Monday, April 28, 2014

Neurosurgery adjustments, Cardiology, Boston Marathon wknd

Sometimes I put off these updates where it seems like it might end up being long, which even though I kind of like writing it's a matter of putting thoughts together from all the things that have occurred since a last post!

Although I didn't expect to have to I ended up seeing Cardiology today (Dr.Earing) after I called to give them the usual heads up that my Infusion Nurse was running the pre-ERT INR result across the path from ERT (Genetics Clinic, Curative) to CHW's lab.
Jane, Dr.Earing's Nurse answered their phone and I gave her a run down of some of the symptoms that have been occurring over the past week and few days (the shunt or pleural space seeming irritated/uncomfortable when breathing, worse when laying down. The feeling like I am breathing under-water sensations, (also intermittent) which worse at night and HR being very high despite the BB med + weight being up quite a bit over the past few days (I don't weight myself but Michele my ERT Nurse also commented on the big jump today pre-ERT).
Jane in turn talked to Dr.Earing who asked if I was able to come over after ERT which I was and so I saw them.
Per Dr.Earing Magnesium and BNP levels where fine (magnesium can be affected I think by the high Lasix dose and BNP is one sign of heart failure they look at). B/c these where normal and b/c he was hesistant to increase the Lasix (gets fluid off) dose any further (quite a high dose already 2x a day) and I've not tolerated a higher than 80mgs dose of Nadolol (beta blocker) + this med hasn't done a lot to get heart rate down as it is. He also did not want to adjust the Spironolactone and didn't really want to switch to a Calcium Channel Blocker (CCB) due to the affect these have on blood pressure and mine already being very low. Instead he opted to add a low dose of Digoxin which is a med that helps a "stiff" heart to pump better I think was how he explained it.
This is a med he had talked about last time and feels it is the best bet with the least likely of addt'l side effects.
He made the comment that he wanted to do a Chest Xray but like anytime he sees me he wanted to be cautious in any decisions he made and make only baby steps compared to how he might treat most of his pts regarding what interventions he might add or try. He added that b/c of the MPS he always feels like he is learning from me, "you make me think everytime" and regarding the Chest Xray he said "I won't likely know how to interpret it" (given the TPL Shunt). He said if he needed he would access the Care-WI network for UW's Med Records and compare this CXR to ones done recently post-surgery by Dr.Bragg.
I am just grateful he is usually willing to admit if he doesn't know and he isn't overly aggressive about treating new symptoms but at the same time belives me and want to try to improve quality of life.. I think really those are 2 of the best things you can ask for from a provider!?
He did say he was surprised the Heart rate hasn't come down yet given we are this far out from the 2 open heart surgeries (OHS's) and that in most patients Sinus tachycardia-post Heart surgery improves within a few months. His big concern is why the Mitral valve worsened so quickly after the 2 OHS's when it was mild after both and from what he has read it isn't standard even in MPS pts for it to suddenly just progress after another valve is replaced. He did comment as he has in the past that the fast heart rates likely has some do to with the mitral valve gradients increasing.
He added something along the lines "You con't to do things your own way" (he was joking, regarding my body, the MPS makes it's own rules).
I keep the normal, as scheduled f/up in a month to see him/Nancy and actually forgot to ask about what the Coumadin/INR level is and when do we need to re-test that (done at ERT so is easy enough).
Regarding if I had any issues with the Digoxin or problems between now and next months f/up he commented that given I am at ERT (same hospital) every Monday to just keep them in the loop if there are any issues and he was good with arranging his schedule to just see me after ERT (hopefully won't need to) =)

I think I wrote an update the other week after seeing Dr.Bragg about how we where going to post-pone making any adjustments to either shunt last week and she had said if I wanted I could see her on Tues once I got back instead of waiting till Thurs. I initially had thought I would do this but it turns out I got very little sleep Sun night in to Mon and got home very late Mon night last wk with very little sleep again in to Tues morning.
I just was to tired to want to drive to Madison. Hopefully whatever we try tomorrow will help. The uncomfortable-ness (is that a word!?) around the TPL Shunt and especially when I lay down has cont'd so I am hoping maybe Dr.Bragg will have some thought on that to. <--- We turned the VP Shunt down by 2 settings to 50 last Thurs I believe while the TPL Shunt is at 40. This helped some, not entirely but still some is better than nothing!

Thurs, April 24 -
I saw Dr.Bragg today (almost a wk ago) and initially we where going to adjust both shunts by one setting but then giving the odd and often strong discomfort I feel along the TPL Shunt we opted instead to leave that at '40' (30 is the lowest) and adjust the VP Shunt from '70' down to '50'. She said if I cont'd to have symptoms I should call and schedule to have the shunt adjusted again although it could be w either of her NP's to (whom are also good). As stated above there is a small change in headaches though I still feel so tired. Really I often feel like I've slept about 4 hrs when I've been going to bed earlier and though also having to get up earlier than usual I still managed 9-11 hrs the past few days. It could be a combination of all the various symptoms going on though, who knows. I don't know if I should have the VP Shunt turned down another notch or not. I thought maybe I'd wait till mid-to end of this current wk to see.

(This was written a few days ago, before today's Cardiology Appt and before knowing they would want to see me).
Probably not related but have been feeling symptom of short of breath (the feeling like you are trying to breathe underwater or have held your breath to long and can't get enough air now, not the 'I just ran really hard' SOB feeling. Seriously who knows who symptom like this belongs to though?
Honestly I often don't know which specialist a symptom 'should' belong to but this started worse yesterday especially when I went to bed but tonight occurring even while I am sitting completely upright in the chair. Hoping it will just improve and if not I guess I can ask my PCP tomorrow who she thinks I talk to or what does she think in general.
Monday i'll re-check INR for Cardiology so while I seriously hope for pete sake this is no longer occurring then if it where I can mention to them when Nancy, the NP calls with the result and any med adjustment (if needed). AGGHHH sometimes!!!!

(Boston) Marathon Weekend

I left for Boston on a Fri (Good Fri!) and though the flights where a bear (I also didn't have a direct route so took longer) I was happy to be in Boston once I got there; my friends the Siedman's whom I was staying with again picked me up from the airport.
Sat I was able to sleep in (thankfully) some and met up with Jessi in Copley Square after Jenn dropped me off at the train station which I took in to town. From there we took some pics near the finish line (the BAA 5k was just finishing and was a ton busier than it had been last yr so harder to get as close) after which we went and met up w some of the Genzyme-RFRD's Team members who where also getting manicures with us. Jess and I and one or two others who got their nails done opted for MPS purple and one nail done in Genzyme green w/a purple MPS ribbon decal on that one finger! AWESOME!

From manicures Jessi, Colleen (another runner) and myself headed out to Allston, one of Genzyme's manufacturing plants (the very first time I was to Allston back in 07' Aldurazyme was still bottled there but it is no longer) where we helped finish setting up for Sat. nights GRT-Patient partner/Family dinner (very fun!).

The dinner was a good time, at the table Jessi and I sat at there where about 6 other people including off hand 2 other runners, the GRT Coach and us + another family. SO FUN!

As far as actual race day Jessi finished in under 4 hrs (3 hrs 45mins I believe) and did AWESOME! Although I missed being home for Easter I was also sooo happy to be there to root for Jessi and so happy to see her smile when she crossed mile marker 14 (our Genzyme/Running for Rare Diseases Team headquarters), we quickly got to hug and say a very quick few words and then she was off again! I had a semi-early flight (there weren't great options for that Monday night when I booked) so didn't get to congratulate Jessi after but we've exchanged emails, texts and other messages since! I can't wait till next year again!! THANK YOU Jessi and the entire GRT Runners! I posted some pics throughout this blog entry and added some to the slideshow at the L of the blog updates.

Jessi is mailing the poster/picture that Genzyme/RFRD had at the Banquet of Jessi and then myself and Ben (IMO if you look closely at the pic means 'In memory of') which was I'd guesstimate about 2x the size of an 8/10 frame! It is sooo cool!!!! Thankfully the Siedman's will also be able to get of the poster/print made as I think according to Stuart and Jenn it seemed to mean a lot to them that Jessi also ran for Ben's memory. This whole weekend w the Siedman's and with Jessi + the Team was simply amazing!
As a really cool side bonus Jenn took me to the Church where Ben's funeral services where held for their Easter Service Easter morning and the service was simply put BEAUTIFUL! The sermon ('The 1 lost sheep (how the Shepherd left his 99 to look for the 1) and 'it is good') was one of the best I've heard in a very long time, this is saying something as I've felt the Pastor's at my Church are very good! The Choir and instruments where beautiful and the Church was gorgeous + the day was fitting! I pretty much slept all Sun afternoon but then Jenn made a dinner which was VERY good and some friends of all of ours came in that night from NY (their sons also have MPS III). Such a great wknd!
I am so very thankful to the Siedman's, to Jessi and Genzyme + the Genzyme Running Team and to Vanderpool's + all the wonderful memories from this wknd! I was happy to be home late Monday night but at the same time even though it meant missing Easter here I am so grateful for all the memories! Paying what I did for my plane ticket was even worth it! =)
As an added bonus My Mom made a Easter-ish dinner again yesterday so those of us kids who wanted would come over and be together!) =) Zander, my nephew spent Sat night and after Sun School and Church he and I went over to my parents where several of my siblings and there families also came. All was good!

Thanks for patience in getting this update written,
God Bless, take care,

Thursday, April 17, 2014

Blessed by Looking good while feeling bad..

What a week this has been; I didn't feel all that great (but certainly not all that bad either) when I got discharged last week and I was able to finally catch up on sleep by Fri and Sat. Over the wknd though I really started to feel pretty bad and though I don't honestly know this is what it was the discomfort started it felt like along the TPL Shunt line increasing throughout Sat., worse when I would lay down (keeping in mind I don't really lay flat) and by Sun though I cont'd on with the things I do the discomfort just wouldn't go away and headaches where seeming to increase. VERY frustrating! Non-the-less you mostly have to just keep on keeping on b/c what else can you do? =/
Non-the-less Monday I went to infusion but was only to happy to be home and just didn't go to Ladies Aid, (which kind of stinks b/c I liked it when I went last month =/) Tues I cancelled dinner plans with friends as I felt even more awful and other than taking a shower pretty much did nothing all day. Super crappy!!! I don't know if I have felt as depressed as I had most of this week in a long, long time, if ever.. =/ I genuinely try to find the positive in most things but even this week, even with Boston coming up tomorrow I just really struggled with how bad I was feeling a vast majority of the time and not feeling really depressed.
The title of this blog is pretty much self explanatory, I often hear "You look really good" (which isn't a bad compliment when you feel bad, I just sometimes when you feel bad you do wish people knew how bad you felt I guess although I also don't want sympathy).
I've often been told I do "looking bad really well" which I guess isn't a bad compliment to hear? Sometimes I just wish it was easier to get answers, for instance I do get tired of the shunt issues and am a bit skeptical that our trying to adjust the shunts is going to help but I guess I hope it will be that simple.
Sometimes I wish there was easier ways like when we used to just tap the shunt (to see how flow was) or if MRIs worked (they don't really in most of us MPS pts) and often it is intermittent flow or obstruction which isn't visible on imaging. In other words I am just frustrated by these headaches and symptoms that just never seem to stop; I mean we do get the shunts working great for awhile and then it's like back to square one for what seems like no reason. To boot I think the MPS storage doesn't help as that isn't visible and I think partial revisions aren't as good as the full revisions as I rarely ever feel as good as when the entire shunt is just replaced. In any case it doesn't matter (it does but it doesn't I guess) as I can't control what decisions are made in that regard, really. =/ I just want the shunts to work fully!!!

I had a f/up Appt with Dr.Bragg today, she has known I was going to Boston for some time and really was trying to make it so I would feel better which I truly appreciate of her. Anyways so today we where trying to decide what if anything to do w either shunt given how bad this week has been (Weds was a little improved, at least I was able to get to PT and I made it over to my parents later on, it really was the nights and mornings by Weds that where so bad-during the day I didn't feel supreme but I also didn't feel as terrible as the wknd/earlier in the wk.)
In any case although I would have liked to adjust either shunt (I think one or both needs to drain more and I do think something is going on w the TPL Shunt as I've NEVER felt this kind of discomfort before, just never and it is disconcerting how uncomfortable it feels. In any case even though I wish in a way we could have changed the setting on either shunt and known it would be ok I also think Dr.Bragg and I made the right decision by not changing anything for now and i'll see her next week when I get back.
We tentatively made an appt for Thurs but she said if I needed to just call when I got back and she would meet me in clinic on Tues (a non-clinic day for her) and we could make a decision on what to do w the shunts then as far as adjustments. For those of you reading if you believe in prayer just pray flights are tolerable tomorrow and this wknd is good; I am both looking fwd to going to Boston and admittedly a little nervous. I also am sad about missing regular Easter goings-on here at home and not seeing my nephew and niece. I wish oh how I wish the Marathon had been on a non-Easter weekend this yr. =/

I'll update sometime next week, for those of you on fb I'm sure i'll have updates or pics on there throughout the wknd.
Thanks for stopping by,


Thursday, April 10, 2014

Home sweet Home (released post surgery 3/28)

I was released yesterday; we dialed down the TPL Shunt to 40 (it had been at 50) so just a small change but one I felt we needed to try and Dr.Bragg was ok with. Per Dr.Bragg one of the Neurosurg. Residents and her NP took out the stitches which where both dissolvable and the one head incision (the one that kept opening up) removable. Total there where 5 incisions.
Last night was not the best (threw up a couple times a couple hours after being home) but then once un-packed I pretty well just went to bed.

I had to be up fairly early this morning to be to a Pain Mngmt f/up Appt in Milw that had been moved to this morning from mid-afternoon today. Needless to say I like that provider but her office is seriously a mess and I am simply amazed the Nurses and Staff at the clinic she practices at (not her actual office) don't lose sleep/get gray hair over her Secretary.
Not only was my Appt change not on their schedule (they still fit it in, I just had to wait 2 hrs for Dr.B to get there, NOT Awesome!) AND I overhead 2 other patients/families who had the same issue, they had scheduled appts but the Secretary 1. never wrote the appt down (seriously!?) And yet I believe it as this has happened to me multiple times. The other patient was taken off the schedule but not notified.
I am not sure where those patients come from but I drive an hour all appts including this one so needless it is frustrating as h*ll! She is a good dr but soooo in denial about how her office is run!

I follow up with Dr.Bragg next Thurs so until then probably won't update unless something changes.
Other little things in the next week are I am teaching Sun School on Sunday (I knew I could take a break but I really like our girls and we have off for Easter and group Teach the following Sun.). Monday is ERT + re-check INR (same hospital) and tomorrow (Fri) I have PT starting again. Then Tues next week dinner w 2 friends.

I am realllly, really praying I start to feel some better,
If you believe in it say a prayer (or 2 or 3)
I leave for Boston next Fri (Good Fri) so am sincerely hoping I feel more human or atleast less worn out/less headache issue by then!


Tuesday, April 8, 2014

Feeling discouraged..

This has been a semi-difficult and not altogether enjoyable past 1 1/2 wks - sure being in the hospital is never fun but being here when your trying to figure something (the shunts) out and feel you keep running in to a brick wall is down right depressing. This is definitely not anyone's fault just the way it is with MPS and complex underlying issues w the Hydrocephalus.

I can completely have patience to make adjustments and give it time but admittedly haven't felt very good since mid-late last week when we made the 1st (2nd maybe) adjustments to the VP and then TPL Shunts; the first 2 times w noticeable improvement as they where over draining but since then it feels like we neither make headway and symptoms remain un-changed ie afternoons are ok but nights and mornings I down right dread. I joke that maybe I should just not go to bed but really it wouldn't matter as mornings are difficult due changes in dynamics with how CSF flows and/or is absorbed at certain times of the day.

I don't want to be here in the hospital but I also dread the next few weeks at home; I mean at a certain point you just have to push through symptoms but certainly still doesn't make it any easier when you feel 'driven over by a semi truck' tired and vision + head are so miserable due to the pressure changes and in my opinion one or another of the shunts just not working at full capacity.

Honestly in talking to other friends both whose kids have MPS and Hydrocephalus and non-MPS Hydrocephalus parents I feel like even though our shunts typically show as 'working' once partially revised (at the proximal or valve locations) but there (again my opinion, my friends opinions) must be something playing in to the overall drainage ability of the shunts to cause the entire shunt(s) to look as if they are flowing (and they are) but isn't flowing as well as when complete shunt replacements are done?
Why this is I have no idea but several of us wondered in the case of MPS is the protein in CSF along w the GAG storage cause the shunts to get 'mucked up' but still able to flow partially? In non-MPS Hydro cases it is likely similar issues probably having to do w protein or something else in the CSF of some of us pts. along w for all of us likely compliance issues in our brains (so inability to adjust between working fully and partially working?)
I have a terrible memory for asking people like Dr.Bragg questions but am hoping I will remember to ask her what she thinks we might be able to try if another small adjustment to the TPL Shunt tmrw doesn't work? I believe she is off until after Easter (good for her, bad for me!) so the next couple wks including my trip to Boston are likely going to be exhausting and probably a little depressing..
Another MPS parent asked me the other night if I thought IT-Aldurazyme would help our shunt/compliance issues and honestly I don't know but I know Drs. Patti Dickson and Emil Kakkis both feel it might help us. I would give just about my L foot to try it and see if it helped!
This is another thing I need to try and remember to ask Dr.Bragg (if she thinks there is anyway possible we might be able to get a single pt.-off label IRB to try the Aldurazyme in to the VP Shunt? It's something I've written about here, that Dr.Bragg has expressed interest in but felt it would be really difficult to get an approval here. Drs. Dickson and Kakkis have said they would help but I don't know. I wish b/c these shunts are going to drive me to start drinking (I don't much like the taste of alcohol)! =/

Needless to say this has been a fairly difficult couple wks, again not anyone's fault just kind of depressing how very good the shunts can be when they are good and yet so frustrating when we try various things and no or little overall improvement. I did have hope at first till probably Sun/Monday the adjustments would help but I am also not holding my breath that when we adjust the TPL Shunt down tmrw it will make a significant difference
I haven't given up hope just feel discouraged I guess as well.

Anyways sorry to write such a down update,
Thanks for stopping by,

Saturday, April 5, 2014

Still in-patient 8days; Hard post-op on many levels

I don't know why I write these posts but then either never publish/share them or finish them vs coming back multiple days later and finishing basically different thoughts. Non-the-less this is from both earlier in the week (below) and the past day or two.

It has definitely been a rocky week with plenty of up's but seems like many more down's due to cycling between high and low pressure issues. Tues I think it was we re-programmed the VP Shunt from the lowest '30' setting to '40' and then on Thurs we re-programmed again by 2 '10' increments to '60' which helped some especially during the day with the low/high pressure swings.
Nightime cont's to be a challenge so i've spent many of the nights avoiding going to sleep only then to wake up every couple hours due to pretty significant headache symptoms.
Today (Sat morning) we opted to turn the VP Shunt up by another 2 '10' increments to '80' which tonight will be the true test.
Honestly when I feel good I feel ridiculous being here and am bored out of my near skull but then when I have the symptoms I know being at home would be nothing but a waste as I would just end up back here.
I am truly hoping and praying we are able to figure this out, that Dr.Bragg cont's to think outside the box/have much patience and she doesn't get upset at me for this being a less than smooth recovery/symptom recovery.

It has been really disappointing that with both shunts working completely we haven't gotten back to the really good, almost symptom free way I felt pre-the past month or so? =/ I don't/can't blame anyone as it is just the way it goes with MPS and especially with when you have brain compliance issues (which means your brains pressure needs can change often). It is often very frustrating!

On a different note, I am praying we figure this out as the Boston Marathon is in 2 weeks (2 weeks!) and I soooo want to feel better for that!
Probably not related but heart rate has not been under the high 80's and mainly in the low-to-mid teen 100's (100-118ish typicaly) since Fri/Sat a week ago so that is more than a little annoying and causing skipped/double heart beats. Additionaly I itch like crazy nearly head to toe, worst being the incisions on my back and head; not much I can do except ice the areas, try to do lotion around but not on the back incisions and otherwise just ignore it (not easy!)

Written 4/3 -
I've talked about this quite a bit in the past and typically related to surgeries; I have a lot of issues with trusting providers (people in general) even though I know deep down in the case of like Dr.Bragg I really do think she wants to help and probably wouldn't give up.
I think it is so many bad previous experiences, so many other Neurosurgeons who didn't believe me, told me I was making my symptoms up or who initially would see me but then when the answers weren't clear cut they wanted no part of my care.

I know I need to work on my trust and especially my trust in Dr.Bragg, I need to do a better job of talking to her about my concerns (I am getting a little better) and I probably need to not keep so much to myself as she/her team can't help if they don't know. It is just INCREDIBLY hard though; so, so difficult to let down your guard and in a way be vulnerable b/c I do feel like when I do that, will that be when she to would stop helping? Probably not but like I said in my last entry, rational thoughts are not always that easy when your over tired or not feeling well and you have a hard time to begin with gauging someone's thoughts on your situation/care.

Last night (Tues or Weds) I hadn't talked to Dr.Bragg or her team yet but wondered due to how the symptoms where occurring if we could consider either adjusting one of the shunts (likely the VP) or perhaps tapping the shunt to see how well it was flowing. My Nurse was able to page the Resident and they talked to Dr.Bragg, who had been by earlier in the day but I must have been sleeping as Dr.Bragg said today she didn't have the heart to wake me as I looked exhausted and typically I will wake up when she is outside my room but had not this time.
She told the Resident that we could definitely try re-programming the VP Shunt valve but she'd feel more comfortable if we did it in the morning (this morning + the programmer was locked in the NP's ofc) which we did today vs one of the Resident's trying to do it.
Needless last night/early this morning where pretty aweful with wild swings between high and low pressures and a lot of sleeping/pain meds but thankfully our resetting the VP Shunt up by another '20' to 60 (goes in '10' increments) made a pretty significant difference. Although I still have the swings in pressure between high and low it is nothing like the past few days/much of the week had been.
When I talked to Dr.Bragg tonight she said depending how tomorrow morning was we could reset the VP Shunt another notch up if need be. Based off right now (2:45am) I think we'll likely have to do that as mornings are typically one of the worst parts of the day and my stomach currently feels like it is riding a roller coaster. Yuck!
One thought Dr.Bragg had regarding why the sudden need to dial the VP shunt up (she wanted to do one shunt at a time which makes sense) was with our replacing the ill-working previous VP Shunt (she feels based of the revision last week that it was either partially blocked or completely blocked) the new shunt would basically be "wide open" and free to flow so having it set at the lowest pressure would be to much and my non-compliant brain would then cycle between high's and low's in pressure (dumping and filling) causing the symptoms I've had.
Made total sense to me and was something my Mom and I both wondered since the surgery last Fri though symptoms progressively got worse over the almost week since that surgery.

When I was telling Dr.Bragg about my fear that she'll get tired of the never ending (so it seems) shunt issues I said a few things and stated my concerns but also said about knowing I need to learn to trust her more given it has been a rocky almost 3 yrs and she still has been by my side. She made a funny comment to the effect of "Your stuck with us, we're not giving you up" and then said a few other things. Somehow she always knows how to make me laugh when in all honesty I feel really uncertain..
She feels like it may take us a bit but we'll have to figure out what my brains "new normal" is now with these new revisions and adjustments.
I really tried to share my feelings to Dr.Bragg's 2 NP's today, which is something I've done hesitantly in the past to, because I do feel like it is the only way they know how scared some of this all makes me (I am not the worrying type but I do worry about being abandoned shunt wise again). Since we've gotten the shunts at least working better for periods I've been able to be involved in more things (teaching sun school, church committee and book club, seeing closer friends more, traveling more and of course my part time work w Gene Spotlight).
When Dr.Bragg came by later this afternoon I really tried to be honest about how uncertain I feel, how I know I need to trust her better and how past experiences have made me more jaded towards what providers say.

This is a link to an article I wrote about my friends, the Siedman's whose son Ben had MPS III and who passed away in Febr as well it is written from my perspective about my Genzyme-RFRD partner Jessi:
Thanks for stopping by,

Tuesday, April 1, 2014

(My) questions about the VP Shunt, has been a trying few days..

So on my part I know quite well I don't always process information correctly especially when I am tired or not feeling well. Well, needless this admission I haven't been feeling great since Sun (sun night I think) as far as being over-tired, uncomfortable and especially the headaches and related (I think) symptoms. Biggest issue is not only the headaches but then vision (intermittently fuzzy), bladder is kind of whacked this time ie to go even though I have to pee I have to really focus on the effort/strain (vs being something that just occurs) and some other things like my head feeling very heavy/in the clouds and as if there is a giant balloon filling from the inside out or a very tight (all over) head band.

I had wondered where these symptoms due to the VP shunt now being fully functional and thus set at the lowest setting (like pre-surgery) was the shunt draining to much and thus intermittently occluding? Pressure once CSF drains and the catheter collapses (my theory) does pressure then get high enough to force the catheter open again, temporarily relieving symptoms? -
Dr.Bragg did opt to reset the VP Shunt today and turn it up a notch, to drain less which hasn't made a significant difference but does make me wonder if we turned it up 1-2 more notches would that help? I don't know but have thought about asking if she would either feel the reservoir to see 'refill time' or would she tap the shunt and see how quickly the CSF fills? I truly dislike asking her things like this though, not because she cares that I ask (I don't think she does) but because honestly I feel intimidated and I worry enough that I am going to cause her to not want to keep helping. Irrational perhaps on my part but when you feel really crappy rationale isn't always the first thing that plays out..

I did try to get the Aldurazyme sent here, to UW but that is a whole other rigamarole in and of itself with what feels like each Pharmacist telling a different reason why they couldn't get it. Needless to say I was pretty frustrated (add to the list) and I said to the Pharmacist who was originally supposed to have ordered the drug (weekly enzyme) last wk that if this where Chemo or dialysis or some other related drug pts. would never be told "to just skip it" but b/c MPS I works more slowly it's as if it doesn't matter? So frustrating!! The docs in the MPS Community who really know/understand this drug always say pts need to try and get it as consistently as possible and it DOES make an impact missing a week, whether there are significant symptoms noticed when pts miss or not. =/

Thankfully in all this I've only thrown up once and otherwise pain from the various surgical incisions is healing some/not as painful. Ice also helps the back incisions a lot + I sleep with an ice pack on my head (I sleep semi-propped up).
That's pretty much it for now,
Thanks for stopping by,