Trying to hold on to hope..?... Peds Neurosurgery (UofMn) ), MPS Conf. -

I haven't written in a while for now reason other than I just haven't known what to write about. No I still don't feel great but who wants to read about my constantly whining about not feeling great? No one I'm sure thus I don't write and honestly there hasn't really been that much other than status quo stuff going on.

Shunt / Neurosurgery
Headaches (really it's more  pressure than it is actually a headache though I call it a headache. I get what I refer to a 'mini migraines' when dealing with the pressure and those are a headache - it's odd I guess to explain. Best I can say I suppose is if you had on a really tight, uncomfortable  headband that went all around your head and that begins to affect your vision. That is what my headaches (pressure) are a lot like.  Not very comfortable and needless the only type of headbands I wear are soft cotton ones or I have several that are plastic but 1/2 of the band is stretchy material (bc I hate having hair in my face).


UofMn - National MPS Society Conf.
Weds I head to Mn for the National MPS Society Conf which runs Thurs-Sat but 1st am seeing the Peds Neurosurgeon at UofMn Children's that I've know quite a few yrs though have never met (he was the Nrsgn who helped Dr.Dickson when I was in her Intrathecal Study yrs ago and she/Team suspected a shunt issue then already. She'd talked to Dr.Guillaume (who was then in Oregon) and he in turn referred me to who I see now (and that whole amazing fiasco of an appt. yrs. ago).
Anyways so Dr.Guillaume came to UofMn a few yrs ago and over the past few yrs has helped Dr.Bragg and I with questions we had re my shunt issues (and she would reach out to Nrsgn she trained under).  Dr.Iskandar my Nrsgn since Dr.Bragg moved last yr had asked me to get an opinion from someone in my MPS Community so I emailed Dr.Guillaume (who is good friends with Dr.Iskandar, I can't decide if that's a good thing or not given Dr.Islkandar won't seem to listen to what Dr.Bragg's seemed to try to tell him? I'm not sure. I'm honestly not sure how I feel about this appt other than frankly nervous, some scared (bc what the hell happens if he doesn't know/have a suggestion or something else? Shunt taps show fairly clearly there's a shunt issue and the recent Chest Xray done for Cardiology shows no fluid in the pleural (lung) space which there should be some I'd think if the shunt where working right? I don't know is all very confusing to me being quite honest.

I just pray Dr.Guillaume can give us a suggestion that Dr.Iskandar would really listen to. That Dr.Islamdar really would go in and look at the lumbar and thoracic (low and mid back) scarring which is apparently pretty severe and figure out a way we could re-position that shunt to give better relief and to calm the incessant radiating low-to-mid back pain. It stinks being really blunt!

HEART RHYTHM
We've been adjusting/titrating the (not so new anymore) Heart med dose while keeping all other heart meds the same with very little success for PVCs (skipped beats) and new fluttery rhythm. About 2wks ago the dose was upped to the highest dosing at 3times a day (I totally screwed that 1 up and had only been doing it 2x a day. 3x a day is a PAIN!).
Non-the-less I am tired of calling Dr.Kovach's Nurses reporting 'no real change' as I feel like a pain in the arsh and frankly I just hate continually needing to ask any of my drs. for help. I'm not good at that.
I guess the next alternate med Dr.Kovach recommends, if my Cardiologist (same clinic as Dr.K at CHW) ok's it is Amiodarone. Another awesomely awesome med choice I'm sure (and I don't say that to be negative it literally just has a terrible reputation, even my Team is hesistant about it and leaving that and 1 other as last ditch). Lovely, lovely, lovely.  I'll call sometime this wk, maybe tmrw.

New Infusion Site:
This has been going well - about all there is to say! The new site is well and my Nurse + the back up Nurse I have (if mine is out or not there a day I need to change)and the other Nurses + the Pharmacist are all very nice. Hast been good. =)


Sunday School -
Hard to believe but have been working on this for here and there for a few wks now. I love doing it and honestly I love that it's a constant reminder of all my God does for me and what I can (try) to do for others by sharing God's word! I love that I get to teach kids and witness through my own personal struggles to my SS kids that our God really is with us through thick and through thin.
I did get a heads start on things with SS, earlier than probably needed (so I could work on various things like rdering our teaching materials, sorting the materials, getting ahold of Teachers, etc  I needed as I had time and work with our new Pastor on whatever he needed me to do) but still hard to believe roughly only a month till Sunday School starts again! I always look fwd to this though (not so much to Fall and cooler weather though I just love seeing my kids). Some yet to do on my part but the part that I stressed the most (organizing Teachers for each grade) should be worked out! That was a HUGE relief!


Humira study:
Not much to update here other than I signed and sent back the consent forms, sent in the records needed (minus 1 for joint range of motion, which I am seeing my PT next wk to get the measurements) and scheduled to go out for the initial study start the end of Aug. We initially where going to do Sept but Dr.Polgreen and Team asked if I'd be ok coming out the end of Aug which should be ok. At this study they'll do the rest of the study enrollment then draw whether i'll start on placebo or humira (though I and Study team will all be blinded to which I am and no matter i'll have to do an self injection every 2wks).
At wk 16 then Pts on placebo switch to Humira and Pts on Humira switch to weekly injections and conts to wk 52 if I remember right.
In total there are 3 or 4 visits to CA (Harbor UCLA, Los Angeles) for the study.
                                      

Will try to update next wk re the Neurosurgery Appt. I do then see my local Nrsgn (Dr.Iskandar) re the Appt this wk in a couple wks to see if we can come up with a plan. Can't even tell you how much I am praying we could and trying to be optimistic. It's really hard to not be. =/  Say a prayer. I so need this to go well but am nervous (and I don't get nervous! I wasn't even nervous for the 3 Open Heart Surgeries which is CRAZY!!!!!)

Thanks for stopping by,

Erica


                                                         

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!

Erica
                                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)