Wednesday, September 24, 2014

Being skeptical vs trusting

I was just paging back through my blog looking for a piece of info and have to be honest I am kind of astonished how much more optimistic I feel like I was 3 or 4 years ago.
It's not that I think I am really, terribly negative now but I don't think I have the same easy going, relatively simple belief in people that I used to. I wish I did!
I think I used to just believe what people (or Providers) told me without much second thought and only after something didn't happen did I (maybe) think about it.. Now I feel like I analyze everything people (friends, providers, some family) say to me and I wish I didn't, I wish I was more of the trusting person I used to be.
I never liked making calls or asking for help but I think I still believed when a Provider said something they meant it (and probably most of the time they do) but now I feel like I constantly have to remind myself they aren't going to purposely try to do something to hurt me or that just b/c I may not get an answer doesn't mean it's about me and instead it's probably about lack of time.
What it really boils down to is I don't want to be that person who needs to be reassured but I do feel like I am less trusting and question what someone really means/says and will they really do it or are they just trying to placate me at that moment.. Here is where I wish I could just trust!?! I know I often forget to ask the right questions at the moment and I sometimes say something in a way that at the time may not come off like I meant it to so I do worry sometimes am I offending Providers, especially those ones I really count on? I hope not but you never know..

Last week Dr.Bragg (Peds Neurosurgeon)asked that I call her Nurse or Secretary this wk and have them remind her to call me regarding her talking to her Codman Rep to see if they (Codman) had any smaller (lower profile, not sure what that means) valves that might suite our needs better... Ironically our having turned down the VP Shunt worked splendidly for the 1st few days and now isn't as bad as it was but it also could be better. It's almost as if we just need a way to simulate what taps do to be able to take off or drain more CSF consistently..
I did leave a message for her Nurse today but also happened to read her last clinic note tonight (I usually request copies for my records but more importantly so I can give them to my Cardiologist and to my Pain Mngmt dr). Anyways in it she talks about the cardiac issues and the affects she wonders if this is having on the headaches (I can't help but have wondered the same over the past few days actually which is odd since I wasn't quite aware that was her thinking!?) Anyways she just mentions in her note if the valve adjustment doesn't/didn't help she would probably talk to my Cardiologist and discuss with him possible need, sooner for cardiac surgery (oh joy) as she wonders if there is some oxygenation issue affecting headaches due to the cardiac issues potentially atleast partially affecting the headaches (so multifactorial)..
She mentioned the possible Cranial vault expansion (bone thinning) in her note but further talked about the cardiac issues and suspecting was this having an impact on our H/A issues. I do think there is some confusion about the degree of the cardiac (mitral) valve stenosis/leaking (it is moderate on the verge of severe) but given high heart rate often I could see how that could potentially affect headaches to some degree.. I also know I am not always the best communicator though some of the info was gleaned from my last blog post; as far as the headaches I don't think the VP shunt isn't working (another comment, part of why stated for tapping the shunt) but to me as in the past I think it's intermittently functioning or partially occluded and thus not draining at full capacity. This boils down really to I don't always convey what I am thinking or trying to say all that well though so isn't that big of a deal. A comment during the tap made last wk was how pressures where within normal though even Dr.Bragg commented that doesn't always mean a lot as my pressures rarely (though occasionally) get quite high when there is intermittent shunt issue but in the note it does mention "normal pressures" during the tap. I admittedly am a little confused by that but overall it seems like from her note she is wanting to try and help be it we con't to look at some shunt approach or be it that the headaches are partially caused by cardiac reasons and will she and my Cardiologist talk. I am hopeful maybe she really will call me sometime this wk (or sometimes in the past she would email whatever info she had which I am ok with to) and we can talk about the valve adjustment helping some and overall a potential plan be it shunt/cardiac/etc..
In reading through past posts while trying to find some info I happened to come across several posts I wrote not long after I began seeing Dr.Bragg and which talked about our early issues with the CSF fluid collections but also how these helped and oh how I wish there wasn't a way to make those happen!?! (not really possible).. It does make me wonder though as much as I am against the bone thinning idea (just seems scary from what many have said) would that be a more permanent means to recreate this fluid pockets/space for CSF? I don't know, maybe I just need to meet w this Plastic Surgeon? It certainly doesn't mean I have to agree to the surgery but gathering info isn't always a bad thing? I am not entirely sure if Dr.Bragg has yet talked to the Surgeon she would want to use.. guess I should ask.. Kind of scary stuff but gathering isn't consenting I guess..

I saw Physical Therapy yesterday which it had been about a mo., which we sometimes space out appts and in this case I had had to reschedule a couple due to other shuffling.
In any case he asked what was going on with the low back/Leg symptom(s) if anything so I filled him in on the great debate going between my PM dr and my Neurosurgeon.
He is normally a fairly conservative Provider ie he tends to try to help his pts avoid a lot of invasive procedures when possible but even he thinks we ought to give the injections a try and see if it either improves/resolves the symptoms or tells us the area of concern may not be what we should be looking at.
I've not heard anything back from my Pain Mngmt dr if she is or is not willing to do the injections now so when I pick up a copy of her most recent office note tmrw i'll ask her Secretary if Dr.B has mentioned anything.
In any case, will update when there is anything new,
We have a SS Teachers mtng tonight and are sorting out some potential changes there with how it is done week-to-week/month.
Thanks for stopping by,

Thursday, September 18, 2014

Peds Neurosurgery Appt (last of the week!)

I was admittedly nervous for today's appt. but it wasn't bad. We sort of are at a wall as far as what to do/try so she (Dr.Bragg) is going to go back and talk to her Codman Rep again I guess and, see if there isn't a lower profile (in thinking about that after I am not entirely sure what that means?) Codman valve we could try. I am not sure if there isn't another brand of valve (non-Codman) that might work but am guessing not if she hasn't suggested it.. She just asked that I call her Nurse or Secretary next week and ask them to have her call me.
Dr.Bragg did opt to tap the VP Shunt which if I got it correct the flow wasn't great but there was spontaneous flow? She isn't concerned that it is obstructed is what it comes down to.. It's good that the shunt is "working" but admittedly (not that I want surgery, I DON'T!) I also know I'd sure feel better as I know with absolute certainty it is a shunt issue and has something to do w/ how well the shunt is or is not flowing. Sure when we do revisions we may not get 100% H/A improvement but what we do get atleast gives me somewhat better energy, better appetite and I don't feel so tired so much of the time..
In a way I wish it would just have been clear cut or even better yet more than about anything I wish the shunts would just work optimally! I have zero doubt the shunt is atleast partially occluded as these symptoms mirror every other time I've had shunt obstruction/partial obstruction. Super frustrating!
As far as after tapping the (VP) Shunt we opted to reset shunt to the lowest setting (30) which she was hesitant to do but I think she also wanted to try offer some relief. I can almost always tell a difference within a 30-60min period if it is going to offer any relief and whether it was b/c she took a bit of fluid off when she tapped the shunt (I am not sure if she did) or it is from actually lowering the VPS setting from '40' to '30' but there is a bit of a difference. The only problem with this is we usually run in to problems the lower we set this shunt as it seems to drain, collapse, pressure builds forcing it open more frequently. Who knows hopefully it would last for some time? I am sure hoping though never hold my breathe (that may sound negative but just being realistic).
I am supposed to go to a Meeting in Minneapolis tomorrow and then fly home Sat. night, the meeting is for Lysosomal Storage disorders which is usually informative (mostly geared to Professionals so isn't dumbed down). I admittedly do wish I felt tip top, even with the shunt adjustment I don't think i'll feel as great as I do when the shunt(s) are working fully. Anyways, I often think I try to go about my life as normally as possible including the things I am involved in or have planned and sometimes just take extra breaks to nap or rest a bit. In any case I sometimes wonder, does going about these things hinder getting care (ie do Providers think if you can still do something like a mtng. you must not feel to bad (and it is true if I where really, really continually sick obviously I wouldn't go) so thus you don't get taken as seriously? I don't really know the answer to that but do wonder?
Because there's always something I forget to ask I forgot to ask if she had been able to talk to her Study Coord. about the potential to try to get IT approved at her hospital. She even asked if there was any other questions, I guess it just doesn't matter my mind gets a little overwhelmed remembering everything. I am just curious if this is a complete not going to happen idea or if there is any update..? I guess if I do talk to her next week I can ask her this then..
One of the other things Dr.Bragg brought up, which we've talked about before and I've written about here was the skull bone thinning (cranial vault expansion) and is something we circle back to frequently.. I think still we are at the same time trying to cross off or look in to any other options to help first.. Her thinking behind that is to create a bit more space for the CSF to circulate and thus help the shunts work better. I don't think it's even that rare of a surgery (actually in an MPS fb forum I just saw a little boy diagnosed w MPS I whom is having craniosynostosis surgery which I think may be similar as his Surgeon(s) are trying to avoid the need for a shunt and create space..

On an un-related not (I have lots of those) =) I've always written this blog as a way to share my experiences with MPS (and the related issues) with other families and secondarily as a way (in a way) to have a place to go back to to be able to look up a detail or past surgery info fairly easily. I never write with the intention that Med people might read it but apparently some do (that's almost a little intimidating) based off what the Cardiac Anesthesiologist had written in an email she wrote to me last year and what we talked about at todays Appt.
I don't really know who reads this or if all the time but even knowing a Provider(s) read it some is I guess in a way a good thing(?); a way for Med people to see we are human and not just our issues? Thankfully my Providers do seem good at the "whole picture" vs. 'Patients as just a diagnosis' but non-the-less I guess this is one place where I really share my truer feelings (good or bad?) on things.. I don't share a lot of my non-medical life on any forum but there are glimpses of it in some updates and in pictures here. Just is something I guess I've rarely thought about(who is going to read it) and maybe should more when I write appt/medical updates?
Anyways I am hoping whatever it was that helped a bit today really lasts as I want to go to the Mini-WORLD Lysosomal Mtng in MSP Tmrw/Sat! The Foundation I work for is sending me with the 1 request I take notes on anything that might be of interest to MPS I/research.
I am hoping to feel reasonably ok????

Tuesday, September 16, 2014

Echo, Cardiology Appt.

Today's Cardiology Appt was good - the Mitral valve gradient hasn't changed from last February (is at 9, moderate leaking, moderate stenosis (narrowing) which Dr.Earing cont's to be concerned about but is good it hasn't increased.
He said something to the effect of "your valve is at the range where we could intervene but I am not in your body and can't tell how you feel so you have to be a part of making the decision when to intervene and when do we go back in a 3rd time." (apparently a 9 gradient is right at the tip of being considered severe, if the # where to be a '10' per Dr.Earing they would consider the leaking and stenosis as severe and likely intervene). As far as things he advised to watch for and would necessitate coming in sooner than our otherwise 6mo Echo/Appt f/up would be rapid weight gain within a few days or over a period of a week or 2 and any changes in feeling short of breath.
He commented when we do decide to replace the mitral valve this valve to would be a mechanical and given it would be a 3rd open heart surgery (OHS) that would (could likely) present addt'l complications in any addt'l valve replacement but there is no doubt in his mind we're going to have to do it at some point. Having 2 mech. valves would mean any other surgeries would be a bit more involved with Lovenox/blood thinner injections given anytime you are not in therapeutic range and/or leading up to any surgery + (I think) some kind of heparin has to be given right up to surgery.
For now the various cardiac meds (various med classes) I take are doing a semi-reasonable albeit imperfect (again his words) job of keeping the heart rate and thus the stress of the heart pumping functioning and stress of the blood going back through the valve better controlled. The higher the heart rate the higher the overall stress on the heart and especially when there is narrowing of a valve so with the various meds Dr.E is trying to target different aspects of the heart function to minimize to what impact we can heart valve function.
I currently take several different classes of meds which as I said above semi-reasonably but imperfectly control heart rate (his goal is heart rate below 70, mine most often sits in the high 80's to mid 110's).
The meds I take include "water pills" including a high dose of Lasix twice daily (though there are occasional days when I am going to be somewhere out and about I skip a dose), Spironolactone which is a longer acting form of a water pill (Lasix typically lasts about 4 hrs each dose whereas Spironolactone, also twice daily seems to last closer to 8-10hrs and a different overall affect on fluid).
There is also the beta blocker which combats heart rate and is a long acting med 1x daily (the least favorite of mine of any of the cardiac meds which is saying a lot). Additionally there is Digoxin a reasonably high dose which has in my opinion and it seems Dr.E seems to think as well done the best of all the meds to control heart rate. This is also the newest addition of all the meds.
Last but not least (?) I take baby aspirin and Coumadin each once per day for the mechanical heart valve. The Coumadin is why we have to do such often INR-blood tests to try and get the blood level in therapeutic range.

I guess the other thing we really talked about was an article on the drug PTC (Ataluran) I gave him which I'd copied out of a Medical Journal I have (I frequently share MPS related cardiac articles with him).
After a few questions he stated and which I (we) already know from being on ERT for the past 9+ years is that Aldurazyme was thought to help some of the cardiac issues in MPS I pts. and yet has shown little benefit. He feels (as do I) Ataluran will not reverse the damage that is already present and likely we're to close to the point of needing a 3rd OHS (open heart surgery, valve replacement) that this drug isn't going to help that issue. More than likely the same for the CNS issues (atleast the Hydrocephalus) but if the drug prevented new damage once available that would be good enough to me! =)

If you could say a prayer I have an Appt with Dr.Bragg Thurs., that the Appt. goes well.. Jane, Dr.E's Nurse said to me today "You probably get more stressed about those appts than anything?" I just replied sort of laughing that I probably worry to much about the things that are pointless (non-medical stuff) and not enough about the things I should (medical stuff) perhaps b/c I've come to realize I have a decent team of providers, I can only advocate so much and worrying if something is or isn't going to happen serves little purpose other than to stress me out. Instead I just try to stay on top of what I can and learn where I can.
That said I do hope Dr.Bragg has some idea how to help the headaches/symptoms as I feel so worn out (tired) and headaches and everything - in other words kind of miserable a lot and I am 99.9% certain it is shunt related... I was supposed to have dinner w/1 of my former ERT Nurses after my Appt and after she got off work today (she works at CHW) and I just felt so tired and yucky so instead I came home we'll shoot for next wk instead.

In an un-related but related note last wk when talking to Dr.B. at that appt. she had asked if a temporary increase in pain med. might help w the intermittent but long (in length) occurring headaches which the meds really don't.
I just said since it doesn't impact the symptom we ought to not make any changes and she commented something along the line of opioid and related type meds really don't touch increased ICP type symptoms and all the more concerning to her that what I am intermittently experiencing (more frequently) is due to shunt issue. I know she really wanted to be able to help but I also appreciate that she recognizes the differences in when I am having symptoms a med might help and when I am having symptoms that are likely shunt related.

In any case, enough from me. Thanks for stopping by,

Thursday, September 11, 2014

Pain Mngmt Appt. -

Today's Appt was long but went fine and in reality was productive I guess you might say. My Pain Mngmt dr. is good at what she does and given she has a wide background (trained in Anesthesia, Hematology/Oncology and certified in Pain Mngmt) she brings a somewhat unique perspective to my care Team.
One of the many things we talked about was the low back to L leg symptoms and Dr.Bragg's wanting to try spine injections (epidural or steroid) to see if the area she is concerned about is in fact the area that is causing the symptoms. Due to a fluid (it seems) pocket around the area where my former LP Shunt was and where we previously had issues w CSF fluid pockets Dr.B is first considering an Ultrasound but done by one of the Radiology docs she works with. She asked me to send her the MRI reports (I guess she didn't get the ones faxed) and she'll talk to her Radiology Colleagues.
She isn't ruling out injection(s) but first wants to gather information both from potential ultrasound (of the soft tissue/what she is concerned is CSF accumulation although an on-going small area and not new, just not absorbing either). Also would want to talk to Dr.Bragg and make sure she and Dr.Bragg where on the same page + Cardiology and verify they where ok (due to light sedation).

Dr.B formerly did all MRIs (ordering) for the 2.5+ years she had seen me prior to my starting to see Dr.Bragg so she is familiar with how my spine looks (varies from a "normal" spine and as well has done a series of previous spine injections both steroid, epidural (lumbar/thoracic) and Radiofrequency Ablation (RFA) in my Thoracic spine so she is familiar from that avenue what my spine is like to get in to.

I said that I feel a bit badly b/c honestly I have been a bit burned out lately on trying to coordinate drs., communication, setting up appts., canceling and re-trying to coord. (canceling one to make another work, though thankfully in the 1 case my Cardiology Team is fantastically flexible and my Neurosurgery Team is as well).
Dr.B gets it and though she deals w the chaos of how much medicine has changed from a drs. perspective (how they have to see so many more pts., how drs such as Dr.Bragg employed by Hospitals have to see so many more pts., and yet how they all still have to squeeze in seeing in-pts, dictation and any outside things they are involved in on top of if they are married (Dr.B is not but most of my Providers are and in Dr.B's case she talks about (due to my interest) in all that she has going on with the Am. Academy of Pain's advocacy but trying to fit that in and never feeling quite adequate at all of it. (She is amazing and though).
sometimes her wait times can be long, but when she spends 45mins trying to get to the bottom of an issue I never have a problem w/waiting and have learned w all my drs to plan accordingly as it just means they likely also do the same for other pts. I'd rather have a caring dr. that falls behind than an asshole for a dr. who doesn't give a shit and is always on time (to be perfectly blunt).

(LOL, I was definitely tired but we had a good chat, I was able to answer questions she had about Dr.Bragg's perspective and answer questions she had about symptoms, was it an infection (we know it isn't or atleast from right after the last surgery CSF was clear) to which Dr.Bragg was happy with this though commented "I am happy to know that but it's always nice when it's very recent" (I don't think she thinks the low back/leg symptoms are an infection is more that she is just concerned what is the fluid-ish/puffy area. She said and I think she is right it has been there a while just a curiosity I suppose why it doesn't go away).

One thing Dr.B. did say and emphasize repeatedly while indirectly trying to get me to let her I think admit me to her ER (I politely refused) is that it isn't normal to "just walk around with increased intracranial pressure and vision changes" and she personally wished it would be addressed. I said that is really in a way my fault b/c 1. I have a way of making light of how I feel, 2. symptoms are intermittent and so while they are lasting much longer lately they do intermittently go away. I think if I really told Dr.Bragg how bad the headaches get she might do something (I could be wrong) but Dr.B then made the comment (as I know she appreciates that someone finally besides her could see the symptoms for what they where 3+ yrs ago) that she has been glad that someone like Dr.Bragg listens, is proactive and repeatedly tries to help despite our many shunt problems. I think really in a way Dr.B just worries about her patients (it's very obvious if you knew her) and is glad that someone else keeps trying.
Personally I just am not at a point where I feel I need to be pushy or maybe it's not that but maybe it's that I just need a break and I am not ready to call Dr.Bragg's office and be seen sooner. I do have an Appt next Thurs., I could have seen her a wk ago but just needed a break from drs in general. Nor do I think it's bad enough that I can't manage. Sure when the headaches are there it sucks big time but I've been around this block enough I know how long to get up before I have to be anywhere, I just don't do as much in general and I feel worn out-but on an energy level not on an emotional level.
I think we ALL need a break from MPS and all the b.s that goes along w it sometimes!?!? I promised Dr.B if I still felt like this next Thurs I wouldn't make light of it and I would try to be as honest as I could with Dr.B and see if we could do something for the headaches. Another comment she made was: "How much can one person take", which I think when it really comes down to it you can take a lot (what's the other option?) and you either let what you have rule your life or it just, no matter how you feel is a part of your life. Perhaps not a welcome part but non-the-less a part.

Somewhat interestingly she to like several other drs both mine and ones in the MPS community feel very leery about the Cranial Vault Expansion talks and in Dr.B's words, not that she in any way was trying to say she knows more than Dr.Bragg (nor are these other drs especially the ones who know MPS inside and out) but "why borrow more trouble when you already have a list a mile long of issues to sort through" (meaning she is concerned it would cause other unforeseen complications). I don't have an answer but having talked to atleast 1 parent whose child had it done (non-MPS) and knowing they would not chose to do it again it is not tops on my list of options.
In any case it is interesting how that particular topic keeps resurfacing with many drs. though thankfully it is on the back burner so to speak with Dr.Bragg atleast for now.
I see Cardiology Tues and Dr.Bragg (Peds Neurosurgery) Thurs., will try to update after either.
Thanks for stopping by,

Here's a pic of the 1st day with my new Sunday School class - 3rd/4th, I had some of the kids last yr.
'God creates Man and Woman', the stars where the '7 days of Creation' w the center being 'God rests'.

Wednesday, September 3, 2014

A year ago..

Believe it or not exactly a year ago at this time I was still in surgery for the 2nd (repeat) OHS (Mech. AVR, Konno, VSD re-do) - hard to believe actually! I see Cardiology in a couple wks for f/up Echo (generally done every 6mo) and hopefully the mitral valve will not have progressed since the new changes where found (mitral valve) in Febr.
I never did get off any of the cardiac meds I was on prior to the 2 OHS and in fact doses where increased + another med added due to heart rate being high and concerns with the mitral valve changes. I'll be happy if the valve is stable though and no changes!
Otherwise in the year since the 2 OHS I've had approximately 5 or 6 other surgeries for the shunts both to replace the TPL Shunt back in to the Pleural Space, to close open incisions (VPS) and to shorten the TPL Shunt catheter. It's sort of been a wild year but then again haven't all recent years!? =)
I talked to Dr.Bragg's Nurse for quite a while earlier about both my Pain Mngmt dr not necessarily agreeing with Dr.Bragg's plan/thought regarding the Lumbar/low back and L leg symptoms + as well about the headaches seeming to increase in frequency/length and vision being such an issue with reading (=/) though the intensity thankfully isn't a lot worse.
Regarding the headaches she gave me the option to see Dr.Bragg tmrw but for several reasons (including so I can talk to my PM dr a bit before the appt next wk) I took next wks appt instead. Dr.Bragg's other appt day is Monday's but given my Infusion even she commented right away that wouldn't work (LOL, clearly I've seen these guys to much). Her Nurse was also going to fax their most recent clinic visit notes and MRI results to Dr.B and asked that I have Dr.B include them in her dictated office notes in the future so they can know what Dr.B is thinking.
Hopefully Dr.Bragg might have had a chance to talk to her Research Coord. and hopefully we can figure out a solution or plan for the low back symptoms be it injections (I am leery of them though not for it being painful but at the same time I guess I can't see why it might not help us figure out if the area Dr.Bragg is concerned may be the culprit is in fact the culprit..) or whatever.
I also hope we can figure out some plan for these headaches, originally we had planned to wait to deal with these till after we dealt with the low back symptoms but I am not sure we're going to figure out a solution to these symptoms any time quick? Guess we'll see..

Otherwise Sunday School starts up again this weekend; this will be my 3rd year teaching which also seems a little hard to believe. The one bad thing about this is it's early-ish in the morning which means getting up even earlier to give the headaches time to improve (=/) and I am not one of those people who can fall asleep early or easily!
I also have a plane ticket bought (the Foundation I work for reimburses) and hotel reserved for the upcoming 'Mini-World' Lysosomal Mntng in Minneapolis-St.Paul (next to Mall of America). That mtng is towards the end of Sept. should be interesting and if nothing else is nice to see friends from Genzyme there and some of the Med Providers I know (gives a chance to talk to them in person).
Thanks for stopping by,
Erica <