Friday, August 26, 2016

Pre-op (surgery) update, few other things..

Pre-op Appt was the other day (lol this is ridiculously sad (really sad!) but less than 1.5 wks till the shunt surgery now and am waiting.. picturing the fluid draining off and a bowling ball like weight coming off my head - hoping this helps for awhile again)! My PCP asked if Dr.Iskandar planned to look at both shunts or just at the VPL Shunt which I am not actually sure. I know he showed me on MRI where if he finds an issue he thinks it will be but I have to admit I have wondered some about the LP Shunt to. I have little doubt it's working more just wonder is it working fully?

New Neurosurgeon -
Dr.Simpson asked about the new (apparently now at UW) Peds Neurosurgeon and if I planned to (request) to meet him which I said to her I didn't really know how  to go about this? I worry I'd be burning bridges if I asked to meet the new Nrsgn (Dr.Ahmed) given Dr.Iskandar seems like he's planning to keep my case? Honestly I would just like to the meet the other guy, see his personality. What I REALLY want out of a Nrsgn again is someone that picks up the phone and talks to (especially) my Cardiology Team and who doesn't kind of give the pretense they are super familiar with my disorder when I know they are not.. Or who I tell them something that's well known fact in the MPS Commnunity, that Dr.Bragg, the Nrsgn that referred me to her and drs in our Community all know but then the dr. goes against that in their own notes? I realize this may sound bad to say it like that it's just what any of us in a rare disease communing with a issue that's not all that common to our rare disorder want/wish for.. And when you had a Provider that was pretty phenomenal at communication well it's hard to not want that again1?!?

She (Dr.Simpson), suggested I ask to make an Appt with the new Nrsgn and just gauge his personality, get to know him a little, see if we get along well (does he take an interest in my case).  Per a former Patient of Dr.Bragg's (the Mom's little girl was the Patient) appts are starting to be scheduled with the new guy so I am hoping i'll meet him when in-pt for the shunt surgery? I feel weird about this whole situation as it was originally intended I'd go to the new guy once he got here but then shunt issues sort of got in the way.
 Shoot I even could work with Dr.Iskandar if he'd work with the rest of my Team and I felt a bit more like it was a partnership.. I do appreciate atleast that he's worked with Dr.Bragg (even if I sense it drives him a little crazy I'd kept in touch with her, not sure). I don't know? I just want  a Neurosurgeon again that it's a full partnership... Not one where they say 1 thing to me and another in notes/etc..
So I'm just not entirely sure yet!?!

Cardiology - Surgery (meds, blood thinners)
Cardiology - Lovenox (Enoxaperin technically) right now the plan is Dr.Simpson is talking to Cardiology to find out if they want this injection both the night before and the morning of or to skip the morning of. Given a mitral valve now technically I guess it's recommended the injection be given even the morning of procedures but she's going to find out and let me know and send the recommendations to Neurosurgery. This to... totally something Dr.Bragg would have double checked herself.. I guess it was just the comfort that she wanted to know herself? It's not at all I don't trust my Team b/c I do just knowing everyone communicated was HUGE.

Other meds i'll skip the morning dose of Lasix but take the longer acting Spironolactone to keep fluid off/prevent fluid overload. Per Cardiology and my PCP will do the same as I'd do with Dr.Bragg w surgeries and stop the Baby Aspirin 5 days prior to surgery.

I'll admit it bothers me a little but given the on-going heart issues and Dr.Iskandar being new to my Team/new to MPS that he hasn't said anything about reaching out to my Cardiology Team.
 I know this was a huge reason my Cardiologist liked working with Dr.Bragg (she frequently worked with them, asked their opinion or just kept them up to date on what she intended to do/did they have any concern from heart stand pt.
She didn't always talk to them but if there was a new med or issue she'd reach out and confirm. As any one in our MPS Community knows having docs that do this is HUGE. Dr.Earing appreciated this as well,  I think he just likes to know his Patients Providers are keeping the whole picture in mind and Team player?

Side Note....
Kind of on a side note, I was thinking about this earlier but I fully realize several of my Providers read this blog (not a bad thing I guess, perhaps unnerving bc this is where I go where I feel like I can share what I am feeling about some things but still good I guess for Providers to see inside their Patients worlds outside just a medical appt... Even if I primarily only write about medical stuff here I guess is still another perspective on a Patients life?.. But that said sometimes I am really honest about my frustrations, worries or fears here in ways I don't share elsewhere. I guess read at ones own discretion (if that's the right word to use, not sure)? ... I'm probably never going to stop writing here what I feel b/c this in a way is my sanity (writing).
Elmiron, Anti Inflammatory:
 Dr.Simpson and I talked about this med and possible use, about what it does (it is now believed there is an inflammatory component to MPS I, which makes sense really bc many of us w MPS I are originally mis-diagnosed with some form or multiple forms of arthritis and muscle related disorders). She is going to reach out to my Genetics dr. in Boston re dosing as he (Dr.Bodamer) and my Cardiologist (Dr.Earing) have been talking about starting the med for about a yr now to see if it could help some of my issues.
Elmiron crosses the BBB (gets to the brain) and has been shown if I remember right to affect GAG storage which is what we do not break down with MPS I due to the missing/deficient enzyme. Perhaps this could help our Hydrocehaplus/shunt and heart issues some. It does have mild blood thinning properties so that  part would have to be sorted given we already have issues stabilizing INR (currently just doing Lovenox (Enoxaperin) injections vs oral Coumadin for this very reason to provide a stable low blood thinning threshold. We'll see. I LOVE that Dr.Simpson is AWESOME about talking to and working w any of my Providers to sort any given issue out!

Otherwise I had 3mo botox today, which at the last Appt we had added an area more central so do shoulders, neck and mid/mid-low back area and while that newer area isn't particularly comfortable getting injected it all works so well! I really do need to go back to PT sooner than later to help overall leg/hip and the muscles that control posture but the botox does work well!

Hydro Association (1 day) Conf. - Phoenix, AZ
Lastly I both know an MPS Family that goes to the hospital Dr.Bragg is now at (they are seeing her next wk) and a few months ago met on fb another shunt Mom who was moving to AZ (not an MPS Family) and I follow the PCH/Barrow fb page (actually kind of an interesting page, they do a good job updating and interesting Hydro pieces here and there).  Anyways it turns out PCH is collaborating with the Hydrocephalus Assoc to host a 1  day Hydro Conf.

I'm not yet sure if i'll go or not but the Foundation I work for has offered to send me if I do chose to. This is in Oct so not the greatest timing what with Sunday School starts back up in a few wks (Sun after Labor Day), the shunt surgery is the 6th (not a huge recovery) but then we're also doing the Heart Ablation procedure in-pt at CHW again so likely another Sun. I'd miss of SS topped by there's another Sun. in late Sept. i'll miss.

This all said other than is EXHAUSTING traveling (but maybe i'll be in the (atleast from shunt aspect) feeling better mode so that would help? Would be fun to see friends out there and Dr.Bragg's new hospital.  I'll likely decide sometime mid-Sept so we'll see?

In any case, thanks for stopping by,



Thursday, August 18, 2016

Appt, Heart Rhythm Team.. Take 2 Heart Procedure (sinus node. heart rate)

This update shouuulllddddd be shorter (but then again I say that and usually end up not being)!

Heart Rhythm Appt. Take 2 (t-minus shunt surgery 1st)
Anyways saw Heart Rhythm doc today and (atleast I was somewhat surprised) he is recommending once we get through this upcoming shunt surgery to re-try the Heart Ablation study (with same idea to try and kill/deaden abnormal (scar?) tissue creating abnormally fast rhythms from the sinus node.

The Sinus Node is the hearts natural pacemaker so is same risks as last time (biggest being the ablation causes to slow of a heart rate which in turn require a Pacemaker) but Dr.Kovach and Dr.Earing (my Cardiologist) feel the benefits out-weight the risks and especially since (as Dr.Kovach put it) they did a similar, less intensive approach last month and it did slow heart rate for about 8hrs.

Why the initial (Ablation)  response?
I can't remember if he said why he thought we would have gotten that initial benefit (besides I jokingly said to him our heart issues are a bit like the shunt issues - we get good results then back slides again to not being effective/not working well enough, lol my body just likes to stay it's status quo I think.. Change? Why would we want to give this girl's body permanent, effective change?! Is how I imagine my body, lol if it could have conversations with itself).
He just commented, as 1 of several comments (but the only I remember) that this area they ablated last month "woke up" after about 8hrs. His hope is if he goes in this time I think he said using 3 cathethers (but not absolute on that part) and he plans to insert one of the catheters in my neck via an artery to get a different vantage point/possible treating point that might give them more efficacy. I cant say I got all of that absolutely right other than I remember the "woke up after 8hrs" (I remember lol) and his mentioning approaching this via both groin and neck this next time.

Shunt surgery 1st... Heart Ablation to follow..
He asked that once the shunt surgery is done and I am released/feel up to it I let him know and contact his Nurses so they could initiate getting the next EP (Heart Rhythm) study set and scheduled.  He asked right away about the shunt stuff and was happy it was being done though he to understands this like the other shunt surgeries likely won't be a long term fix either to our on-going shunt problems. It more he'd like to wait to do this procedure till after the shunt surgery "so you can lay more comfortably flat and isn't as miserable after for you for the procedure and post-recovery" (indirect quote).
I am game with that as last time my Anesthesia dr (as I've mentioned before is the same Anesthesia (airway) dr whose done all 3 of the Cardiac procedures and the 3 Open Heart Surgeries in the past 3yrs) opted to do sedation for the 4hr period in order I would be able to lay flat (I still am amazed by that,  I totally would have said I was awake after). Is so weird the things we don't realize!

So in any case i'll let him and his Nurses know once the shunt surgery is over and they'll work on scheduling/coordinating it. Kind of craptastic this all ends up falling just as the Sunday School year is about to start gearing back up! In any case will figure it out as necessary and if both the shunt and the heart surgery and studies help improve symptoms then bring it on I guess.

Pre-op is mid-next wk for the shunt surgery, will update sometime soon. Thanks for stopping by,


Wednesday, August 17, 2016

Peds Neurosurgery Appt (A plan is had, surgery to explore the shunt(s)), Heart Rhythm Appt. (Thurs)

Sorry for not updating last wk's Appt with the Peds Neurosurgeon;  I had a post typed out but apparently didn't save it right.

Peds Neurosurg. Appt  - Sorting the new (to me, sort of) Nrsgn

I guess the Appt went ok? Frustrating at moments and I feel as if I may have a fair amount of  sharing or helping Dr.Iskandar understand a few things about MPS (primarily the lack of eye-optic nerve not being indicative that other changes aren't/couldn't be related (given occurred each time when there was issue w/shunts) and I know multiple people who've said they or their kids eye have similar where their eye glasses script changes when they deal with shunt issues. I just didn't go and get the script adjusted right away and of the times.
 I'll try to update this as best I can re the Appt though - I was soooooooo tired that day and will admit (not bc of sadness but bc of frustration not that any dr would know the difference and especially one that doesn't know a Patient) but literally was trying to hold back tears as I was trying to explain some things and trying to explain many of the alternative things I've tried or done (from far to many meds, especially before I started seeing Dr.Bragg and some meds more than once to the hands on approach my PT does (counterstrain, lymphatic and myofascial approaches - he always says "I can really help you here and you can chose to do the stretching or not at home (I do). I also do botox and have for yrs + have done Chiropractic (a bit limited there due to my neck and I don't do it anymore as PT was far more successful) + take various supplements.

There where a few things  he brought up potentially trying that I pointed out we've tried and I think he said he would talk  to Dr.Bragg again and would look back at our records. He brought up potentially doing a ETV which I commented is not recommended in us MPS Pts. and he seemed to think might still be an option bc sometimes drs just recommend against things  but they can be done.  - I'm not so sure I'd agree to doing one though. It's something I'm sure we'll talk about amongst other things he mentioned at future appts though.
It was definitely  a long appt (not a bad thing, atleast we both answered each others questions and I think a chance for him to try and get to know me though perhaps a day I am less tired would be better. =)

A Plan was had - Surgery we'll try (Shunt exploration)
In the end he wanted to try still other alternative approaches 1st given we have troubles keeping the shunts working long term (due to compliance issues, Dr.Bragg has always felt the same) and I am willing to try botox for occipital nerves (do I think it will work? No, frankly I don't but i'll try it and my PMR doc is ok with submitting the PA to get it approved - I see them next wk or the wk after so we'll sort out that then.
Still I kept coming back to I felt we needed to do something now/sooner as does my Cardiology Team given the symptoms and our having such troubles keeping INR stable (see below but Cardiology Team opting to stop the Coumadin altogether for now) and so after much back and forth (literally we must have went back to this no less than 3 times until I pushed enough) we are going to do a "shunt exploration" in a couple wks and Dr.Iskandar pointed out on the MRI where he thinks he may find an partial obstruction.

I was waayyyy over tired but ultimately we came up with a short term plan (explore VP Shunt as I mentioned above though thinking about it although Dr.Iskandar showed me where he suspects he'll find a partial occlusion I need to ask him before the surgery date (Sept 6th, pre-op Aug 24th)  if by some chance he doesn't find occlusion at the VPL Shunt if he'll please consider looking at the LP Shunt as the leg-sciatic seeming nerve pain is something fierce at times (thankfully not constant!) and that has always been related to the spine shunt. Is all a little crazy!

No I do not want surgery and is about the worst timing (seriously!) as I start Sunday School that following wknd (probably line up a sub and hope I'm out in time) to still teach. I've missed so many 1st or last days of SS if it wasn't so sad it might be funny!

Surgery and Sunday School  -
I don't entirely know how I ended up in this role but given our 1 Pastor moved to NE earlier this summer to another Church and he is who always coord Sunday School / Teachers Mtngs / Materials, etc. I had worked with him before he left to get our school year materials ordered, and a few other details set so I guess in organizing all that and working with the Pastor there now (our other Pastor, a call has been placed to have a 2nd Pastor again) I sort of just became whoever everyone comes to. I don't really mind and isn't difficult just various things to make sure are set before our 1st SS-Teachers Mtng and the SS year starts next mo.

I am praying this surgery helps and then i'll con't to work with Dr,Iskandar and Dr.Bragg  (yes she;s remaining on my Team even if she's in AZ now) to possibly come up with other ideas to help the shunts potentially work better.
 I am working with Sue the NP to coord. getting the Aldurazyme in-pt as it is Labor day that wknd before (there to, lol it is sadly funny how many surgeries I have had the day after Labor day!?! - not just shunt either).
Given it's a holiday wknd and the friend who used to access my Port from time to time, her daughter no longer has one and she is working I am going to ask Sue if she can help me arrange with the UW Access Team to see if they can potentially come down to pre-op the day of surgery and just access my Port. I am hopeful or otherwise will sort something out as it is sooo much easier to use that then try to get a periripheral or when Anesthesia does also need a peripheral  IV they then can place it once I am sedated/asleep and they have their airway stuff set.
I also talked to Dr.Bragg some about anesthesia issues to see if there was any particular drs she could recall she liked best but I think I am going to bring the info my Cardiac Anesthesia dr. sent me after this last Heart (Rhythm) ablation study that she found with airway changes and what she used.

Blood Thinners - Cardiology
Lastly re the blood thinners, per Jane, Dr.Earing's Nurse bc we've been having such a difficult time keeping INR even remotely stable (it literally is like a rollercoaster though more time than not lately not even close to being in-range) so they are stopping the Coumadin altogether and only con't the twice daily Lovenox injections. Once surgery is done they'll re-evaluate.
As we did with Dr.Bragg and surgeries i'll stop the baby aspirin 5 days prior to surgery and the Lovenox their preference is to stay on it right up till the morning of surgery then  skip that morning injection.
I was driving when we where talking (I know not ideal)  so I couldn't take notes and didn't think to ask (I do pre-op next wk and my Primary dr. is great about getting specific recommendations from Cardiology so she'll outline that in her pre-op note and share with me) but imagine the recommendation will be to restart the Lovenox injections soon after surgery.
Lastly in a message to Sue I asked that we make sure Dr.Iskandar is aware of what Dr.Bragg would do post op surgeries as even with the smaller surgeries it's not always about incision discomfort or solely about that but is about positioning on the table (they generally do great care with trying to use padding, etc) but my body just does not like very many positions and takes a few days to calm back down.
I want to make sure Dr.Iskandar is aware of this and that with my MPS/joint issues our staying on top of post op discomfort for the 1st day or 2 or so is better than trying to play catch up.

 They talked, Glad Dr.Bragg is working w/Dr.Iskandar -
Dr.Bragg messaged me earlier in the wk as she had told me last wk Dr.Isakandar her former Partner/the doc I am working with now asked her to call him. They did in fact talk and she wanted to let me know what they talked about, that she explained to him we've tried literally numerous different valves and she (to him) does not feel adding a 2nd valve in either of the shunts is the right approach as her experience working with me has been I do best draining as much CSF as possible and adding another valve would only stop up what we already have troubles with (shunts draining enough).
I appreciate she's still helping and she can lay out for him what he othe rwise may not believe from me. I feel now a little like I did long ago, trying to convince
I seen him Thurs for a longer appt (per his Secretary) and the original plan was to have 1 of the  the NPs  take off fluid from 1 of the shunts again but he decided against doing that which sucked actually! The relief may only be short term but is works REALLY well for that short term and literally I swear gives my body a much needed, temporary break... The taps only help the headaches for maybe 1.5days at most but still seems to reset some of the other symptoms longer. He's conservative compared to Dr.Bragg was always VERY think outside the box for my care so I appreciate she's seeming to have an affect on him but I wish he realized how good these help even if just short lived!
I really have way to much else going on and don't entirely want to but may call and ask if we could do even just 1 shunt tap in the interim between now and the surgery date. I need some sleep!?!
Essentially what Dr.Bragg told me she and Dr.Iskandar (her former Partner, the doc I am working with now in Peds Neurosurgery) talked about was how my seeming 'normal' pressures for me tend to be to much as I do better with more drainage, or basically what would be excessive drainage for many is where I feel best. She must have brought up to Dr.Iskandar about 1 of the best successes we had with shunts was the very 1st surgery she did in which I ended up with a spinal fluid (pseudomeningocele) pocket that allowed fluid to drain, absorb and do it's own thing independent of the more rigid nature of shunts.  

Oh the blood thinners ?? -
 On  a separate note to all the shunt stuff (but related) Cardiology called Monday with the INR results  and bc it's been low for so long and Coumadin dose was getting fairly excessively high (keeping in mind I am not a big person) Dr.Earing cut the med dose back to 16mgs daily while continuing Lovenox injections 2x's per day.
 Per Jane (Nurse) if level remains low and depending on Thurs's appt outcome (Cardiology really feels its a shunt issue,  Dr.Earing is considering dropping the Coumadin altogether for now and just doing only lovenox as he feels the issue is malabsorption (sp) due to intermittent throwing up. This is all NUTS!
Cardiology had asked that I call them back after the Neurosurgery Appt. to let them know how the Appt went and what if anything was decided which I did (easy enough, lol)  just letting the Nurses know what the plan was, Dr.Iskandar thoughts.
Jane (Dr.Earing's Nurse) when she called back a couple hours later said to me (she knows me pretty well after our working together for 12 yrs now) commented 'you sounded sad or depressed in your message'.
 I probably did though not intentional by any means! I think just being so over tired due to so little sleep especially this wk (has been horrible, actually!) and a little bit having to do with my feeling both frustrated but optimistic (I know how is that 2 even possible, right to feel both emotions) but I just said to Jane in response mostly the way I sounded was just being over tired.
Anyways she commented in response to the effect 'it's been hard with Dr.Bragg who knows you so well, huh?' - she isn't kidding (!!!) though still I am grateful to Dr.Bragg for remaining so involved in my Team, for answering questions I have and for working with Dr.iskandar as I truly think she helping Dr.Iskandar is significantly going to help my care and preventing reinventing any wheel.

A bit of info on ventricles / Brain in MPS or Complex Hydro -
Related to the appt. a little info related to ventricles/brain compliance in someone like my case info on brain and ventricle:
In MPS the Ventricle size or the brain often becomes a rock instead of a sponge which in turn leads to  non compliant, no reserve in brain for sudden changes. A non compliant brain is not adequate to maintain someone headache free all the time and unable to change w/ various activity though in this regard those rarer periods we have the shunts working really well (for instance when we put in a completely new shunt set up) I don't get headaches with movement, spinny things and is mainly onl from laying glat to sitting up. With my  2 shunts they work better but  the 2nd takes some pressure off of the VPL Shunt draining.
Lumbar drainage = independent of Ventricle size, is shunting sub arachnoid  (apparently doesnt affect it 1 way or another) helps the 1st (VP) Shunt w/a narrow range for pressures

Something with the MRI if I understood Dr.Iskandar right is what leads him to believe that is where he may find a partially or completely obstructed area in the shunt catheter based from vent size. Again I'm not sure I got that completely right..

I suppose really there's a lot more I could share but this will already be long enough.

Heart Rhythm Appt - Dr.Kovach -
I see Dr.Kovach, Heart Rhythm doc tmrw (Thurs) and  will try to do a separate update after if anything note worthy. We have since re-started the lower dose Ivabradine a few wks ago with heart rates ranging from 90s-130s so I am curious what his thoughts will be.
Stay tuned!?

Thanks for stopping by,