on surgery apparently ) where a little worried how it would go and would this be ok.  I am beyond happy it's went so much better than perhaps thought and getting out quicker than was thought (original 10days-2wks)!

1 of my Pastors came to visit at CHW Weds. and always reminds me how grateful I am to know God as my Savior and in my life! I truly can't imagine knowing the high risks that where w this surgery and not having faith in  my life, I'd go nuts I think?
So grateful for what I know and have (God as my Savior), a much better place than our earthly life awaits us one day when God deems the time right.
I am so grateful and humbled by all the people who offered prayers, thoughts and support going in to surgery last week! I may not have talked to anyone about what I was feeling/thinking really but I will say I am grateful to be here and still be able to help others! Heaven will be a glorious place but I am grateful I still get to be here to help others and be with family and friends!  Even on the bad days I find peace in having God beside me, helping me!!!

Signing off but grateful  is what I feel!
Thanks for stopping by,


Sunday, January 24, 2016

Post 3rd OHS (Mech. Mitral valve), 3.5days out

Sorry for no updates here after surgery.
The mitral valve is in and have been off the ventilator since 24 (or maybe 2 days?) after surgery.
Pain well managed, just 1 chest tube left in due to cont to drain a fair amount of blood from it yet.
Worst issue since surgery has for sure been a crazy, bad UTI. Ugh!
My Surgeon told my Parents it was a good decision, a needed decision that we did the mitral valve replacement when we did as it was in really bad shape. I guess given 2.5yrs ago there was already questions about this valve and should they  do the MVR when they did the aortic valve and aortic root enlargement (they felt the risk was to high if I remember right due to how long I was already on/going to be on the heart lung machine so opted to wait and try other things 1st.

I guess I get the record for 1st person with MPS to have 3 OHS if nothing else? Not particularly  a record I'd have cared to hold though!

Given we knew how bad the Mitral valve was then is probably no surprise the valve was in such bad shape. The increased/high pulmonary pressures where another sign pointing to our needing to get it done.
Thankfully we got it, decided to do the replacement when we did (I'd been having symptoms for some yrs but the symptoms greatly worsened since last Spring-ish and got even worse in the past month leading up to this surgery last wk.
I am definitely glad to be on the other side of OHS again!

Monitors are going off like crazy, some unusual or bad rhythms I guess and fast heart rate.
For intubation Dr.Taylor, my Anesthesia Dr went with the endotracheal (breathing) tube being down the mouth vs the last 2 OHS she had done intranasal intubation. I thankfully remember very little of this!
The Arterial line and another central access  line are still in place but may come out Mon.

Otherwise not much to update - thanks to everyone who reads this but is also on my fb and left kind and thoughtful posts there. I think they are a huge support to my Parents.
1 of my Pastors came Thurs and some other friends came to visit today as well as my Parents of course.

I should receive the ERY Infusion tmrw while here, the drug per my Genzyme Case Manager shipped out for here on Fri.

I don't know a whole lot else right now so will update again sometime soon.
I'll post other  pics in the next update.
Thanks for stopping by,

PS: A few of the OR pics are from 1 of my Providers.
My Dad took the sedated/intubated. pics in my Patient rm.

Tuesday, January 19, 2016

T-1/2 day? Pre-op Appt. Few hiccups but plans in place.

Just a quick update.
My Mom is updating my fb and a friend will copy those updates to here for those not on my fb but who wanted updates.

Pre-op Appt went OK today. Seemed like it took fooorreeevvverr but that partially was I just wanted to get home and get a few things I had to do yet done and pack! Overall I didn't leave CHW till at least 5 though I think after getting there at 11.

Tests that where done in addition to what's already been done include Chest Xray, Labs, Ultrasound of abdomen  (for catheters used in surgery ) and then I ended up having to come back in to CHW after I'd gotten out to my car due to questions with the Chest Xray.

The PA for surgery showed me these and his quest. was what was a shadowy space which I right away (lol, I was pretty sure but not absolutely sure) asked if that area was the pleural space and was it from my VPL Shunt. B/c this fluid hadn't been there 2 yrs ago on a CXR he wanted to make sure. A Chest CT Scan was done which confirmed my suspicion it was just fluid built up from the VPL Shunt. I kind of knew that shunt has been doing well! Tim (PA) ran this by Dr.Mitchell as even Radiology was unsure about the fluid (funny I'd have figured they'd know) and Dr.Mitchell confirmed he wasn't worried  and they didn't need to drain the fluid ahead of surgery which was something several of the Providers had mentioned ad a possibility.  All I could think to myself about this  was nnooooo  I just want to go home yet tonight! Thankfully I was able to! 

Dr.Mitchell was going to try to get ahold of Dr. Bragg but she was not at the hospital  per my own info so I am unsure if he did it not. I had told his Nurse he didn't need to call me either way  as I'd talk to them in pre-op tmrw. I figure whether he talked to Dr.Bragh or not they'll figure togs out with that fluid, the shunt, etc and surgery.
The biggest thing to me was just knowing he'd talked to CHW Neurosurgeons, they have a plan (Neurosurgeons there will be on standby if needed to externalize the VPL Shunt) and their plan would be to if all went well re-internalize it before I was discharged if all went well. Ideally it won't need to be touched though.

I don't know if this bigger amount of CSF (spinal fluid ) sitting in he lung space now will change anything but if anything will find out in pre-op tmrw. 
Otherwise the plan is arrive at 6:00am, surgery at 8:00. The 1/2 hr earlier than usual CHW arrival time us due to the Port access and so they have everything in place to give the steroids an hour before going back to the OR. 

Honestly it seems hard to believe this surgery is tmrw! But that said my entire Team really does seem like they've all communicated well and have various plans in place.
Going to sign off, see ya all in a few days (God willing) and in interim my Mom and friend Jamie  will update here and my fb.
I am not nervous for this surgery, I have my questions answered. Sure i'll for sure miss Dr.Bragg and my UW Nurses as they know me so well with this surgery (they are shunt stuff, different hospital ) but I do feel trust in the Team I have for this heart surgery and care and hopefully all the little things we did ahead of time make the difference this time! 
God bless and best wishes to all, remember God gives us challenges but that doesn't mean he isn't always beside us, walking with us!

Monday, January 18, 2016

T-2.5 days.. As ready as ready can be? Providers all seem to be planning together..

So I've written about how my Anesthesia dr. and how she was going to try to come to one of my pre-OHS Appts. She emailed me yesterday and in turn asked that I have her paged today, while I was next door at FMLH and she would try to come over.
She works at CHW, which is where my OHS will be this coming Thurs and I am next door (connected by an underground skywalk) at FMLH for my weekly ERT Infusion so she was able to come over later in the afternoon. This was nice actually as we where able to talk about the surgery planning as far as anesthesia goes. We talked about what she's considering as far as intubation (start with least invasive, work her way to more equipment which she realizes she'll likely end up using based on the past 2 OHS and my UW records/past but as she said (something along the line), I want to try and do the least invasive first. Overall I think we ended up talking for close to 1/2 an hr or more and as much about the surgery and other things to. She's one of those people I trust immensely.
Once intubated she's said she'll have to use almost an entirely different anesthetic regiment, if I got that all right than she has in the past I guess, due to my heart rate issues but this to she seems to have thought about a lot and re-reviewed my chart again. To quote her (indirectly) 'You've been in my thoughts a lot lately as I've thought about your surgery'. This is sweet and nice she admits as much and honestly I trust her anyways (sort of funny but she said to me something along the line 'I hope you feel confident in my care and trust me' (of course!) but is very nice to know when ones drs are putting their all in to making sure a surgery and care is as successful as possible! I am very grateful for this about Providers like her and Dr.Bragg, they'll admit these things and said as much to her.
Overall my entire team of Providers is pretty amazing, I feel really blessed for this and thank God before every surgery for these Providers! They make what could be really, really hard easier! I try to let my Providers know I appreciate their care and kindness and I appreciate that they truly care about helping me - I've always felt this is important. I said to Dr.Bragg how many of my Providers comment about her, either about 'how does Dr.Bragg do it' when asking how to do something (Aldurazyme most recently, how does she generally give it post op after surgeries in-pt) or how they can tell she is invested in what she does in my care. She in turn commented to me something along the line 'just think I know you very well', meaning I think she knows how to help, to read me and how to help make things better when it's not always straight fwd. - I feel really blessed that my other Providers realize how good she is and I feel blessed in reality to have an entire team of Providers who in many ways are like her, they all show they care and want to help.
Anyone with a large team of Providers (in my case 14 or more Specialists and my PCP) knows how unusual this is! For some reason Dr.Taylor and I got to talking about this today (our conversation really was all wide spread) =) and I said, to me it's important to realize (and I often say to other rare disease families who comment to me they wish they had a team like I do) that to have a good Team of Providers one generally has to be choosey and most often ones Providers are not all going to be at 1 hospital system. - Mine are spread in 3 cities and 2 states.
Sedation wise she (Dr.Taylor) had commented in her email she felt it was important (as she has commented in the past) we do it differently after this OHS, perhaps like we did after the 1st OHS, either longer intubation and sedation to allow better healing (biggest issue we feel waiting till the hest tubes are removed) or leave sedated initially with intubation but then extubate probably after 24hrs as was done after the 1st OHS but stay in the CVICU longer so sedation can be kept and pain mngmt be better kept on top of while chest tubes are still in place. -
Admittedly I had not realized before today that apparently after I was extubated after the 1st 24hrs following the 2nd OHS they must have moved me from the CVICU to a different floor where sedation wasn't kept (this I knew) and pain not able to be kept on top of as well. I remember a post my Mom wrote on my fb, something about being moved but I never knew what that was about, guess I do now. =) Funny the things ones mind blocks out, probably for sanity sake!
As Dr.Taylor said and I completely agree, the sternal incision where they cut down your chest is uncomfortable but not as painful as some surgeries I've had, (ice works/helps well) bc there aren't nerves there. The discomfort I had initially after the 2nd OHS was definitely, initially from the 4 chest tubes in place yet and being completely awake. And as Dr.Taylor said when they offered to give sedation before removing those 4 chest tubes I should have taken up their offer - that removal was a beast, an aweful one and partially I/we believe what made recovery so aweful!
She's talked to her colleagues, both apparently in Pain Mngmt (she commented the person on call the day after surgery is really good and she feels will be really good for my care/any needs, she gave them a run down on our past experiences). I am glad she's talked to her various colleagues!
As far as anesthesia she said in her email yesterday due to the mitral valve and being sicker I guess than both OHS prior her anesthetic mngmt will be vastly different than both OHS 2.5yrs ago. Part of this having to do with our heart rate issues and one of the meds she used the other heart surgeries can affect or increase heart rate which is/has been a significant issue for us and something they want to avoid during this Open Heart Surgery/MV-Replacment so she plans to avoid that and use a different sedation plan.
This in turn means she is formulating a different plan and approach if I understand it all correctly as I mentioned above. The other concern Dr.Mitchell has had was the small potential a pacemaker could be required after this OHS due to my already fast heart rate and of course- though none seem to concerned that they have to place a chest tube in the R side pleural space, which is where the VPL Shunt drains now. Hopefully, a chest tube in fact won't be needed..
I often think it is sweet how many times Providers of mine have said or commented to me something along the line my ability to negotiate my care, manage decision making and get this in place. Dr.Taylor in her email the other day commented something along the line about this and (my paraphrasing ) 'You could teach me a thing or two'.
While I doubt it's true I could teach my Providers anything about Healthcare and managing it/complex care, it is nice to know when they think I am doing a decent job given I do this mostly on my own - (getting things in place, getting insurance coordinated, getting my Providers- who are at multiple different hospitals and health systems to coordinate with each other (most are great about this! ) and just making sure little and big things are in place. I should add I do have a pretty great Team of Case Managers of course with Amy, at Genzyme re the Aldurazyme when needed and Marge, at Dean for Prior Auth's when needed. I do appreciate both of them and my Team of Providers and their Nurses, Secretaries and NPs!
I don't know if she has and from talking to Dr.Taylor today I feel confident in what she has told me/who she has informed of our past issues and upcoming surgery but my Pain Mngmt Dr. (Dr.Bratanow) at last wks Appt was talking about sending her recommendations to my Heart surgery Team so they know to reach out to her if there are any questions with post op pain mngmt.
My NP at Dr.Earing (my Cardiologist) called Thurs last wk, she had talked to Dr.Mitchell (my Heart Surgeon) Nurses who must have heard from my Genzyme Case Manager re the ERT (Aldurazyme) drug I receive every wk and ordering that.
Nancy (NP) in turn had some questions for me about how it's given, what they may do with it after surgery, etc which I am glad she called and glad they are sorting this all ahead of time! Super grateful to my Genzyme Case Manager for working on this and helping get it sorted w my Cardiology Team!
On a side note I think I will be happy when this OHS is over and perhaps just sitting in the chair reading I even feel short of breath? This is not a constant issue, thankfully (!) though but still occurs frequent and often enough!
In all honesty it seems a little hard to believe but less than 3 days from now and surgery should be over, for that matter 3 days from now the surgery should be over and recovery in process? God willing! I was talking to 1 of my Infusion Nurses about what to do with next weeks infusion whether to cancel it or somehow put a note in the system that I likely wouldn't be there but being concerned if say surgery where to get cancelled when Dr.Taylor came in. She overheard and commented, 1/2 joking "It's only getting canceled if you cancel on us" - lol, I am not cancelling. Can't say I am looking fwd to the surgery but as I said to Dr.Taylor I am looking fwd to it being over to.
On a totally separate note, Headaches have been better since we put the 2nd shunt back in and things improved once we adjusted the shunt settings down. Not perfect by any means but they had been better. Oddly enough, perhaps the best thing about this (and this is way TMI) but bowel movements had totally normalized - this always occurs when we have the shunts in a really good spot. They've just started reversing back to a more abnormal again after headaches have been a little worse in the mornings again so that is a little crappy (lol) but hopefully not a sign of shunt problems to come? I am hoping, I so want these good times with the shunts to con't, even if mornings are a little worse it's still overall been better than it had been in a long while!?! I am hoping stomach and overall doesn't get worse and overall stays good!!!!
If anyone wants to visit (I know a few friends in Milw are planning to as are my family) I will be at Children's Hosp, - visitors always welcome! Any and all =)
In any case, I will try to do a short update after the pre-op Appt Thurs., and my Mom will likely do short updates here and on my fb for the 1st few days after surgery.
Thanks for stopping by,
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Monday, January 11, 2016

Crossing T's and Dotting I's?.. Plan and prepare.. Heart surgery..

Nothing to big in this update, honestly just a few things I forgot to mention last wk (I think?) and a few things I have been mulling over.. I kept questioning my drs., re the shunts and this upcoming heart surgery, should fluid build up around the heart and around the lungs after the MVR is done (Dr.Mitchell's bigger concern with the VPL Shunt) and a intervention end up being needed what would they do with the shunt or how would they manage it all? If fluid builds up post heart surgery in pleural (lung) or around heart  Dr.Mitchell at the appt last wk was uncertain but concerned this could affect the VPL Shunt and is why he wanted to have a plan in place w the Neurosurgery Team at CHW.
He wasn't sure and couldn't say if fluid in these areas would affect shunt function but felt if this happened (which happened w both prior OHS) the need to place a chest tube in the lung spaces would probably be a lot higher. The big question is would they need to move the VPL draining (distal) end of the shunt then.
I don't know what they will decide if notjing else will definitely be asking what the plan is at the pre-op Apps the day before surgery. I am confident  my Team will have 1 in place but I still want to know what it is!
I just want really to know what the plan is between my Cardiology / Cardiovascular Team and the Neurosurgery Team at CHW, whoever Dr.Mitchell ends up talking to, simply bc I'd like to know ahead of time. The surgery is the 21st so not all that long out, a wk from this coming Thurs.
I see my Pain Mngmt dr. this coming Thurs so had sent her a message to see if we could potentially work out a (open ended?) pain plan I/she can share with my Cardiology Team at CHW which they can use along with what they did last time /the past 2 open heart surgeries as a primer of sorts for managing post op pain? I know my Anesthesia dr. has been thinking about this and hopefully with have thoughts in mind to but knowing the Pain Team at CHW for whatever reason after the 1st OHS asked my Parents if I was 'abusing drugs' (paraphrasing what my Parents later told me as I was still sedated at the time I think) and didn't seem all to willing to contact my Pain mngmnt dr. or perhaps see what we've used at UWHC I feel like it might be a good idea to have a tentative ideas in place?
The Pain Team at Children's doesn't have an entirely great reputation and personally when my own Anesthesia dr has brought up how she thought pain after the surgeries was an issue I feel like we need to have ideas in mind/in place!? The Pain Team seemed hell bent they where going to do their own thing and thankfully while I remember pretty much none of it from both being sedated (but must have been some awake at times just intubated? Not entirely sure?) Anyways I know from what my Parents have said and even from things my Anesthesia dr. has said the post op after the 2 OHS was not great but definitely worse after the 2nd one mngmt wise.
So anyways I figured I would try to work with my Pain mngmt dr and give them an outline to potentially work from this time?
What it comes down to is I am just trying to cross all my T's and dot all my I's as much as possible ahead of the surgery day.
I also talked to my Genzyme Case Manager (Genzyme distributes the ERT I receive every wk), Amy was just getting back after being out for close to a month for her wedding but was in the process of reaching out to the Nurse in Genetics at CHW re ordering the enzyme drug for when in-pt at CHW after the Heart surgery and helping to get this all coordinated. She (Amy) will talk to the Nurse I worked with last wk when I met with Dr.Mitchell, at CHW and try to see if she and Yvonne can make sure things are squared away for after the 21st that drug can be ordered and given while in-pt. 
She (Amy) did email me today to say she would be talking to the Nurses at CHW herself bc she knows I'm a little nervous about not knowing who exactly I talk to to make sure a plan is in place re the ERT. Amy said she would be sure there's  a plan and any questions ironed out as much as possible ahead of time. Originally the plan was she was going to talk to the Genetics Nurse who would then (hopefully) coordinate all this. I am grateful to Amy and for her being on my Team, especially that despite her being on the edges in a sense of my Team (regarding this Heart surgery ) she's still staying as involved and trying to make sure this are as in place as possible. 
She was going to arrange it so Aldurazyme would be able to be ordered for however many wks I'd  need it at CHW (hopefully just one given Dr.Mitchell felt would be 7-10 days in-ptbif all goes well).

The Aldurazyme (ERT) was arranged for the 2nd OHS in-pt stay at CHW and given (not the 1st, sort of a long, ridiculous story) and for that matter Dr.Bragg's Team was able to sort it out a few yrs ago, that I could start getting it while at UW so while I am cautiously (figuratively) holding my breathe an I feel semi optimistic it will work out again the drug can be ordered for at CHW- when in-pt. With Any, my Genzyme Case Manager helping I feel much more optimistic about this. =)
So the schedule for surgery so far is:
Pre-op Appt: Jan 20th, 11:00am, this includes a ultrasound/scan (I think for catheters they have to insert, but not entirely certain, it was just added last wk).
Meet with the Nurse and PA and (typically, she was trying to arrange her schedule) Anesthesia. 
I am hoping they can tell me more about Neurosurgeries plan 'in case' if needed. Surgery plan (estimated length, if it's in fact just Mitral valve, size, etc-just details I like). And any other details they feel are important.
Labs, labs and oh Labs?
Whatever else I am forgetting. Thankfully the bigger tests where done already (Echo, Cath, Heart Rhythm, etc.) so we'll see and whatever else they add.
My Heart Rhythm Dr had emailed  a few was back he planned to stop by at some pt while in-as he is curious how heart rate will do after this MV-Replacement. It can take weeks or months to know true extent but they get some idea coming off the heart/lung machine and in the days after how heart rate and rhythm are doing. He is watching for the sake of our potentially being able to cone off the Ivabradine (for heart rate control, CHF).

I think if anything makes me nervous about this upcoming 3rd OHS it is the shunts, not yet knowing the plan yet (but I'll get it) and hoping the headaches stay what they are at? Not perfect by any means but also a little better than they had been? I think the bigger issue w headaches right now is still nights/mornings (but I plan to ask they leave the head if the bed elevated if I am out for any period of time which will help at least a little) and I keep getting (at least relatively short) migraines which I only get when the shunts are being stupid. I do think still the shunts are working some better than they have in a while though, the migraines seem certain part of the day.

I hope to if they do need to do anything with the VPL Shunt they communicate with Dr.Bragg, keep her updated.. I know I can when I am awake but just be nice given she IS my Provider and manages these shunts otherwise!
Otherwise last but not least, this coming Sunday is my last week teaching Sunday School for a few wks. Some friends are taking my class for the wks I am out, hopefully only a couple wks. I love seeing my kids every wk and learning with them so I am hopeful it won't be a long period I ma out!
Our craft for this wknd, unrelated to our lesson (3 men in the fiery furnace, how God protects us in our faith) is wooden snowflakes I found some wks ago at Michaels. I'll attach a picture from our craft last wknd which was beaded snowflakes, a craft I'd found online. 
In any case that is all, nothing really to important, more or less just my thoughts/feelings in this post.
Thanks for reading if you did, 
I'll  try to update 1 more time at least next wk after the pre-op Apps Thurs night (night before the heart surgery) but otherwise my Mom will likely update my fb and short updates here for a few days after the heart surgery.
Thanks for stopping by,


Tuesday, January 5, 2016

Appt w/Heart Surgeon; Plans in place, details being worked out..

Oh Lol, going to start this with a good laugh (on my part) - Dr.Mitchell and I and his Nurse/Scheduling Coord. where talking about where the VPL Shunt is implanted (in back on R, parietal location), I was thinking it was still on the L side, valve with catheter crossing across skull to the R, running down to R side of chest to pleural space but in fact it is implanted in back and it does run down R side.
Anyways I say this b/c Dr.Mitchell as I mentioned above was asking about what side it was on and I totally goofed. Lol, I guess atleast I was semi right in where it's placed? I think I am so used to formerly being able to feel the reservoir and this one although apparently there is a reservoir on the shunt I cannot feel it and the shunt blends in well till it gets to my collarbone/runs down behind my ear. Just a stupid funny on my part. =)
So anyways as far as surgery goes, the plan is as I knew to use a mechanical mitral valve (also a St.Jude, same as the Aortic valve brand) although the 1 difference with this valve is unlike the aortic root where they could enlarge the aortic root making it possible to implant a larger size valve with the Mitral valve this is not an option so he said he would have to place a pediatric size valve.
I asked both about the Port (would it be ok in surgery) and he does not feel the MVR will affect the Port at all. The Nurse felt we'd be able to likely use the Port for one of the IV access sites (they typically then have to place a couple more peripheral IVs).
VPL Shunt wise Dr.Mitchell is planning to talk to the Peds Neurosurgeons at CHW to see what they think but as far as the actual Mitral valve replacement he did not think he would need to do anything in surgery that would directly affect the shunt.
His concern was more after the OHS is over and likely fluid collecting where the VPL Shunt is, which occurred with both prior OHS and he was as yet unsure if this would affect the shunt function and/or would they need to do anything with the shunt. Remains to be seen I guess, right? I have this question in to Dr.Bragg, does she think fluid building up around the heart/lung would affect the VPL Shunt? Atleast he is talking to the Neurosurgeons there and if needed my Cardiologist talks to Dr.Bragg often enough and can get ahold of her... I've been keeping her updated on this surgery and plans.
Dr.Mitchell talked some about the risks with this surgery but also how he felt we'd get through it and have a good plan in place. He brought up about the endocarditis (heart infection, I have to admit, obviously I knew it was treated as this after the 1st OHS and post the 2nd but never knew it was officially considered as an actual heart infection) and anyways he just said they'd do their best to make this surgery go smooth, to try and avoid any complications/infection or problems.
I have to admit I like him as a Surgeon and felt like he genuinely tried to apologize for some of the errors in the past 2 OHS, 2.5 yrs ago (not communicating well after surgery, the surgery having to be re-done) but I also feel like as much as those things made me a little frustrated at the time I DO know he's a VERY good Surgeon and I know to, clearly from today's appt he recognizes little mistakes where made on his part or things he could do better to make communication go better and that speaks volumes for me on his behalf..
I do not believe I should fault him for something that happened years ago when he clearly was apologizing today and on my part it's just not even a big deal anymore.. It's a little like my Neurosurgeon's Partner, I may never love the guy but I also don't feel animosity towards him as I used to bc while I would never by choice chose him to be my Nrsgn I do know he's good at what he does. Sometimes it's good to just move on.. And in the case of Dr.Mitchell he IS a good Surgeon and clearly cares.
One of the bigger issues Dr.Mitchell seemed to think he might have to deal with was scar tissue both in the chest from the 2 prior open heart surgeries and from the VPL Shunt/Pleural shunt surgeries. He said he expected this likely would take the most of his time to dissect and get rid of prior to being able also dissect and take down the current mitral valve and in turn be able place the new mechanical mitral valve(after they hook up the heart lung machine and of course prior to all that after they get a stable airway secured).
After all these many steps and some others (whatever chest tubes they need, pacer wires if they add them which they did with both prior OHS, etc) are done they then start the process to implant the new Mitral valve. Some scar tissue was removed back in Nov/Dec back when the VPL Shunt was placed by the General Surgeon working with Dr.Bragg in that surgery so perhaps that will/could help make this surgery a bit more smooth? I honestly don't know and remains to be seen I guess...I know scar tissue was one of the thins he mentioned would likely be a concern for him.
One of the other concerns he had given this is going to be my 3rd Open Heart surgery and I do already have issues with fast heart rhythm he commented he'd worry about the need for a pacemaker after this heart surgery and is some thing they monitor for during and after surgery. He said they can tell often coming out of surgery if the patient is in heart block (not entirely sure what that means but having to do with heart rhythm/conduction) and was something he said they monitor for in the 1st week post OHS especially and typically know whether one would be needed by the 1st wk post op or not.
One of the other things he mentioned and we talked about was before/after the 1st Open heart surgery there was a lot of talk about whether they should replace the Mitral valve right away to and apparently one of the Staff Cardiologists even wrote a note with concerns about this. I think she may have been a CICU dr., not sure. Anyways, Dr.Mitchell said had it not been for our needing to do the highly complex and very indepth already Aortic valve replacement and additional Konno procedure (enlarge aortic root, repair VSD) they would have went ahead at that time and replaced both the aortic valve and the mitral valve all in the 1 surgery. I understand why they didn't as the 1st and 2nd OHS where already extremely long (10 and 12 hrs I believe or maybe it was 8hrs and 10hrs?) but I remember my Anesthesia dr saying to me some days after the 2nd OHS how she'd had to very nearly replace my entire body volume in blood products during the 2nd surgery and just in general how difficult those 1st few days after the 2nd OHS where. I thankfully don't remember it but from things my Parents, Dr.Taylor (anesthesia dr.) and others have said to me.. For whatever reason it was in God's plan we do these 2 valves separately I guess..
I talked to the Nurse/Scheduler today about Genetics, about our working to get ERT for when I am in-pt and if possible how I'd prefer my former Genetics dr. at CHW not be allowed to make decisions on my behalf given decisions regarding my care when in-pt. I am working w the Genetics Nurse some re getting ERT for when in-pt so her I don't mind at all but that's just it she's working w/me. This other dr, isn't a part of my Team and I never talk to/see him. My real Genetics Team is in Boston and if anything I said to the Nurse my Cardiologist could get ahold of them though I'm not sure what they'd need Genetics advice for in general. In any case the Nurse I worked with today was going to work to make sure it was in place re the Genetics issue.. I really liked her!
To end on another note, to make me smile a bit. Dr.Mitchell asked me if I had any travel planned in the next 2wks before the OHS to which I said no, but I have plans to go to a Conf/Mtng the end of Febr. in San Diego. =) This is a mtng for the WORLD Lysosomal Mtng, something I've gone to the past few yrs for the Foundation I work for and is all drs and researchers/Health Providers whose focus is Lysosomal Storage Disorders.
This is usually a good mtng., a big plus is it's in warmer climate - this year San Diego again! I am hopeful i'll be up to the travel! I went to a Conf. a month after my 1st and 2nd OHS (would have been a month after my 2nd OHS, approx. 2mo after my 2nd OHS) in San Antonio so if this surgery goes well I think I can do it. =)
Anyways I think that's about it, hard to believe it's only 1.5 wks away!? Yikes!

Older update -

1st let me say I have to apologize for all the typos in my last blog - wow!  Yikes, sorry about that! I thought I edited it but was doing it from my phone partially and I guess I did not edit well!

I was laying in bed (on the couch lol) trying to fall asleep when I had been writing (the other day), knowing it was going to be waayyy to early of a morning w the early bird (Zan) sleeping over who gets up by 6:30-7 every time he's here (vacation doesn't much matter to him, he stays on his school wk schedule albeit that's even earlier I think).
Anyways I'm not sure why, I guess bc it's what I do when I can't sleep (?) and distraction from the headaches (worse at certain points of the day, have been literally all over the place, some moments really good and others Not.So.Much but I was thinking about the coming Coumadin changes, about Sunday School and the 2 Teachers that are subing (sp?) for my class while I'm (yes I know random and all over the place thoughts) while i'll be out.-I am hoping will only be a couple wks but anyways for whatever reason then was thinking about how the change in INR/Coumadin mngmt will (may?) affect future surgeries and this in turn I was thinking about surgeries and procedures  i've had in 2015. ..
This included (which sort of seems like a lot but was actually in some sense mild compared to some yrs)
-Heart Cath/Heart Rhythm Study (July)
-L Hand (3rd) CT releases/other nerve surgery- last Jan/Febr. (the R was done a yr or 2 prior to that, just prior to the 1st AVR/Konno surgery.
-This is a little fuzzy but TPL Shunt revision (Last Winter, I think? May have been earlier)
- Shunt Infection - (both shunts removed) -May
-EVD put in (when both shunts removed)
-LP Shunt replaced (May), 0 press. Valve
-0 pressure valve removed, Codman valve put in (a wk or so after 1st LP)
-EGD (Stomach scope ), May (prior to the EVD, Shunt removaslls/replacements).
-VPL (2nd Shunt) put back in (Nov) - imperfect but some better than 1 which I'll take.
-Lots and lots of shunt taps (but hey they don't hurt and they work so well!) =)
- Who knows if I am forgetting something but what a year!?
Not all bad though, I added a great Genetics Team in Boston,  I still work for GSF (love it) and I participated for a 3rd year  w/the Genzyme  Running for Rare Diseases Team - this past yr both in Boston and in Providence  (R.I.)

Definitely a year of up's and some downs, things I didn't really expect (heart!!!) and at the same time I have a Team of Providers who are pretty amazing.. I have a few who are outright exceptional and that I am beyond grateful for! Who as a Patient and especially a Rare disease Adult can honestly say they have more than a dozen Specialists and like every single 1 of them?
I certainly couldn't up till a year or so ago!
In addition this is my 3rd year teaching Sunday School and I honestly never thought in the beginning I'd love it as much as I do but I genuinely look fwd to seeing my kids each wk. I miss them the wks we don't have class (these past 2 wks) and I am grateful how kind and caring the majority of the kids and their families are!
I guess this post is what I was not feeling the other day? =)
For 2016 I'll hope (especially ) for a good outcome to this coming heart surgery. For (perhaps) better w the shunts but if that is not to be God's will then I know in the bad days or bad moments I am still blessed w a Neurosurgeon and her Team who truly care.
For my Parents I hope they are able to weather this upcoming heart surgery and it for them goes smooth so they not have to deal w unneeded stress. I pray they have strength in their faith and always remember God is by their side. I pray for Zan and for my Sister the times that are perhaps hard get a little easier and the good times remain in both their vision as the 2 mean so very much to me!

I also am on the Running for Rare Diseases Team again this yr, being partnered for the Boston Marathon which that is not till the 2nd or 3rd wk of April. I finally called and made the appt at Boston Children's with my Genetics Dr the Thurs before Marathon wknd.

Friday, January 1, 2016

To a New Year.. New (post <3 Surgery) Blood thinner changes, New Year, New?

This post has been written over a few days - I should really finish it =)
I sent my Cardiologist an email, asking what the upcoming post MVR (mitral valve) blood thinner dosing would be.
It's a question I hadn't really thought to as at the Apot when we where discussing this next open heart surgery yet I know the INR level is different after 2 mechanical heart valves are in place and I  know it is handled a bit differently with surgeries.
Anyways he resonated back in fairly short order and just said INR has to be monitored more carefully + anytime INR falls below 2.0 we'll have to bridge when lovenox or heparin injections.
I am not entirely sure how that works then we surgeries pre/post a surgery but hopefully for instance in Dr.Bragg's cade she'll do as she's been awesome about doing and work with my Cardiology Team as needed was shunt stuff and in the case of my Primary Dr she's always been great about talking to my Cardiology Team and then to whoever is the Surgeon (say Hand stuff) to iron out pre/post op details w blood thinners. It all should be OK but I just like details!
In any case I really am going to sign off - below us the original, full post  from the past few days.
Happy New Year - May 2016 treat each of us well!
May the grace and kindness of God be with each of you,


Not entirely in a reflecting mood but I guess that's what a New Year does? Went out with my Sister, her hubby and a friend last night to a bar (totally not my thing, I don't really drink and was way to tired, feeling kind crappy to boot) but was ok. Honestly if anything it's a people watching galore and good for (if nothing else) reflecting on the past yr - which I know sounds weird but...
New Year's Eve prior to going to my Sister's I'd run to Madison, I had some labs (the gene mapping blood work and somewhat more routine (to me anyways) MPS/uGAG retest (something done every so often  my Genetics dr. in Boston and PMD had been wanting) so I finally got those done. Exciting stuff ;)  If noting else a little grocery shopping to.
Anyways so I wasn't really going to go with my Sister and hubby but they kept asking, lol, both know I don't really drink and my Sister wasn't really drinking either but what the heck, I guess, right? We played some pool, and pretty much just listened to the music (was decent but  loud). Was an ok NY-Eve and nice enough way to ring in the New Year though I admittedly was happy when I got home!

I was kind of thinking on and off the past few days about this past year and various things that have went on, changes I've made in my Medical Team (nothing to big, mostly the Genetics component), some of the surgeries (shunts especially, heart stuff and the upcoming OHS, the 3rd L hand surgery last Winter) and some of the ups  ups and downs we've had with the shunts.  - The really good moments, even if they don't last long enough and the seeming more often periods where the shunts are just stupid - they appear to work and likely are or atleast to some extent but is more (probably?) the shunts draining (which is good!) but this in turn likely causes the ventricles to collapse which in turn causes headaches as pressure rebuilds till it gets high enough causes vents to open and shunts to drain again over and over-repeatedly. A good-frustrating cycle which I wish there where an easier way to help!
On top of the shunt stuff, and the shunt infection (last May) which totally took us all by surprise last Spring shortly after I got back from Boston from the 2nd Marathon (Boston was 1st Marathon w the RfRD Team, the 2nd Team Marathon was in  Providence) and our at the time only putting 1 shunt back + trying various means to get away with 1 shunt building up to this past Nov. (I think that's when we put it back?) when we opted to in fact replace the 2nd (VPL) Shunt.
This although it's better at 1st and in a way now is just different is on another hand different then other times when we've had some issues but isn't quite as good as when we had 2 shunts before (which was never perfect then either but was some better than this).
This said I am STILL glad we have the 2 shunts back in place. I realize it might seem like, well if you have 2 shunts and isn't as good as before then why is it even any good and believe me it frustrates me but hopefully, maybe we'll figure it out and in the mean time I am still grateful for what is?
And well because the shunt stuff and any other stuff wouldn't be dull or exciting enough of course the heart stuff has taken up it's own good efforts in 2015 to? Between the heart rhythm/to fast of rate issues and trying to slow these down (I think in 2015 we tried no less than 4 different meds and eventually now (since last Summer) ended up with the Ivabradine (heart failure/heart rate) med (newly approved to market back in May), the Digoxin (helps heart function better), Lasix and Spironolactone which both work to get fluid off and then the 2 meds for the heart valve.
The hope had been that with the addition of the Ivabradine we might be able to help slow the continued progression of the Mitral valve narrowing and leaking but that did not happen. On Echo's done last summer and then this past Nov/Dec. the valve had worsened several more gradients putting it in the severe range along with we knew already last summer when a Heart Rhythm Study and Heart Cath where done (invasive tests to measure various things in the heart and heart rhythms) showed the blood oxygen from lungs to heart, having to do I guess with oxygenation (I think that's how it works, I am blanking on the right term/word off hand) where very high which is not normal.
This all taken together along with symptoms continuing to worsen we opted at the Appt back in Dec. (or maybe it was Nov, I can't remember off hand) to go ahead with the 3rd open heart surgery and replace the Mitral valve.
This is a surgery that in and off itself has been done before in an MPS Pt. but 3 Open heart surgeries have never been done in an MPS pt. so it's sort of an unknown but I also have 1 of the best congenital heart centers and Teams doing the surgery (and exact same team as before - Heart Surgeon, same Cardiologist, and Same Anesthesiologist as both prior Open heart surgeries and the other 2 heart procedures) so that goes in our benefit as they are all familiar with my heart and my Anesthesia dr is familiar w/my airway, endocrine stress hormone needs, etc. We'll manage. And most of all God is by my/our side and he knows his ultimate plan.
That surgery is, right off hand I believe Jan 21, I meet with Dr.Mitchell this coming week, pre-op Appt is the day before surgery and the Anesthesia dr. was working with her scheduler to try and arrange her schedule so she could be at either the appt with Dr.Mitchell or at the pre-op Appt. Dr.Taylor (anesthesia) has felt pretty strongly (which I am grateful fior!) we need to have a better immediate post-op plan in place (ie how long they'll leave sedated/intubated which varied a great deal between the 2 prior heart surgeries post op mngmt, etc) and wants to try and iron that out ahead of time. I appreciate she has thought about this and would like to try and have a better plan 1 in place!
I guess the other (potential) bigger issue with this heart surgery which Dr.Bragg (Neurosurgeon) and my Cardiology Team (both at different hospitals /cities ) have talked to each other about is my newest replaced VPL Shunt drains to the pleural space which is where chest tubes are frequently needed in heart surgeries. When she talked to my Heart Team they felt they may not need to place a chest tube on the R side so all agreed and opted to leave the shunt to drain where it does and if needed it could be moved temporarily in the surgery. I am a little uncertain should this be needed (it need to be moved) what they'll do with the shunt and plan to ask my Surgeon so I know they have a tentative plan in place, just in case but God willing it just wont be needed!? That said I just like to know my drs have back up plans in the off chance!
Otherwise I posted the questions I have for my Team which I'll ask at Tues appt /in coming wks in my last post and think those answers are the most important as far as knowing the plan and knowing my Team has their plans in place ie VPL Shunt (should it be needed), immed. post op mngmt, etc. Stay tuned :)
I guess otherwise I don't know much, will try to update after the appt w Dr.Mitchell this next wk.
I am also supposed to update Dr.Bragg/her Team on symptoms so we'll see I guess. I don't think there's anything she can do so there to not sure.