Potential plan re Tues shunt replacement.. to be decided..

I guess if nothing else this past 2.5wks has again validated re shunts that I know what I feel and so many around me have commented they could tell a difference in that it was so obvious to them I didn't feel good and that they hope whatever we do this time works better.
Dr.Bragg has said she felt the TPL Shunt wasn't working at all and that the VP Shunt was not working optimally. I feel like this should be validation to myself to really stick w what I think is wrong.. I know Dr.Bragg was helping me try to stay on top of the headaches and I was/am very grateful to her for that but I do wonder if I should have pushed harder, sooner to look at the TPL Shunt? I guess at the time I wondered but I also knew that shunt wasn't exactly the easiest to test.
Per Dr.Bragg surgery to take out the EVD (see below depending what we decide to do) and replace the shunt(s) is officially scheduled for Tues morning, 1.5days basically!! Even when she rounds she has jokingly teased about my well known excitement at the countdown and potential end in sight to this shunt stay..
I am counting and waiting and as sad as that sounds (it really kind of is, lol) I am excited for Tues. Not so much for the surgery or discomfort that goes w it but for getting freed from Neuro ICU, not having the external drain (see below, depending what we ultimately decide to do) and being able to wash my hair soon. Right now I've been able to wash parts of it in the sink which is sort of gross in itself but is by no means ideal and could use a regular shower! I may just be thee happiest camper around when I can shower and wash my hair again! Ideally I could wash my hair normally before surgery Tues but fairly certain that isn't going to happen.
As far as what Dr.Bragg is thinking for surgery she had been talking about putting the 2nd shunt back in to the Lumbar space albeit I think she would still have routed it to drain in the pleural space though that was 1 option she was considering and I knew by no means the final decision. She had also talked about Valve wise for the shunts a newer valve, made by Medtronic I believe that is similar to the former Medtronic Strata valve we had used at 1 point but she said this one had more settings and if she could get it she felt may work well.
Today (Sun) she came by and had a different thought after talking to her partner, whom I may not like but I appreciate they can swap ideas. Anyways so now she asked me to think about and consider just putting 1 shunt back in place, in the lumbar space (bc I seems to have the most amount of CSF there) and would drain to the peritoneal cavity but we would atleast initially try only placing the 1 shunt again. She commented in a way it would be like coming full circle since when I started seeing her 4yrs ago I had 1 shunt, though a medium pressure valve in the lumbar area.
Valve wise she brought up a prior small valve we'd previously used when we tried having 2 valves on the VP Shunt, I can't remember for sure but think it is something like a 'shunt assistant' and acts more like a barrier to CSF backing up in to the body (say an infection occurs they prefer to have some sort of barrier to preventing that infection from re-circulating I think it was) than it does a valve. Ideally we'd go without a valve but given infection risk it's not an option she really wants to entertain which I can understand.
She did say flow wise using only this very small, device that is a valve but yet isn't really she thought we might be able to get really good high flow drainage (lower setting essentially then even the programmable valves we've used albeit it isn't programmable). This is 1 thing that may be ideal for my body as we know I feel best when I drain a lot.
She said she would likely leave the EVD in place but clamped this way if we ran in to problems like 2.5 yrs ago where I couldn't tolerate 1 shunt we could un-clamp it until we could go back to surgery to replace the 2nd shunt. .. It seems she's really leaving the ball in my court and just said to message her with any questions I have + she would respond. She is not rounding tmrw so I am trying to think this over before I message her anything.
In reality I've had decent results w the Codman Hakim valves is just a matter from our experience they don't work for more than a few months in my case and this last time in Winter we really didn't get great results but perhaps the TPL Shunt was already failing some then? Back in Nov. after that revision I'd felt the best I had in a long time.
I have to admit I am not to keen on coming back to Neuro-ICU nor having to do a 2nd surgery (3rd really this stay) if 1 shunt with this alternate valve didn't work and I feel a little skeptical BUT then I think what if it did work? I really don't worry much about it overdraining as I can literally dump 10-15cc of CSF at a time and feel fine and if we've kept the EVD (drain) leveled I've been waking up feeling much better the past couple days.
I so appreciate that she trusts me to think it all over logically but I also admittedly feel like I know what the right thing to do is (try 1 shunt and keep EVD clamped) but I don't want to do it, lol b/c I want out of N-ICU, to shower and to have a better end date in sight!
I've been talking to my Nurse today who I really like and 2 things she also thought I should ask where 1. how long would we know before 1 shunt was enough or we needed to put back in a 2nd (I think last time a different valve we may have known by the 2nd day but can't remember for sure.) The other question she thought I should ask is, say the 1 shunt isn't enough how many days would we have to wait to go back to surgery again for the 2nd shunt to be replaced?
I am unsure what's up w the potential gallbladder/General Surgery issue ie is she having them look at the area or no during surgery Tues. I guess it doesn't really matter to me it's more that I do have a consistent side pain (but that seems a oddly atypical gallbladder symptom) though it is only apparent when I breathe. I am sure whatever she decides she'll have put thought in to it and talked to whoever she needed to get the best opinion on this.

On a different note it is sort of funny but having talked to a few different friends whose kids have shunts this wk, when they've pm'd me all remark repeatedly if they where in WI they'd have their kids going to Dr.Bragg bc "she sounds like such an awesome Neurosurgeon". Anyone who deals w this specialty knows how uncommon she is personality wise and in that she genuinely seems to care.
A lot of Nrsgn's even the Peds ones don't have the best bedside manner and not always willing to take in to account what the Patient says/feels. She definitely has an ability to think outside the typical 'Neuro' box.
When my local Genzyme drug rep visited at the hospital last wk she happened to be here when Dr.Bragg rounded and Carol said to me after they all figured she was great but she couldn't believe how down to earth Dr.Bragg was, talking about her life outside work and Carol said she could clearly tell she cared. She was impressed =)
Ironically after that she (Carol) was going to talk to one of the Metabolic Genetics drs here at UW that several MPS pts and a Pompe pt where having issues with w/basically being unreasonable about how he'll treat pts/what he'll allow them to do infusion wise.
I'll update either before surgery if I know a certain plan or if nothing else on fb and then a post-surgery update here following surgery.
Wish us luck if you would, say a prayer,
Erica

Neuro-ICU, still externalized.... booorrreedddoommm

Not really much to update, just hanging out here in Neuro-ICU till the CSF infection clears/we get the all clear to replace both shunts.
Per Dr.Bragg who works with Infectious disease drs (who in turn round each day and I have a chance to ask questions or just get their perspective) we do have a clear CSF cultures and now is just a matter of waiting 7 days to make sure cultures stay clear. This would bring us to Fri so Dr.Bragg was going to look at her schedule and likely we'll put the shunts back in place next wk. Unfortunately next wk is Memorial Day wknd which means yet another day here. By the end of each day I am about at the end of my rope! I like being left alone and there's a reason I live alone!
Since Dr.Bragg talked to them and explained my pressure situation the Nurses have been much more laid back about my getting up and moving around leaving the drain un-clamped for short periods when I stand to dump more CSF (which in turn provides greater relief of symptoms).
Sunday night I went to sleep (have been sleeping in recliner) feeling ok, not perhaps awesome but atleast ok. I woke up at early morning, around 2 throwing up and with a bad headache which kept on throughout the rest of the night till Monday. When Dr.Bragg came by she lowered the drain to -5 which has helped significantly with symptoms and my standing up with the drain open for short periods = between these 2 things feeling the best I've felt in awhile. There are many times I fall asleep despite it being in the recliner that I wake up w a headache but this I can get on top of with standing up to let the drain open and release bigger amounts of fluid.
Now if only we could better replicate this w the shunts! =)

All day Weds I had been thinking it was Tues when really it was Weds but even that I could deal w surgery next wk, thinking maybe it would be on Monday though I do know that is a clinic day for her. What I had completely forgotten about is that this wknd is Memorial Day wknd so my guess is surgery will be Tues or Weds... Hopefully tmrw Dr.Bragg will have an idea but honestly this realization was really, really depressing! Being stuck here on a 3 day wknd is really going to suck. It is also the 1st wknd that Cedarburg Flea Market is held (held 4 times a summer/fall) so will miss that.
Probably in reality it all wouldn't be so bad but some of these Nurses Neuro-ICU drive me crazy, mostly bc some if your asleep will do their ICP # check (every 2 hrs) and the vitals (every 4 hrs) when your asleep and I am not a heavy sleeper so never sleep through this. Some nights are not bad at all given some Nurses will skip 1 or 2 of these favoring sleep over #'s and that really makes a HUGE difference on how the next day is. - thankfully this atleast was changed to everything only has to be done every 4 hrs and at night only at midnight then not till 8am.

I am hopeful when we replace the 2 shunts it will equal having 2 working shunts in place should equal feeling good for awhile!?!
I keep forgetting to ask Dr.Bragg if she has decided on staying w the TPL Shunt or if she's still thinking about putting the shunt back in to the lumbar space where we did seem to have better luck and if we'll stick w 1 shunt draining to the pleural space. I've not heard otherwise so assume she'll go back in to the pleural space to drain with 1 shunt and 1 draining to peritoneum.
Otherwise it's just been a really, really long week! Will update sometime soon,
Say a prayer surgery is early next wk and I don't die of extreme boredom this wknd. =/
Erica

Neuro-ICU, shunts removed, EVD. drain. Am extremely boring!

Yesterday's shunt removal surgery went ok, per Dr.Bragg the TPL Shunt wasn't working which maybe this explains the change in leg-nerve symptoms and overall how i've been feeling nerve symptoms lately but also explain why Headaches had been much worse. It does make me wonder when we had the sudden, severe shunt failure in Jan, could the TPL Shunt have not working then already?
Dr.Bragg was able to remove the VP Shunt and replace both shunts with a temporary EVD which is why I am in Neuro-ICU.

In order to get the shunts removed Dr.Bragg had to remove 3 connector pieces during surgery in order to be able to pull out the catheter. I couldn't remember why we used them at times but Dr.Bragg explained bc my skin is so thick and hard to tunnel catheters straight through she often had to use these connector pieces to get the VP Shunt in to the Peritoneal cavity (where it drained) and get it all from brain/ventricle down neck/upper chest and on down to abdomen.
She also said there was a brownish crusty (I think that's how she described it but ) material at the TPL Catheter (at the end part of the TPL Shunt. I think per ID drs and Dr.Bragg they cultured this shunt catheter and send it to lab to make sure no infection or that there is not a 2nd infection going on?
In any case we have the External drain in place when they took out the shunts Fri, (yesterday) and I am in Neuro-ICU till infection is cleared and we can put both shunts back in.
The actual infection found earlier this wk is not yet cleared per Infectious Dis. drs, though everyone says it should be a relatively easy bug to treat and get rid of. It is the same infection I had 2 other times.

there are 5 incisions, 2 on my back, 1 on collarbone and 2 on my head. Sad part about that being my hair had finally gotten to where the new growth was long enough it didn't stick up under my other hair anymore.
I know it's onlt hair you have little control over anything else in a hospital hair had just gotten to the pt from the last revision in Jan that it didn't stick out from the rest of my chin length hair! I know it's vain but little things like that matter when
Last night and this morning I was pretty tired so needless when the Access Team person came and woke me up at 4 this morning I sent them back out and asked that they come back later. Vitals where every 2hrs last night and all machines where hooked up, with everything going off every 1-2 hrs.
I also had asked the Nurses if we could change the setting on the EVD and they where like 'oh no we cant do that you'll overdrain. Having been through EVDs before I know how they work and thankfully Dr.Bragg told the Staff here I could get up on my own and clamp/un-clamp the drain on my own.
Definitely glorious to have gotten the turban wrap off this morning and to get un-hooked from all monitors minus the ICP monitor. A little independence goes a long way! l feel crabby today but no where near yesterday and I took a nap which helped a little and hopefully will sleep atleast semi-ok tonight!

I don't know what Dr.Bragg's plan is re: replacing both shunts other than she thought we'd replace both at one surgery (we did it a bit differently last infection 2.5yrs ago). I am not sure if she'll attempt to tunnel the catheter back in to Lumbar spine again vs where it currently sat in the Thoracic Spine. I can't imagine she'd want to put both in to the same (peritoneal/abdomen) draining area.
Pharmacy needless to say is driving me bat.s**t. crazy. First lets pump someone who normally restricts how much they drink and pump them full of fluids. It's not so bad when fluid is very minimal through the IV per hour but at times this has been ALOT of fluid was being given.
I'm not sure what these Residents think but gee if someone is on high dose Lasix AND Spironolactone wouldn't that suggest they shouldn't get a lot of unnecessary fluid? I do have the PCA pump so a small mount of fluid has to be given but not the high doses of fluids they are running in.
To me a patient being on those meds would imply their hesrt is working overly hard already, fluid only makes worse these symptoms!
To top it off the N-ICU Pharmacist changed up the way my meds are given during the day (there's a reason why I take certain ones at certain times of day!) Triple pissing me off is the Nurses and Pharmacists keep saying "oh we can't give you your cardiac meds and especially the Lasix/Spironolactone bc your blood pressure is so low?!? Hello my Cardiologist has me on these meds for a reason and low blood pressure is not his worry! Today they wanted to not do any of the water meds (Lasix, spiro) and restart it tmrw. WHAT?
Believe me I brought up every single reason why I am on all these cardiac meds and why, no we can't just skip them. I have enough cardiac issues already much less making it worse!
B/c some of the meds, especially the Lasix/Spironolactone where omitted yesterday and yet I wasn't asked or even told about it till I asked if I could please have these before 8 or 9 tonight! The Pharmacist finally is calling the Resident but let me tell you if they don't given it it's quite possible I'm going to ring someone's neck be it Nurse/Pharmacist or Resident!
It is 8:15 now and they are just sorting this out so I'm sure much sleep won't be in my cards for tonight due to having to get up so much to pee!
I just don't understand why this Pharmacist(s) wouldn't 1. follow the D6-4 med plan AND talk to the Patient re any changes, most of us DO know our bodies far better than some dr that's met you a couple times at most!
In any case if anyonreading this hasn't figured it out I HATE being in Jail (aka ICU) due to so many restrictions. In any case, not the most positive update (atleast from an I hate this perspective) but hopefully each day in here will get a bit better.
Thanks for stopping by,
Erica PS: Thank you to everyone that wore purple yesterday, sent pics and or checked in to see how things where going. Is funny how friends who are long distance whether MPS or not tend to be more supportive than some of my family and for that matter friends at home. I'll try to post pics soon in a different blog post/update.

Infection; Surgery to remove all shunt hardware. Fri. 5/15, MPS Awareness Day!

We've been treating with IV antibiotics this week, after Tues's positive shunt tap result (which was confirmed in another tap and lab test) the antibiotics where switched to one more susceptible to this particular bug.
I haven' felt particularly great so have spent ALOT of time in the recliner I have in my rm., sleeping in this is easier than in the hospital bed. I do have the PCA (patient controlled pain control button) which along w morphine, (longer acting pain med) and the Pain patch I have on daily has helped a lot.
The 1st surgery is scheduled for tomorrow to take out all shunt hardware (2 shunts) and place an External drain to control headaches until we have the CNS infection under control and can replace the shunts. This likely won't be atleast till mid-next wk or as the ID dr. stated "down the road". LOL, hopefully everything heals fairly quickly.
I can't remember for sure but I think they ID drs and Neurosurgery like to wait for 3 days of negative cultures in a row before they schedule surgery to put all hardware back in place.
Infectious Disease just stopped by (they come by daily to in addition to Dr.Bragg) and they believe the fluid collection that is at the base of the VP Shunt is likely part of the infection. They'll obviously do the IV antibiotics but before replacing any hardware said they'll repeat an ultrasound to monitor the CSF collection and make sure it is gone/no residual infection if that is what the fluid is caused by.
At least when over here on D6-4 although it is boring to I know the Nurses and Patient Care Assistants so they help keep it slightly less dull. Several have promised to stop over at the N-ICU to say 'hi' so that is nice atleast.

Sunday School
Unfortunately this Sunday is our last Sunday School class for the yr., I am bummed to be missing it and especially given this was my 1st yr being there on the 1st day! So much for starting and ending the yr.
Some of my kids will be in VBS though 1/2 of my class moves up to the next Teacher (5th/6th grade SS Teacher). I'll of course see some of them in Church to when back. I talked to a couple of the Parents and told them when I am out of here i'll get the kids end of yr gift to them (photo album with pictures from the last 1/2 yr).
I guess God has a reason for everything, will update again sometime after tomorrow's surgery.
International MPS Awareness Day
I did get a text msg from my Genzyme Case Manager (she coordinates Insur. from Genzyme's end) earlier this wk, there is a banner hanging from the top of Genzyme Center's mainsteps (a huge flight of stairs running from ground fl to 1st). Anyways the banner is for Intrn'tl MPS Awareness Day and of 3 of us with MPS I. Neat!
Please wear Purple tmrw for MPS and for myself, I won't be able to given I have surgery so am asking those around me if they will! If I had purple ribbon I'd atleast tie that in my hair after surgery but don't have any. I also have purple ribbon pins at home but not entirely sure where those are or I'd have had my Mom bring some!
Take care,
Erica

Local ER, PCP, UW-ER, Admit to D6-4 (Neurosurgery), Shunt Infection and?

May 12th - Updatung (see below older update)
See below for backstory on this update ie what has transpired since last week and how I ended up in-pt. Today an EGD (upper scope of the stomach) was done which I don't have the results yet.
I got back from this test and shortly after Dr.Bragg and her NP Emily came by and had some news (rather un-expected to both of us I would say!) that the CSF shunt tap that was done Fri and sent to the UW Micro Lab came back positive for the slow growing CSF bug Dr.Bragg had mentioned but neither of us really thought would be the end issue. (A Shunt tap draws off spinal fluid, the stuff my Shunts drain off my spine/brain).

Given this positive result we'll have to remove both shunts do a week or so of IV antibiotics while they have an 'External drain' in place (this acts as a internal to external way to drain CSF) until we get the all clear.
Once the infection is cleared we'll schedule another surgery and replace the shunts internally. Dr.Bragg mentioned she is considering moving the TPL Shunt back from it's current location to where we formerly had it in the lumbar spine given we did have somewhat better results w it working better when fluid drained from there to pleural space.
I'll update on all of this hopefully sometime in the next few days, Dr.Bragg said the shunt externalization would be the 1st case Fri and as far as the gallbladder issue I can't remember for sure but I think she said if they want to she will try to coord. their doing that with this surgery.

Local ER, PCP, UW ER and Admit to D6-4 (Neurosurgery)

Last time I signed off of here I don't think I had any clue the days after would be quite this eventful, wow!

See below for what's going on but talking to Dr.Bragg this morning about General Surgeries plan and tentative thoughts she said to me (which in turn made me almost bust out laughing albeit that hurts like heck to do!):
"Your body reads the 1st sentence of the manual and then makes it's own rules" - Dr.Bragg
So funny and kind of pretty darn true given not much ever presents classically!
I was in the local ER Thurs morning, due to side/abdomen/back pain that started earlier in the week as intermittent and turned out to be gallstones apparently. I honestly had had not idea what it was just that it hurt really bad and since Monday, the beginning of the wk. the symptoms only getting worse.

The opinion of the ER doc and Radiologist at BDCH was it was 1 large gallstone and would not pass on it's own so I needed to get surgery as soon as I could but the ER doc said 1. There's no way we would do it here given my complexity (nor would I ever have surgery there optionally) and 2. You'd probably have to have it where your Neurosurgeon is due to your shunts and especially due to the VP Shunt end runs right by where they go in for a gallbladder surgery.
My mistake when at the local ER was NOT having that dr. talk to Dr.Bragg to try to better sort this out and where did it for sure need to occur. I instead spent Thurs afternoon pretty much camped out in my recliner, moving as little as possible and eating/drinking very little while making calls to Dr.Bragg's office, my Primary dr's office and to my Insur. Nurse Case Manager while also sorting other stuff from Weds's Cardiology Appt.

Needless to say by Thurs night I was at a little bit of a loss and unsure what to do but a tentative plan with my Primary dr. (PCP) was to see her NP on Fri morning and they originally planned to direct admit to Dean St.Mary's Hospital. Later Thurs night I got a message from Dr.Bragg better clarifying what she felt about the whole situation and so re-thinking Fri's plans. Thurs night I pretty much drugged myself asleep which is something I NEVER do but was so tired and knew otherwise I wasn't going to be able to sleep.
Fri morning I did see my PCP (the NP) who was working w my actual dr. and is Dr.Simpson's Nurse Practitioner. This Appt went ok but was slightly frustrating albeit I don't blame it on my drs.
They had changed their mind once we had the info directly from Dr.Bragg and decided we sould try to get a consult from a Dean Surgeon "to make sure it's covered by your Insur." which I did understand their thinking but I also knew it was unlikely I was going to get through this wknd given I was not drinking or eating much of anything.
Even walking even short distances causes the pain/discomfort to worsen to an extreme in my abdomen/side-radiating to back.
I made some more calls once I was done at the PCP office as the soonest the Dean General Surgeon's could do was Monday and I wasn't sure if I should just go to UW's ER.
I really felt most likely they would admit which would negate the Insur. out of network issue (this per my Case Manager) and I knew I needed help with this.
I did end up just coming over to UW around 1, checked in at the ER and after about an hour was taken back to the initial screen where the Nurses start IV, do an eval, put in for the dr., etc.
Thankfully atleast this Nurse also was willing to use my Port!
Much waiting around later the ER doc came by, she did her exam and ordered labs and the 1st ultrasound.
After all this I came back to the actual ER and waited some more which was long but honestly I felt so bad I just did not even care. The Neurosurg. Resident(s) on-call stopped by "because we saw your name and wanted to say 'Hi' and talked about what they thought the problem might be. Needless to say their thoughts where not Dr.Bragg's thoughts!
A little while later the Neurosurgery Residents would get more involved in my care while in the ER including doing a shunt tap Dr.Bragg wanted done which was to look if there was any infection.
I hadn't realized it but Dr.Bragg wasn't actually on-call Fri night and so she really didn't have to take the time to call her Residents or get up to date on what was going on with me.
Her Residents told me she specifically told them regarding me they where to call her directly.
I think I heard her call them no less than 3 times and who knows if it was more regarding questions she had or something she wanted to tell them about my care specifically. I love that about her, that obviously she cares and she takes the time when it's really her time off!
I think it was around 11-11:30 I finally came up to the Neurosurgery Fl. once the Neurosurg. and General Surgery Teams worked out where they wanted me to go. If nothing else if I was going to have to be here honestly I am glad atleast it's on this floor b/c many of these Nurses just make it a lot better and a little easier to get through!
Sat., I didn't see General Surgery at all but Dr.Bragg stopped by and just let me know what her thoughts where, that she wasn't going to give up till we had answers and that we'd figure it out.
I saw Dr.Bragg's Partner Sunday morning, they take turns rounding every other Sunday and he just kind of reiterated what Dr.Bragg's thoughts had been that they didn't think it was the shunt related but Dr.Bragg would work w the General Surgeons.

Sunday evening the actual General Surgeon on my case stopped by, she seemed very nice and especially like she had put in the effort to get up to speed on my history with Dr.Bragg + especially she laid out that she felt the various scans all looked really, really good but based on others findings and her own exam she did feel we should investigate the gallblader or area around it and explained what a few things could be potentially in this area causing symptoms.
First due to my risk especially w the VP Shunt she felt we should re-do the ultrasound (to look at and see especially if the wall around the gallbladder was getting thicker though I can't remember what that would mean).
This dr had ordered what's called a 'HIDA' scan on Sat, this also looks at gallbladder function through a long series of PET Scan images.
The other test she felt we should do is an upper endoscopy - or also known as an EGD, a scope of the upper GI system and I think airway (but I may be wrong on that second part, I don't know that it has anything to do with airway other than they go down your throat I believe w the scope which if anything like the last TEEs (also uses a scope down the throat) I sure hope this drs/Tech's use the smallest probes they have!

This EGD is to rule out an ulcer or other problem that could be causing the symptoms though she does not feel that is the case and is doing it more as a precaution to try and absolutely rule anything else out before they take the risks involved with gallbladder laparoscopic (I assume) surgery and the fact she had concerns with this and any knick to the gallbladder or leaking of bile goes directly (apparently, who knew) in to the peritoneum where my VP Shunt catheter sits/drains it's CSF.

Dr.O'Rourke just said if we have any concern of contamination your VP Shunt will have to be externalized and I am sure you would rather not have to sit here even longer with a catheter hanging out of your body. Although I do feel better when the shunts are externalized and my headaches have been down right wicked here the past few days I also absolutely do not if we can avoid it want to go through an externalized shunt to!
I fully realize it could happen but am glad Dr.O'Rourke and Team are trying to minimize these chances where possible for shunt contamination!
At this moment we just started Plasma infusion to reverse INR and they'll also give a dose of oral Vit K. as INR is still 3.4 I believe my Nurse said. If we can get it down in-time they'll do the scope yet today. Prior to any procedure such as this scope I did make sure the Nurse let the GI people know we need to give Solu Cortef injection stress dosing so hopefully that won't be a fight w this procedure! I never hold my breathe though.
Unfortunately this wknd is my last Sunday School class for the year, till Fall.
I missed this past wknd due to being here and missed the wk prior due to being in Providence. I am hoping most of my kids will be in VSB as I do have a small gift for each of them and I really loved my class this year. =/
Will update again when I know something further, thanks for stopping by,
Erica

It's been an eventful week, to say the least. I got back from Providence Sunday night which I might add was very fun and I am sooo glad Melissa (1 of my runners) talked me in to going!
The atmosphere out on the course just totally different than the Boston Marathon due to it being a smaller marathon, less turn out and far less people cheering.
Pretty much every single person our (somewhat large!) RfRD cheering group either cheered on, high fived or whatever showed some kind of emotion be it high fiving, hugs, many whispered thanks of appreciation, etc.
There is just nothing that beats that atmosphere and I personally believe any person who feels sorry for themselves whether healthy or not should experience a Marathon!
The after party held at the hotel was great fun, so, sooo many pictures with my runners and Jessi, my runner from the last 2 yrs and then all of us together which was cool, I literally felt like my running portion of 'Team MPS' was complete in that moment!
Cardiology Appt F/up
Yesterday I saw my Cardiologist for a 1/mo fup from his starting the Cardizem last month. With the heart rate logs I was able to email him he already had a plan in mind as soon as he walked in the exam room which was kind of nice.
Because my heart rates despite the Cardizem are so high yet (high 80's to upper 120's, some much higher than that) and he is concerned overall heart muscle isn't getting enough time in-between each beat to get enough properly oxygenated blood he opted to increase the Cardizem dose and as well he is adding a new med, that is recently FDA approved for Heart failure and inappropriate sinus tachycardia.
He is getting back to me about that med but I figured out what it is likely and asked my Insur. Nurse Case Manager if she could find out if it is covered by my insur. If it is not due to being so new than Dr.Earing will go about doing whatever he does to get it covered.

In addition he feels we should add a heart rhythm dr. to our team to (I guess?) help manage the high heart rates and if necessary should these new med and new Cardizem dose not work he mentioned the potential for an ablation procedure where (I believe) they go in with a catheter in the groin and thread up in to the heart to the problem area and essentially kill/deaden that part of rhythm system.
Overall it seems he thinks if we can slow heart rate down this could help with the heart failure symptoms and the overall mitral valve and perhaps if I understood correctly give us more time to needing to replace that valve in another open heart surgery?
I F/up with Dr.Earing mid-June for an Echo and Appt re these new meds and although he was trying to coord. the EP dr Appt for the same day I see him it wasn't possible so that appt is June 4th.
ER
In other exciting news b/c why should life be dull and pain free I guess?
Earlier this wk I started getting pretty bad BUT intermittent side pain that came and went with little rhyme or reason seeming to it. Over the past few days has gotten worse to last night was really bad, already by yesterday I couldn't straighten up fully, I'm sure I looked like a little old granny walking and the pain was starting to be pretty constant.
By last night it was full fledge. I seem to (knock on wood) be experiencing this different than most people but anytime I move an inch, get up, turned over when I was trying to sleep (I am a restless sleeper so that makes for a really long night!) or walked around my side hurt so freaking bad I can't even explain other than is kind of like someone stabbing you repeatedly or twisting a knife?
This in turn for whatever reason seems to be aggravating the Leg/spine nerves quite a bit which has been less than pleasant.
Not sure what to do and somewhat uncertain if it was shunt related or not I went to the local ER where they did a CT Scan and labs and the scan shows a "large gallstone" that the ER dr recommended be removed as soon as possible. This in turn began a VERY maddening day of trying to figure out if this surgery needed to be done at UW as the ER dr thought or not. Talking to my Insur. and to my Primary dr. and in general trying to just deal with the discomfort.
I heard back from Dr.Bragg's office this afternoon, which I might add if your ever in the ER and the ER doc thinks you need a surgery but done where another of your drs is for god sake have the ER dr call that dr before you walk out of the ER!

Per Dr.Bragg's Secretary there was not anyone she specifically recommended at UW, she feels the "shunt just has to be protected" (what does that even mean?) and can be done at St.Mary's. After this I called back my PMD's office for I believe it was the 3rd time (they had specifically asked that I call w/ANY update I had big or not) and b/c I didn't want to go to St.Mary's ER tonight I am seeing Dr.Simpson's NP tmrw morning and they may direct admit to Dean St.Mary's Hospital from there.

All fine and dandy but I am telling you if the Anesthesia drs there give me so much as a hint of issue regarding airway mngmt I am fairly certain I am going to blow a gasket, I am tired of bullshit this. wk. When on the phone w Dr.Simpson (PMD) Nurse I had to choke back tears and they weren't tears of sadness BUT tears of complete and utter frustration at all of this. I.am.sick.to.death.of.there.always.being.something.and.not.feeling.good between the stupid shunts and headaches and Leg stuff and what has been an endless stream of Appts and phone calls this wk for a lot of things.
My INR is also outside the normal range at 3.3 so that would have to be managed before any surgery and brought down. I'll admit I am not always a fan of change and feel comfort in surgeries that take place where either Dr.Bragg is b/c I know she watches like a hawk and chimes in if necessary as far as how something is done or where my Cardiologist is close b/c he to is familiar.
I do not like change and as anyone w MPS knows we have valid reason to be nervous with airways issues and surgeries!
Given I am not sure what her office meant by "the shunts have to be protected during any surgery" i'll talk to whoever is doing this surgery be it tmrw or whenever and perhaps ask that they talk with her to make sure nothing is screwed up with the shunt(s)!
I don't need anymore shit to be wrong! (pardon the language but is true).
My biggest concerns with any surgery at a new hospital/new Anesthestiologist(s) is 1. they think they don't have to use the airway equipment to get an secure airway. 2. The usual argument over steroid pre/post surgeries or procedures, 3. INR mngnt (will have to be brought down) and they talk to Dr.Bragg so they don't in fact screw something up!
I am supposed to see Dr.Bragg in clinic Monday, which I had changed that from today bc of the mtng tonight (ironic given now I'm not going to the mtng!) so depending what happens w the gallstone I guess in the next few days will talk to her then. I do really appreciate that she has been doing the intermittent taps, those atleast help temporarily and calm the Leg-nerve stuff temporarily.
Obvously like always I wish we could just fix whatever is making either shunt not working as optimally but what she has been doing is better than nothing.

I was supposed to go to a Genzyme Mtng tonight in Madison for Lysosomal diseases and pretty disappointed to be feeling this crappy and thus not going. Pretty damn sick of this week and sick of cancelling one plan after another every single day so far (Book Club Monday, (may not be the most exciting but I like it), dinner with friends Tues and yesterday I just came home and did nothing, read a book for the afternoon on and off (Large print is great!).
I did get a lot of phone calls and stuff related to the Cardiology/EP stuff done. Even if it was still related to medical stuff I really enjoy these mtngs held by Genzyme occasionally here in WI and will miss seeing my (Genzyme) Insur. Case Manager and Genzyme Team especially. =/

Will update again when there is anything to update,
Thanks for stopping by,
Erica

Another VP shunt tap done; is so good! - Neurosurgery Appt.

Just a short update, nothing to important from seeing Dr.Bragg just a few things I thought I'd share. =)
When I had seen the Metabolic Genetics dr the other week at Boston Children's one of the things they where interested in was if Dr.Bragg might be willing to test CSF for GAG levels. I received the clinic portion of Dr.Bodamer/Dr.Berry's notes in the Patient Portal today and fwd'd those to several of my drs including Dr.Bragg.

While Dr.Bragg was tapping the VP Shunt and slowly letting CSF (fluid) drain off she was asking me if anything had been changed/added since that appt a few wks ago so I brought up to her about Dr.Bodamer wondering if she might be willing to test fluid for them. Dr.Bragg said she was more than fine sending them a CSF sample from one of these next times she taps the VP Shunt to drain off fluid.
I'll have to get instructions for her how she would go about sending a sample, I know Dr.Bodamer had said something about he could send her the supplies and instructions if she where willing. I think it is really nice she is so open to working with other of my drs. and she is so interested in what other drs. might add to my care or might be thinking!
After the tap was done and as we where talking about the symptom relief (for whatever reason even though the headache relief is relatively short lived these periodic/repeat taps to remove fluid seem to be causing the Spine/L leg nerve to stay calm for longer periods of time, each time after we've done a tap. That is AWESOME on my part!
She said she was thinking about why this might be as she was draining fluid and figures the symptom relief may be better w the shunt taps similar to how I get better relief with the External drains b/c there is no resistance when she is draining fluid vs with shuns the pressures have to reach a certain '#' (pressure) before the shunts will drain so I get continuous intermittent symptoms throughout the day due to my brain compliance issues and the build, drain, collapse, build, drain, collapse.
I honestly thought she might say we shouldn't do anymore taps for a while given I am not traveling anywhere now after this wknd BUT she asked me as where done if I wanted to come back next wk to do another.. Crap, I know your likely thinking 'why would you want to go back and see your Nrsgn again and have a needle put in your head?' The simple answer is I never want to go to an Appt BUT the relief is so, so worth it and especially with the Leg/nerve symptoms seeming to calm down for a little bit longer of periods after each!
I know she totally doesn't have to keep doing these and she even jokingly said something along the lines of 'I know some would say I am crazy for continually doing the shunt taps but I know your getting relief and you feel better and that is a good enough reason to me to keep doing them for you'. Have I said she is amazing?

If you know Nrsgns you know she is not your average Brain doc both in personality and in how she approaches caring for her pts! I am so, so grateful for her and will always be! I may doubt at times but that doesn't mean I'm not grateful both to her and for her for her persistence and as she has even commented about several times over the last wk, for her believing me when many other Surgeons didn't b/c my situation and presentation is not classic.

She never gives away details but she was using as a comparison another little pt. of hers whose had some on-going complications for which she was able to figure out today and she commented something like 'I know he was in a lot of pain to but you'd have never known it and his case reminded me several times of you and of your high pain tolerance as you deal with a lot of issues and pain but most would never know it looking at you'. (totally not how she put it but something like that, having to do with high pain tolerance and just dealing with these headaches everyday which would take many people down and make them complain I guess but I just march on)?
I am not sure entirely but I appreciate actually that she said that, that she seems to think I deal with the pain/discomfort fairly well as honestly I don't always feel like I deal with it all that well albeit I know I rarely complain.. I guess maybe I tend to feel like if I dealt w it really well I wouldn't ask her for her help maybe?
One thing I thought of while driving home from Madison and while thinking about some of the stuff Dr.Bragg and I had talked about (I love conversation with her, while I rarely think of questions related to what we talk about at the Appts I feel like I always learn something and come away w just a little more information)..

Anyways so Dr.Bragg was talking about the resistance issue w the shunts and like I said above why the shunt taps give better relief albeit short term than the shunts in general do and she believes it is b/c of the resistance from the shunts. Soo on my drive home I couldn't help but think about all this and I wonder can there ever be a shunt system that doesn't have a valve in it? Ie could we revise one of the shunts potentially to have no valve and it just be free flowing?
I am not sure if it would be even quicker to clog/collapse but I'm definitely going to ask her about it!?! Can't hurt to ask, right? We know after all 1 of the shunts will clog, it always does is just a matter of we never know when or if it will be a sudden full occlusion or as seems to be now intermittent partial occlusion that leads to partial permanent occlusion and either way requiring revision when symptoms are bad enough.
We talked about how MPS high pressure/Hydrocephalus issues are not really the typical Hydrocephalus issues, our pressure needs typically change over time (ie I started out w a medium pressure valve on a single shunt being good and now have 2 shunts set pretty low and ideally could use to drain more fluid).
Her feelings are the overly thickened skull/bone and compliance issues are a big part of what causes our on-going issues w the MPS and pressure and for some of us with shunts but also why symptoms don't always present classically. I know I've talked about that before but it's something we always come back to and for good reason! The more in-depth note from Dr.Bodamer (the other note was written by his colleague Dr.Berry) I think will cover more of the study stuff, family stuff and other thoughts he had? I am not entirely sure.
Appt wise right now, I am gone this wknd, Dr.Bragg is gone Monday-Weds for a meeting she is presenting at (baclofen pumps and spasticity, I actually bet it would be an interesting topic to listen to her present on) and so I will see her Thurs. as is scheduled now.
When I scheduled this although I was looking right at my calendar (I am rather dense sometimes) I scheduled it for late morning but I have a Genzyme Lysosomal Mtng Thurs night also in Madison. I called back a couple hrs. later and left a message for her Secretary to see if I might be able to switch the appt. date to the following Monday. If I am able to do this i'll switch ERT (infusion) to Tues or Weds that wk.
Anyways, thanks for stopping by, thanks for the various emails and comments on my last blog. =)
Will update again next wk sometime,
Erica