Monday, April 30, 2012

Lumbar Peritoneal programmable shunt and Dell XPS (a potential valve reset)

So I dont know if I ever wrote about it here but in pre-op fri while we where waiting for my neurosurgeon who was finishing up  a surgery over at the children's hospital (skywalk walk-way) the anesthesiologists and resident where milling about talking to us as was the nurse (same one from the week before and multiple times before that!) and the anesthesiologist commented that the particular valve Dr.Bragg was going to be using was actually on it's way from Chicago and not yet there. It turns out this valve was being delivered by the Medtronic area neurosurgery/shunt rep and he was driving down from his home/office. That all worked out fine and he stayed in during my surgery to help check and set the new Medtronic Strata valve which they opted to set at 1.0 during surgery so we would have some wiggle room to go up or more likely down on the amount of CSF (cerebral spinal fluid) drained.  (sThis is the same valve as my VP shunt but a different programmer is used). Fast fwd the weekend to today, monday and I was having some symptoms yet and especially vision wasnt improving much so after Dr.Bragg stopped by we decided to try going down the last bit to .5. She talked to the Medtronic rep who was around and will be around all week and her NP + those 2 came by with the LP shunt Strata valve programmer. This is done differently in LP shunts but uses the same general device set up.
They got everything set and positioned over my right flank/side valve and turns out after re-checking it a couple times the programmer in the valve has reset from 1.0 back to 2.0 which means alot less CSF was draining. Thankfully I have the VP to pick up some of that slack and I could tell when the VP shunt would kick in + when it wasnt draining right but still the symptoms clearly ment I wasnt draining enough although I had assumed it was just needing to adjust the amount of CSF down another notch. They confirmed with Dr.Bragg and after gathering some info reset the valve back to the original 1.0 it had been programmed at in surgery fri. They believe it was reset by my laptop as it is known that the IPAD 2 can reset programmable shunt valves and my particular laptop is a heavier-duty, stronger set up that most laptops so likely has a fairly equivolant magnet strenght if held close enough to the valve. They checked my VP shunt and that was still fine at .5 but I could say definitively I had on several occassions since fri had the laptop either right near or on my lap as well as next to my hip especially on Sat when my nephew was here and he was curled up in my hospital bed watching a butterfly documentary with me. Yikes!  I will have to not sit on my bed with the laptop balanced in my lap anymore (one of my favorite ways to sit and watch movies or even sometimes sit and type things). I'll adapt. :/ :)

Before we realized the valve had been reset to 2.0 from the 1.0 Dr.Bragg was going to reset it to .5 which is what my VP shunt is set at and she said it was possible at some point we'd maybe have to even consider taking out the valve all together to allow a more 'free flow' CSF dynamic. Hopefully this correct setting or being able to go to .5 will do its trick though!
The Medtronic rep said that my having the VP shunt at its lowest setting and then feeling best at draining 15-18cc's an hour of CSF with the external drain was pretty impressive and a very significant amount of CSF (cerebral spinal fluid) to drain in any one person as someone my size typically produces 20cc's an hour of CSF not the 25cs's I originally have been quoting/writing about.

I commented to Sue (NP) that it was nice to not feel like maybe I was going a little nutty in not getting much relief from the 1.0 setting as it wasnt even set at 1.0 for long it appears and made me feel alot better. :) Even Dr.Bragg said this morning though before all this that she was very confident we would get to a good place given how good the lumbar external drain was and our past experence with the External Ventricular (EVD) drain and improvements we've had. Neither of us feels like we are chasing a pipe dream in other words. (I think thats the right word to use???)  and once again I take my praise to God for all he does in often mysterious ways!

Typically Dr.Bragg has always said it takes about 12 hrs to notice improvement with any shunt valve adjustment so by tonight I hope to be feeling really great again!!
Will  keep updates soon,
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Saturday, April 28, 2012

Surgery #15 Laminectomy, L:umbar shunt placement

Sorry for the delay in updating here, some have been following on fb and so hopefully most have atleast a general idea what has went on since surgery yesterday early afternoon. Ihavent updated here sooner as I was on bedrest and having to lay completely flat or at most below 30degrees for the past 28 hrs, In pre-op yesterday Dr.Bragg talked about how she had looked more in to lumbar shunts and talked to some of her former colleagues and while she initially thought she wouldnt be able to use a programmable shunt for the lumbar peritoneal drain she wsa told and research confirmed that programmable shunts can often be used in both types of shunts. She was going with a programmable Medtronic Strata valve just like my VP shunt is and initially planned to set it at 1.0 which is the second to lowest setting as she preferred to have some wiggle room for going up or down with pressures and amount of CSF drained if need be. She thought there would be a good chance we would end yp turning it down to .5 which is what my VP shunt is at but will discuss that in the coming days. She also felt optimistic she would be able to get the Lumbar portion of the catheter in to place without havbng to do a larger incision and laminectomy but more on that in a minute. I had the same anethesiologist and resident as one of the previous shunt surgeries and they went with the standard fiberoptic intubation and where nice enough to write down updated numbers for me on airway grade and tube size, etc. I have copiesof their actual anesthesia records from previous surgeries but is still nice to have the basic, more important information written down to share at other hosptals when I have surgery elsewhere.  Surgery was scheduled to take 1 1/2 hrs or less but started around 2 and I finished around 6 6:30  or so so 4 5o 4 1/2 or 5 hrs. The reason the surgery took longer is Dr,Bragg did end yp having to do a laminectomy (removal of all or part of the lamina which is hte bone that covers the spinal cord area and prevents CSF from leaking out.). As I said above she mentioned it was a possibility shw would have to do this but felt optimistic from looking at previous MRI scans again that she shouldnt have to especially given how easily she was able to get the external lumbar drain in last week. Once again with this surgery like last week she inserted the lumbar portion o fhte catheter at the low lumbar spine and then instead of just threading it in a small ways up she threaded it up to chest level height (but in spinal cord/sub-arachnoid space) as this will prevent the catheter from backing out of the sub-arachnoid as happened last fall (a quite bad thing).
Because of the laminectomy along with the shunt placement (virtually same surgery happened last May with my first surgery with Dr.Bragg). She has commented on this before but brought it up again before surgery yesterday how tough and almost glue like (not exact words but meaning skin doesnt stretch/elasticy like a normal-non MPS pts)  my skin is and how much tougher that makes it to get catheters positioned and passed/tunneled under the skin from one area (say lumbar) to side where the valve/reservoir are positioned and then to abdomen where a 3rd incision is made and the catheter is unneled in to the peritoneal area.The laminectomy and scarring was likely more to do with the pevious spine surgeries (though shunts where never that low), less CSF pressure in the area this time and overall compression that occurs in MPS I pts. 
Ive had to lie flat for the past 24 hrs which has been semi-hard due to breathing issues (but we used oxygen at relatively small 2 liters over-night and took that off late this morning before I went down for a shunt series of the new lumbar peritoneal shunt and of the VP shunt pathways to make sure no obvious issues where occuring with connectors or fluid buld-up, kind of standard after shunt revisions/placement atleast with my neurosurgeon.

I had emailed the family that my neurosurgeon and her partner treat whose little boy has the LP shunt and VP shunt and whom my CHW infusion nurse also knew. This Mom emailed me back last night and asked to talk on the phone so we texted back and forth a bit and she called me earlier plus we talked for about a 1/2 hr. Her little boy has as my neurosurgeon said a lot of the same symptoms I do like autonomic signs/symptoms, possible has possible endocrine issues
  and the 2 shunts of course.
Dr.Bragg said earlier today that when the residents came by tomorrow they would likely say I was going home tomorrow (sun) but that I should correct them and tell them she looked at their notes and the earliest she plans to do is mon. Given ive yet to be able to pee on my own (also very similar to last May's surgery) and pain is so wildly up and down (treating with multi-med approach including combo IV and oral meds. I am glad she is being cautious and would rather make sure there are no side effects first from the laminectomy. Hopefully now that I am off of laying flat as of 30mins ago and able to get up and walk to the bathroom with a nurse in another 10-15mins I will be able to return to peeing on my own and not having to be to straight-cathed by the nurses. I do not want to go home having to self-cath like last year nor do I want UTIs again as I had just been thinking the iter week it sseemed like we finally got on top of these recurring UTIs from last May's spine nerve injury issues. I have 1 year urodynamic testing in early June at would be so great to go in with a more working bladder and spine nerve signals even though I know itt isnt fully normal.
 Zander had the munchies when he came to visit with my parents and sister and so was eating my buttered noodles. I love, love, LOVE this kid!!!!

               Curled up next to me in bed - miss and love him to much to even begin to explain!!
                                                   Zander nad his Mom, my sister Sara.
                                                       Goofing around with Zander
                                               Chilling out with my best buddy. :)

                                          Zander reading about his bugs, such a smart one he is!

All for now, more soon.


Thursday, April 26, 2012

Surgery #15 @ 12:15/2:15, multi-part update

Wow, tomorrow will be surgery #14since the end of last May and #17over-all not including a few in-pt procedures that preceeded surgeries.. Yikes!

This is another of my multi-day updates so I apologise, I think ive dated each section appropriately. :)
Today I had a Pain Mngmt appt (normal fup) in Milw so I went and before that had worked out with one of my infusion nurses who is on the CHW Access Team to have her re-access my Port so I didnt have to leave that in all week. She and I met in Radiology and from there another of her access team nurses met us - this nurse it turns out is one my nurse talks about often and is married to the Director of CHW's Pediatric Neurosurgery dept and President of the Academy of Pediatric Neurosurgery (one of the top groups). My nurse had been telling this other nurse about my on-going issues and apprently talks about me alot and the other nurse was curious what was going on given the on-going shunt issues. As ive said in the past shunts alone are not a black and white business and then add in MPS with its typically small ventircles, non-expanding ventricles and of course the actual glycogen (GAG) storage and shunt issues tend to be a problem. Many people of course who have no background in shunts dont realize this and then if they have no knowledge of MPS they are going to be even more lost to why the issues are so complex. In a way this other nurse and my nurse I think thought my neurosurgeon just wasnt doing a good enough job at first and that maybe I needed to get a 2nd opinion (my neurosurgeon has continually talked to the former neurosurgeons she worked under/trained under in Utah as well as talks to and works with her partner - a highly respected person in the Hydro community on my case.) She is personable, down to earth, funny, smart and thinks outside the box which is something I didnt have on my team for to long as far as my shunt goes. She also works with and has no issue with my other providers giving their in-put on the endocrine, cardiac and other issues that play in to her surgeries and she when needed, when we've had issues has always gotten outside help, from other providers/specialists regarding new or on-going issues such as the bladder problems and talking to her Neurologist about my case as well as referring me to a Neuro-Opthalm to keep tabs on the optic nerve and eye issues. I also have heard nothing but great things about her from the people ive worked with at UW for APFAN advocacy as well as the floor nurses and another friend who incidentally saw my neurosurgeon without our each realizing the other was seeing the same provider and this friend also thought she was great to her daughter.
I appreciate actually that my infusion nurse apparently likes me well enough to talk my case over with her friend and fellow nurse whom is married to the neurosurgeon and I think this other nurse realized by the end of our meeting that I probably was in good hands and that my case just isnt cut and dry. As I say from time to time it's good to be surrounded by people who are concerned and care and whom we trust in many different facets. I realize more and more how much of an impact I apparently have made on people and how little things like this blog and my just trying to not be snide but also be informed makes many providers and many families appreciate that I am just trying to do my best too.

Earlier this week I got an email from a Dad in the MPS community who is doing MPS fundraising and big behind some of the most recent research in to MPS I and he was laying out some of the newest things going on and asking if I would be willing to be a part of advocating and getting families involved. His child is affected by MPS I as well and he commented that he thought I was someone in the MPS community that alot of families respected and whose story alot of families and individuals follow and so he felt like I could really rally troops to help get people involved. I agreed to help and while I dont know that I am a leader in this MPS I community I do appreciate that people like him and so many others ive heard from recently think I am doing a good enough job that they want me to help them and/or they just want to be able to keep reading things like this and have someone they can kind of in a sense look at for what the future might be like. There is absolutely nothing better than in-directly and directly helping others!



verb (used with object), val·i·dat·ed, val·i·dat·ing.
to make valid; substantiate; confirm: Time validated our suspicions.
I think and have found that provider trust comes more easily between those providers who take me seriously, who consider me a part of the team (imagine that, a patient as a part of the team?) and who listen to me from the get-go. Not that I think I am always right and not that providers need even to always acknowledge everything I might think but instead that they are willing to either 1. admit they may not know or 2. admit they may not know and will try to find out but still may not get an answer. or 3. They realize that sometimes patients do know what they are talking about and we arent all dis-illusioned, ill-informed and not willing to take responsibility for our own issues and learn about what we are dealing with. 
I was thinking about this earlier today while I think I was driving and I just couldnt help but think that I have 2 groups of providers 1. the group that I feel like I could trust and although still not easy ask their help from and group 2. those few providers who are either very good but I dont know well and have never been close to (sometimes thats an ok thing) likely bc I dont see them often and thus they arent in the 'core' group or a very slim few providers that I have little say in seeing them (usually bc of what they manage) and thus I likely dont actually see them outside of the actual thing they manage and wouldnt put them in the core group of providers either. To me the core group, which I know may sound a little funny is just those 4 or 5 providers who manage the bigger issues and who when there are big issues such as a surgery going on or new issue going on I then will send a group email update to all and these are the ones who I try to get records for plus if they asked me for info for something I would really try to help. The whole reason I feel like I trust these providers is 1 simple (atleast it seems simple to me!) reason - they offer validation and they admit when they dont know. All I think most complex chronic disease patients need is a provider(s) who will listen and who will validate that yes, sometimes patients do know what they are asking about and providers who take just an extra minute to agree when they think something the patient brings up might be right or concerning (vs deny or ignore it which are the things that make me not trust a person) or that they, the provider as I said above may not have an answer for the patient but still letting  the patient know they are a part of  a provider-patient team and the provider isnt going to give up. Just letting the patient know the provider isnt going to abandon the patient (happens way to often) as far as finding an answer or solution if no easy answer is available or if an issue continues to occur, letting the patient  know that the provider cares I tihnk is a big part of trust and creates a better partnership in which patients are more likely to try or do what the provider thinks and the provider in turn is going to generally, probably have an easier time working with the patient.
Pre-Op went well and not to much to go over since we just did this same pre-op just shy of a month ago and a month before that and before that.... Good thing this is one of the providers I do like :)  Labs where the only thing that had to be done and we talked about some just recent tests + a relatively new med (beta blocker) and otherwise just the actual logistics of the surgery itself ie where the 2nd shunt will go, more about last weeks external drain, etc.  That was the only thing I had going on medical wise today and so was nice to be done and headed home and even nicer to be here at home writing this. :) I was definitely exhausted today so while I got a few things done errands wise before and some cleaning done before leaving home I think bed will feel nice tonight if I can fall asleep reasonably well.     Thurs., I have something else going on (fup with Pain Mngmt)and so one of my infusion nurses at CHW who is also on the Interventional Radiol.-Access Team at CHW is going to re-access my Port then which worked well so then I didnt have to leave it accessed from mondays infusion to fri's surgery and especially after having had it accessed from last week mon's infusion through to this past sat. when I got the drain out and got discharged.

This is about all I know, other things to write about but will do that at another time. Will update on fb fri night and on here fri night or sometime sat.
Stay tuned -
All for now,


Monday, April 23, 2012

Actual shunt surgery scheduled, 2 part update

Wow, what an up and down day with right now being pretty down (as far as symptoms/headache) and so im not sure tonight is going to be very productive but maybe this will turn around!? Anyways, Dr.Bragg's office called and initially where just scheduling an appt for next week but then when I asked her Secretary if we where going ahead with the surgery right away this coming fri or waiting till after I saw Dr.Bragg she asked to call me back. Turns out Dr.Bragg had to go out of town for a family emergency and so they still where able to get ahold of her and she (Dr.Bragg) verified that she would like surgery for fri. So that is scheduled for they think as of now early afternoon with arrival around 10 or 11 (verified the day before as usual) and to place the 2nd shunt in lumbar spine to peritoneal. Pre-op is scheduled with my PCP for tomorrow.
Lisa, Dr.Bragg's nurse called back a bit ago and she asked if I had any other questions plus to say she was wishing me luck (very sweet) and that she had heard from Emily, the NP that she (Emily) was pretty excited how well I felt last week and how big of a difference the drain had made.
 Dr.Bragg and I emailed  last week about potentially doing MRIs while the lumbat drain was in this a  but then I kind of forgot about it possibly getting done and although I even had it written on a list of other things both related to the drain and non-drain related, non-shunt related things (stuff to reschedule, some records to get copies of for another provider, info I wantd to look up for another site, schedule conference hotel, etc) in true me fashion I didnt think to ask Dr.Bragg if we should do the MRIs. I guess it doesnt matter and we can get them done at some point.  My reason for asking her if we should do them was the lumbar spine discomfort, leg symptoms and feet intermittent-numbness and those all did seem to really improve with the external drain so hopefully all symptoms where just related to the high pressure from the CSF on that area.

I think what im most nervous about with this new shunt addition will be that with the External Ventricular Drain (EVD) we had complete sytmptom resolution and I worry a little that could it be the not enough CSF draining from just the VP shunt is from some aspect of peritoneal issue? I dont know and I do trust Dr.Bragg as ive said before but I think I just realllyyyyy hope we get as good of a result from this actual LP shunt as we did from the external lumbar drain. The other thing I wonder and I think will just be situational likely is how do they know which shunt is causing the problem if a problem does occur again? I imagine she (Dr.Bragg) would do a shunt tap or check the reservoir refill time but am not certain and will ask that. All these things a person as ive said or atleast in my case just doesnt think of when your actually talking to your dr. I really cope well when I have information so I think that is why I think of some things later on and not when actually talking to my drs and I also just need time to process information.
Ooh well a  person can have nerves sometimes, right!? :)

These pictures below are from sunday at my parents with my niece and nephew in the backyard/field.
                                            Red Admiral (Zander's catch)

                                          Zander and Jordan's Butterfly catchin'

Sunday April 22, 2012
Well it's sunday and this has been one of those "it's a work in progress" updates but needless symptoms have continued to retun over the past 24+ hours since the lumbar drain was removed Saturday morning. Right now those symptoms include the headaches/pressure, vision, GI, low back/leg numbness and other sutonomic symptoms. It is of course a bit discouraging to go from such complete symptom relief to knowing you have to go back to having symptoms to actually having those symptoms and knowing it will get worse but I also know I have a neurosurgeon who has ideas and has a plan and hasnt given up over all this time so instead of feeling sorry for myself  and why do I have to deal with this I just focus on 'it could be worse' and the usual 'one day at a time' as far as symptoms go. Instead today I went to church and a couple errands and then went over to my parents where my sister-in-law and brother, niece, sister, brother-in-law and nephew + niece also where and a nice way to semi-forget how you feel.  Tomorrow i'll work on figuring out this week and what will happen when as well as what do I need exactly to do before the actual 2nd shunt is placed ie pre-op, do I need to make an appt with Dr.Bragg to get couple stitches from external drain removed or can they in fact wait till the actual surgery, other calls, etc.

Both the EVD and Lumbar external drain worked wonders for symptom resolution - if we cont to have problems after this 2nd shunr is placed it makes me think we have to re-think the distal  (currently peritoneum) location of the shunt tubings as these are the 'other' varables? hen I had asked Dr.Bragg why she thougt the pressure would have been so different/high at both brain and in lumbar spine despite the VP shunt she mentioned perhaps it could be compartmenalized but didnt think that was the case and just couldnt say.  Bc we by-passed the peritoneal portion of the drainage everytime we did the  external drain it makes me wonder if there isnt a connection there if we would have future on-going problems.. I sure hope this works as well as the actual drain though! The other question  Is it more a distal issue vs brain compliance issue or a combination im not really sure of.

Anyways those where just some thoughts or things I couldnt help but think of, will update when there is more,

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Saturday, April 21, 2012

Day 4 Post-Op: Drain (lumbar) removed; moving ahead on 2nd shunt

Dr.Bragg came by this morning and we opted to remove the Lumbar drain today + i'll go home for a few days, she I think to is excited how well of a response I had to the drain and said no one could doubr we where doing the right thing given my symptom resolutiion, energy increase (even with so little sleep!) and the amount of pressure that was built up when she placed the drain catheter on Weds. She said she felt bad i'd have to return to feeling bad w/headaches and symptoms for a few days but still felt it was safer by going home for a few days in between the drain being removed (stitches placed and drain removal was pretty easy just a little nerve irritation/symptoms when it was being pulled out.) and she said she'd like for me to fup with her early in the week to get the few stitches removed as she knows my skin usually gets irritated from these and this to will prevent a potential infection source. She said her nurse on mon would help figure out a time for the  actual 2nd shunt placement but she thought maybe they could even do it on fri next week. The following week, a week from this coming up thurs my parents are going on vacation so Dr.Bragg is trying to work around that but still not make me wait to long.

 I asked her if I would have to get a new pre-op done as this surgery we slid under the 1 month time frame by a few days again and so it is expired and wont carry over to this next surgery. Isnt it silly that you can have surgery one week and only a month after the last surgery but yet bc that original pre-op order was from just over a month ago you have to get a new one done. :/ Its not Dr.Bragg's fault is just a stupid rule in my opinion in cases like this where ive had so many surgeries almost consecutively/close together. She was trying to think of a way around this and thought maybe they could do it but ultimately we figure it probably has to be done by my PCP. I guess I know what i'll be doing this week. (re-scheduling, calls, paperwork).

I am hoping and praying the headaches and symptoms atleast stay resolved until sun or even mon but will see. I can hope!
All for now, will update if I know something on monday,


Friday, April 20, 2012

Increase of CSF drain/flow

Dr. Bragg and her NP Emily stopped by a bit ago and she asked how I was doing at the 15cc and they where teasing me as I was picking something off the floor to throw away + rearranging a few things. Emily said she could definitely tell I was feeling better! I love that other people can notice it so much especially since in the last 48 hrs ive slept at most 4 hours and so am tired but still feel heads and tails better than before this external drain was placed!
Dr.Bragg asked if I wanted to take out the external drain today or leave it in another day while they work out details/plan/timelime and I jokingly said i'd just go home with the drain still in place. She turned to her NP (Emily) and said I  told Emily you would say to us that you would just leave the drain and take it home because you feel so much better! Ahh she knows me to well. :) I told them that was it nuts that bc I feel so much better with the 2nd drain in place id crazily rather stay an extra day then go home today and have to deal an extra day with the pressure building up??  Both said they completely understood and thought that i'd think this bc they can see how well I feel. Dr.Bragg upped the amount of spinal fluid to be drained every hr from 15cc's now to 20cc's an hour till tomorrow and we'll see a trial of sorts if this is even better.
She and Emily are talking to Dr.Bragg's partner to get his in-put on how long to safely wait after the external drain is out and in order to let CSF build up (so the actual shunt catheter is easier to get in place) without making me wait to inordinantly long and in discomfort. They are working out those details today.  As ive said before how did I get so lucky to have an awesome neurosurgery team like Dr.Bragg and her nurses????
So I took this picture of the staple gun Dr.Bragg/Emily brought in for when they take out the external drain and have to close the incision. Yikes so apparently this will be used to close the lumbar drain incision tomorrow! I dislike staplers unless I am srapling papers!!

I'll update when I know more on the plan!


Thursday, April 19, 2012

Pictures and CSF rate drain increase

So the news for today which really isnt news is that Dr.Bragg did opt to increase the CSG drain rate every hour from 10cc/hr to 15cc/hr which the nurses I talked to said they most they ever drained was in a male as I think I mentioned yesterday and that was 18cc/hr. I apprently like to build up CSF quickly f we have both this drain working nad the VP working and finally symptom resolution! The nurses have also commented each several times how much faster I drain that 15cc every hr compared to most External lumbar drain pts they have as we get mine drained in a matter of mins and some pts they said they take as long as 20mins for their body to drip out very similar and often lower CSF count per hour! I dont know what that says about my body besides just plain weird!
                                             Collected CSF
 Actual insertion site and drain/catheter for Lumbar exteral drain

 A tie blanket ive been working on here in the hospital for my nephew "bugs"
                                         Initial collection chamber before drained in to bag.
I'll update tomorrow fri when/if I know more about overall timeline. I honestly now that I feel this great (minus no sleep in close to 2 days now) am a little depressed at the thought of having to take out the external lumbar drain and knowing symptoms will return and until we get the actual 2nd shunt placed. Certainly not that I want a 2nd shunt but I cant begin to even explain how nice it to so feel more human!
Thanks for stopping by,

Wednesday, April 18, 2012

Revision/surgery #14: Lumbar drain surgery details. Good, Good, Great!!

Surgery being at 12:15 was kind of nice since I didnt have to get up till a bit later and be at my parents house till a little after 9 in order to get to the hospiital at 10:15. The surgery itself even started about 15-20mins early. In pre-op when Dr.bragg stopped by she mentioned she thought anesthesia would probably opt to do general anesthesia with fiberoptic intubation (essentially a camera) given she didnt know if the drain placement would take 5 mins or take a lot longer. Anesthesia opted to do fiberoptic. Dr. Bragg also said she had been thinking about my situaiton alot again over night and this morning (how can you beat a surgeon who cares that much?) and said she would be a little sad if we got a resolution to our on-going issues (but happy to get me feeling better) and said something like it woiuld feel a little strange if I wasnt coming to see her every other week bc she really likes working with me and her nurses really like me. :)  She also said her team and her had definitively decided to have me on the regular neurosurgery floor (vs neuro-ICU) and that when she was up on this floor earlier in the day the nurses had asked her if I was coming back and she told them yes, plus they told her while they hoped we got it figured out soon they also really liked having me. Again how sweet is that???  I thought that was INCREDIBLY sweet and makes me LOVE her even more both as a surgeon and as my provider and a person!! I had a different anesthesiologist and his resident and even though the actual surgery ended up literally being like 5 minutes to place the drain they didnt know this would be the case ahead of time (Dr.Bragg had reviewed previous MRIs and decided to go below my previous lumbar surgeries/scarring vs above it as below is less risky) and so Dr.bragg said they where almost in disbelief when she told them she was done so quickly, not that they didnt want anesthesia to go safely just that we all if in hindsight (which is always 20/20 and we would have had no way of knowing for sure) had known would have done it awake given how easily the catheter went in. .. Dr.Bragg did say both in almost disbelief (bc it has never happened to her before) and excitement that as soon as she got the catheter in to the sub-arachnoid space the CSF (cerebral spinal fluid) literally shot out the top of the manometer/catheter and hit her in the chest all over her gown and when she looked down it had run all down the front of her and her gown and soaked her pants and shoes. She actually thought this was kind of amazing and good bc normally CSF drains relatively slowly or slow enough to get the catheter attached to the needed drain/measuring device. She said this was definitely a clear sign that pressure was obviously to high yet despite the VP shunt and a likely sign we need a 2nd shunt. When she came by she was dry and still amped up about it all which I thought was kind of cool since as she said for once we had a clear cut sign  of where/what a problem is/was as well as she was very confident I would have symptom resolution which I do!!

                                                          CSF Drainage

                                                  External drain placement

All today Dr.Bragg has been draining at 10cc of spinal fluid every hour (the nurse comes in and over about 3 mins they drain CSF which they all comment how fast it flows and quicly that 10cc drains compared to others they've seen) . This drains in to the above bag or essentially like a IV bag and attached to a device that hangs it from the IV pole. She thinks in a day or two she will try draining at 15cc/hour CSF and see if that is even better or if worse we'll go back to 10cc/CSF.  With a lumbar drain and draining pressure she has no real way to know how much fluid to drain so this is a guessing game in a way and she goes with other experiences. The nurse I had today said the most he has ever seen someone successfully drain at was 17cc/hr and he said that was a larger male. She also said if as she further suspects we end up doing a lumbar shunt (the VP will stay in place) then she will likely do a low resistance valve on the LP shunt which just means essentially the CSF has less it has to overcome before pressure builds up and drains. Programmable valves arent a real option with LP shunts so she wasnt sure yet which valve setting we would go with ie low, medium or high pressure setting valve.drainage.  She did say we wouldnt neccessarily have to wait very long to do that 2nd shunt placement if we decide on it. My parents are going on vacation week after next so I hope we could do it soon but will figure that out with Dr.Bragg when we know more about the setting/valve type we'd use. Otherwise it has been lovely to feel better and walk around the floor - albeit a low back pain from drain placement and vision which will resolve in time I am super excited!!!

More soon,


Tuesday, April 17, 2012

Surgery time, External drain

Surgery is at 12:15 tomorrow (Weds) with arrival at 10:15 in pre-op. We left the Port accessed from ERT (infusion) on monday so that part is taken care of unless the anesthesiol. decide they want to also use a Peripheral IV which some do given the pre-dosing with both antibiotics and solu-cortef (aka stress dose cortisol replacement).

I had emailed Dr.Bragg, my neurosurgeon last week but knowing she was out of the office and might not get the email. She replied back today (love that about the providers of mine who will use email!) in response to 2 questions I had. I had asked her if she thought we should do MRIs while the drain was in to make sure CSF is flowing corerctly and no compression and also if the external lumbar drain worked would we do the 2nd shunt placement right away this admission a few days after this drain placement or would she do it at a future surgery-admission. She felt that it was better to remove the external drain and even if we see benefit from it/symptom resolution given the risk of infection (and my previously having had a CSF infection back in Nov/Dec for which we had to take out both shunts and replace the VP shunt + 6 weeks of oral antibiotics) we should do the 2nd shunt placement at a future date and I guess we will figure that logistics out if this works. She plans to leave the external drain in for 2-3 days.

This is both good and bad - bad because it would mean having to do another pre-op physical (simple enough but still takes time and yes, I can piggy back it with something else but it's still another appt) as well as another admission and surgery. Maybe I am getting ahead of myself since we dont know that the external drain will even work I guess its more that as a patient I hope to be done with this soon and done with having to bother my neurosurgeon (not that she ever says anything and we do get along well and she truly seems to care as does her team) . In that mean time of course if the drain does work I would feel better, then feel worse then feel better again. Yikes! 
On the good side of it is that Dr.Bragg obviously has been thinking about this and cares to think of the little details like out past surgeries and issues and cares about trying to prevent infection/infection risk as well as not overwhelming my body. I can and do appreciate that about her.

Overall I guess it will just be a 'lets see how it goes' kind of week with this external drain and i'll take the rest from there. With the VP shunt in place we do get some symptom relief and benefit. -- Otherwise today I was supposed to see my Pulmonary dr at FMLH but rescheduled that as I was purely exhausted (even with going to bed earlier than normal the previous 2 nights). My PCP and Cardiologist want her opinion on the breathing issues see if she can add any thought and I am curious if she has any suggestions for these episodes (which we think are cardiac) of feeling like I just am not getting enough air/difficult to get air in enough. I currently take the beta blocker and lasix (water pill) partially for this as well as Xopenex an inhaler at night and when needed during the day.  Other things coming up which ive rescheduled for this surgery include with the new Orthopedic dr the end of the month, the Neuro-Opthalm. and then various other appts like Cardiol. which are otherwise just scheduled. We will see!

I'll update sometime tomorrow or soon after.


Friday, April 13, 2012

Minor musings

Tuesday's Nuclear Med Cardiac Study seemed to go ok although admittedly not the greatest or longest ability to walk/run and actually more due to hip/knee and low back discomfort than due to actual cardiacc symptoms. Ironically yesterday I had alot more symptoms cardiac wise without any test than I did on tues with the testing. PET scans where used before the test an hour after dye was injected and then I did the stress test and 30 minutes later after further dye I did another 9-10mins of pictures. I follow up with my Cardiologist in May so if I dont hear results before then (I dont really expect them to be abnormal) i'll get the NM-Cardiac stress test results at that appt in May. Sometimes testing results like the sleep study where released on mychart so that may also happen and I will get a copy of the medical record. So one way or another I will post the results is the long winded point I am getting at! :)
**Test results where released on mychart and appear to be normal which actually isnt unexpected. Ejection Fraction (or how well the heart pumps) was 60% which normal is 55-75% and circulation was normal throughout the heart on the pre and post-exercise scans. I fup with Cardiol. in May and neither of us expected this to really be abnormal but non-the-less he wanted to fup after the test and cont to keep an eye out on overall symptoms. I dont know what this means for overall symptom/valve issues other than we know coronary arteries are so far ok.

Yesterday the one of the nurses from the neurosurgery floor called and did the admitting quesitons (usually takes about 15mins) and then today Pharmacy called to go over meds I am on although we both had to laugh (in as much as you can laugh at this) that I was just there 2 weeks ago for the last surgery. = / Ironically in such a short time as proof I suppose of the craziness and ever-changing nature of my health care I have had med changes in that time. If you where to look at my med list it looks quite long (due to several PRN meds) but as I was sorting out my nightly meds earlier tonight I I couldnt help but appreciate that atleast on a routine basis I at night only take 3 regular meds (lyrica, bisoprolol, topamax) and 3 supplements (fish oil, zinc/copper, biotin) and then 1 pain patch I change every monday and 2 meds (lasix, MSIR) I take in the morning. Then I take Xopenex PRN which has been more often lately at night and vesicare is written for every night but I take PRN. Anyways that is still alot of meds but there have been times it has been worse.

As I was getting the IV contrast on tues for the cardiac stress test/nuclear study and then during the actual test I couldnt help but think again about how uncomfortable things like that make me. Having someone else who clearly is just doing their job but be so close to you and asking questions about my life some of which are things I am asked a million times over (or so it seems) and others related to the test and that I think most people wouldnt find that uncomfortable. I couldnt help but just think how distinctly noticeable it was to myself my discomfort at much of it but it not being physical but instead just because ive in all these years still never gotten used to someone being in my space. I know that sounds ridiculous and it is ridiculous really and I know it is far worse for me (this feeling) when  I am over tired or havent slept well in days or have the headaches and it is people I dont know or dont know that well. There are some providers I dont have a problem with talking to or atleast I can talk much more comfortably  with male or female I suppose because I know them reasonably or somewhat well and then there are some providers I have no problem talking to but I still if I had a choice would keep them at arms lenght away (so to speak) although I suppose its more because as much as I talk about my life and sometimes really personal things here no one is touching me and I get to chose what I write about. I cant give a rational explanation for why many things health care still make me uncomfortable but they sure do! And along with being worse when I am tired I also find it harder when other people are around - I mean stupid things like to ask a question or bring up a basic topic; a topic that wouldnt be that big of a deal. I often think to myself I cant even imagine what peole with Asphergers feel bc I would like to crawl out of my skin sometimes and I am just quote, un-quote "normal". Again I dont know if it makes sense! It sort of makes me laugh that id be the one that ended up being in these advocacy positions and having given talks that is for sure. I think in those situations though you can get lost in whatever it is you are talking about or what is being talked about and even if I am talking about myself to a group of people it's less personal than if it's 1 or 2 or 3 people?

I think one question I really hate I guess because I feel guilty still (why I dont know) is when people ask me what I do for a living and I reply  that I am on disability and do the advocacy work on the side.  Even though I do know my life is busy and  I keep busy with the various activities I have going on in addition to helping with my nephew on top of managing all of the stuff that goes along with having MPS ( multiple insurances, paperwork, appts, coordinating/scheduling and time spent going to and from as well as at appts/ERT)  it still feels like because I 'look' normal I should be able to be more normal. I know what is normal anyways and how can anyone expect to understand another persons life (they cant but plenty im sure still think they do)  or why do I feel guilty when I know I am still doing quite a bit in society and managing a life despite not always having alot of control. I think I just have a hard time not feeling guilty because I know I look pretty normal on the outside and to people who dont know me (and plenty who do) I dont look like I really have anything wrong. I dont really have a good explanation for why I hate this question.

Anyways, enough rambling from me - i'll update next week either with surgery time or if anything changes with the Pulmonary appt on tues. (my Cardiol and PCP would like my Pulmonologists opinion to see if she has any suggestions on things we could be missing breathing symptom wise other than the cardiac issues).


Friday, April 6, 2012

Lumbar drain scheduled, Nuclear Med-cardiac test

Anna, Dr.Bragg's secretary called  earlier today (quick scheduling, I was kind of surprised actually to have heard from her so soon!) and they are scheduling the external lumbar drain placement for the 18th + Dr.Bragg must have talked to IR or Anesthesia as she has opted to schedule the placement in the actual OR vs in Interventional Radiol. This is a procedure that is otherwise typically from what ive read done at the patients bedside so clearly she has some concern about good placement and placement difficulty as well as comfort during the procedure... Anna, her secretary said they scheduled it for 3 days in-patient and I am unsure then if it works if she will place the 2nd shunt (LP) right away during that same in-patient stay. This drain does not require being in the N-ICU which is a nice thing,

Otherwise Interventional Radiology- Nuclear Med Cardiac stress testing dept called earlier about tues and that test will take 3 hours and they advised no food after midnight which was a little (lot, actually) surprising and I cant say made me very pleased. There is very little way I would make it through the stress test and dye studies without atleast a little food so I will eat something simple like toast. I cant understand how food could affect this and the lady couldnt really explain it. Strange for sure.

Will update again when there's more to update,


Thursday, April 5, 2012

Lumbar (external) drain, etc

I saw Dr.Bragg today and we are going to place the external lumbar drain likely the week after next although it will be done under/with interventional radiology due to the spine issues in that area and the difficulties they have had in the past with getting a catheter in to the lumbar spine + previous laminectomy. She also kind of wants to do some kind of  light conscious sedation but isnt sure anesthesia is going to go for that given the airway issues and commented that she didnt want to cause breathing issues but just a light enough level that I wouldnt remember. She was going to look in to that and her concern was just because of the size of the needle they use to place the external catheter for the drain as I mentioned to her ive been awake for all previous lumbar punctures.  Either way seems ok to me, I did do the Port-a-Cath awake and wonder if it can be to much more than that? And with the Port the IR provider also had to go through a bunch of previous scar tissue as he placed the device in a previous pocket that  I had a cardiac implantable recording device in; the only difference is of course with lumbar your going through/have the potential to hit nerves I suppose. We are looking at possibly doing the week after next as her nurse is out this week and Dr. Bragg is out of the office all next week so it might be  a little difficult to get it all arranged for that next week. Im unsure how long the drain would be in as in typical my not thinking fashion I didnt think to ask until later after the appt but imagine it really would  be dependent on the results. I also am unsure if she would place the LP shunt right away that same stay if the results where favorable/symptom resolution but from past conversations I had gotten the feeling she would; I can always ask her nurse this when I talk to her next week about scheduling this all and I can if really need be always send Dr. Bragg an email later on with that ques.

She asked if I could still hear the shunt draining and I said no to which she commented she wasnt sure that was a good thing or a bad thing but symptom wise I havent gotten worse since last week and this also isnt the first time ive been able to hear the shunt the first couple days and then not hear it after that. When she depressed the reservoir it was quick to refill as well which is of course a good sign to both of us. Im unsure just say that we do place the 2nd shunt and down the road I had issues again (heaven help us) how we also would know which shunt it was? These, my dear readers are just the questions I cant think of ahead of time and never think of during appts while we are talking! I think I am usually processing information and doing my best to remember what we are talking about that actually thinking of questions is not somethung ny mind can do, lol.

Earlier the week of 3/3 ------- ------       --------    -------- -----------          ---------            ---------   -------
I was reading another blog the other day and have been thinknig of this ever since; it's not a new thouhgt to me but non-the-less something that bothers me at times and something you cant help but think about at times. Obviously not everyone but alot of people assume if a person looks normal they must 'feel' normal and must not really 'feel' how they say. Or they must not really have issues like they say. I just always feel like my normal may not/is very likely not other people's normal and we as a society do a pretty pitiful job of listening to each other, judging based off of looks and assuming just bc of how someone is. . Saying 'you look good' might be meant as a compliment and i'll try to take it that way but sometimes it hurts when people cant look past the surface and just assume or outright tell you that you dont like other people they've seen (lol to that, bc what is that even supposed to mean!?) and so how can you even have the symptoms you have? As another blogger wrote "you cant see cancer", that doesnt mean it isnt there.. Well thats exactly what most of my issues are "they're still there".   As ive said before typically the worse I feel the harder im going to try to make myself look better; makeup, nicely dressed, cute shoes, hair done, etc. My schedule is never the same and is hard to maintain what I do with APFAN as these shunt and cardiac issues have been going on but I always try to have something I make myself do each day in addition to phone calls and paperwork for medical stuff. Somedays that is going to my nephews for the morning/part of the afternoon or his coming to spend the night here on weekends even if I feel really tired. Sometimes it's background stuff with APF or now small things with the ACHA group. It's variable but I feel much happier when I accomplish things for myself and need to do things for myself and wish people could understand that. What would society be if we all where the same and all did the same things, anyways? Do they think we who do have issues dont wish we felt good enough to go to a regular job or have a more predictable schedule? Sure, my life isnt your/their life but people dont need to say condescending remarks about "how nice it must be not to have to work" or "you wouldnt understand, you've never been in the workforce in a real job (i'd like to ask what working as a CNA was? What was waitressing in high school?) Lots of people are full time waitressess; just bc it may not be what you the person thinks is "good enough" doesnt mean we havent had "life experiences". In some ways I would just like to counter that ive likely experienced more in 29 yrs traveling for talks for MPS, having worked as a CNA, having waitressed, and meeting new people from all walks of life on an almost weekly basis with MPS and for APF than do many people who think they are "ooh so great" in a lifetime. As the saying goes; it isnt a matter of what you've done but the way you do it". (or something to that affect.) My point in writing this is just to make people stop and think before they say something. We all do it, I know there are times I say something and then I think about it and think 'wow, did that really just come out of my mouth?" or "how do I really know?"

The way I cope is by having information and being as prepared as possible about whatever it is I am dealing with and so although I dont always think of the 'right' questions at appts I do try to become as informed as possible before making decisions and I try to become a part of my provider team in making decisions and this in turn helps me to help others. It also helps me to feel as prepared as possible for new issues or changes and to feel like I have some sliver of control. Not everyone is the same and that is ok but I feel like the more information I have the easier I can rest about a decision and I feel like the better I can be turning around and trying to explain it to someone else. I definitely cope only becomes I can be informed about my care and because the decisions are made in the end by me with the help of providers and what they think.

3/3/12 -----
I saw the Hand Surgeon today and we're going to give it a month but he said it's a good possibility based off my last 2 surgeries that we'll end up doing more release/clean out work due to the   - tenosynovitis (build-up and inflammation). Tonight I have my one thumb that the tendon transfer was done on taped up as it makes it feel better and he wanted me to splint the wrists for atleats part of the time for this month so although I can only do tape for short periods it is easier to deal with and does make my hand feel better. I think he is a little amazed at the amount of build-up/recurrance that MPS causes as I dont think he's had any other MPS pts before and this would be the 3rd surgery on the L hand and 2nd carpal tunnel/synovitis release on R hand in addition to the tendon transfer that was done on that hand. Such joys. I do think MPS should only be able to strike one issue at a time! Wishful thinking, right!?

This is about it; will update when there is something further on any end,
Happy Easter!