"You do feeling bad really well", "You look great!" - the seeds that sow self doubt

Sorry this is one of those updates I started last wk and never finished....

The thought that's on my mind today and admittedly quite a bit lately, (but today especially due to a well meaning comment by a former Nurse of mine). "You look really good, I thought you'd look a lot worse given your posts on fb". To that I just made the comment (b/c it's been said to me so many times) 'I do feeling bad well' meaning I get up, shower, get dressed and do the minimal to show up.  I often think about (or often enough anyways) how my doing this "feeling bad really well" I do think hurts my getting answers quicker often as how often do drs when they see a Patient that genuinely looks bad//wears how they feel on their face react more to helping quicker? When drs whether they mean to or not see a Patient who is dressed, independent (something I strive for, I give up so much to my MPS that where I can I refuse to rely on someone else as much as possible) and still functioning do they (drs) think 'well it can't be that bad, she looks fine'?... When for instance the with the shunts they are in complete shunt failure its very much noticeable to (I'd guess) anyone but with this partial failures I think it's much less obvious..
These past few wks that have been plain miserable have been really difficult but that doesn't mean I can stop living and just not do the things I have to do.. I can't.. I think this thought is particularly on my mind  after today's comment and then yesterday  I stopped by my Parents after an informational insurance meeting I went to at the school district Tues night and my Aunt and Uncle where there (whom I love). My Uncle said essentially the same thing that my Nurse said to me today, along the lines "well you look really good". I cringe when people say that BUT I also know people say it in a well intentioned way so it's not even like I am ever offended. I'm not.  I suppose it's just me doubting myself and doubting if people really believe me then and do they or even (especially )  my drs think I'm just over blowing symptoms or whining or can't handle things?
Like I said I way over think this sort of thing but how can I as a Patient not when it's so difficult to get answers and I feel like we know what 1 of the issues is based off the shunt taps but my Nrsgn only sees the better times of day and so doesn't see the times of day that are worse and intermittent throwing up and affect vision and appetite and nausea especially?

Tomorrow (Thurs) is the Lumbar (low back) and Thoracic (mid-back) MRIs so I am praying I can make it through them. They offered them under anesthesia which I declined as I want to be able to drive myself there and home but I am hoping each doesn't take more than (hopefully) 1/2 hr and in the time waiting to get the shunts re-programmed after the scans any pressure will subside.
 The 2 MRIs are atleast scheduled with an hour break in-between each (my choice, I thought maybe would make them easier). I get the results then next wk.
I can't even say how much I have been praying there is some clear guidance to why the low-to-mid back pain has been so (incredibly) bad! The headaches are really bad at some points (and so clearly pressure related as they subside when it feels like my head finally clears) and other points not quite as bad. The low-to- mid back is something else though! Holy heck!!!! 

Last week, new Infusion site - Dean Fish Hatchery Clinic
I think anyone who had anything even remotely to do with the Infusion site switch (weekly Aldurazyme-Enzyme Replacement infusion) knew I'd had plenty of reservations (doubts?) about the new site and switching to a site that had no experience w/my MPS I. It's not that it's even unusual for any given place to not have experience as there are only a couple of us here in WI who get this sort of treatment and especially for my MPS I but as much as I'm willing to train new/young drs I wasn't all that keen on training a new infusion site.
D-FH from what I know had no experience prior to my starting there yesterday (Tues, a week ago) with any of the Enzyme Replacement drugs, seeming no experience even with drugs related to mine (synthetic enzyme replacement drugs, such as Fabry, Gaucher, Pompe).

That all said from all the people who'd taken time to call me, to talk to my FMLH Infusion Nurses and who seemed like they where working with my Dean Case Manager and PCP to get things squared away I was pretty ok with the site change by the last few wks. Much more at ease anyways then I'd been when I 1st found out about the pending site change!
Although it's different than how it was done at FMLH (but there is no right or wrong way as long as the drug is mixed and infused the same way I had it done at FMLH)  i'll have 1 Nurse (Jill,) as my primary Nurse at this site and so weeks I may need to change my infusion day (earlier this wk, my 2nd wk) Jill and I worked together to pick a day that worked with the days she is there as she prefers to be there as much as possible especially in the beginning she said.
                                 New Infusion Site (West Side Madison) Dean Fish Hatchery Clinic

Infusion itself went fine. The Pharmacist who'd conference called the other week along with the infusion site Nurse Manager stopped by and introduced himself as did the Nurse who will on the occasion if Jill is gone do my infusions.
The site itself is smaller than FMLH, most rooms are also single Patient-infusion rooms though from what Jill said they did have an area that was for multi-Patient infusions (I'm not sure how they decide who goes in which, maybe based off infusion run time and how busy it is that day?). I didn't ask.

My Primary dr. which I think I had written about in another post is in the same building as my new infusion site so she had stopped by last Tues (my 1st day there) to check in. 
The only question I'd asked the Pharmacist and forgot to ask them last wk was Aldurazyme is supposed to be given based off weight (mine is fairly stable give or take 5 sometimes 10pds any given few days or week) but I want to make sure they are doing that atleast once a month just to be sure. I did ask Jill my Nurse about this drug based weight this wk at infusion and about checking weight once a month to which she replied we can monitor it every wk. I am fine with whichever and not to worried about the dose changing but just so we'd know. Right now I actually receive an extra vial of Aldurazyme more than my weight so we do have room.

                           Sunday School, our 2nd last wk for the 2016/17yr (hard to believe!)

Insurance, I may have updated in an older post (can't remember) but this is changing come July 1st from out current Dean Health Plan to Unity. At this point we are planning to go with a PPO plan so I should be able to keep my Providers the bigger question will be where will my infusion be, will the new (Dean) site keep doing it or will my Genzyme Case Manager and I have to look at going back to FMLH or something else (Unity is typically aligned with UW Health System though with the PPO plan I have wider Provider/care options.

That's about all but also enough! =) Will update when/if I know something and please say a prayer we might finally get  clear solution to whats going on/causing all the irritation/tremendous pain?!?!
Thanks for stopping by,

Erica

Insurance change, calls, planning, uncertainty / Switch 1 for another (shunts, irritation)

Should be just a short update but no-less kind of a update I'd hoped I'd not be doing this yr! I knew from my Dad that the school district, where he works was again considering switching who they go through for their insurance and this time through Unity. Non-the-less I hoped Dean, our current insurer might come back with a better or comparable offer.

No-less this morning I got a message from Dad that come July 1, insurance is changing to Unity, which (depending what kind of policy it is, but guessing an HMO, we don't know yet) which likely means all the time I/my Dean Case Manager and my Genzyme (Aldurazyme, Biotech Company) Case Manager have spent setting up and coordinating the new infusion site at the Dean-Fish Hatchery site may be short lived.
Marge, my CM at Dean is looking in to this, if say I drop Unity and just keep Medicare as my primary would Dean, the new site still do the new infusion? Amy (Genzyme Case Manager) and I have made numerous Conference calls to look in to if I keep Medicare as my primary will that affect my being able to get Aldurazyme when in-pt? And will this affect cardiac care if there where any more in-pt procedures/surgeries (hopefully isn't but still info I feel like I need to know)?
              
                 My Infusion Nurses (most but not all of them)  / Funny comment from DHP Case  
                                                          Manager   -will miss her!

Has been a flurry of calls, Conference calls and emails between Amy, Marge, myself and numerous other people at numerous other places trying to verify and double check and triple check various info! In addition separate but related the Infusion Clinic Manager called me today to give me a little info and try to see about switching an infusion I'd asked about in 2 wks and she was seeing if the Nurse that is assigned primarily to my infusions might be able to switch what days she is off (that astounds me but amazes me in a good way they'd even consider that all so the Nurse would be there the day I infuse!
I was really skeptical about this new infusion site at first but between everything my Primary dr. has done, my Dean Case Manager has done and my Genzyme Case Manager, the Dean Med Director, Pharmacist and the myriad of other (fill in the blank) Directors I've talked to have done I actually feel pretty comfortable it will go ok. I'm going to miss my Nurses at FMLH but seems like the new site is really trying!
I am equally impressed my PCP wants to be involved and has asked that I have the Nurses let her know my 1st day so she can stop by.

I had my last infusion today at FMLH (start at Dean next wk) and some of the Nurses and I where lol'ing at the fact it's quite possible i'll be back there in a few months time anyways depending what the Dean-FH clinic says about my insur. change.
                                                          
Otherwise the only other bigger thing and oh is it an issue, HOLY SMOKES, today at infusion I must have asked the Nurses for atleast 1/2  a dozen of the throw away ice packs. Low-to-mid back radiating pain (hoolyyy shiiittteeeee!!!!) that's far worse when I'm on my feet but certainly there a good percent of the time when I am sitting to. Improves when I sit certain ways and worse when I stand or sit certain ways. Ironically as this got far, far worse the headaches have gotten a bit better. AGGHH!!!
I see Peds Neurosurgeon anyways Thurs so am praying he will accttuuualllyyyyy listen.. And maybe do something besides just Xrays? Argh.I.Just.Want.To.Be.Able.To.Function.Better.!

No-less will try to update sometime soon. Have started the new Heart med (Verapamil), am hoping it will help. I really do not look fwd to calling Cardiology back with the update they'd requested and stating no effect if it does not. I. Do. Not. Want. To. Be. A. Pain. In. The. Arsh. But. I. Feel. Like. One!

Erica
                          Sunday School - Love these kids, love teaching them!                                               

Cardiology, Heart Rhythm Appts + New Infusion site, Shunts..

                                            "Liquid Gold" aka Aldurazyme (crazy expensive)

Saw Cardiology today, which I don't always record Appts but days I am overly tired I do. I haven't slept very well in quite a few days which after awhile makes my already, often crap memory worse so these kind of days I do record the appts and then delete once I listen and take notes at home. I have to admit from listening to the 1st Appt and what Dr.Earing emphasized I make sure Dr.Kovach knew to that actual appt with Dr.Kovach and then listening tonight I realized I don't actually think I said much about the symptoms these endless PVCs (skipped or double heart beats) cause. Oops!
     I think did an ok or a decent job emphasizing I super hate asking for their help/needing their help and really didn't want to start a new med (we stopped the Beta blocker I was on, wasn't doing much) but with symptoms and the PVCs literally feeling like they are an endless stream-near 75% daily occurrence (maybe not that much but is a ton) I was willing to try something see if we could get under control.
 
I know I've written and said this before but even though I know the symptoms are very real (and very evident to both Heart drs when they each separately listened) it still makes ya feel a little crazy! Any of this chronic issues stuff does, be it heart, shunts, etc!
 It is nice atleast both Heart drs. really work together and try to tag team each other but non-the-less even with really liking both their Nursing Teams and both drs I'd still love to not need them!
But no-less I guess since I do need them I am glad it is the 2 docs I have as I like them both and both seem dedicated and especially keep my whole (bigger MPS) picture in mind!

I couldn't help but think at the appts today, while I am very glad we did the ablation because slowing heart rate has had some impact on both breathing (yay! Isnt perfect but it's somewhat better and I will take that!!) and on how the newest (Mitral) valve seems to be doing so even if we have these issues now with PVCs that are a tad relentless, we'll figure those out! I think the best thing about these 2 (Heart) drs is each will admit when he doesn't know but then they will try to figure it out. Neither gives up either and that with a disorder like my MPS (type I, Hurler Scheie Syndrome) is HUGE! I also can't say what it means as a Patient when your drs really listen and want your opinion and are actually listening to what you have to say.

Re the PVCs as I mentioned above we'll try this Calcium Channel blocker (Verapamil) and say a prayer it works better on these than the CCB class of meds ever did on slowing heart rate! Otherwise if it didn't work/where to con't as bad as it is (I'm thinking positive just listing what  I learned) the options aren't all that great. We could try heavier duty arrhythmia meds (but side effects of their own that sound pretty abysmal) or a really last option and 1 we'll try to avoid is doing another ablation to try and capture/deaden where these PVCs are coming from.
       -Dr.Kovach did say it's possible a targeted ablation wouldn't be successful either as these could be coming from the L side which is where both my aortic and my mitral mechanical heart valves are and if I remember right isn't possible to target those areas or maybe not easy to target (can't remember exactly).
Concern with another ablation which if it where done would be a 3rd for me (lol 3 OHS, 3 ablations - I think i'll try to avoid tying it up =D ) and much greater risk of permanent damage and again that risk for then needing a permanent pacemaker. Amazingly (but awesomely!) we've come through all 3 Open Heart Surgeries as well as 2 sinus node ablations with no serious damage yet. Wouldn't be bad to keep it at this (no damage, no need for Pacemaker).
A Pacer to would be complicated (?) b/c both of my shunts (LP, VPL) are programmable and dialed to drain a certain amount of CSF. I actually do not know how say the shunts could be re-programmed with out affecting a Pacemaker or vice versa? I'm sure there's some invonvenient way and hopefully we'll never need to find out what that is! =)  

                                             Sunday School kids - 1st-2nd, 3rd-4th

Otherwise per Dr.Earing he's hired a newly graduated Adult Congenital Heart doc (Jennifer Girard, MD (sp?) who did her training he said at UofMn and is now finishing up (I think if I remember right) her Adult Congenital Heart fellowship in Atlanta (Emory I'd assume? Will have to ask some friends there if they know her).
Dr.E seems really excited to be setting up a dedicated clinic specific to MPS Pts and our needs and as he put it "a real clinic not just a sort of 1". =) I think he seems to have a interest in these/my MPS disorders which I always think is cool. Given he diagnosed me 12 yrs ago  I always appreciative of what he knew and what he's all learned + that he'll admit when he isn't certain (which has been more than a few times in my case) and he'll work to figure out whatever the problem is.
CHW isn't considered an MPS treating center but is a top ranked Congenital Heart Disease treating hospital with superb experience and survival for really tough cases. I think it's perfect there will be another dr. here who is interested in MPS issues and seems to have quite a bit of experience and especially so in Adults with MPS as there are very, very few ACHD Heart drs who are experienced in MPS and/or interested in it!

-I'll end on this - Dr.Kovach's Nurse as we where wrapping up the appt with him (I love his and Dr.Earing's Teams! This Nurse is super sweet)   but we where joking about needing a new body and she commented:
"We just worry about you" when she asked me to call and update them in a wk. Funny but very sweet. It means a lot actually when our drs Nurses and for that matter our drs., to, if they care and they can see us as more than just the issue we are there for! =)
I sometimes feel like a pain in the arsh as a Patient (not that I think I am just it's more b/c of needing their help and frankly having to rely on them at times for things 1 would rather not need to rely on people for).
I appreciate when my Providers let me know I am not the bother I often feel like. =)

                          Randy (Fabry dx) and I, we've infused together the majority of my 12yrs

 

ERT - New Dean Fish Hatchery clinic (start there 16th)

Thanks for stopping by, if there's nothing before I'll update after my 1st infusion in 1.5wks at the Dean-FH site. I finish 1 more infusion under the current (expiring) Prior Auth at FMLH then it's to Madison and the infusion site within my Primary drs. ofc. bldng. She (Dr.Simpson) has asked I have the Nurses page her my 1st day at the new site so she can stop by. Given she really wouldn't have to seems kind of her!
Per the Nurse Manager of Dean Infusions there is also 1 specific Nurse assigned to my case. I'm not sure if that's just initially or she'll be my Nurse primarily? Hopefully she (Jill) is nice and to they quickly realize I do my own thing during infusion (calls, paperwork, Sunday School lesson, etc - basically anything I need to get done for the next wk) and non hover to much. =) Dr.Earing was asking if I'd started at the new site yet which I haven't of course and we both agreed (b/c they seem a little nervous? Not sure =) ) as long as nothing with my infusion rate or volume is changed should all go smoothly.
I know when to take the Lasix I already take every day if needed during infusion (if any breathing/coughing symptoms creep up) although that I especially try to avoid (sometimes not possible) but Lasix and infusion = endless bathrm trips. Eeew (I hate public bathrms, lol)!!!    Shunts... Ouch!!!

Re Shunt stuff, Dr.Bragg is brainstorming a little for me. She had an interesting idea, given my 1 (Lumbar-thoracic) Shunt causes so much irritation and there is significant scarring and apparently some compression (unsure about this, just what Dr.Iskandar mentioned) maybe see if Dr,Iskandar would take out the LP Shunt and put in 2 VP (from brain) shunts. Don't know that he'll go for it and admittedly I do think as very imperfect as it is when the 2 shunts are working it has been good to have 1 draining from brain (head, VPL Shunt) and 1 draining from my back (Lumbar, low back) Shunt.
While he'll likely say no to this idea I may ask him about this at next appt. I need the 2 shunts but  the LP Shunt is wreaking havoc on my spine topped with secondary to my MPS narrowing and compression is already not unusual but the current LP Shunt and prior Spine shunts have caused per Dr.Iskandar severe arachnoiditis (several decompressions to clear scar tissue and remove bone have already been done over the past 5.5-6yrs for this).

Anyways will try to update soon,
Thanks for stopping by,
Erica

Whatever will be, will be..? Heart, Shunts/Back, etc..

I know my God is a gracious God and a loving God and I know it is the Devil that causes me to doubt and worry but I have really, really had to remind myself this this past week and have reminded myself repeatedly it is ok to ask God for help with the many things I feel like I am struggling with.

                                             

Between the headache/shunt stuff which has been pretty bad and very little of each day that I don't feel just worn out (tired) and pressure (certain parts of the day are better but it still leaves me feeling exhausted due to not very good sleep even despite sleeping in my recliner) and then the low back to mid-back pain that is so very unpleasant and uncomfortable and disheartening really (b/c I feel like there's no hope in sight) has been  a kind of unpleasant few wks piled on top of not all that fantastic few months.

Cardiology/Heart Rhythm Appts:
Heart stuff while heart rate has been better (more often in 80s-90s vs before Jan's 2nd heart ablation was most often in the high 90s-100s which is a good thing!) but for whatever reason I've been having a increasing amount of PVCs (basically extra or double beats) which at 1st was more of an annoyance than anything but as it's continued to build in frequency and occur far more often is causing other symptoms (because you know any 1 thing never ends!?! Not to play woe is me, just is kind of true). We trialed a beta blocker along with the other heart meds I take which didn't have much of any effect so after this last heart monitor our next step is likely to try a Calcium Channel blocker.
I follow up with my Heart Team (Cardiologist and Heart Rhythm dr.) Thurs, later this wk.

Infusion Site Change - Dean, FH Site:
I have just 2 infusions left at Froedtert then will move to the Dean-Fish Hatchery clinic site which is where my Insurer and Team have been working the past month or so to set up my infusions (an in-network site, while I don't wish to change where I go I do get that the cost savings will be substantial for how much the Aldurazyme (enzyme) costs my insurer and thus that in turn impacts what insurance premiums and costs are to everyone.  This site is where my Primary dr is and although I don't think she'll be actively involved in infusion she'll be on site (bldng) where anything to happen I guess. She'll be off for maternity leave from July for about 3mo I think she said but imagine there shouldn't be any issues anyways.
The perhaps true irony of this change though is our insurance (through my Dad) may change anyways come July 1 as the school district is looking at another option pending if DHP comes back with a comparable/better offer than what the school district has received.
I am stressed enough about several things so really praying the insurance stays the same as losing my Case Manager at Dean (she's been great) and the Med Directors who are familiar with my case would really suck actually. Sure there are things that at times annoy me about Dean but isn't that so with just about anything in life?

I also don't know if I'd take my Dad's new insur., then or not if it did change or just switch to using my secondary insur. as primary. At the moment my Genzyme Case Manager and I are looking at all possibilities. My Dean CM has said she'd help with the transition if it does change which is sweet on her behalf as clearly she wouldn't have to. I am just praying it doesn't have to change and Dean comes back with an offer. I know they did last yr., so while I am worried I am trying to be hopeful. Ugh!!

Audiology:
Last wk I saw Audiology (Hearing) which went back down some so back to hearing aids. I don't really need them for quiet situations is more when in noisy areas/a lot going on (which is often enough) hearing aids can help that. I had these yrs ago and admittedly didn't love them but did use them at times. My insur. does apparently surprisingly cover them so my Audiologist is working on whatever that entails.

Otherwise just ALOT of stress re my nephew and family stuff. Holy crap!!!!! I just have been really, really trying to lean on my faith as this past wk has really been hard. I miss that kid! I LOVE all of my nieces and nephews but Z admittedly is just a little closer than all, probably b/c I've watched him since he was a baby and spend significant amounts of time with him over the years. =/ I am so praying whatever happens he really will remain part of my and my Parents life. God has a plan right?

Will try to update after the 2 Cardiology/Heart Rhythm Appts later in the wk.
Thanks for stopping by,

Erica