Wednesday, June 28, 2017

Loss and Change (in a Rare world)

I don't know about most people but I don't particularly like  change when it comes to my care and my MPS. Sure there are times a new Provider is added, a new issue crops up (all to often actually on this front) or something is added but in general I like things staying kind of the status quo. I don't like changing Providers except when it's really necessary (and sometimes it is, sometimes I am the 1 to make the decision to change a Provider or 1 of my Providers suggests adding another Specialist to my team).
There are other times though when change has to happen and I dread it, even worry about it. Last year, a year ago when losing Dr.Bragg (atleast locally) was a perfect example of that. Was similar when my former Metabolic (Genetics) Neurologist left his practice at UofMn and went in to research with no patient contact. We as complex, rare patients don't always know how to replace these providers and sometimes frankly like with Dr.Bragg they are irreplaceable.
I share these thoughts b/c as I've written about a few times my primary insurance is changing (unfortunateliy)  July 1st and with that I will lose the Case Manager I've had for the past 4 yrs at Dean. I know (believe me I know!) it's probably a little weird to be sad at losing someone you work with at an insurance company but when you have a disorder like my MPS that's fairly complex and requires a LOT of care and Providers and thus dealing with insurance often enough (especially when primary insurer was an HMO) having someone, a point person at the insurer can make working through that 10x easier. Marge was completely that person for me at DHP.
I have been working on it for some time but heard yesterday from the new (commercial, through my Dad's employer) that the new Insurance we'll have come July 1st is agreed to cover myself under their policy as well (disabled dependent due to my having been on disability before age 21).

I am grateful for this and breathed a small sigh of relief admittedly - it's not that I don't have other insurance (I do, Medicare and Medicaid) so it isn't as if I would have went without care it's more (the biggest reason) the uncertainty would I still have been able to get the  IV Enzyme drug I receive weekly when in-pt? My Genzyme Case Manager and I did our best to try and find this information out but could never get a solid answer, just a 'we think so, b/c it would be medically necessary'. With coverage under Unity that question is answered.
 Some stretches of in-pt stays have been long and 4 or 5 wks of no access would have a huge impact (I can notice a difference after 2wks). Thankfully that part is now sorted!

I share all this because my Case Manager at Dean was the point person I worked with there. She did loads of paperwork, calls and coordinating on my behalf which meant I in turn did not have to do these things. Regarding about 1/2 or more of my Providers where out of Dean's limited HMO Specialist network and so could conceivably with many insurance companies meant having to switch Providers (Dean does not have most of the types of sub specialized Specialists I see)  but with Dean and with having Marge/a Case Manager we where able to submit out of network prior auth exceptions. Years back when our plan 1st switched from a PPO/POS Plan to the HMO I had to switch Primary drs (but my then Case Manager at Dean who was also very good asked around and got a few good recommendations and of these I reached out to 2 and heard back from both). Of those 2 I picked  the PCP I have today and who has been fantastic (and I don't say that lightly!).

Having a Case Manager hasn't meant I gave up decision making and being my own voice nor being able to have a say (again making the decisions re my care)but it HAS meant getting my out of network Specialists covered as in-network Providers and being able to for 6 o7 yrs receive my infusion (till it was set up recently in-network at the current site) at a hospital that had experience. It meant having my 3 Open Heart surgeries and the numerous heart procedures covered despite these also being at out of network hospital. Same with so many of my Providers. My Case Manager was a link to the top essentially at Dean, so the Medical Director was even familiar with my case and for instance with this recent infusion site switch the Med Director amongst many other Dean Directors took time to be on the call regarding the infusion site switch. If I where just a Patient trying to do all those things my own they either A. wouldn't happen or B. would be a helluva lot harder and a whole lot more work on my part!.

 I'll call and see about having a Case Manager assigned at this Insur. Co similar to how I worked with 1 at Dean. (see below, I met her last wk - so sweet and reeeaalllyyyyyyyyy going to miss her!)
 I really get it that not everyone has great luck with Case Managers and I've had a few over the yrs (not at Dean but in other capacities) I didn't do much if anything w/bc I could do whatever needed being done as easily on my own (there weren't great). With Dean having been an HMO the past 4 or so yrs it was a godsend to have a Case Manager like Marge (and Peggy before her) who made getting Providers I see out of network covered much simpler, they did vast majority of paperwork and she (and Peggy initially) where the 'go-between' between the policy, the Medical Director, myself and getting my out of network Providers covered. I can't say enough other than very grateful and I really am going to miss her!

                                    Marge (DHP Case Manager) and I, Dean-FH Infusion

Perhaps many don't  but I can cope with all this uncertainty and frankly often (with some aspects of my care) not knowing what is going to happen by surrounding myself with people who learn my situation and who care. I don't mind admitting I greatly appreciate those people like Marge and some of my Providers who take an extra step and get to know me while they try to help me. I feel being able to trust someone makes working with them a lot easier (and sooo much harder when as a Patient we don't necessary know if we can trust a Provider)!

Unity (New Insurance) -
I found out the coverage information re Unity while at Infusion yesterday (I was uncertain as I am past the age of 26 and given I do have other Insurance so wasn't sure if they would con't to cover me as Dean has) so gave my Primary Nurse as well as my PCP a heads up.
They apparently have already talked to Unity and confirmed no referral is needed for the weekly infusion (it will now be back to an out of network site at my relatively new D-FH site) but per what my Nurse told me and makes sense given our Insur. plan is a Point of Service (meaning we have the option to go out of network for coverage just is a different coverage tier) which is what I wanted given as it is now I see a few Providers in-network with Dean.
I'll keep most of my Providers the same whether they are in network or out of network but as it with my current (Dean) insur. provider most of my Providers will fall under the POS out of network coverage.  With our Unity plan being a POS I won't need referrals but the Providers I see out of network will still be covered (this includes PCP, ERT (Infusion), Cardiology/Heart Rhythm, Neuro-Endocrine, and a few others. My Botox (PMR) Provider at Dean had left his practice this summer anyways so I am switching back to the PMR dr I originally seen at UW and will go back to seeing Neuro-Opthalmol. there as well.
Some to be sorted yet but overall seems to be falling in to place fairly well. For that I truly give praise to God as I cannot even say how many times I have prayed about and worried about this all!

Shunts (B/c I wonder will he ever actually fix this? )

Someday I hope to be able to write here that we are finnalllyyy going to fix the Lumbar-to Thoracic Shunt (LP Shunt but sits in Thoracic space) but as it is that time is not now unfortunately. I feel like everytime I talk to my Nrsgn he wants something different but this time he wants to talk to Nrgns other MPS Families see and I am seeing the Nrsgn at UofMn next month (early August) to get his opinion for my Nrsgn on the whole shunt situation. It's all a bit shitty being quite frank! It's like others on my Provider can see and know the issue but he's so determined we can't fix it b/c it will just fail again and "your not that sick". Fantastic (note sarcasm)! The worst honestly is how everytime we deal with a longer on-going shunt issue is how it affects vision, this time being no different. I've already had my glasses script increased 2x in the past year and no sense getting it adjusted now till (God I seriously pray and I truly mean this) we do. I realllyyy like reading and is so much more difficult when these shunt issues!

In any case will update again when there's anything to update. Happy 4th of July! Be reminded this (coming) Holiday wknd all we have to be grateful for!

                                VBS kids and new Hearing Aid (L side in process yet)
                                                             VBS - 5th / 6th Grade
                                             Pretty Nifty Hearing Aid I have to say (Resound brand)

Friday, June 16, 2017

Team work.. Team effort? Wishing it where a little faster..

Mmmmm what to even say sometimes? This MPS stuff can be and often is so very frustrating!?! I know it's an on-going, on and off issue we deal with, with these shunts but I do not understand how the symptoms I have been and am dealing with are just ok to deal with. SOOOOO Frustrating!!!!
Neurosurgery, LP Shunt, Extreme irritation.. -
Because frankly I was at  a complete loss what else to do and my Primary dr. thought maybe we needed to get the opinion of another Neurosurgeon so I reached out to a few docs in my MPS Community.
It amazes me (but I am always very grateful) every 1 of the few drs I reached out to emailed me back and was willing to give their thoughts. The Peds Neurosurgeon at UofMn (who is friends with my Nrsgn, unsure being completely honest if that is a good thing or will just cloud the picture) agreed to give us his opinion and will see him when there in Aug. Given symptoms are seeming to get worse still again, vision being affected now to (and I've already had my glasses script changed 2x in the past yr so this stinks actually! Its an issue that several friends of mine or their kids also deal with, when they have shunt issues their glasses scripts change). Super frustrating as I LOVE to read and this makes it more difficult and 2. no point in getting vision checked/script probably changed till after shunts where (God willing =( ) fixed at some point. =/ I've avoided calling but will have to do so next wk I guess. SO DAMN FRUSTRATING!!!!!
-Dr.Iskandar did email me back earlier this wk re the other drs emails and says he's not opposed to exploring the Lumbar space (space where the LP Shunt formerly sat) at some point, but wants to get these other drs thoughts 1st. His concern being scans show the lumbar area where I've never had problems with pain is more scarred than periods when Dr.Bragg and I dealt with the LP Shunt (of the 2 shunts the LP Shunt was the 1 her and I had very few problems with other than the period where we (like now) had moved the catheter to sit in thoracic space as it does now. Again SO FRUSTRATING!!!
Then like now I experienced the very same radiating irritation, pain and discomfort in spine and radiating pain down my R leg (I can't remember for sure but I think at that time it was affecting L leg, likely due to where the catheter sits positioned closest to (which nerves I guess?). I cannot recall for certain but I think we ended up moving that shunt due to a shunt infection) and though it surprised her after the irritation I experienced then completely resolved when that shunt was removed and did not recur when we replaced it in lumbar (low back) area (from thoracic, mid-spine) as it is now.
Actually thinking about it now as I type this I can remember asking the Infectious Disease doc (I've worked with the same 1 or 2 for each of the infections I've dealt with w/the shunts) if he thought the irritation where caused atleast in part by the formerly unrealized  infection and he had thought it did affect it. This said we do know CSF (Spinal fluif) is clear, based off somewhat recent cultures when the shunts where tapped.
I guess all I can do is keep pushing and frankly (b/c that's what it feels like I'm doing which STINKS) complaining to try and get this resolved. =/

Humira, Dr.Polgreen (inflammation, MPS I Study):
Re Dr,Polgreen's study for the anti-inflammatory injection drug, I did the consent and initial phone interview earlier in the week.. Initially she has funding for 4 patients if I remember right (4 MPS I Patients) and once she is able to locate/secure the rest of the funding the Humira study will include 15pts total ages 5 and up. My Team is okay with my doing this study so as long as I meet other criteria once I go out for the actual  study enrollment (late July/August she's thinking) then will be 52wk study and if actual drug then that starts right away other ways is placebo injection every 2wks for 15wks if I remember right followed by actual (Humira) drug for the remaining weeks.

Genetics (Dr.Bodamer) - Boston
Having saw my Genetics dr earlier this wk when in Boston we talked some about the drug/study he is working on (will not replace Aldurazyme but be a complimentary/add on therapy, with the thought if it works as it has in animal studies it will help increase the enzyme my body does not make naturally and should cross the Blood Brain Barrier which is a significant issue for me). We also talked about the Whole Exome testing I'd done a little over a yr ago (b/c it was done as part of a study the results take a-llooottttt longer to get back). Even this, b/c of being done as part of a study we (my Team) likely won't see all results and if there is something that gives clues or possible additional answers to my issues Dr.Bodamer will pass those along to my Team.
 He just affirmed again his suspicion b/c of my typical yet very atypical MPS issues he suspects (as has my Cardiologist) an separate connective tissue disorder though he hasn't ever said if there is a specific 1 he/they suspect just that there are some of these CTDs (Connective tissue disorders) that have treatments and if that where the case he feel it could provide a good benefit to my situation. In this case 1 does not wish for another diagnosis and yet if it where something fairly easy and had a treatment or way to manage it that made my overall situation easier to manage then it perhaps would not be a bad thing. Who knows, it may very well be the testing won't show anything, only time can tell! =)

I just have to say I love Boston though felt pretty crappy so other than dinner with my Case Manager from Genzyme and as well dinner with my runner from last Fall (Kai, he ran the Connecticut, Hartford Marathon for the Running 4 Rare Team on my behalf  even though I was not able to be there) I didn't do a lot this trip.    It was nice meeting Kai as that was the trip I'd have loved to make (I've never been to CT) and was duelly, really disappointed when I missed going to AZ! Was really nice meeting him and learning a little about him and what he does at Genzyme!

Heart / PVCs
Heart wise, we've been dealing with PVCs (which in and of themselves aren't dangerous) for months now, though they didn't actually start till a few months after the 2nd ablation done in Jan. Anyways we've been using Verapamil  to try and control the symptoms these cause with some but not a lot of success and when we'd tried Beta Blockers the side effects from those as has been the experience in the past was to significant. I'm of the belief if I am helping 1 symptom but feel far worse on another hand have I really gained anything?  I don't really think so and so we had stopped that med. 
My Heart Rhythm Team is suggesting 2 different meds (options), though 1 seems less bad then the other. Amiodarone can have it's own side effects but then some never experience any so i'll hope that is my case. The 2nd med, Flexainide apparently Pts. have to be admitted in-pt to start it initially as it can apparently cause other problems with heart rhythm. Lovely, totally lovely, hey? Unsure yet what to do but have to try something and not real keen about going in-pt so we'll see I guess but probably the Amiodarone. Yuck, just yuck what we as Pts have to put in our bodies!

 Pulmonary / Audiology -
I emailed my Pulmonary (Lung) dr. re the testing typically she orders (sometimes Cardiology does) and she is setting up that and an Appt. She is the dr. along with my Cardiologist who 1st suspected the MPS and though I don't see her often is 1 I really like and respect. Her clinic is based out of FMLH (where I used to infuse up till a month or so ago) .
Audiology is set for next Fri, I'd rescheduled this as I agreed to teach VBS (which I am still working to figure out lesson plans and best way to present/teach given I/my class won't be in an actual classrm)! Anyways she (Hearing dr.) was ordering the new hearing aids (red, oh so red they will be =) ) and will start the process to get those set right.

Insurance Change
And last my Insur. changes July 1st so my Case Manager from Dean (current primary insurer) is coming to my Infusion clinic Tues to meet. I can't say enough about her and how easy in many ways she's made working with an HMO seem and how many endless questions she's answered!  I am going to miss being able to pick up the phone or type out an email to her (Marge) and when things my Genzyme Case Manager and I sorting her and I being able to call/email Marge! I can do most everything on my own but really when you don't have to and is easier to have someone's help, who has inside knowledge, why not! I am hoping at the new Insur., i'll be able to have someone of Marge's same personality, caring and incredible knowledge!

All for now, please say a prayer if you wouldn't mind we could figure this shunt stuff out sooner than later... Much sooner would be great..


Friday, June 9, 2017

Same ole' same ole' same? Nothing new w/shunts. other stuff moving along

Peds Neurosurgery  -

It's been  a busy few wks and yet a not all that productive (on one hand) few wks. Between still trying to get my Nrsgn to reconsider the LP (Lumbar, low back) Shunt and the incessant but intermittent  irritation that's been causing since last Jan (you know time... great how it's worked so well to make this feel even an ounce better (note sarcasm, I am not being serious) ) though I've come to the conclusion and my Primary dr. from having talked to her about this several times seems to feel pretty similar.  She feels our best bet is to get a 2nd opinion elsewhere though our issue is we don't exactly have a wide (or even small) pile to pick from re Neurosurgeons who deal with complex cases. So frustrating!!!
    I've reached out to 1 Nrsgn in my MPS Community (though I know he's a good Nrsgn I'm not very hopeful he'll have any suggestions, from what I know from other MPS Parents he's pretty conservative to. =/
We need drs in our MPS Community who aren't afraid to think outside their typical and yet Neurosurgery wise there just aren't any.  - I keep in touch with Dr.Bragg (former Peds Nrsgn till she moved last yr) and I think she's tried to help repeatedly from afar (by talking to my Nrsgn now re her and I had this exact experience and what finally helped it) but doesn't seem much luck there either. Stinks being perfectly honest going from someone like her who was willing to try even if we didn't always get perfect but she trieeedd.  As a complex Patient that to us Pts means a lot when our drs just try!

1 of the docs in my MPS Community is going to try to help so we'll see. I appreciate his help atleast! I can't even say how much I wish we could figure this LP Shunt out and move it! I regret all the freaking time ever having agreed to the position of how the shunt and valve/reservoir are.
                              MPS I - Humira, Dr.Polgreen (Study)
On a separate note, I'd been talking to this dr for some time, helping reach out to families in my MPS I Community to let families know Dr.Polgreens study would soon be recruiting again, the details, etc. I heard from Dr.Polgreen the other day again and she had just received the new IRB approval (IRB has to do with a study site approving the logistics of a study basically)  plus is now enrolling individuals. Her and I are talking tmrw (Fri) re the study, details, etc. The downfall is it would require 3-4 trips to CA (Los Angeles) in a 52wk period but the rest of the study whether 1 is placebo or 1 is actual drug (Humira) would be done from home.
  I can't say on this for sure, although I have read the study protocol, requirements, etc but I believe those who start on actual Humira (vs placebo) will do injections every other wk. Then those who are randomized to placebo would receive the actual drug I want to say 1/2 way through the study.
      It looks like the study site hasn't been updated yet but for any MPS I Pts or Families interested I can send you more info if you like or contact Dr.Polgreen.

  The 1st initial phase (2 Patient I believe, this is the one I'd been asked if I was interested but at the time we where dealing with Heart surgery or Ablation yet) article is below.

          I heard from my Audiologist (Hearing dr)  re the Hearing aids and coverage was as we suspected.                        
  We knew  the 1 hearing aid bc of the hearing test changes a month or so ago would be covered by my primary insurance but that the 2nd would fall just outside their parameters. After my Audiologist turned in/submitting the various paperwork we now have approval for the 1 side (which was the more urgent to get through given my insur. changes July 1 and my new primary insur. if I am accepted to stay under my Dad's new policy does not cover hearing aids. Re the 2nd Hearing Aid she was turning in the forms to my secondary insur. for coverage and hoped to have that by the time I see her for the 1st side (1.5wks from now, other dates she had didn't work for my schedule).

I did actually have hearing aid(s) some yrs ago but then hearing had improved (yay!) for a few yrs (which was awesome!) till now. My hearing issues as it is fall in what's called SSNRI (Sensorineural hearing loss, hearing at the higher levels)  so in quiet situations I can hear pretty fine.

Unity, Insurance  switch (from Dean Health Plan)
Re the new Insurance change, I've been working with Unity some and the paperwork I need to complete has been mailed late last wk, just waiting for it to get here! Thankfully I have secondary insur so if it came down to it my Genzyme Case Manager (they help with Insur aspects of the enzyme replacement IV Infusion drug I receive every wk given Aldurazyme is so expensive and given weekly for a Patients lifetime).
Anyways so I've been getting a little info from my Case Manager at Dean re what Amy (Genzyme CM) and I should or can do re the insur change and my current infusion site.  I am REALLLYYY going to miss having the CM I do! I don't care what anyone says, having a Case Manager when you get a good one makes ALOT of things a bit easier! Especially given my primary insur. has been an HMO the past 3 or 4 or so yrs my CM has worked wonders with helping get many of my out of network Providers covered as 'out of network exceptions' so they where covered.

My CM (Marge) and I are going to try to meet in a couple wks at my Infusion site which is relatively closer to where the Dean Health Plan is (she'll come down to my infusion site)  which will be kind of cool
. It's always a bit funny how much time as  a Patient I spend talking to some people and yet if I where to pass them on the street, by site I'd have no idea who they where! =)

 Picture from earlier in the week. My Infusion site is now in the same bldng as my Primary dr (Internal Med, actually her office ironically is in the same clinic space where I used to see my Peds Cardiologist yrs ago - I seriously LOL'd the 1st time I went there to meet her/see her and realized this!) =)
    Anyways she has stopped by infusion a few times and this time I'd asked if we could take a pic (I am a total picture person, I like having pictures of all the experiences and people I meet or work with when possible). =)

Wednesday, May 24, 2017

"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,


Tuesday, May 9, 2017

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!

                          Sunday School - Love these kids, love teaching them!                                               

Thursday, May 4, 2017

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,

Monday, May 1, 2017

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!!

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,


Tuesday, April 18, 2017

Insurance; Infusion site, Conf. Call (Insur Co heads, Team). new Neurosurg. Appt.

Earlier today, after what felt like a mad dash to get my infusion going (my Nurse was amazing!) and things settled I had a Conf. call with the Dean Insurance Med Team as well as my Case Manager from Genzyme called so we could talk ahead of the conf. call.
Altogether there where  6 people from the Insurance Company as well as my Case Manager from the Biotech Company (Genzyme) that markets the Enzyme infusion drug I receive every wk. If I remember right it was the overall Insurance Company Medical Director, the Infusions Medical Director, the Pharmacy Director (all for Dean Insur.), my Insur. Case Manager (for Dean), my Case Manager for Genzyme as I mentioned above and admittedly I am forgetting who the 6th person was from Dean. Impressive though that all these people took the time to call in and talk to us!

Non-the-less as Amy, my CM said to me when we hung up from the call and she had called me back (on her day off no less, I am blessed to have her on my Team!) she has been on a lot of these types of calls with other of her Genzyme Rare Disease Pts and never before has she been on a call where so many from the Insurer took the time to familiarize themselves with the case  or the history. For that I was and am very grateful. All seemed to chime in, answer my questions and bring up very good points or things they are working to have in place. Although it's very likely it will be denied at the very end of the Conf. call (about a 1/2 hr in length) my Dean (Insurer) Case Manager (CM) mentioned I can request a new Prior Auth (PA) be done (I have) to request re-approval at FMLH to continue infusing there. This likely will be denied given Dean's push to bring Pts in-network, to their sites but then she also mentioned we can write up and submit a "appeal" to any denial if we wish.

Ultimately although yes I do wish my infusion where staying at FMLH as they have experience and have been able to manage my (especially) cardiac-fluid issues without needing to stop or miss any infusions I can understand why it is a huge benefit even to us Insur. plan holders to go in-network for the infusion as the cost savings to Dean each wk is very significant (roughly $42,000 now a week at FMLH where it was roughly $13,000/wk when Dean paid for the drug before it shipped/I infused). I'll also still be able to get it out of network when in-pt say if at UW or CHW.

I guess if I'm going to find any light in this (believe me I'm really not, I really don't want to change sites, the drive to FMLH is MUCH easier than is the drive to the Fish Hatchery site every wk) but my PCP works at the FH site so if there where any issues other than the 3mo span she'll be off (maternity leave) starting around July she'd be able to (her words) come over/help manage any issues.
On a different note (sorry this is ending up a lot longer than I thought) Peds Neurosurgery called Monday and a new Appt is set for a few wks from now w/Dr.Iskandar. I am not entirely sure what she meant but seems he's reconsidering/maybe has something else in mind (maybe she meant he wanted to re-test the shunts again? I'm not to sure, I was driving when the Secretary (Anna) called though I did pull over). Anyways so any prayers appreciate that goes well.
I think I may have wrote about it in the last post but he emailed me he was reconsidering the ICP Monitoring which I agreed with. Thinking about it since then I think even if we did do this him and I have to have an agreement what #'s range he would be looking for to in-fact show 1 of the shunts isn't working (which the shunt taps seemed to show). I and Dr.Bragg know well what (very small, very low) range I feel best at but I'm still uncertain if Dr.Iskandar completely gets this.


Will update sometime soon.
Hope everyone had an Happy Easter! He is Risen, He is Risen indeed!



Thursday, April 13, 2017

Is it really have to be this hard? Shunt frustrations

Why does life as  a Rare Disease Pt have to be so difficult? Why do some Specialists who are good at their fields have to be so dense to the not straight fwd/not as simple' aspects of rare disease issues?

I feel so frustrated, actually distressed in a way this past 2 wks. I do not normally experience anxiety much and I have to say I feel distressed. Frustrated that the answer is so evident, the NP got it, Dr.Bragg I think (?) believes its shunt issue (she originally stated she felt a revision was needed not sure now after she's seemed to talked to my Nrsgn. Regardless the shunt taps where very abnormal. My Primary dr. wondered how it isn't clear (that its my "normal" pattern with shunt issues) but this Neurosurgeon seems to have no clue?
Why can't he understand that in my MPS we often have difficult Hydrocephalus that isn't very straight fwd. Yet why also can't there be/or isn't there more drs in the MPS Community who treat this sort of issue-why isn't there more Neurosurgeons aware? Is Discouraging waking up every day feeling like this and little hope. =/

Between this shunt issues, the pressure being so typical to me when shunts not working right (pressure worst at night, early morning, slowly improves as the day goes on till finally about dinner time I feel better albeit imperfect). And then the Insur.-Infusion stuff. It's so tempting to me to just drop my primary insur. and use Medicare as primary thus I wouldn't be as stuck re where I could infuse at and I could stay at FMLH. The local community hospital has no business doing this infusion and the drive to my Primary (whom I really like) is more difficult than is my drive to FMLH/Milw.
 Neurosurgery wise unfortunately there is no good option, the Peds Neurosurgeon that works with mine now (replaced Dr.Bragg) feels the same as my Nrsgn does, that there's nothing he can do to help. Dr.Bragg of course isn't local and for that matter apparently can't treat Adults at her new hospital so seeing her in AZ isn't even an option. That makes me sad (wouldn't be ideal even if could but atleast I'd have an option).

I JUST FEEL DISCOURAGED AND ANGRY AND UPSET. Why does it have to be so difficult?
Sorry to basically just vent,

                     Reminder to myself. There has to be someone who can help, who will help, right? =/ I pray God leads me to whoever this is. =(

Friday, April 7, 2017

Neurosurgery (Shunt taps, clear but not clear result), ERT (Infusion) Changes

     This wk has just been a huge barrel of fun (or b.s, maybe a huge barrel of b.s?)

Peds Neurosurgery / Shunt:
This Appt was no less frustrating then any of the past ones with this Nrsgn have been. On one hand he says himself he has no experience with my MPS and thus the different way it presents Hydrocephalus in my case but then he treats it just like he'd treat any other complex Hydrocephalus Pt. SOOO FRUSTRATING!!!!
The shunt taps done on both shunts at Thurs's Appt showed the VPL Shunt they where only able to get around 2cc's I believe the NP said and she wasn't able to pull anything off the LP Shunt other than there was flashback in the needle (nothing able to be taken off).
I ran this by Dr.Bragg and she raised the concern a shunt revision likely needs to be done. But yes lets confuse the picture even more by repeating ICP Monitoring which won't be clear (b/c my pressure issues even in complete shunt failure aren't normal "high pressures" but is to much for me. And with atleast partial shunt function I completely don't get what the aim of this testing is... If he doesn't fix something after I do not know what I am going to do. I really keep questioning if I should try to get a 2nd opinion from his Partner (the guy that replaced Dr.Bragg at UW-AFCH basically).
This testing is set for a few wks., from now and between this and the infusion stuff I'm not sure which issue is frustrating me more!

ERT Infusion:
I don't even know what to say!?!?! This wk has been so freaking frustrating/stressful/irritating! Not only is my primary insurer trying to switch the infusion I get every wk to a site that has ZERO experience with my MPS I and enzyme infusions in general but these are the same sites that not 3 yrs ago said they could not do it because they didn't have the capacity (experience I guess)?!?!
I am fighting this all the way, I've been working with my Genzyme Case Manager and talking to my Case Manager at my insurer who unfortunately is in the middle as she then acts as my and my Teams voice what our concerns are.
Multiple Providers have told me they do not feel this site, which has no experience should be doing the infusion and my Cardiologist raised the concern they will not have a Geneticist on site. FMLH also has been able to adjust my infusion rates and volume around the cardiac issues b/c they have experience with enzyme replacement infusions in general.
1 of my Providers (whom I love) commented along the line 'they'll only do the infusion if they feel comfortable' which does not make me feel comfortable b/c I do NOT want there to be issues when we have not had any with infusion in years!

Will update sometime soon. Sorry it's been awhile in-between updates lately. I do tend to write a lot less often when I am not feeling great which I am not.


Wednesday, March 29, 2017

Exhausting and a little hard.. Shunt stuff. Late Cardiology update

I apparently started writing a update a few wks ago and never finished (see below) so will just tack on to that.
It has been a bit rough of a few wks, not that the past few months have exactly been great but atleast when I had the Spinal Fluid (CSF) leak from my low back (LP) Shunt I had great headache control even though the low back pain/discomfort was pretty intsense any time I was on my feet. That CSF has resolved or healed itself unfortunately now for a few wks and so not only do I still have pretty bad back pain to leg (radiating) pain I now have some pretty freaking (not) awesome headaches that occur mainly at night and certain parts of the day (when pressure is highest). Has stunk really bad!
I have been talking to Dr.Bragg some, getting some advice from her (I miss her so bad, I literally am tearing up just writing this. I miss having a dr. that really listened and thought outside the box. A dr. who realizes my underlying MPS makes my Commun. Hydro just different in some ways than are 'typical' (whatever that is) cases of it. She never gave up and she'd try options like intermittently removing CSF to give relief while we figured things out. Did I say I miss her?

Non-the-less earlier this wk or last wk I emailed my Neurosurgeon (her former Partner) and asked if we could do a shunt tap. check the function of the 2 shunts. I don't know but I do know I feel crummy. Thankfully he responded and agreed we could check the shunts function and see if both are working right.  That is set for next wk. I wish it would be a simple answer! I literally have felt a little depressed some days just from feeling so crappy and frankly uncertainty in answers. I struggled when Dr.Bragg was here, trusting her enough but atleast I knew she wouldn't stop trying to help/figure things out.  Now is just hard.

From a few weeks ago... -  Heart Rhythm Appt
Follow up with Dr.Kovach (Heart Rhythm, CHW) was Thurs an went ok. We opted to add back a low dose (milder) beta blocker, see if this along with the Ivabradine can help calm some of the funny/off heart rhythms. Heart rate itself has averaged (on the past several heart monitors) 80s with some upward spikes in the 100s but overall rate itself is better following the heart ablation in Jan.

Re the beta blocker this was a  joint decision, between Dr.Kovach and myself to trial it. I appreciate my Heart drs. want or ask my input and don't just do something without a joint agreement! I have to admit I may not get this part right or entirely right as I didn't write down any notes and I didn't record the appt (when I do this I delete any recording after I write down any notes) but anyways he mentioned a few options we may have though he's not eager to do/try should we not be able to get these wonky heart rhythm issues to calm with meds. As anyone whose followed this blog knows we haven't exactly had the best of luck with heart meds! That said any further ablations which I genuinely think is unlikely (that we'd try again) but if we did the risk is greater still than the other 2 times. This time especially so b/c we've had a few short episodes of heart block (where the heart stops beating for a brief period or maybe it's that the beats come in a sporadic, inefficient pattern. I honestly can't remember).
   Non-the-less right now it's not on our 'being considered' and I get the feeling is pretty unlikely we'd try again unless things change or got worse.
Will try to update sooner, I genuinely like writing (is easier than talking about how I feel to people and I am glad this blog seems to help so many, who have said as much) but I just don't do it as much when I don't feel good. I guess b/c it's energy I don't really have.

If I sound depressed I promise I am not, just a little discouraged and a little sad and not sleeping to great (I still LOVE my recliner though!) doesn't help.
Thanks for stopping by,


Monday, March 6, 2017

(Cardiology, Neurosurgery, etc) Wonder when we'll figure it out..

Going to try to make this short - I know, stop laughing. =)


Cardiology I had the 48hr heart monitor which I mailed back the other day. Usually takes a wk or so to hear anything. I did have some symptoms during it and in general haven't been feeling all that great for the past few days or week or so so who knows. Between lightheaded episodes (but very odd), heart rate that's a little odd and the low-to-mid back stuff (irritation, radiating discomfort) topped now to with the CSF (Spinal fluid) leak in my low back from the LP Shunt seems like it's pretty much headed. That stinks a lot! Headaches still aren't as bad as they are sometimes, like when the shunts are completely not working but that leak definitely makes/made things much better!

Neurosurgery wise going off above, I seen my Neurosurgeon today at UW Children's and he is basically at a loss. I said to him the risks of doing more surgery with the LP Shunt may be real (and I know they are to an exent given the stenosis/narrowing and severe scarring) BUT that shunt location right now SUCKS! Now to the CSF leak from around that shunt being pretty well healed makes it all even worse.

I have thought a lot lately about this weird life I live, both normally and so abnormally abnormal. I watch or see people including really my own family, siblings especially going on about their lives, functioning normally, doing normal life things every day meanwhile most or many of my days are spent dealing with insurance stuff, endless medical stuff and all that goes with both of these. Shoot I even fully realize with having my Case Managers at Genzyme and at Dean I think I have it pretty decent compared to many. I think it's just the fact even with these 2 helping (and they do help a lot!) I still have to communicate with them, sort paperwork, deal with scheduling drs. appts., figuring out when f/ups are needed (today's Neurosurgery Appt? I have no idea in hindsight what the plan is other than Xray was done and he wanted to look at some prior surgery notes (for which I sorted through my binder of Neurosurgery Med records and sent this to him and to Dr.Bragg. So much easier keeping copies of all those notes! I called UW Med Records to have a copy of recent spine MRI and Xrays mailed to me which no big deal any 1 of those things but altogether it takes up a lot of time!
I talked to my Insur. Case Manager while still at the Neurosurgery Appt to sort prior auth for the Xrays done (PA is covered for a yr at a time but something was messed up today) and several other phone calls or messages before I'd ever gotten home.
Once I got home? More phone calls, emails, answering messages, editing a document for the interview/photoshoot I did a few wks ago (Levi and the guy he works with do a write up to go with the pics, I somehow messed up the changes I'd made so will have to re-do that in a couple days). This on top of a few 'more normal' things and the day has just been busy and kind of nuts but one of those days you wonder 'what did I do' even though I know I actually did a lot!
Anyways so I guess I've just been thinking a lot about life and trying to figure this LP Shunt irritation/symptoms out and in general pondering life and my life and maybe especially so b/c of watching people around me and how normal their lives seem (even though I realize there are so many whose lives are equal as out of the ordinary as is mine).

Happy 34th Bday I guess and hopefully a year that I in a way is a little less frustrating than this past yrs has been! Was definitely nor all bad and plenty of good sometimes just would be nice if it where a little easier at times! I know if it be God's will we will figure this shunt stuff out, the seeming heart (rhythm) issues and i'll con't to hold my faith, the best I can (failing often undoubtedly) that tomorrow and the next day and the day after that will bring answers and better days. For now I am just grateful to have all I do and even though I don't feel particularly awesome I know I am blessed by those around me whom I love, my Team of Providers and even Providers, like Dr.Bragg whom are no longer here but choose (so much gratitude on my part for her!) to stay a part of my Team and so willingly offer so much help.
 I am grateful for all around me who (to be perfectly honest) sometimes pick me up in a sense when I am frustrated, tired and at times feeling a bit at the end of my rope. I am grateful for all who offer advice and frankly optimism that I sometimes am just barely otherwise holding to (re finding answers).
 I know we've done this so, so many times but even if I felt uncertainty other times atleast I knew in my heart Dr.Bragg would help me figure something out. Now I don't feel that certainty and that stinks. I do think Dr.Iskandar is trying, much thanks to Dr.Bragg for that but I don't think he really gets what it's really like in the harder moments and it just seems or feels like she got it in a way that made even the uncertain, difficult times a bit easier to handle. This all said I'm not giving up, I can't but it does get hard!

Thank you for stopping by, sorry to vent, sometimes it just helps!

                          San Diego, Loma Point Beach (I think that's what it was called) - was beautiful!
Thanks for stopping by and no matter, ultimately I know my God knows the plans he has for me. For that I am grateful.


Tuesday, February 28, 2017

Rare Disease Day - a year ago.. Now vs then

I am not a worrier all that much or the things I do worry about are things that in the grand scheme don't matter that much but 1 thing I do think about often enough and worry about some is (especially when it's something seeming new or uncertain) will my drs. think I'm making something up? Will they (do they?) believe me?
I can't really say why that's something I think about but I do and I guess maybe it comes from all the days when no one knew and from time to time I did get a dr. who in as many words questioned what I said and/or felt.

I am wide awake (but certainly tired!) at 5 in the morning, this Rare Disease Day, many things on my mind and some iffy/odd/weird symptoms occurring (very) intermittently over night and it ALWAYS makes me cringe! I'll wait to say anything, see if symptoms con't or not but to this day I STILL hate calling drs offices, trying to explain something (I will be 1st to say I often struggle  trying to explain symptoms or how I am feeling or if something different what it is exactly. Hopefully in this case the symptoms where just a passing thing!

It is RARE Disease Day (capitalized b/c come on it's RARE DISEASE DAY!) 12 yrs ago this day didn't exist so it's a pretty neat day to me, even if all i'll be doing is driving my sleepy self to infusion, working on paperwork and phone calls and 6ish hours later driving myself home (to hopefully crawl in my pj's and go to sleep I hope!) On this Rare Disease Day I am grateful to have this infusion and even if it's an (basically) entire day 1 day of every wk, week in and week out I am GRATEFUL I have a treatment and to that better treatments are being studied and in studies!
None the less, if you are healthy be grateful. If you know someone with a rare disease, wear your jeans (for genes, the faulty errors for those of us with genetic issues in my case the error falling to a single gene (4p16.3) which was something both my Parents happen to be carriers of)! And if you are like myself walking around with a rare genetic disease well then celebrate this day in some form or another as we are alive and though some or many days may be difficult we've made it so far!


ps a year ago earlier this week., I had heard from Dr.Bragg that she'd accepted the position in AZ at PCH. I can still remember how I felt, how many times my Providers said to me after that (and still say from time to time) they didn't know how to replace her and "there will never be another Neurosurgeon like her". Doesn't mean other drs. aren't good just that she was mighty special for her field!
Reading the various things I wrote that day and the days after was a little heart wrenching (I was so scared, undertain, afraid I think) but I also wrote that I couldn't wait for it to be a year from then honestly for the simple reason that things would be better settled, I'd know who my Neurosurgeon was going to be and almost above all I'd know or have a better idea of what things would be like. I am glad it in fact is a year now! I miss her terribly but she's thankfully has in fact kept in touch.
I'm working w/her former Partner now and though that's not always been the smoothest (I guess she just was a good listener, she didn't try to pretend she understood it all but she learned). - Things will get better with the Neurosurgeon I see now, he's told Dr.Bragg and I he's glad her and I keep in touch as she helps both of us. Above all I get it, he's just a different personality type than she was, but if he tries I can keep on trying to.
 Just forgive me when I get frustrated and when I feel like I'm banging my head on a wall. It's simply b/c I want to sort things out and most of all, above all I just want to feel better (head is pretty good, the leak makes a huge difference, it's the low to mid back radiating pain and the deep-again radiating pain down my R side and at times now when on my feet my L side as well. God has a plan, right?

Another year from now, God willing who knows what will all have occurred, what i'll be up to and what better things may in fact be an option or closer. God willing!
Thanks for stopping by,

ps It may be to late but wear your jeans or wear your blue!

Tuesday, February 21, 2017

Cardiology update, Neurosurgery, WORLD Mtng

Cardiology -
Due to some on/off (but very intermittent and very short lived thankfully) lightheaded/dizzy symptoms that started only in the past few wks or so (though before we restarted the Ivabradine)
Dr.Earing, my Cardiologist is concerned could our having restarted the Ivabradine post this 2nd Sinus Node ablation back in Dec. be causing intermittent episodes with the heart and abnormal pauses.  This concern is because of short episode(s) (ie a short run heart of heart block) I guess they saw on a recent Heart monitor (Zio patch) episode(s) occurring and should I be on this now?
We'll decide I guess based off the Zio patchc(24-48hr continuous EKG, records heart rhythm though 1 done since then was clear.  On the other hand I restarted the Ivabradine before these symptoms started and to heart rate while not drastically slower seems like it's pleasantly different.  Maybe that's a mind game on my part, who knows but even while the lightheaded/dizzy episodes which occur for a few mins. at a time, very intermittently are occuring the skipped or extra beats seem like they are improved. Who knows!

Dr.Kovach (my Heart Rhythm dr.) responded to an email I sent him updating on this all and he thought perhaps if symptoms kept on he'd want a 30day Heart monitor to see about any episodes over a longer period. I don't f/up with him until I believe May so we'll watch to see how things play out.

The Echo done at this last episode  showed a very slight improvement in the Mitral valve gradient which not significant but it puts the new valve back in the mild range vs. being moderate stenosis so if that where to stick (stay improved) that wouldn't be a bad thing!

Peds Neurosurgery F/up - few wks ago -
I seen Dr.Iskandar a few wks ago  which wasn't particularly useful (not the right word but can't think of what word I'm looking for)  and honestly the Medical Assistant had called a few days prior to confirm the Appt and say something about I'd need to schedule an MRI after the Appt (I forgot to ask Dr.Iskandar about that but assume he changed his mind as he didn't mention anything re doing imaging before next fup). This MA said he was planning for a 3mo f/up after the Appt. LOL, To that I just thought to myself  'so why am I going to the Appt if he has a plan in mind?' Needless in fact not much came off the Appt., I'm tired of saying over and over what an impact this is having on day to day life so was pretty quiet needless at the Appt though I made sure to emphasize to him this insn't normal, this isn't even entirely what Dr.Bragg and I dealt with after her and I 1st surgery together (symptom mirror then some but not all that much anymore. Hard to explain the difference).
Something I did realize just the other day, though of course several days after the Appt. is that a few years back Dr.Bragg had moved the Lumbar Peritoneal Shunt to drain from the Thoracic (mid) spine versus where it was in the lumbar spine and at that time I had virtually the same symptoms I have no including radiating leg pain, hip and significant low back pain and started in just 1 leg but spread to encompassing both legs after some time though 1 leg at that time always remained worse than the other to as it is now.
The only way I realized this was an old blog post, written about this same issue a few yrs (from this very blog =D ) was it showed up in my facebook timehop (a daily thing that shows up with memories from that same day the past 5-6 years). Needless I asked Dr.Bragg about this and she to felt I should mention it to Dr.Iskandar as he from what I remember placed the LP Shunt so it starts in my low back but the tip ends up in my mid (thoracic) spine now to.

Gawd I hope he will listen!!!!! Hi Secretary when I had called to make the f/up Appt last wk made an Appt for 1mo from now not the 3mo Dr.Iskandar had recommended and she just said if by some chance things are improved (that would be AWESOME!) then I should just call her back and she'd reschedule. Hey, can we can hope but I appreciate she also is insightful!

WORLD Meeting - Injectable anti-inflammatory study.
Otherwise I was in San Diego last wk for the yearly WORLD Mtng., which is 1 of my favorites to attend and was good. A few studies either started or will be started including one looking at a arthritis (and several other indications) injectable drug which is being studied by an Endocrine dr. formerly of UofMn and now at Harbor UCLA in CA. She's helped my Endocrine dr. and I a few times and a genuinely nice doc.
Last summer or so they did a small study with this drug of which I had been asked if I would want to participate but there was a lot going on here with heart and shunt stuff so I chose not to. Things now are a tad smoother so I have already talked with this dr. more, sent her several test results they'll need when looking at if Pts (myself) qualify and b/c they just got a new round of funding (yay to the Foundation I work for!) she expects they may be up and starting again in May. She'll keep me updated which I appreciate (b/c I know these docs and doctors in general have a lot else to do besides just update interested Pts like myself!
This would require initial travel to CA at study start and then any addt;l travel if there is any I believe is limited to the end. I may not be remembering that completely correct but I know the weeks during after initial enrollment they mail the drug to Pts.

Otherwise I did a photoshoot with Levi of 'Living in the Light of Rare' Foundation, whom photograps Pts, tells their story and has displays of these pics and stories at various Pharma/Biotech mtngs. These are used to raise awareness but also to give Pts a voice. The photoshoot took us to the Bay area of San Diego (about 20-30 mins from our hotel), and various area around there incuding some cliffs (maybe not the right way to describe), Navy ship area and to lighthouse on the Bay. Was fun, exhausting and so well cool! Some of the pics attached either I or Levi took with my phone but he'll send me the ones he took when he has them set (edited maybe or downloaded, not sure). Either way was really rewarding! We had dinner afterwards and he humored me by going to a more basic food place (Cheesecake factory) vs a (I think it was but can't remember for sure) Mexican restaurant (I have nothing against this particular one just didn't seem to have any basic items.
The night before that I'd had dinner with the Foundation I work for and with the CEO and Study Coord. for another study that is on-going using the Aldurazyme (drug I receive every wk) reformulated to cross the BBB. Was informative and interesting as well as the 2 people where extremely down to earth to talk to!

Will keep things updated here on any given 1 of the above on-going issues or study,
Thanks for stopping by,