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). =)