Neurosurgeons (Shunt/Spine docs) have talked! - Possible plan..

Going to try to keep this short and honestly there's still a fair bit to be worked out but alas. Dr.Iskandar emailed me the summary from his, Dr.Bragg and Dr.Guillaume (UofMn Peds Neurosurgeons) Conference call the other day.

Basically the long and short is they are thinking we focus on the Lumbar Shunt (shunt that sits in my low back and right now tunnels up to Thoracic (mid) -spine and rthe valve +the reservoir. These 2 add on devices control how much spinal fluid drains and the reservoir is used similar to my Port for the weekly IV ERTbut in the case of my shunt the reservoir is used for testing the Spinal fluid (CSF) and/or anything else that's needed to be done with that shunt to test it somewhat less invasively (without surgery).

Plan..
The plan atleast at the moment though I don't know actual timeline (I emailed Dr.Iskandar back and will call his Secretary this wk to find this info out) is to explore the lumbar (low back) area of my spine, see if there is an area that has more CSF and less scarring than what we've come across and that would allow us to move the current thoracic based catheter to low back where we virtually never had problems in the past. I am praying there is a more favorable area!!
I don't actually know how they explore the low back area, I mean imaging has been done so do they have to actually cut a long scar (my lumbar scar is already pretty lomg!) or can they go in somewhat less invasively with a scope and peer around various parts of the spine? If there's an area that is less scar filled and more CSF then Dr.Iskandar would move the cathether back down to low back space. This is the area we virtually never had issues with the LP Shunt in the past so I am praying.

Try adding 'On/Off Valve':
The other thinking was we try adding  a 'On/Off' valve separate from the whole spine exploration portion of any surgery (but during the same surgery) and then Dr.Iskandar I guess could remotely turn this on or off. This I am unsure how that works 1. they just do it at any random time? I do need to function so say its turned on and they suddenly turn it off and I did get really sick, what happens???? I definitely need to clarify that part.
 It's completely up to me I guess if we try this or not. Both Dr.Iskandar and I know we need both shunts so I'm just not sure as Dr.Iskandar even said in his email this part was my choice to try or not.  The only bonus would be in the slim chance we could do 1 shunt then we'd be able to get rid of the LP Shunt catheter which is of course the part causing endless irritation right now.
Big problem I could see there is out of the 2 shunts when I only had 1 shunt the VP Shunt failed more often than did the LP Shunt whereas the LP Shunt was frequently enough a problem but less often was due to complete failure.
                        

Do we try? Do we not?
Related to that in his summary email Dr.Iskandar seemed to question even doing this part (adding on/off valve) and left it up to me if I thought we should try it as he commented our experience last fall when we clearly showed I needed the 2 shunts.
Due to this and several other times we've monkeyed with the shunts or 1 shunt failed and only 1 shunt was working or when we had just the 1 shunt in place I KNOW we need both shunts and Dr.Iskandar seems to get that. I'm kinda confused on this part.
He just commented in this part of his summary if we did show I needed the 2 shunts but we where not in fact successful getting the LP Shunt back in lumbar area the last resort and far less than ideal/more risk area would be placing the LP Shunt to drain from my upper spine (catheter placed internal of course) or from Cisterna magna  (base of neck). I'm really not very sure on any of this, is allot up in the air yet and much remains to be seen and to be figured out!

As any one who reads this and has read this for awhile may have figured out the hardest part for me of any of the MPS Stuff is not even surgeries or treatments (not that any of these are all that fun!!!) but the hardest parts are the unknowns and the uncertainties!
 Have to trust my God knows the plans he has and his plans are the best plans, right? I am grateful it at least finally feel like we are getting somewhere in trying to figure this all out!?!...
                                                  

I head to Los Angeles tmrw (Weds) just for a couple days. Will update when I get back if there's any news.
Thanks for stopping by,

Erica

Brain, Spine, Heart - the docs have talked, thoughts will be shared... Prayers

I've not yet seen anything from my Peds  (Pediatric, if you wonder why Peds it's b/c this specialty of Neurosurgery tends to deal with the complex, congenital (from birth) disorders more than does the Adult Neurosurgeons) - anyways so I got a msg from Dr.Bragg last wk (my former Nrsgn) and then an email from my current Nrsgn (her former Partner cc'd to Dr.Bragg and to Dr.Guillaume re the Conf call they'd had that day). They want to type up a summary of what they talked about and then email it to me to get my thoughts.
 Dr.Bragg gave me the very briefest of idea what they may be thinking (something to do with my Lumbar (low back) Shunt. She seems optimistic i'll like what they are thinking so while I am trying to not get my hopes up I am most definitely praying and cautiously hopeful? Maybe, just maybe they'll have an idea for something we can do or try to help this shunt or shunts? I am really curious to see what they are thinking... When Dr.Iskandar had emailed to let me know they had talked he said they'd get their summary together this past wknd and send it to me so I am trying to remain hopeful i'll get it in the next day or 2?
I CANT EVEN SAY HOW MUCH I WISH WE'D COME UP WITH SOMETHING TO MOVE THIS SHUNT AND THEN A WAY TO HELP THE 2 DIFFERENT SHUNTS TO KEEP WORKING BETTER?? I can hope!?!?! <---- Sorry to basically yell, not really meaning to but I did purposely put it in all caps. God has a plan, right?

Heart Rhythm - Amiodarone Testing:

I  talked to my Heart Rhythm Drs Team last wk and emailed a bit w/my dr., and have agreed to go ahead with Labs and whatever other testing they want (not sure if there is any other they want 1st as baseline, I did just do an Echo not to many wks ago for Dr.Polgreen's Humira study, the Echo was done locally at my Cardiologists clinic). B/c of my travel to CA this wk (Weds -> Fri) I made the HR Appt 2 wks from now.
I'm really not all that eager to start Amiodarone if in fact we do add it BUT I know if side effects are to bad I can stop it and honestly I need to try something. These extra beats (PVCs) literally occur like 50% of the time. It's CRAZY!
We've tried numerous other meds with little success. I do not at all regret doing the last ablation (Cardiac cath to slow heart rhythm) as that has helped immensely in other ways but would be great if the PVCs never started (I'm not sure they are a  result of the ablation but could be due to scar tissue that occurs).
Regardless we'll figure out at this Appt if this is the route we are going to try. Amiodarone can have a lot of side effects apparently and I already take a fair amount of heart meds and meds to get fluid off so is always a balance.

Insurance
Separate from the above 2 things, Its scheduled for tmrw and I won't need her for much but I have a new Case Manager assigned at the new (commercial) insurance. - The plan at the moment is the person whose been helping my Genzyme Case Manager and I to get a sole point person assigned at Unity and the new CM + Amy (Genzyme) and myself will talk. We really wanted this so anytime anyone from my Team has questions all of us have 1 person we can talk to whose familiar with my case vs everytime I or someone from my Team would call we'd talk to a different, unfamiliar person. Hopefully this person is as good as my last 2 CM's at DHP!


                           

Old posts, Old thought... Old reminders...
Reading an old post on fb from last year regarding the 1st surgery I had with Dr.Iskandar and communication I had with my PCP nad my Cardiology (Heart) Team going in to that shunt surgery it reminded me (again) how grateful I am for the Team I have!
As I wrote above my Neurosugeons (Dr.Bragg, Dr.Iskandar, Dr.Guillaume (the Peds Neurosurgeon I saw while in Mn a month or so ago) all talked this week, Dr.Bragg gave me a brief overview of what they discussed and thinks i'll be pleased/happy ( I am hoping so, I so have been praying for a solution and to feel better).
Dr.Bragg let me know and then Dr.Iskandar emailed me that they are typing up a summary of the conversation + wouldl email this to me (hopefully) over the wknd for my to review. I pray we really are able to do something to help the LP Shunt (out of the 2 shunts that's always typically been out better working/more reliable of the 2 shunts and least amount of issues). I am saying prayers!!!

In reading the old fb post I was struck though just how much I've missed my Primary dr. over the past 1.5mo's that she's been out on maternity leave. She'll be back in later Oct I believe and I am genuinely happy she gets the time to be with her kids but she makes such a difference for my Team! I try to not take for granted the Team of Providers I have and have been reminded many times really that even with change I am still lucky (blessed) as despite Dr.Bragg leaving WI last year, she stays in touch and involved and though he's never been my actual Neurosurgeon Dr.Guillaume at UofMn has always been willing to answer my questions over the years. The same goes for so many other drs in the MPS Community.

This is long enough already, i'll update soon when I hear from Neurosurgeons and if anything to update from the initial CA visit.
Thanks for stopping by.
 Please say prayers for good options from my Neurosurgeons and soon,

Erica

Knowing what we know if we don't know...

I can't even say that this post is going to be short/shorter so I will just apologize ahead of time and ask that if you are reading this you bear with me and understand why I am sharing the conversation I had, with my Pharmacist (owns the Independent Pharmacy at the Children's Hospital where my Heart Team is at). I also can't promise this will be the most entertaining update (but I doubt I am ever the most entertaining in the way I write!).
Jake has taught me a lot over the past 12 yrs and been an immense source of knowledge for my (I am sure) sometimes endless questions. He has always been willing to answer questions I had or to help me/my Team work out how to get a medication including sometimes brand new medications covered if my insurance was balking.
Anyways, so the other day I was talking about something I was frustrated with regarding my insur on my fb (and a drs office, not actually about the insurers) and how was it I could know how this particular issue we where dealing with worked but the drs office had no clue? Needless Jake commented (and he wasn't being rude, he was simply reminding me and I am thankful =) ).

So anyways, see below for what Jake wrote, if you have any thoughts please share in the comments section or respond via email. I really am curious others thoughts. I know many of us deal rather endlessly with insurance, insurance issues and working with (atleast in my case, though I am grateful for all the people who have been in my life and are still in my life regarding the insurance stuff ) a lot of Insurance folks, be it Case Managers, Medical Directors, and all the Hospital and Drs office people for our insurance to.

I've shared what Jake wrote re what I had posted on fb (he gave his permission I share this, please don't think I am sharing his words bc I think anything grand of myslf. I do not). I have learned what I know b/c I was blessed to have many fantastic people involved in my care who where amazingly knowledgeable and who where always willing to share what they knew with me. I am sharing what he wrote bc I had never heard of this effect he writes of and find it interesting!

                                           

-
Jake Olson Erica, you know more about insurance than 10 insurance people.
Have you have heard of the Dunning Kruger Effect? There are 2 sides to it, and you are a perfect example of the second part that is not as well known. Basically, the more you know about a subject, the more you assume everyone else knows the same thing, so you underestimate your ability and extremely high level of expertise about a subject.

You are an insurance expert. Very few people know as much as you. Take pride in that, but you also have to lower your expectations of other people.
Once I learned about this effect, it really helped me to be less frustrated with other people. I stopped assuming everyone else was as knowledgeable about a subject I know a lot about.
If you listen to podcasts, listen to this. It has seriously changed my life. (There are 3 stories, the part I am talking about is the Dunning Kruger Effect which is the second (or 3rd) part.
https://m.thisamericanlife.org/radio-archives/episode/585/in-defense-of-ignorance

I have started to use this concept with my pharmacy students. I give them a complex topic to research. When they come back to me with an answer, I ask them "Are you confident with you answer". If they answer yes, then I tell them to keep researching, because they probably missed something that they don't know they don't know. (The Trump part of the Effect if you google Dunning Kruger). Over confidence in a subject without all of the information. I have them keep going until they start finding the reason why I asked them the question (because I knew there wasn't a 100% correct answer). Once they have learned enough about the subject to start doubting their answer and they have a few options if the first answer doesn't work, then I tell them they are ready to answer the question.

I think this happens a lot in our life. We think we know enough about a subject to provide an answer or have an opinion, but we don't know that we have a gap in our knowledge that prevents us from fully understanding the situation. Then, once we do know a lot about a subject, we start to doubt our own abilities and get frustrated with people that don't know what we know. We also think that everyone else obviously knows as much about the subject as I do, so the frustration sets in.

I think you could apply this to our country's situation right now. Everyone has an opinion about North Korea, or Steve Bannon, or why police seem to be heavy handed in certain situations. We don't fully understand the complex issues because we don't know all aspects of the problem, so we are overconfident in our opinions about them. I always think to myself when I hear a news story, if there is an obvious answer to why something happened, I probably don't fully understand the situation.

I have thought a lot about what Jake shared this wk and definitely will make me think even more!
----------------

Pediatric (Peds) Neurosurgery,  Heart Rhytm -
There is plenty else going on, I'll do a better update on the rest soon but I exchanged emails with Dr.Guillaume , the Peds Nrsgn I saw while in Mn a few wks ago and he had just gotten back in to the ofc on Weds last wk. He will try to set up a Conf call with  Dr.Bragg and Dr.Iskandar though apparently he and Dr.Iskandar must have been at the same conf (curious Dr.Iskandar didn't say anything about this when I saw him last wk? He just asked me to let him know this wk if I hadn't heard anything and he would set up the Conf call btwn the 3 of them. Odd!?

Dr.Kovach  (Heart Rhythm) also emailed me re possibly starting the Amiodarone which non of us are to eager to do but he's talked to Dr.Earing my Cardiologist and they feel doing a series of baseline lab work and apt 1st makes the most sense. I go to CA next wk for 3 days for the initial Humira study and then again later in Sept + other things going on so I'm guessing will be a few wks till we get this scheduled.
                                                         Bay Beach, last wk

                                 
                    
Last but probably best is our 1st meeting of the year for Sunday School occurs tmrw night (8/21) w/our new Pastor. I am looking fwd! =)
Thanks for stopping by,

Erica

Another opinion; Peds Neurosurgeon UofMn. Nat'l MPS Society Conf.

                                                              AWARENESS

Last weeks Appt with the Pediatric (Peds) Neurosurgeon at UofMn seemed to go ok. He’d clearly done some research through my med records, looked at imaging they had requested and remembered the various conversations we’d had over the past 8 or so yrs. As a complex Pt these are always nice to hear from new drs and saves a fair bit of trying to explain!

He made many of the same observations Dr.Bragg has over the years about my skull bone being overly thickened (he commented how noticeable it was from the imaging he had looked at), how he also feels that likely has played in to our not being able to keep the shunts working well for long periods and how my brain wouldn’t have the capacity to adjust for even mild pressure changes be it weather, elevators, anything that people with normal skull compliance their bodies can automatically  adapt to.
He brought up and  commented about  cranial vault expansion (but its not technically that procedure, in my case he seems to think like Dr.Bragg has that IF it would work (he said he wasn’t sure it would be the best approach for my case and is a pretty big surgery) this could create more room in my head for the spinal fluid (CSF) to circulate and thus help the shunts work better by creating more room.

He did make the comment that my ventricles are not slit like (not slit ventricle syndrome) though I am not 100% sure what imaging he was looking at (older imaging I assume) and would be curious actually what my ventricles look like now. They do not routintely get very big but when in shunt failure my vents do enlarge some these days.

I had known this before the Appt but he is going to be out of the ofc for about 1.5wks but said he wants to set up either a 3 way call or Video Conference with Drs.Bragg and Iskandar to discuss my case and hopefully they can come up with a treatment plan. I mean this in the sincerest way, but I pray to God, sincerely they are able. I have had about enough of the headaches (pressure) but as much as that even more sick of the low-to-mid back pain, it  literally feels like a searing pain. I can only pray.
 It did seem from his comments that he is interested in staying a part of my Team or if needed potentially even doing any surgery but I am not completely sure on this. He seemed very passionate about trying to help figure out a longer term plan.
As my Mom said when I’d talked to her the other day after I was back from Mn, in as many words ‘It would be nice if someobody would do something to give you some relief while they figure out a longer term plan’. I couldn’t have agreed with her more!

Re the Lumbar-to-Thoracic (LP) Shunt he made the observation that he also like Dr.Bragg and like my 1^st Neurosurgeon with Lumbar Shunts places the valve and reservoir (valve controls how much CSF gets through, Reservoir is used much like my Port-a-Cath for my weekly infusion to draw off CSF (Spinal fluid) when needed and/or a direct way to test how the shunt is functioning). Anyways he commented he never places these on low back as mine sits now and positions them along the shunt but to sit on a Pts side. – Yet another Nrsgn who does not do as mine currently sits. He seemed to feel the current placement could have some to do with my significant low back pain.
Now if only someone would do something about it!?!?!? I can only hope and am certainly praying..

 I actually get that I likely look very good to most people and most can't/don't know me well enough to see the subtle ways when I don't feel good that I am different but those who know me best know and can see it. Oh how I just wish to feel better! For the shunts to work better again!!! I reallllyyyy miss Dr.Bragg, atleast she was always thinking and trying to figure something out to help. I pray Dr.Guillaume will be able to set up the 3 way call with Dr.Bragg and Dr.Iskandar and something comes of it and SOON!!!!! 


                                                            Peds Neurosurgeon (Dr.Guillaume)

                                                Friends and Fun - National MPS Society Conf.

                 NATIONAL MPS SOCIETY CONFERENCE

Re the Conf. I think I can say this was the closest Conf I've been to yet that's had at least a little focus on Adult issues though mostly/mainly young adult issues/transitioning. STILL it's slow progress!
 The Adult Committee with the MPS Society has been looking for new members and many Pharma/BoiTech and Families asked me at the Conf why I am not on it/why I wasn't talking on the Adult panel so I guess i'll try to get involved. I definitely have enough other things going on  but I do think I can help and that I have a good voice (experience) as an somewhat older adult with these disorders so I did send in an email about possibly joining this and adding another older adult voice (kinda funny b/c really I'm not actually that old but in our MPS/ML world 34 is kinda old-older). =)

I think to me the best part of the Conf though some people don't apparently like that they are there is talking to and meeting some of the new/newer Pharma/Biotech folks and getting to learn about the various new research coming down the pipeline. There are several studies either going on or starting (Armagen, with Aldurazyme re-formulated to cross the BBB, Sangamo just recently started a study looking at a gene editing (sort of like gene therapy) study. RegeneX Bio is another newer company I talked to a bit and who wants to do a Conf call next wk (they and I meant to meet up to talk more indepth at the Conf but ran out of time)  to get my opinion or thoughts I guess on their study and to share some about what they are working on.
The Sangamo and Armagen folks also talked to me and shared info about their studies. Dr.Whitley with Sangamo had a copy of the Sangamo gene editing consent form he had given to me with his # on (he's been trying to recruit me to this for awhile, I had given him quite a bit of personal data to use when he presented before the FDA for study approval).
 I unfortunately/fortunately (however you want to look at it) am already signed and consented for Dr.Polgreen's Humira (anti-inflammatory) study.

Perhaps at some point i'll share a overview of what each of the studies are about. Matthias, the CEO from Armagen (whom I've met before, VERY nice guy!) also stopped for a bit as he was leaving the Conf to give me his card and chat a bit. He emailed me again last night and I have to say seems like a genuinely caring type of person.

Honestly I feel like the HUGE take away from this Conf. is that SOOOO MANY Biotech, Pharma and Researchers are working on my MPS I  and though it seems as though everything takes forever and a day there is more HOPE!!!! Potential treatments that may help some of my brain (especially) and heart and spine issues for which Aldurazyme is currently not. Atleast I have a treatment but it's kind of exciting stuff!
Almost above all I pray some of the other disorders that do not yet have treatment will be able to get treatments sooner than later. I am grateful I atleast I have something even if it is imperfect.

I feel like there is so much to say about this Conf and yet I've written a reealllyyy long post already. So instead i'll try to update again when I know anything re the Neurosrugery  stuff. If you believe in prayer please pray we can sort something out!

Dr.Polgreen, Humira Study
Lastly I just heard from Dr.Polgreen's study coord. this morning, they are needing to do 2 separate initial visits to CA for the study (we where trying to avoid this) which kind of does bite but this will take place Aug. 31 and Sept 13th if I remember the dates right, right off hand. That is kinda going to stink (I don't typically mind flying just that with the headaches/pressure it's harder and all around flying is exhausting!). Is what it is I guess though to try and get something to potentially help some of the areas. 

Will try yo update soon. Thanks for stopping by,

Erica