Wednesday, June 25, 2014

MRI's, Appt with Dr.Bragg

Today's MRIs although incredibly hard (took about an hour for full spine MRIs), thankfully the Tech's let me sit up about 1/2 way through for about 5mins. I was getting a little anxious and overly hot then chilly. By the last 20mins I had to pee something fierce and head was not all that pleasant feeling. I really tried to just think about other things which worked a little to distract but these where some of the more miserable imaging I've been through.

I posted on my fb about feeling a little discouraged and that sometimes you just need something or things to be better, I really was feeling this after my appt w Dr.Bragg. It really had very little to do with her and more that I feel like I have these symptoms but 1. since I have 2 shunts (so even w what I think is 1 shunt not working well enough the other shunt picks up some slack whereas prior to having 2 shunts I would get really, really sick and perhaps was more noticeable? I rarely get 'so sick' so I think I look fairly reasonable at appts and I sometimes wonder if that hinders getting answers sooner? I don't really know to any of the above.. I am grateful that 1 shunt seems to help or pick up slack when/if the other isn't working as well but does make it harder to get answers sometimes..
I'm sure also I look fine, even when I don't feel fine which isn't a huge help and I try to stay smiling even when I might feel like anything but.. I guess I figure even if I feel bad I don't want to make someone else miserable?

I just wish there was an easy answer once. As I figured they have no real idea about the intermittent fevers (they're unusual), and I think the thinking is it's probably just a virus (?I have no other virus like symptoms and would a virus be so intermittent?)
Honestly these fevers seem similar to other times I've had issues so is admittedly a little concerning to me. Then I feel like a hypochondriac even though I absolutely know the symptoms are real. =/

As far as the Leg/nerve pain the MRIs did look pretty good other than I think (and Dr.Bragg wasn't really concerned about this) narrowing of the spinal canal in some areas.
She thought maybe the leg/nerve pain (she did say the symptoms I described are classic for what other people also describe) could be possibly from the TPL Shunt sitting on/draining nerve root (causing irritation).
She thought it was possible the chest/shunt site breathing pain might be caused if the shunt was unintentionally reset (can happen) and irritating the spine nerve root as the catheter sits closer to one side of the spine canal. Since this same issue occurred a month (maybe it was longer?) ago and symptoms improved w our re-programming the (I believe) TPL Shunt to a different setting Dr.Bragg thought maybe doing the same today would help.
So far I've noticed no difference and though she may have said, I don't know how long it might take for nerve irritation if that's what it is where going to improve. Given actual headaches virtually always improve within a few hours of our re-setting/programming 1 or both shunts I do feel a little discouraged that symptoms con't on and not at all improved.
I don't know if I should call her office tomorrow (=/) or wait till next week, she mentioned something about if today's re-programming didn't help to call and come back + we'd either try a different setting (I think we are 2nd to lowest setting on both shunts though =/) or she would tap the shunt. Given only the VP Shunt has a reservoir to tap I am not entirely sure how this would help us know if the TPL Shunt where working or not? She must know though and I trust her thought.

In any case the discomfort with breathing is all along the TPL Shunt now and really irritated + uncomfortable + seems almost (as ridiculous as it may sound) as if their is air or fluid or something bubbling as I can literally feel something when I breathe sometimes. I miss just breathing and no 2nd thought about it!!!!

Anyways, thanks for stopping by,

Monday, June 23, 2014

Lots going on (but not all medical)

I meant to 'publish' this a few days ago but will add a quick note before doing so since I didn't.
INR for Cardiology (which checks clotting time for the mechanical heart valve) was within normal again, for the 2nd time in 2 weeks! This is remarkable for the fact it took us over 2 mo to get the level back to a normal (for Coumadin/Mech. heart valve) level!
Per Jane, in Cardiology they'd like to re-check the level in 2 wks (I thought maybe I'd get to go a month but I think if it stays in-range this next time they'll move to that) and dose stays the same (6mgs 5 days, 8mgs 2 days)

It has been a busy (but not to bad) few weeks.
The past few weeks for several days and nights each week I was staying at my Sister's (about 40mins drive)while she did her clinicals for Phlebotomy (lab draw) over a 3 wk period. The 1st wk I just had to then take my nephew to school in the mornings and the past 2 wks the days I was needed I watched him till he went to his Dad's.
This past week in addition to that several nights/days I also had VBS M, W-Th and ERT on Tues. Thurs night was closing service for VBS which was done in-service w/the Christian Contemporary band 'Koine'.
A really pretty service! I sat by several of my students and their families and they all looked so happy!
VBS ran in the afternoons last wk so it actually worked out pretty well, I also really liked the group of kids (22 total) I had. Although I really struggle with names I was able to with the use of name tents and a master list I wrote up of my students do a lot better w remembering each kids name this yr.
That said I still struggled a fair bit and many of the kids thought this was really funny + would randomly quiz me on their or others names. =)

Otherwise on a MPS front things have been fairly quiet, last weekend pretty much out of the blue I had fever, much worse headache and I ached to the point sitting or standing was uncomfortable.
I think the body aches was un-related to the other 2 and more b/c of having been on my feet most of Sat. Thankfully by Monday night I was starting to feel better and VBS still went fine.
Headaches otherwise have been hit and miss, the damp or rainy weather does seem to have some affect and otherwise I am not sure honestly why some is worse than others. For whatever reason weather changes is a fairly common complaint in Hydro pts although for me I would have to say it is really only an issue when I seem to be having some issue (small or otherwise) with 1 or both of the shunts.

From a Cardiology stand-pt the breathing issues con't on and off, for instance I've felt pretty well fine the past few days and then other days I just don't, breathing is almost a chore. I am definitely grateful when it is normal!!!!
Since waking up Sat I've had an uncomfortable feeling again, similar to a month or more ago when I was getting really uncomfortable stabbing like discomfort when I would breathe in.
It isn't at all with movement just with breathing in so I tend towards taking shallower breaths when this is occurring (on and off since Sat).
At the same time I was playing a bean bag toss type game w/family on Sat. and that neither made it worse (I throw L handed though and discomfort is R sided, seems near the TPL Shunt but may not be at all related) nor did I feel more sore on Sun from this so I don't think it is muscular.
When Jane, from Cardiology called she asked about the breathing issues to which I gave her the mild run down (really good days, and days where it seems really bad but weather seems to have 0 affect as it occurs at seeming random on both hot/humid days and on some of these chillier days).
HR still in the higher (for me) range and some of the random skipped or double beats but not always to bothersome.
She wanted to run all of the above by Dr.Earing and said she'd either call or email in the morning/after she was able to talk to him see if he had any thoughts.
Although the breathing issues are definitely bothersome when they occur I am also grateful the med changes we've made over the past few months (digoxin 250mcg's, Nadolol 80mgs, Spironolactone 25 x2/dy, Lasix 60mgs x 2/dy) have helped some!

The only thing occurring this week is full spine MRI's for Dr.Bragg and then she'll reset both the VP and TPL Shunts to the correct setting afterwards.
These are both for MPS sake due to spine issues that can commonly occur in MPS I pts.
I've had the c-spine decompression/fusion and then 2 other smaller un-planned lumbar and thoracic decompressions while Dr.Bragg was doing shunt surgeries so am not new to my spine having issues.
She was concerned for potential herniated discs in lumbar or I believe c-spine due to the very many surgeries I've had on both spine areas. I do have some spine symptoms but tend to think there probably isn't anything big going on. I am grateful Dr.Bragg gets this disorder though and tries to be proactive.
The MRIs are very, VERY early Weds morning and then I see her shortly after for re-programming of the 2 shunts.

Thanks for stopping by,
Will try to update again soon,

Wednesday, June 11, 2014


This week has been fairly quiet minus watching my nephew several days and many phone calls/emails. I am also registered now for the Disney Conf. in Dec through the Foundation I work for. =) Cardiology we re-checked INR which was 2.24 on Monday and is finally in-range, the first time since I believe a week out from the last shunt surgery end of March!
I talked to Jane, Dr.E's Nurse Monday afternoon when she called w the results and will re-check INR again in 2 wks at ERT just to make sure the level isn't to high (INR # doubled in the 1.5wks since Cardiology Appt), so they want to make sure I do not suddenly get to thinned.
As far as breathing Jane called me back today and Dr.E isn't sure what to make of the symptoms (makes 2 of us!?) so they'd like to keep meds the same (which is good as I honestly don't think I can tolerate any increases in any of the cardiac meds). They'd like to give it a few more days and Jane will call me "1st thing Monday" to see how symptoms are/if any change. The breathing issues are very intermittent, it's just when the symptoms do occur is almost stifling and seems as if there's no rhyme or reason to when it comes on.
If no real change (=/) then she said Dr.E would likely want me to come in and see them (also =/) I guess re-eval or something. Sooo frustrating given it's been 2 weeks, TWO WEEKS since I was just there!
I am praying the symptoms will just improve as it is a little hard especially b/c I know the vast majority of people would never even know when these symptoms are occurring (a few have commented). I will just hope and pray symptoms improve again and was just a fluke!? ..

Neurosurgery, some headaches the past couple days (after a fairly good few wks since the last adjustment) although symptoms not to horrible by afternoon (or having been up awhile). I can deal with them for now and like the breathing I just hope these shunts adjust themselves to drain better.
I do think it is just a partial obstruction in one of the shunts and sometimes these can fix themselves or I wondered if we maybe tried re-setting 1 of the shunts to higher (draining less) temporarily and then lower again (drain more) could this flush any small blockage out.. Not really sure about that. ironically I had literally just said to Dr.Bragg in an email and to others that the H/A's where doing really well for once! Hope it improves again on it's own!
Due to some shooting leg pain/muscle type discomfort Dr.Bragg is ordering a series of spine MRI's. This is not un-common as MRIs are recommended in MPS I pts anyways due to our propensity to have spine compression and other issues. She (Bragg) wondered if it could be a herniated disc in one of the areas I've previously had spine surgery but is also possible maybe it's also a temporary thing? (symptoms have been occurring for over a month.
Her Nurse will call to schedule those and then appt afterwards, likely w one of the NP's to reset both shunts.

I have been watching Zan several days last week, this week and next week. VBS also starts next week Monday afternoon. For VBS since I am teaching I was able to get my infusion switched from Monday next wk at CHW to Tues with my former ERT Nurse agreeing to come in and do it for me.
We had been planning to figure out a day to have lunch so will do this instead. =) I should only miss 1 of the 4 days of VBS this way and atleast this year it won't be the 1st day!

On a different note my Genzyme Case Man. emailed today to get the contact info for my Insur. Nurse Case Man. so she (Amy, Genzyme CM) could check on the progress of the Aldurazyme out-of-network Prior Auth and make sure this will be approved in time for July 1st when our insur. contract year starts over.
She mentioned in her email that she, the Patient Care Liaison (Karen) and Carol, the drug rep would be in WI for about a week in July and want to have lunch/dinner (something Karen and I had talked about last I saw her). I've not actually met Amy or Carol in person (both are fairly new) so will be great to see Karen and meet the other 2! =)
Otherwise that's about it, I'll update whenever there's anything new to share.
Thanks for stopping by,

Sunday, June 1, 2014

The things we notice, Appts, etc.

Just about every week has some appt. during the week in addition to normal (Normal!?) ERT Infusion every Monday which is like all other appts out of town and at minimum an hour drive for me. I also have been doing PT again on/off (missing a week here and there due to other things) for the past 1-2 months though that is more local.

This last week it was a Cardiology Appt (a f/up) from the addition of Digoxin to cardiac meds a month ago (dose was subsequently increased a couple wks ago). This wk i'll have ERT tmrw (mon), Neuro-Endocrine (FMLH) Tues, and Pain Mngmt on Thurs (also Milw) w/PT on Weds in BD. In addition to all of that I am watching my nephew W-Fr early mornings (Brandon) till i'll take him in to town to school.
Dr.Earing and I (Cardiology) given symptoms are reasonably well controlled (ability to feel like I can breathe/get air more easily is some improved) con't to agree, as I've I think written about before that waiting as long as we can to replace the Mitral valve is best.
Some pts with the symptoms I exhibit would have their valve replaced (apparently) at the range I am in but given 1. having 2 mechanical valves would mean a more difficult pre/post surgery regimen (injection lovenox), admit the day before typically and I believe any time INR is out of range (near constant in my case) pts have to do the self-injections which that part wouldn't be that big of a deal on my part as I already do solu-cortef injections directly in to the muscle when sick whereas lovenox inj. goes subcutaneous, typically the stomach.
A 3rd OHS as I've written about before though would be even more difficult given the 2nd one was bad enough and per Cardiology they had difficult re-starting the heart (had to shock it out of abnormal rhythms when re-warming). In any case that all aside I am non-to eager to under-go any more heart surgery for as long as possible anyways! I f/up in 3 months which coincidentally (or not?) is the 1 year mark for the 2nd (repeat) open heart surgery.

Since my car accident last Febr I have to admit just about every night in my prayers I pray for God to be with me each new day when I awake, to be with me if I am driving somewhere and especially to help me make good decisions in my driving as I am scared to crash again.
This isn't to say I don't like driving, as I do, actually I typically, most days find it to be a good time to just think over the day or week but that doesn't mean I am also not feeling like I am on hyper alert for what might happen or could happen as there is so little about driving that I actually control beyond my own self in my own car. I feel a lot more comfortable in the car I have now (smaller than my Grand Am was, not as wide) and easier to see out the back but I still feel nervous (an otherwise unusual feeling for me) everytime I have to go somewhere!
A cross between enjoying driving and praying for God to help me to drive safely and be safe in order that I may make it both to my destination and then home safely! I've to say spending so much time in the car driving to and from Milw and Madison (typically 2-3times a week), my Sister's in Brandon (35-40min drive) and to my parents who are closer at around 15mins away I do hope I eventually get past this really nervous feeling albeit I don't want to lose the trying to be on extra alert (though I am quite sure that drives other people nuts when I drive w them now to).

Earlier today we had book club at Church which is once a month typically, the book we are reading is called 'You'll get through this' by Max Lucado and related to Joseph's time in captivity when turned over by his brother's.
At the start there where 2 of my good friends and I, )these are the 2 I also teach Sun School with), the 2 whom have most been there for me during medical issues, either taken me to the ER, picked me up when released multiple times in the past year and come to visit when they can. Anyways, somehow we got to talking about the things people do that no one around them realizes and Joan (we co-teach SS) brought up how she is always amazed and thankful at my ability to network within the MPS/rare disease community, to connect families to Drs. or Specialists and help others find resources.
She talked about how I have been able to learn so much about who does what in our communities (b/c others have taught me, I have listened and paid attention, tried to file away details) and thus from her view I in turn help others when otherwise it can be such a daunting process to find this information for a general medical issue much less she though for something less common/more rare.
While it was un-expected to hear her say this I have to admit it meant a lot and I only share this here b/c it really means a lot when you realize other people are paying attention to the things you do or are involved in, are paying attention to what you are involved in in your 'other' (medical) life and that some friends care enough to notice things like this. She made the comment to Connie and I, along the lines of "almost no one in our Church would know the time, effort and detail you put in to advocacy which is a shame b/c you are making a big difference and doing a lot of great work" (wasn't exact wording but something along that lines).
I have to admit I didn't really know what to say to that other than I really appreciate she shared her observation, sometimes it matters that other people notice!

On a different note, I am not sure what made me think of it the other day but Zander (my nephew) will be 8 June 7th! This coincidentally is also the month and year I first received a shunt. At the time as I've talked about before the Neurosurgeon I was seeing placed a Lumbar Peritoneal (LP) Shunt. The next 3 surgeries where w that Adult Neurosurgeon at FMLH (Froedtert) in Milw. The 3 surgeries w that Surgeon where June 07,, July 07 and Febr 08' though we knew basically by that Fall the shunt was on the fritz.
3 years later and much heart ache (frustration!!!) I finally found Dr.Bragg and in turn a Neurosurgeon (Bragg as did the Adult Neurosurgeon at UW who suggested I consult w/her given she had more experience w Hydro). Thankfully she believed me when I said something wasn't right!
Thankfully, by the grace of God I haven't had as many troubles with drs (neurosurgery and otherwise) over the past 3 years as I did in the many years before that and I've got some pretty good drs and specialists overall!

Anyways, Thanks for stopping by,