Peds Neurosurgery Appt (Dr.Bragg's former Partner), Heart rates 1wk ->

Sorry for not updating after Monday's Appt with Dr.Bragg's former Partner - I will admit (and most knew/could have guessed) I was beyond nervous and not sure what to expect given (really) he's so different than is Dr.Bragg.

I truly think her (Dr.Bragg) having talked to him on my behalf helped as the Appt seemed to go well - we talked for almost (I think) 2.5hrs w/short interim breaks as he'd answer calls or see a Patient or two while the NPs tapped both shunts (we could get fluid off, he wonders if instead perhaps there's a small obstruction somewhere in the VP Shunt given (especially I guess) ventricle size has increased (actually really unusual in my case, even when 1st shunted my ventricle size never changed - which isn't uncommon in MPS Pts.  - a newer finding from talking some MPS Parents it appears vent size once shunted some yrs will begin to change subtly (no one really knows why, Hydrocephalus isn't exactly a well studied - if even at all studied issue in the MPS world).

None-the-less as I mentioned above  Dr.Iskandar seemed concerned given my symptoms (intermittent throwing up, nausea, pressure worse at night and morning till I am up for awhile/shunts begin to drain better, vision changes especially) could there be a small obstruction in 1 of the shunts (VP I think he thought but can't remember for sure that's affecting shunt function.

 

He suggested we try re-programming both shunts to a much higher setting (higher setting = drains less) and then immediately after re-program them back down see if there was a chance that could help clear  a blockage if there was one. Was definitely worth a shot (to try) albeit no symptom change.

He also suggested we may end up doing a shunt exploration surgery to see if he could find a problem - there to I have to be honest and admit (but assume) he'd start with the VP Shunt. Not sure.

We're sorting it all..

 

I had completely forgotten about it (really it was I was thinking the Conf was a few wks out yet) when I was seeing and talking to him the other day but the Conf I am supposed to go to next wk for MPS for the Foundation I work for is next wk in Columbus (OH) so I  asked Dr.Bragg what she thought I should do or suggestion. - Granted there are a lot of symptoms going on and no I don't always feel great BUT I've always tried reallllyyyy hard to not let my MPS stop me from doing the things I want/enjoy.   

She always worked with me to try and allow me to keep living even if we where dealing with issues and would frequently tap either of my shunts to take fluid off and give temporary relief if I where going to be traveling. It helps with the flights to wherever I was always going and although Dr.Iskandar is definitely more conservative than is Dr.Bragg she suggested I ask him if he might be willing to allow this. She thought to this would be a good way for him to see the good benefit/relief I get from these.

I talked to Anna the Peds Neurosurgery Secretary this morning who in turn happened to see Dr.Iskandar shortly after and he actually thought it was a good idea (I don't actually enjoy having needles stuck in my head but the temporary relief is AMAZING!!!! - Ah I am Grateful!!!) -

 

- Anna also said Dr.Iskandar had apparently called Dr.Bragg this morning to talk to her about what's going on and get her opinion or insights  (yay to their really working together, again AWESOME!),

 I truly think Dr.Bragg has so much knowledge she can help us with of my case/situation! So that larger volume shunt tap is set for Tues morning (I have a Hand Appt that day anyways so while my hair will be gross (is somewhat hideable due to my dark and thick hair atleast) so the 2 are coordinated albeit different parts of Madison (not to far apart though) just different medical centers.

 

I fly out to Columbus Weds morning.  We'll make final or better decision on what to do longer term/manage this issue once Tues's tap is done. I am ok with this plan. Just grateful he is thinking outside the box and working w/Dr.Bragg!

 

 

Otherwise heart rates had trended back up to low 100s within a few hours of last wks Heart Cath/Ablation though the hope I think was this would perhaps trend back down on it's own. If anything heart rates have gotten higher (110-120s seems average, I try to track them for my Team on an app on my phone, which the Cardiology NP and I had cross checked with their machine a few months back).

Given heart rates are up I called Dr.Kovach's ofc this morning to ask his Nurses if he would consider re-starting the Ivabradine sooner. I see him in Aug. so we'll see. He seemed both skeptical but thought maybe (?)  if we restarted the Ivabradine again it might work like it did pre-Jan's 3rd OHS so either way we'll likely try it again, see if helps heart rate before we'd jump to doing another more intensive ablation.

 

Will update when or if there's anything really to update.

Thanks for coming by, <3

 

Erica

 

Modified Sinus Node Ablation / Heart Cath - Anesthesia (an Update, post [post procedure).

The Heart Cath and ablation study to (to try and slow slow rhe heart rhythm / modify the hearts "normal pacemaker" and measure pressures from within the heart and lungs went ok. Inutial good results which my Team was happy about (heart rate came down to the 80s) but then heart rate by Weds night was back up in the 100s and has stayed there since.
A few of the heart meds have been restarted (ones that work on heart muscle function and secondarily heart rate if I remember right)
After a 1 night stay I was able to go home Thurs (yay) and will fup in a month. Should heart rate stay up as it has been then Dr.Kovach/Team will trial re-starting the Ivabradine which he said there's a chance given it's in a sense a new heart rhythm now it may work as it did prior to Jan's Mitral Valve replacement/Heart Surgery.
He did lay out his plan though if heart rate stays up and the Ivabradine would not work which plan would be to repeat Weds Heart procedures again but in a more aggressive Sinus Node ablation (bigger risk of damage to natural pacemaker and need for actual pacemaker which would not be ideal but he laid out his thinking on how this could work if we did end up in this situation. Given my VPL Shunt runs down the R side and the Port (access device) runs down the L side and the  2 shunts (VPL and LP (lumbar spine placed) are programmable (programmed by magnets) which is also per Dr.Kovach how Pacemaker are programmed it would be a tad tricky but he feels we'd be able to sort this and make it work. I know he's been thinking about this even before the modified sinus node ablation we did on Weds so I trust if it where to happen (but hopefully won't) he and my Team would work it out.

As far as Airway this ended up being under general anesthesia (which Dr.Taylor told me ahead of time in pre-op) and went ok.
When she came by on Thurs morning, the day after the procedures  Dr.Taylor, who I've written about before has done all of my prior Heart surgeries and procedures (6 total, 3 OHS, 3 procedures as of yesterday, lol she was ticking them off on her fingers which was kind of funny) explained what she ended up doing (actual endocatracheal tube placement was really difficult  even with equipment we use to ease placement (glidescope) so she switched to an LMA with a newer Pediatric (kids) size tube/mask and I think she said she had a easier time with that. It seems airway has worsened quite a bit in the period Dr.Taylor has been my Anesthesia dr (at CHW, 3yrs)  as she has pictures for instance from 3yrs ago and must have looked back at those and said the difference was pretty obvious from 3yrs ago (this wk ironically) to Weds's airway changes. Good old MPS, it doesn't leave much unaffected/unchanged even despite the weekly IV Enzyme replacement infusions (man made version to replace  what my body does not make). It's known of course ERT doesn't cross the blood/brain barrier (brain/spine) and get in to the heart or other areas but doesn't always mean it's any less frustrating (if that's the right/best word?) when changes do keep occurring.
Apparently  though I have to admit I do not remember this at all (Dr.Taylor, the Nurses and I where joking in the Cath Lab Weds as they where getting everything set/hooked up before starting anesthesia that I was taking no responsibility for anything I said during the semi-conscious, pre-complete sedation (intubated) period. I stand by that (back in Jan I apparently asked her if she'd take pictures which she did with her phone and later emailed to me. Funny but nice really even if so weird on my part!).

Alas though it seems immediately after the Cath/Ablation and due to concern with my positional headaches and inability to lie flat (as they where getting ready/hooking everything up they allowed me to be slightly propped up) but following the procedure it seems they kept sedation going for the period following in which I was required to lay flat. I completely did not realize this and have to admit, although I was in the CVICU for my in-pt stay (Cardiac ICU) i'd have said I was awake during that 4hrs I was required to be flat. Oh my lord I think (the things one doesn't realize/but does think!?!?)
As I mentioned above I hadn't been entirely sure what she was going to use during the procedures but she did general anesthesia which as she was explaning her intended approach she was explaining our history (her and my) to the surgery Nurse who was in pre-op with us and commented to both of us: "your airway still scares me". - Not a bad thing but kind of funny as I think she was being honest but saying it in a way that it keeps her on her toes with planning for my surgeries and procedures.

 I always appreciate she is willing to do each of the heart procedures and surgeries I've been through and is AWESOME about the endocrine stress dose steroids + always seems to think about the positioning (joint/head) and trying to keep joints happy as possible with steroid/pain mngmt during/post our surgeries or procedures.
I could tell after the procedures it must have been a more difficult airway day as swallowing was fairly uncomfortable post procedure but thankfully has pretty much gone back to normal.. Nothing to horrible but not the entire best either. I just didn't eat much between that and the headaches which crept up pretty bad Weds night.
If I remember right intubation wise when she stopped by this morning (I love docs that do that, stop by either after a procedure/surgery or the next morning, it is always nice to hear their 1st hand account of how they feel airway/intubation went) - anyways if I remember right she said she started w a laryngoscope (I cant entirely remember what that means in and of itself for an airway tube but am going to look it up in a bit). and did not have success with that as she could not get a good visual of either upper or lower (I cant remember which) airway once she'd gotten the airway tube inserted a certain point. After troubles with that I believe she said she abandoned that ship (my words, lol) and used a new Peds (kids size) airway breathing tube which must have worked well and I know with LMA she wouldn't have to place it fully in airway as the LMA sits (if I remember right) at the back of the throat/mouth to keep airway open while they do the mechanical breathing and drugs, etc. I am sure I didn't get a lot of this correct but non the less overall was a lot more difficult this time then it was back in Jan and more reminiscent of other times she's done my anesthesia which gives credence to her long belief that airway in MPS, at least in my case changes over time and from day to day though I cant remember if she's said why she thought maybe these changes occur (much worse some days than others) ? Curious and for sure always interesting when she's talking about it, I don't always remember what she tells me particularly well but appreciate the info.

She told me the LMA type/style/size which I wrote down the size (2.5) but did not write anything else down (I don't know why I didn't) so will either perhaps email her to ask or get copies of the Med records to have on hand for my records anyways.

In any case will update again soon - Dr. Bragg talked to her former Partner on my behalf (I am so grateful to her remaining willing to help!) and they called to make an Appt which is set for this Monday afternoon. I admittedly am nervous but praying given Dr.Bragg talked to Dr.Iskandar maybe he will understand my MPS makes the Hydrocephalus just different? Her having talked to him + the MRI changes, CT Scan changes (done by my Cardiologist for another reason but showed fluid not absorbing properly where VPL Shunt is), the change again in glasses script), on-going headaches and of course the issues with keeping INR stable due to intermittent throwing up (for now Cardiology opting to just keep the 2x daily Lovenox injections due to literal roller coastering INR)   I hope will be enough to convince Dr.Iskandar this is an issue and he will help. Like I said I am NERVOUS! The Appt is Monday afternoon.

Will update sometime soon, early next wk.
Thanks for stopping by,

Erica

PS Ironically it is 1 yr ago today I had the Heart Cath/Heart Rhythm Study (to see if an abnormal area could be found, see if the Ivabradine would be appropriate). - 3 yrs ago tmrw was the 1st of the 2 (in 6wks) OHS. That 1st (and then 2nd ) Open Heart Surgeries where for the Aortic Valve replacement and Aortic root enlargement (Konno procedure, VSD repair/patch).