Frustratingly grateful, grateful for Providers who don't give up

I saw Dr.Bragg today for the VP Shunt revision f/up, not surprisingly the incision con'ts to heal well and my hair is growing in, thankfully as the area they shaved is not in the greatest of locations this time. I always think, even if it is vain how much it must stink for people who have really thin hair to have shunts? Although I as always wished we could have figured something out today I know I can call her Secretary or Nurse anytime if things got worse and I am grateful even if the overall situation is frustrating that she stays on my care, not giving up and willing to see me if I think somethings going on or changed. I think the words I used in an update on fb today in talking about the appt where being grateful she is 'steadfast and caring' as she really wouldn't have to be.

I really didn't know what to wish for with this appt., sitting in Church yesterday I prayed silently to myself for God to guide us and for his wisdom to shine down. While with my Sunday School kids yesterday they are so inquisitive and yet so sweet asking if I was back and was I feeling better and that they missed me the week before and several said they'd been praying for me (so sweet). I think I hoped maybe we'd figure something out at this appt but also I have to admit I wasn't so sure the shunt tap was going to show a problem, atleast not yet. (I know thats a funny thing to say, an odd thing to say).
With doing the shunt tap a needle is inserted within the reservoir in the VP Shunt, the part that goes in to the ventricle in my brain where CSF is and with this Dr.Bragg can test how well the shunt is flowing/functioning and also is able to draw off some fluid to see how this would do.
It's one of those things where usually I can hear her drawing off CSF (sort of a mild gurgly or suctioning sound) but couldnt this time, perhaps bc the flow was good and so it took a bit after sitting up to feel the difference but WOW, is it ever soooo, sooo nice that difference when even a very small amount of CSF is removed! I felt a whee bit like I was on high energy this afternoon, running in and out of Target (I only had a short window between Appts) grabbing a few things before going to a Hand Appt (see below but good!), the store and driving home in awesome weather (sarcasm). It was/is nice to feel nice for that short while, to remember 'hey this is what humans feel like'! ;) Perhaps a boost that I needed given this past week has been mildly depressing, not trying to think about it to much but yet knowing I atleast felt reasonably better not that long prior!

My head just feels soo clear - my guess is by tonight it will be back to it's 'more normal' but seriously i'll take the relief I can get when I can get it! For whatever reason this removing a small amount of CSF and getting relief is a semi-common phenomena w MPS pts (maybe most Hydro pts., not sure) and then typically symptoms will return within a few hours to 1/2 a day.
I didn't want the shunt tap when she did it to show an occlusion (would be partial, whenever there is a full on shunt malfunction I know within hours and end up at the ER) vs partial occlusion the TPL Shunt is there to pick up a bit of the slack but on it's own can't do enough to handle pressure needs, which I have to admit i'm not sure I ever put those 2 pieces together before but totally makes sense after this last malfunction 2 wks ago and a few other experiences w complete shunt failure Dr.Bragg brought up today that we've had over the past couple yrs.
Obviously it's good the VP Shunt is working but dang it is soo frustrating knowing basically it is just a matter of time till it will occlude or stop working (more likely partially occlude) and wishing the 2 shunts would work better together to do a better job of getting fluid off consistently, more like when there are EVDs in place!
Even the D6-4 floor Pharmacist last time I was in-pt when talking to me about if she should go ahead and order the Enzyme Replacement or not, not knowing if I'd still be there that Monday finally made the comment "well we can always use it next time your back if not this time". LOL, probably not funny (actually it isn't) but yet it kind of is b/c it seems non of us have faith the shunts will just work for good and instead is always a matter of 'how long do we get this time'?
I didn't think of it till now but this just made me wonder if they (Dr.Bragg and her partner) have decided on who they'll hire as a 3rd Nrsgn on their Team.. Doesn't really matter but is curious.

I don't really know why I was thinking of this earlier but there in reality probably is not alot of reasons Dr.Bragg should have to believe me (I don't know?), I know I don't always have classic presentation while other times I do but thankfully she does seem to and she cares which means as much. I think sometimes maybe I am just amazed she hasn't given up and she doesn't seem like she gets frustrated at me that issues recur.. I know it's not my fault but what Surgeon would really want to have to keeep on keeping on revising someone's issue? Non-the-less believe me I am beyond grateful! My faith may waiver (that she'll not give up) but I trust her and am so glad to have her in my corner.
Her NP brought in the kit to do the shunt tap this morning and commented to the Med Student (a former Nurse, very cool, it would be really curious to know in a few yrs if that Student liked Nursing or being a dr better!) that Dr.Bragg is really unusual in that she likes to get her own supplies and prefers to do her own tests whereas many drs ask the NPs or other Staff (apparently, which I dont doubt) to do it for them. Just another reason to like her (Dr.Bragg) for her being so human, so down to earth. I am glad she is the the way she is though, she talks about her everyday life and she is funny + knows how to put things in human terms which helps put me at ease sometimes when I probably feel a little stresseed out. I have a great team of Providers overall but the very best ones are those ones who care, who can explain things in ways that I can understand (even if I understand most of the medical lingo sometimes their using funny ways to explain something makes it easier to remember!) and who go out of their way to try to help even when I feel like a nuissance.
The Hand Appt was good, I saw the PA which he was happy with the range of motion I have going on despite 2wks in the other splint and a few days in this newer removable one. The bruising is still pretty remarkable he thought (I guess it's more than with other hand surgeries i've had, lasting longer) but non-the-less it is cont'ing to go down and the swelling he thought should decrease in time. As far as the splint I can con't to take it off some at home but wear it anytime I am not home and f/up in a month with the Hand Surgeon. I can probably live with that. =)
Otherwise I just have to make the quick comment and i've written about this here before that I feel like when I am dealing with pressure issues I don't always feel like the nicest person. Well apparently, per reading a few of the ER Med Record notes from a few wks ago albeit I remember almost nothing of it I was still a very nice patient and nice to the Staff despite being pretty darn uncomfortable! I guess that is a good thing! =)
On an un-related and not really relevant but cont's to sort of amaze me observation; at UW atleast in the Peds Neurosurgery clinic and when in-pt Dr.Bragg and staff gown up in isolation measures (for a bacteria resistant bug from a few yrs ago that has not been positive/active in several years in my urine) and anyways yet at outside UW Clinics they do not use isolation measures nor does Dean Clinics, FMLH or CHW. I am by no means complaining (!!!) as it's nice to see Providers in their normal attire but it is mildly amusing how healthcare places are so far apart when it comes to how they handle patients with former drug resistant bacterias!? At CHW when I was in-pt most but not 100% of Healthcare Providers would do the isolation measures but they do not in the Lab or clinics and at FMLH they don't do any of it with me. Again, like I said I am not complaining as I find it a little ridiculous that a bug formerly in a patients urine requires lifelong isolation (gowning) measures but it is interesting..
Here are a few pics from the past few days, I love my niece and nephew! No matter how I feel these 2 are the best blessing I could ask for!
Thanks for stopping by,
Erica
Zan and I looking in Bell's giant doll house, Bell loves playing with my camera.

Bell and Zan (anyone who knows these two, knows how unusual it is to get a pic of them together!)

Sunday School kids

Discharged, revision #30-something. Feeling like crap

I did get released Sunday afternoon, I wasnt feeling any better or worse there than I could feel at home so there was no point in being there. My Sister had my neices 4th Bday Party Sun night and I have to say I just felt tired and not great so felt as if I was watching everything from the periphery. Kind of sucked (feeling so out of it) actually).
It has not been a greatly fantastic week though atleast I am home. I admit I feel kind of depressed at feeling like crap again and stinks that the stupid shunt had to fail when atleast we where having semi-decent results. Atleast I had felt like a human, headaches or not I could live w the way things where prior to last Mon. While I don't feel great I am trying to remain optimistic though that admittedly hasnt been easy as I wake up a 1/2 doz times or more a night (for whatever reason when I have shunt issues it affects my feeling like i have to pee alot at night). I have little motivation to do anything and yet have plenty I should do. I HATE THIS! ='/ Dr.Bragg has said to just call if I thought I needed to see her before next wk and i've been tempted but will probably just talk to her Secretary when they call w the f/up date. I don't want to need Dr.Bragg! I am pretty sure the shunt isnt going to sort itself out this time, atleast other times when it did i'd feel a little better each day and this time I just feel blah and aweful day after day after day.
Last week seems like a blur, especially Monday night to Weds after surgery. I really remember very little of the ER at BD, the ambulance ride to UW and I don't know that I remember anything really of the UW emergency room or being on the Gen Med floor at UW prior to the shunt revision Weds. I do know the hospital was full so that's why I was initially on a different floor and that the Nurses (apparently) specifically made it a point that I had a room back on the Neurosurg. floor following surgery Weds. I apparently had multiple conversations on fb with friends but don't remember these or anything I may have posted which is sooo weird!
As much as I feel like crap now (and I do, ugh it's aweful), reading evem this is near improssible (seems the stuff that is close up) I feel lucky that as much as I hate being in-pt these Nurses and staff have become like extended family and seem to really like me/want to make my stays a little better. As weird as it is I feel like some in the Neurosurg. floor/Team are more like family (in an extended kind of way) than are some of my own extended family.
Honestly it is a little depressing to feel worse now than I did prior to last Monday night when the shunt really failed. I may not have felt perfect up till Monday night last wk BUT I did feel more human and I felt atleast somewhat better, I felt like things where going fairly well for once! Honestly I feel like the hardest part of shunt issues atleast on a mental level is I feel like I am in a fog and like interaction with other people is much harder.
Hard to explain but I know i've talked about this before here. I don't feel like a very friendly person as it genuinely feels difficult to make small talk. At the same time i'm sure in conversations lasting just a couple mins. I just seem either unfriendly or probably aloof/rude or some people likely don't notice. I feel bad about and hate this and yet it's as if I am in a fog so I am aware of my change in personality but to tired to change it.
Today I just felt like there wasnt anything we where doing in-pt that I couldnt do on my own out-pt. Dr.Bragg when she stopped by before I got out commented something along the line if things didnt improve she wasn't giving up and if needed she felt we had options + we could fiddle more with the shunt function/placement or settings.
I did keep forgetting to ask her what settings the 2 shunts are at now post-last wks surgery so I am not sure if we have any room with settings. She to it seems is hoping due to the sudden and severe nature of this shunt failure that it will just take a few more days for my brain to calm down and settle back to a more normal.
As far as f/up i'll see her next wk (a week out from now) probably on that Thurs 1.5wks from now. If things would worsen or I needed she just said to call sooner. I kept wondering this week given the shunt failure was so sudden and so severe is the TPL Shunt working or working optimally? I guess this is something i'll have to ask Dr.Bragg if I don't feel better. Who knows but hopefully it (TPL Shunt) is!? =/
On a side note Dr.Bragg mentioned yesterday they are interviewing a new Neurosurgeon to join her and her Partner in their Peds Neurosurgery practice, it sounded like someone who has been in practice for some time. She was hoping this person would be open to trying new things and could perhaps help her - who knows but I am grateful she still seems to not be giving up. I think that will always be a fear of mine. I do just wish I could feel better though. =/
Will try to update soon, sorry this has been less than awesome,
Erica

Sudden VP Shunt failure, Admitted to UW, surgery Weds.

Well i'll come back to it and update how the Hand Surgery went last Thurs. but first some other unexpected shunt issues began Monday might.
I'd been having slight increase in Headaches over I'd say the last week or maybe more BUT nothing that was so bad I thought 1 of the shunts would quit working. I still was somewhat optimistic this revision done in Nov. was the best I'd felt in a long while which believe me was nothing short of exciting!
Sunday I'd taught Sunday School, visited my parents a bit and went to a Church committee mtng Sunday evening followed by getting dinner w 3 of my Church friends, all feeling pretty fine minus the mild inconsistent but consistent headache that wasn't really new.
Monday was ERT (infusion) day which ran a lot longer than normal due to our continuing to try to figure out the best ramp up and final rates to run the drug over 5hrs. In total infusion ended up lasting 6hrs but I was still able to get home about 7:30(after leaving my Apt about 9-9:30. I think I put my PJ's on and sat on the couch probably watching TV or zoning out or something due to being tired! Pretty much out of nowhere around 8:30-9 I started getting a really bad headache that is one that just nothing I did would ease it. I think I gave up and laid down around 10 before getting u take Zofran and then a bit later a 1/2 a dose of Ativan followed by the other 1/2 not to long later.
I managed to sleep on and off a little but by the wee hours of the morning I was throwing up very frequently. I think I must have done that for about 4-5hrs on and off trying to stay warm (due to chills) and keep ibuprofen and adivan down (with pretty much no success but I kept praying I'd feel better by morning. Needles by around 7:30-8 I called my Dad and asked if he'd come pick me up and take me to the local ER in BD. I still wanted it just to be the flu or something even though I knew it was something shunt related in all reality.
At the ER between throwing up in their bathroom next to the registration desk the Nurse (former HS classmate ironically) I was taken back to the ER within a few mins, an IV started through the Port (as miserable as I felt I could have hugged the Nurse for being so open and willing to use the Port thus our not having to fight with terrible veins. Once that was placed they did fluids and more Ativan + I think a pain med and Zofran. Not to long after the doc peeked in, asked who my Neurosurgeon was so she could do whatever Dr.Bragg wanted or find out if she (Dr.Bragg) just wanted me to transferred to UW. In total although the morning is a complete blur I don't think I was at BDCH's ER for more than an hour or so. The Ambulance came, was transferred to UW and I have to admit I remember pretty much nothing of the rest of the day.
Once they tapped the VP Shunt we knew it wasn't working so Dr.Bragg went back and forth between doing emergency surgery that night (but having to manage to high of an INR) or waiting till Weds for surgery and giving multiple (3 I think) bags of Plasma to bring the blood thinner (INR) level to a safer range. Surgery I couldn't even quite tell you what time it was at but Dr.Bragg did say with their using the Lightascope (sp) went the best she's seen it go in a long while. For surgery if I remember what she told me even remotely close they exchanged out the old valve but used the same Codman (I am not sure and haven't thought to ask her of she opted to stick w this valve bc we've had reasonably good luck w it (clearly not the best but atleast I feel semi-human with it most of the time) or bc having been a unplanned surgery did she not have the newer valve she'd talked at the Appt about on hand.
She did tell my Parents after surgery they ordered and will keep on hand now an extra set of each of my shunts (VP Codman Hakim and LP Codman Hakim which they use in the Thoracic spine) to prevent the delay they had during this surgery.
She did say they had a problem w a tiny part of the shunt (a connector piece maybe, cant remember for sure) so they had to call their Codman Rep who was 1.5hrs away and have her bring a whole new shunt Codman Hakim shunt and in the mean time they just kept me asleep on the table + Dr.Bragg and her colleagues just kept watch while waiting in the OR room.
I think once they had the new shunt system they took off the piece they needed and where able to finish the shunt part changes. She didn't really say why she thought this occlusion would have happened so fast and so (for me) severely but commented that was the worst she'd seen me since 2 Christmases ago when I'd gotten released the afternoon prior to Christmas Eve day and then was brought back that same night also by ambulance. It definitely makes me wonder why some occlusions are so much worse than others, other than perhaps when I am able to go longer w headache and related symptoms the VP Shunt must be workin minimally whereas this time it was completely failed? Not sure but was TOTALLY unexpected! I was actually somewhat optimistic we'd make it longer, maybe even a few more months or something the way I'd been feeling the closest to normal I'd felt in a long time!
Non-the-less I am more than grateful my Dad could come pick me up Tues morning, that the local ER calls and works w Dr.Bragg to come up with a transfer plan without really a second guess or my having to ask them to.
I don't feel as good as I had after Nov's revision but am hoping maybe in a day or two I will. I am really grateful to Dr.Bragg and her Team, to my Family and friends for the huge outpouring of support and concern and that this surgery now to is done and over with!
As far as the Hand surgery that minus some mild disagreeing with the Anesthesiologist over whether the stress dose steroid needing to be given or not I think surgery went well. He didn't come talk to me after surgery so any info I have is whatever he told my Dad.(that always gets on my nerves when Surgeon don't bother to talk to the Patient and explain their thoughts or what they did).
He told Dad I guess the carpal tunnel was definitely severe again (the last release, done for a 2nd time was performed by Dr.D in Milw. about 5yrs ago). That was a pretty extensive release but this time was done even more so and along w the tendon thinning (I think that's what it is) Dr.Bliss did what is called a 'fat pad graft' where they take hypothenar fat (I guess) and use this to protect the newly released carpal tunnel area due to the other surgeries.
I was supposed to have the 1st f/up to get the splint changed on Tues but due to the ER and getting transferred to UW that appt was rescheduled to Thurs (today) but I again had to reschedule. Now i'll follow up next wk., they said if I got out to let them know and they'd fit me in early in the wk but given it's ONLY to change to a new splint and I go back to get stitches out (the splint stays on for a few more wks) on next Fri I may just see both the OT and the Hand Surgery P.A. on the same appt. We'll see.
Right now I am just hoping the headaches improve some more and I get back to feeling closer to prior to this VP Shunt fail/occlusion!
Will update again soon,
Erica

P.S on a more positive note the Foundation I work part time for asked if I'd like to go to the WORLD Lysosomal mtng mid-Febr again (this yr in Orlando) so my plane ticket, hotel, etc. are all booked and planned! It is a good mtng with a lot of info on not only MPS but the related disorders and most if the drsa patient might want to talk to all under rook!

Pulmonary, Hand Surgery

INR (blood thinner level) is thankfully low enough for tomorrow's surgery, arrival is 6:30.

It's defintiely been a crazy week since I last wrote last Weds about the Pulmonary Appt.
Friday I started getting the same coughing and side pain and chest discomfort all over again (it had been hugely, vastly improved even over the improvement since the VPS revision) and the cough that was incessant, occuring literally almost from the time I woke up till i'd finally fall asleep. The wknd was more of the same so at first I thought maybe it was just cold symptoms but didn't seem like it as I had no other real symptoms that would have made it seem that. Non-the-less a pretty not that awesome wknd! I did manage to have Zan (my nephew) spend Sat night, we went to Church on Sun (no Sun School during the holidays) and after I went to lunch with some Church friends. Not altogether a pleasant wknd!
Monday I was beginning to feel somewhat better, the cough was easing up and side pain to so I do think it was just the shunt and my body was fiiinnnaaallllyyyy deciding to catch up on absorbing whatever fluid had drained/or was sitting there.
B/c of Thurs's out-pt surgery (3rd carpal tunnel release in L hand, tissue/muscle graft) my PMD was a little concerned so I gave Pulmonary a heads up. They in turn made an Appt for today with Dr.Biller's NP as they where concerned it was fluid getting worse (I had let the Nurse know yesterday when I was talking to her I thought the symptoms might already be starting to improve) so Xrays where re-done and Appt went fine. Same R side lung is reduced breathe sounds but otherwise the Xray looks a little better still than even last wk. as she said the area was expanded and she couldnt really see any fluid I think is what she said.
Maybe it's just going to be a matter of when fluid accumulates I get symptoms and they come and go (damn will that be annoying) I don't know. Hopefully if that is the case it isnt always as bad as this last wknd was!?!?!
Thankfully ERT running at 5hrs does seem to make a difference to, the cough still ramps up some but not as significantly as it would when we did 4hrs.

Headaches have been a little worse in the last 3-ish days but nothing I can't live with yet - mostly the mornings. The NP asked me today how I knew when the fluid was better (I had said something how I figured there wouldnt be much if any change on the Xrays and that I suspected it was improved from the wknd) and I said "Because I have varying degree of headaches", the look on her face was priceless! She in turn responded though, "that makes sense, I wouldnt have thought of that". =) This is just b/c the shunts to tend to drain better the longer I am up, so I typically wake up with a headache but somedays are worse than others.

As far as this surgery I have to give kudos to the Hand surgery Team BUT most especially to my PMD who does seems to be amazing at coordinating what she feels should be done and for reaching out to Providers when she feels we need more information or answers. Thankfully as I wrote at the top the INR was low enough (1.31, they wanted it 1.5 or below today) as otherwise we'd (per Cardiology) have had to reschedule. Per the Nurse in the out-pt surgery Dept. it was written in my Hand Surgeon's notes to give Vit K. should INR have been higher so it seems everyone likes this med except my Cardiology Team!
I asked my Primary dr if she thought there could be periods where there was no CSF in the Pleural Space (I think thats what todays CXR showed or even less than last wk and the area expanded more) or why she thought it might cause intermittent symptoms. Part of her response: "I would expect some fluid in the pleural space from your pleural draining shunt but the quantity is the problem. I am not sure how much is normal and how much is too much. I would say your symptoms should help drive the answer (cough/ chest pain/shortness of breath). Likely too much fluid. I hope that helps."
I'll try to update atleast a short update soon, will be harder since I am L handed and that is what this surgery is on. I know last time the splint was on for approx 3wks (or maybe it was 4, can't remember) due to the graft portion of the surgery. The things I do for various relief on various body systems!
Thanks for stoppingg by,
Erica