Saturday, September 9, 2017

Hurry, hurry and wait... Communication.. Few updates

What  a week?!?

Re the TIA (Mini Stroke) issue thankfully no one seems to overly worried other than the ER folks. I will say for about the 9-hundreth time I really missed Dr.Bragg this wk (I was able to ask her advice which I appreciated) as she'd have been really up on what had went on and was going on + would make sure the things recommended actually got done in a timely manner.
This said the Humira study 2nd (enrollment) trip is on hold for now till I see the Neurology-Stroke clinic Provider in a couple wks  (25th) and hopefully they will sign off on my going back to normal flying.

Cardiology which was the other "Urgent" thing I needed to get done and get an Echo done they never bothered it seems to pass that info to my Cardiology Team and  Cardiology initially wasn't going to do an Echo at all (I really don't care 1 way or another) but the Neurology clinic literally called no less than 3 times in a several day period asking if I had gotten it done yet and stating they where going to order it to be done at UW if it was not ordered by my Team.
I passed all these messages along to the Cardiology Nurse (who I really like) and she in turn talked to my Cardiologist + he ordered an Echo  + wants a lab test to check dosing levels for the Lovenox (Enoxaperin) injection blood thinner I take. I'll do both of those mid-next wk (I'm sure I could have done these sooner but next wk is what worked for my schedule and seems no rush). They'll let me know the result. I just asked if they could  include a note or something stating if they felt things where fine from the heart aspect so Neurology would be happy. Trying to cross all my T's and dot all I's before the Neurology-stroke Appt!!

In the mean time I seen Neurosurgery at AFCH (It was a UW kind of week I guess) and Dr.Iskandar and I are going ahead with the shunt surgery (LP Shunt). He'll explore my lumbar (low back) and if an acceptable, less scar filled area is available he'll move the catheter down (shunt catheter tunnels up to my thoracic spine now). If this is all to scar filled (my back is a mess and already had several decompressions/laminectomy surgeries for shunts) then  he said he would at least try moving the catheter a down a small bit in my mid-back. I am praying for low back placement!!!! We know this is an area I do well with, with the LP Shunt. Unfortunately he's not willing to move the valve/reservoir (valve controls how  much Spinal fluid or CSF  drains, the reservoir is used for tapping the shunt to check function or infections/how well the shunt is working) but atleast it is something and hopefully should help some!!!
                     Lumbar Peritoneal Shunt placement (same general placement as mine)
This surgery is scheduled for Sept 26th, the Neurology Appt is the 25th and pre-op is this next Tues before I go to my weekly Infusion.  I am sure praying Neurology signs off and if anything gives suggestions how to prevent another TIA or works with my Cardiology Team (this is where I really miss Dr.Bragg and even my Neuro-Endocrine dr. said it the other day, Dr.Bragg would pick up the phone and talk to these Providers).


Otherwise I have a few trips for various reasons in Oct and early Nov., for the Sangamo talk/invite and to AZ (Hydro walk) so saying prayers things are sorted by then and my Team can do what seems rather impossible these days and work together to sort this stuff!
  It's not all that reassuring when 1 dr., has no clue you even where in their hospital ER, their Residents re-programmed your shunts and yet they had no idea till I asked their opinion about the whole issue that the TIA had even occurred!? Kind of the same with Cardiology - I'm glad if he's not concerned but ya know there's not much that worries me and that night I WAS worried! Anyone who knows me even remotely well knows my biggest fear has always been losing ability to drive and losing being independent!

Will update when there's something to update.
Thanks for stopping by,

Erica

PS On a side note Sunday School starts tmrw, almost seems hard to belive!  I am praying my body just plays nice and this yr I  make it more than just the 1st wk (2 wks instead, lol?) before being out potentially a week or few wks. Last year I was there the 1st day and then gone for the next 4wks or so due to surgeries but remember being so uncomfortable (was my choice to go anyways, a good friend of mine will always pick up my class if I am sick/out). I think I'm looking fwd to this starting again!

Monday, September 4, 2017

Brains are a fickle thing.. Brain, Heart.. transient attack?

I got back from CA (Humira study stuff) Fri night, unpacked, settled and back in my norm pretty quickly. I was by my Parents Sat for supper and a few hours after that, after I'd been home a little while I had a sudden onset of some really unusual symptoms which at first I didn't think to much of.
The initial symptoms quickly turned in to numbness  that quickly spread to 1 entire side of my body, thinking was really weird, trying to type a message to my Dad and then to call him (then trying to type in his # on my phone to actually call) took I bet 10mins for something that's 2nd nature. It was WEIRD!!!

Once I finally got my Dad on the phone I just asked if he'd come take me to the ER (which anyone who knows me even slightly well knows I HATE ERs). We went to the ER in BD after I'd called the on-call Adult Congenital Cardiologist who advised getting to an ER and getting brain imaging done + keep them updated.
 In the interim I had posted something on fb as well about the symptoms and a few of my drs., saw the message + where private messaging me to see what was going on and to make sure I was in fact going to the ER and that the ER was going to do imaging.

                                
While waiting for my Dad I managed to 1/2 walk, 1/2  drag myself from my living room to my bedroom (my Apt is not huge but this was quite the process)  to take an early dose of the baby aspirin I take every morning and my Lovenox (blood thinner) dose early + pack a few things just in case.

Once we had gotten to the local ER they did a CT Scan, labs and then we sat there for what felt like eons  (6hrs I believe, the Ambulance guys where a but taken aback by this long delay actually in the transfer though maybe there was a reason I don't really know) till the ER made the decision to transfer  and then eventually decided to transfer to UW.
      UW ER did more lab work, an Brain and other MRI with and without contrast and few other basic tests but the basic thinking is I probably had a transient ischemic attack  (basically a stroke that passes I guess) and since I am already on blood thinners (Lovenox) and Baby Aspirin they would let me go home. The Neurology folks at UW gave me the name of a Neurology Provider I need to see this wk at UWwho fups on all ER stroke Pts and I am  to also have my Cardiology Team do an Echo + go from there.
   Fun, fun, FUN!?!? (Not so much though glad the symptoms and issue passed, even I was a bit worried!!!!)
                                                                                      
I left a message for my Cardiology Team (it's Labor day today) and will call the Neurology office tomorrow. I do see my Peds Neurosurgery Provider later this wk so a lot to follow up on but thankfully most things where just transient and did not last!!!!
If I had to guess given Dr.Earing (Cardiology) mentioned concerns he had about my just being on the Lovenox and not Coumadin and he had if I remember right felt there was a higher risk for blood clots I'd guess he'd opt to add back the (Coumadin)  but also keep the Lovenox injections for some time (or atleast till we got the shunt stuff hopefully sorted).

Neurosurgery Resident - the shunts are a pain in the arsh to re-program and adter a bit of re-trying he not only listened (a good thing!!) he got them!     
Stay tuned I guess?
Prayers are always appreciated! I fully believe it is because of God this episode and other things I have experienced or been through have not been worse. - I am grateful to know my God's forgiveness and love!

Erica

Tuesday, August 29, 2017

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

Monday, August 28, 2017

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

Sunday, August 20, 2017

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

Wednesday, August 9, 2017

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 1st 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

Monday, July 31, 2017

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


                                                         

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!

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)

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..

Erica

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 clinicaltrials.gov 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.
            
    https://clinicaltrials.gov/ct2/show/NCT03153319?term=Humira%2C+MPS+I&rank=1

  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.
                    https://www.ncbi.nlm.nih.gov/pubmed/28119823


Audiology
          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.
                          http://www.resound.com/en-US/hearing-aids
              
         
                                               

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,

Erica

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!

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