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).

Heart Cath / Heart Ablation Study 7/20 at Children's Hosp.

There where multiple things I meant to update here about but admittedly I didn't write any of them down and so now I am drawing a blank on what exactly it was I meant to write!

Non-the-less the Heart Cath and Ablation Study (to try and slow the heart rhythm,  (area of the sinus node) and measure pressures from within the heart and lungs + if I remember right Cardiology was going to use Xray and Fluoroscopic guidance to get a better look at the chest wall or maybe it was the lung space or heck it may have been both. I honestly can't remember but that was/is slated to be done while in the Cardiac (Heart) Cath Lab to look at the chest wall area that has been abnormally elevated since Jan's 3rd Open Heart Surgery.

Ironically this Heart procedures are almost exactly 3 yrs (minus 3 days) since the 1st of the 3 Open Heart Surgeries occurred w/the 1st OHS (AVR, Konno procedure) being July 23, 07'. I don't remember the exact date but last yr., (may have been the 21st) we did the Heart cath and Heart Rhythm Studies (to get a better idea for the new Heart rate/Heart failure med and to look at the Mitral valve  for the expected MV replacement, which at this time a yr ago we knew it would occur just not how soon)..

Schedule wise I have to be at CHW at 6:30 with the procedures slated to start at 8. I am a bit surprised (but also totally ok with!) Anesthesia for the prior heart surgeries and Heart Rhythm Study last yr (both the Open Heart and the earlier, separate Heart Cath) asked that I get to the pre-op area 2hrs ahead of time so they could access Port there (my Anesthesia dr ok'd my coming in with it accessed and said she plans to place an actual peripheral (in the vein) IV once they start Anesthesia as that is more practical for her to use.
I am game with that, just glad she's letting me use the Port initially as IVs themselves don't bother me it's the fact that more often than not placing an IV is like a hunt and search (my veins look great but roll and often the vein will blow (collapse in a sense)  when trying to get IVs in. Sometimes the docs have better luck but since I had the Port and had Infusion today I'd rather they use this initially and do their hunting (for a good vein) when I am out. =)

I am also glad it's the same Anesthesia dr I've used before as I could have done these procedures a wk after my Team decided we where going to go ahead but opted to wait so Dr.Taylor would be available. Many other MPS Individuals or Families understand this (having an doc familiar w airway is 1/2 the battle with any procedures or surgery).

Otherwise it's mainly just getting the Steroid 60mins before the procedure starts, signing consents and going over everything once I am there.

 If you would please pray for all to go well and this to in fact help bring heart rate down! The biggest risk both bc I've had so many Heart surgeries and in MPS our anatomy tends to do it's own thing is this procedure causing need for a Pacemaker (my Heart Rhythm doc has mentioned the potential several times) but I'm not to worried and if it did end up with that well then we'd figure it out in the setting of the programmable shunts and the need for imaging for bone/spine/brain in MPS.
I am staying on Coumadin for the procedures though my level  (big surprise lately, I guess, right?!?) was low (even lower than last wk) at 1.78 or something around there.
Once the heart stuff is done my Team will adjust the dose and we'll once again try to figure it all out. - Dr.Bragg did msg that she received a message from her former Partner and he is going to ask his Office Team  (her former Team)  to schedule an appt "ASAP". - I am truly grateful to Dr.Bragg for her help with all this - still nervous but thankful to her!

I'll try to update Weds night or Thurs., for info on the actual procedures see my last post.
Erica

Sinus Node (Heart) ablation - Weds, 7/20, Shunt stuff cont's

Heart Cath

http://www.chw.org/medical-care/herma-heart-center/programs/cardiac-catheterization/

EP (Heart Rhythm) Study:

http://www.chw.org/medical-care/herma-heart-center/programs/electrophysiology-program/

See below for an article on the Heart procedure next wk - it is  not specific to what will be ablated (killed/burned basically) in my case but the area being located and ablated/killed next wk is the Sinus node or the heart natural pacemaker.

The risks with this or the biggest risk really I guess is the procedure causing to slow of heart rate and thus a need to place a pacemaker (basically a device that controls/mainrains the heart heart rate/function )

Hopefully we won't end up on this route as it would for sure make things like MRIs (which are needed for the MPS bone/spine issues that occur and MRIs help us with things like the Hydrocephalus mngmt  when it comes to surgery planning BUT as Dr.Bragg said a few mo's ago if it did come to this then we'd just have to go to CT Scans and deal w the excess radiation that goes along with these.

 

Anesthesia wise we are using the same Anesthesia (airway) dr  i've had for all 3 of my Heart (OHS) surgeries and the 2 prior heart procedures in the past (now 3) yrs but actual airway wise I am not entirely sure what the plan is.

I know they have the Endocrine (stress dose steroid) requirements from my Neuro Endocrine dr so I guess given it's the same anesthesia (airway) dr as prior and same Heart Team as prior im not to worried and am sure she'll (Dr.Taylor, et all) will  have a good plan.

At this point the plan is to leave my Port accessed after Tues's (ERT) Infusion and use that for initial anesthesia then once under Dr.Taylor or her Team will insert an peripheral IV as she said the Port isn't ideal for use for this procedure.

 

I'll stay on Coumadin for the procedure, so I am not entirely sure how that affects recovery BUT hope they'll let me atleast lay with my head propped up some in recoveryrecovery after the procedures if I keep my lower body flat (to prevent bleeding, especially due to INR will likely be high ( thin blood). - We've had issues keeping INR stable due to the pressure issues but have figured out a few tricks that atleast help some so we'll see where it is when I re-test Tues.

As long as no complications it's expected to be just an overnight stay at CHW. 

 

Below is a mix of copied from an article and my own writing so if it seems weird the would be why! =)

Article on Heart Rhythm (EP) Ablation Study

Related terms: cardiac ablation, radiofrequency ablation
.

Electrical impulses from the heart muscle cause your heart to beat (contract). This electrical signal begins in the sinoatrial (SA) node, located at the top of the heart's upper-right chamber (the right atrium). The SA node is sometimes called the heart's "natural pacemaker."

 

For most people with arrhythmias, medicines work quite well to control the abnormal heart rhythm. But when medicines don't work, doctors may suggest catheter ablation.

 

The procedure is performed in the Cardiac Cath lab) or the Electrophysiology Lab.

 

Once you are in the cath lab, television monitors, heart monitors, and blood pressure machines. You will lie on an examination table, which is usually near an x-ray camera. Small electrodes (EKG) will be placed on the chest. These electrodes hook up to an electrocardiogram machine which monitors your heart rhythm during the procedure. 

 

After gaining entrance into a blood vessel in the groin, arm, or neck, doctors insert several long, thin tubes with wires, called electrode catheters, through a sheath and feed these tubes into the heart. They use a video monitor (like a TV screen) to see the process.

. 

To locate the abnormal tissue causing arrhythmia, doctors send a small electrical impulse through the electrode catheter to activate your arrhythmia. Other catheters are used to record the heart's electrical signals and locate the abnormal tissue.

Doctors will then place a catheter at the exact site of the abnormal cells in your heart. The radiofrequency energy is sent through this catheter to cauterize the tissue causing your arrhythmia. 

 

A Heart Cath will be done initially to measure pressures in the various areas of the heart and lungs before the Heart Rhythm study is started. 

From the last Appt w my Heart Team they had planned to do some fluroscopic guided views of the lung space or maybe it was the area around the lungs to better view this. I admittedly am not 100%  sure what that was but using X-ray guidance to view the abnormally elevated chest wall if I remember right and perhaps the abnormal fluid spaces.

Given the VPL Shunt drains to the 1 Lung space I'm not to sure off hand how that works though at the time it was explained it did make sense.

After the catheter is removed, firm pressure is applied to the site the  groin to stop any bleeding.  To avoid bleeding at the catheter insertion site, you will need to lie very still for several hours, either in the recovery area or in your hospital room. 

 

Catheter ablation usually takes 2 to 4 hours. If you have several areas of abnormal tissue, then the procedure may take longer.

---

Otherwise nothing new in the shunt saga which if its not already been obvious has been aggravating! I don't see the other Peds Nrsgn (Dr.Bragg's former Partner) till Aug. 11 which  given 1 of the shunts isn't working right and we almost virtually know it given both the scans and the CT Scan is nothing short of frustration there!

I asked Dr.Brahh her opinion on this if she tight her former Partner would atleast let the NPs do Shunt

 Intermittent shunt taps till I can see hmm and she was not thrilled this has still not been resolved.. I had an eye Appt last wk to have glasses script increased yet again (2nd time in a yr) as the shunt/pressure issues really affect that. 

Dr.Bragg in turn messaged me back and was going to send another msg to this former Partner, see if he'll see me sooner. She strongly seems to feel this needs to get taken care of sooner than later which its been going on for mo's and several different scans show there's an issue not to mention vision changes amongst other things  so I don't disagree with her!

I really don't want it to come to that  (as much as I like Dr.Bragh and hated her moving to AZ, not being here) but numerous if my Providers really feel at minimum of should contact at least some care w her once she has "Adult privileges" at her new hospital system. It's. A mess right now we the shunts and my care though certainly not for lack of Dr.Bragg trying to help,  she's been amazing and persistent when there's no reason she really has to be!

Dr.Bragg and I have talked about if really needed once she gets privileges  at the Adult hospital affiliated  with her new Hospital I can see her in Phx which I am glad that option is there and I really like Dr.Bragg of course but what a pain (seriously) it would be to have to trek out there. I can remember the 2 times I had surgery in MN which was at least semi close enough my Parents came and then they went home from the 1 surgery for a day or 2 (about a 5hr drive 1 way) and my Dad came back to get me. That was likely one of the more memorable drives for him given a huge snow storm cropped and 5hrs turned in to far longer on his way to MN.

I have friends in AZ of it comes down to it. A now eine really! 

It will suck having to break in a new Neurosurgeon if this new guy coming to UW works out but I also love my Nurses and Team on D6-4 (Neurosurgery at UW) so am praying. I can always do w/ Dr.Bragg like I do with my Genetics dr, see her maybe once a yr but in mean time just have to pray the new guy really is a nice guy and interested in complex cases with underlying genetic dx.

Ironically I ran in to my Neuro Endocrine Dr  (she works with the Neurosurgery Team at FMLH,  where I get my wkly ERT infusion and where my 1st Neurosurgeon was) and 1st thing she asked me when she stopped me  (I'll  admit I was in my own world, on a mission to go home so not paying attn when she walked up to my heading to the elevators:) ) but literally 1st thing she asked was "have you found a new Neurosurgeon yet " which we talked some and she comments along the line "We'll why don't you just see her in Phx?"

I am just praying Weds's ♡ procedures go well, we can figure this shunt stuff out soon and overall the new/former Neurosurgery issue works out best however it's supposed to work out.

Will try to update later in the wk after Weds's Heart stuff.

Thanks for stopping by,

Erica

Appt for nothing (Adult Nrsgn, No where's fast?).. Back to Peds Neurosurg.. / Cardiac Heart (procedures) 21st.

I think the best way I can describe the last wk since the Appt with the Adult Nrsgn is below, what I'd shared with my family.- FRUSTRATION essentially!

I generally get copies of most medical records so I can give them to drs who wouldn't otherwise receive/see them and this way keeps the more "Core" group of  docs up to date re the various issues or going on.
1.5 almost 2 wks since the Appt   I've not yet heard from the Adult Neurosurgeon I saw last wk though I know they did receive the CT Scan images from CHW  Tues last wk. These where uploaded Weds but heaven only knows when they'll be viewed if at all and not sure it really matters as reading this drs note I'm not sure he actually really heard any of the info I told him and wrote down in the new Patient forms (he was even reading back some of the info to me, confirming  various things!?!?) 

I almost never do it (and after writing down notes delete any recording right away) but I recorded that Appt  and I know we talked about the things that are very different in MPS from more typical Hydrocephalus Pts. He even agreed to some of the pts. yet the dictated note says multiple things about optic nerve size hadn't  changed (optic nerves NEVER change!), ventricle size was stable from the prior (Ventricle size on that 1 as noted by the Radiologist was a small change which on the rare occasion there is any ventricle size change such as prior to the 2nd shunt being re-replaced (put back in) last Nov. was also the slight increase in vent size  if I remember right and IS significant for me!).
Anyways it's like the clinic  note was completely oblivious to anything this Dr and I actually talked about including the headaches having gotten worse over the past few months (unfortunately is my case most often, the symptoms will start out more subtle and gradually get worse over mo's). The intermittent throwing up, H/As that are worse at night and morning but improve as the day goes on the longer I am up. Honestly I read the note and just thought (pardon word choice) WTF?!?! I was both pissed and so frustratedI 

Literallywas in tears but tears due to the frustration NOT of anything else!

In reading his note so I could scan in and send to a couple of my Providers I just was a little dumbfounded actually. The note says virtually nothing about the throwing up, but "mild/light occasional headaches" (lol as a friend whose an MPS I Mom said "if daily headaches is "mild and occasional okkaaayy" ) will arrange to have her f/up w Pediatric side".

I know the Peds Neurosurgery Team seemed certain he'd be the one to mange my case long term but I wondered about that and now even that doc said to me and said right in his notes he  was just seeing me since the Peds Nrsgn was out of town but would ultimately send me back to them for mngmt.

I don't even think it's that this Nrsgn I saw isnt a good doc I just (really) think Adult Neurosurgeons deal with Congenital issues so little (?) that my providing the MPS info and emphasizing it apparently went over his head (I don't mean that in a air headed way I guess I mean it like those Adults docs just rarely get our issues with MPS are NOT straight fwd)?

I have been at a complete loss on this and  almost as frustrating is this Dr said to me last wk at the end of the Appt,  "I'll  review the scans and get back to you, we'll come up with a plan" yet I've heard nothing from him?? 

Seriously the least a freaking Doctor can do is call a Patient and let  them know they don't know!?!
We as Patients get labeled in all kinds of crap ways if we don't show up for Appts or for various other things but drs if they don't communicate? Apparently that's ok?

I've talked to his Secretary a couple days in a row earlier last wk but finally just gave up.

It's super nice to feel like you belong no where bc neither side apparently wants to manage care?!?!?

I've said it before but  there's a reason even my Cardiologist is at a loss re  replacing Dr.Bragg. 
Adult Neurosurgeons just don't deal with Hydro much much less genetic/complex  issues and always obvious.

 

I appreciate Dr.Bragg is trying to help  and said she'd talk to /send a msg to her former Partner about my case being less than straight fwd but I feel guilty for keeping needing to even ask her for her help! Sucks seriously needing our docs so much!!! I HATE IT MORE THAN I CAN EVEN SAY!

 

All i could seriously think when reading that clinic note is 'seriously, people just throw up for no reason? Vent size changes for no reason?' And optic nerves for pete flipping sakes it's written right in Dr.Bragg's and the other (1st at UW) Nrsgn note that orptic nerves don't change w MPS not to mention this doc and I discussed that very thing in detail! Have you picked up on how frustrating this has been????

In all honesty in addition to being so frustrated and in general tired it literally feels like I'm back where I was  pre-Dr. Bragg (searching, my drs knowing there's an issue but wait, uncertainty, wait). -
I can't explain why and is irrational actually but I feel like I'm letting someone down  (who I don't know) by even needing to keep asking Dr.Bragg for help and for needing help in general w this with the shunts! Like I said, irrational I know but I still feel it.

This in turn makes me  keep thinking about 'why no doctor/doctors in our MPS Community actually study these MPS Hydro (shunt) issues??? It's not thee most common presenting issue nor does it occur in every MPS I/other type of MPS Pt. but it is an issue that's not at all well understood!?!?

 

I know I keep  thinking about the seeming  (apparent from CT Scan) fluid that's building up or not absorbing properly and how symptoms now seems to mimic /mirror other times we've  dealt with 1 shunt working and 1 not or 1 of 2 shunts not working completely  and that I feel (as I have for mo's) the issue seems less like it's the  VPL Shunt but seems more like the LP Shunt isn't  working right and thus any excess fluid in VPL is likely similar to other times where 1 shunt can't keep up with what 2 normally do? Or maybe it is the VPL Shunt but partial issue or who freaking knows but what I do know is I wish to God Dr.Bragg where still here so she could help!

 

In all of this  I feel like  the difference in Peds Neurosurgery  and Adult Neurosurgeons is alot like the  difference in Adult Congenital Cardiologists (what mine is) vs regular Adult Cardiologists.
Adult Nrsgns don't deal with the rare/complex Congenital issues enough to get its not straight fwd or simple or by any book.

I am super frustrated and feel like I am back where I was before I found Dr.Bragg  (except I know she's still trying to help just isn't easy) and that this frankly scares me not knowing will the new guy be willing to take on a complex case/work with my Team????

IAggravating!!! Dr.Bragg said something along the line "they need to listen to you and help you" - don't I know but again appreciative to her.

 

In any case the Cardiac (Heart) Cath Study and Ablation is next week Weds, 21st. I'll stay on my normal dose of Coumadin (range 2.5-3.5) for the study, same Anesthesia Team and is an expected - as long as all goes well - 1 night stay after. 

INR has been all over the board between to low and in-range so either way they aren't stopping the Coumadin for the study.

 

Will update again soon - please say a prayer we can sort something with the shunts soon, otherwise I couldn't get in to see the Peds Nrsgn until August 11. ---> Like I said, it's the never, ever, ending issue!

  If I sound frustrated I am but life also still goes on which isn't a bad thing.

Erica