Happy (almost) New Year! - Humira Study, Pharma/MPS, etc

                                 

Hard to believe but it is just 31 hrs away from 2018 - this year hasn't been a bad one nor particularly great (but really it hasn't been to bad) - I guess I am ready for a new year though. =) 
Thankfully the past few months up till a few wks ago the headaches had been really good (and even now they aren't as bad as sometimes, just not as great as they where either). I think or am pretty sure the lumbar-CSF (Spinal fluid) leak is healed up and that makes all the difference in how I feel.
I don't really know what I'd hope for 2018. Maybe easier times with my Peds Neurosurgeon? Would be nice when I do eventually call them if my Neurosurgeon could just listen and try to help vs how it always is.
Otherwise just that  day to day life stays decent and those I care for stay well also.
                                   Dr.Polgreen and I, CA   

      
Humira Study:
3months or so after the TIA (Mini Stroke) occurred and I had postponed going back out to CA to do the final Humira (Dr.Polgreen) Study enrollment I made the trip, did the final needed tests and enrollment process and 1st injection is done last wk (right before Christmas, don't ask me what I was thinking!) 
 I /we won't know for about 3-4months (week 15 if I remember right) whether I am on actual Humira injection now or whether I am getting placebo injection every 2wks but alas atleast I am enrolled and it's started now!
 Equally awesome is the Foundation I work for has agreed to fund 3 more Patients for this study so that is awesome to. There is actually fairly wide interest among families though it seems actual enrollments has been wider amongst the more severe-MPS I Hurler Families than it has been amongst Pts with my more attenuated form. That said for pain, inflammation and our arthritis like issues this is a drug/study worth considering! 

Had it not have been for my cardiac issues I was offered a spot in Dr.Polgreen's original, 1st phase of the  study but at the time I was dealing with heart and shunt issues and surgeries so declined/postponed enrollement till this last summer.

Heart wise / Amiodarone:
This has actually been relatively okay. Still plenty of the skipped/feels like my hearts tripping over itself beats but at the same time they occur less frequently (50% improvement?) and between this and the Ivabradine and some supplement doses + the other Heart meds I take it seems to maybe be helping in general a few things. I won't complain when it's relatively better. Not perfect and plenty of symptoms yet but some is better than no improvement!?!

 NIH - Sangamo :
I honestly can't even remember the last time I updated or posted here but has been a relatively quiet (in some ways anyways!) few months and yet really busy to. I con't to work with Sangamo, the Biotech Company (doing the gene editing studies for MPS I and II)  I spoke at in San Francisco a few months back on/off.
The Sangamo Patient Rep I mostly work with reached out to me a week or so ago to see if I would be interested in/willing to work with them in March for a Rare Disease Day related event at the NIH, hosted by the NIH for a Rare Disease Day Mtng. The mtng is actually 3/1, so not actually on Rare Disease Day.
I agreed to doing this if a few things work out, namely that costs are covered by the mtng sponsorts (Sangamo is figuring that stuff out). Honestly if it happens it will be neat! I mean how cool would it be to be a Patient voice speaking at the NIH (Nat'l Institute of Health) to Pharma and Gov't/Research people!?!?!
On the flip side if it doesn't happen I am genuinely okay with that to. Traveling these days is ALOT more exhausting then it ever used to be so while I enjoy it when I go to mtngs and Conferences I am also just as happy to be home in my own space! Whatever be God's will, will happen. =) I just have to say though it's an awesome honor that this Biotech/Pharma company asked me to be their Patient speaker for the Gene editing part and seems to want to keep working together!
I am working some with Regen-X Bio, another Gene therapy Company working on a treatment for my MPS I and have a Conf call set with them for later this next wk as they wanted to catch up and update me on what they are working on. Pretty neat. =)

I never do anything I do for any reason other than I want to help better  educate Families and Providers/Researchers (in the MPS Community and in my own care) and I genuinely like helping Pharma/Industry better work to meet our adult-MPS Pt. needs. But in the process of doing this I will admit it is phenomenally awesome to (it seems) be making such a impact in so many areas (both to Families and to Pharma/Industry)!

Running for Rare Team - 2018 Partner
Running for Rare - NORD Partnership
This will be my 6th yr partnered on the Team? My runner this year works at Genzyme and knew a very little about MPS I as a whole when she 1st emailed me so it has been fun emailing and texting with her, getting to know her. My runner 2 yrs ago, Sarah and I had planned to partner up again this yr but Sarah ended up not being able to do the marathon so I will to this with Sarah and especially her daughter Madi on my mind that wknd. Madi has CCHS a sleep/ventilation disorder (I can't do explaining it justice but Madi requires a breathing tube and must sleep with that attached to a machine at all times or she will (If I am remembering this right) suffocate from CO2 build up.
I am REALLY glad my partner this year Cheri seems pretty fun and really interested in learning and how can she raise awareness for my MPS so I do think it will be a good year! Should be a fun time leading up to Boston!

I'll sign off on that note. - Thanks for stopping by,
Happy (almost!) New Year!

Erica

Sunday School Christmas Party
                                        

Happy Thanksgiving...Restarting (oral) blood thinner, various

  A bit late but Happy Thanksgiving! I will admit I've struggled a bit this holiday season to get excited for the Holidays (I am looking fwd to some parts just struggling with other things) but no less I am grateful for all I have and all who are part of my life.

Things con't to go pretty well with the headaches - I honestly think this is 1 of the longest periods I've had in a long while (perhaps some yrs) where I've not had pretty bad headaches. I am so so glad Dr.Bragg has stayed part of my care and my life and that she con't to work with Dr.Iskandar (my Neurosurgeon since she moved, her former Partner) - together they have made a difference especially this last surgery though really I think this relief is almost an unintended benefit of surgery (no doubt moving the shunt catheter helped significantly, that radiating pain is 90% better and only occurs now on much rare occasion and to a much lesser degree).
Best perhaps of all (though don't get me wrong that relief is fantastic to!) but the CSF (spinal fluid) leak that occurred after this last surgery which continues to a small (or varying really) degree is what truly relieves my headaches. The 2 shunts alone make a significant difference in my headaches but I rarely feel truly great. With the additional CSF leak it is truly like a whole different situation and WONDERFUL!!!!

Heart rate wise, oh this heart rate of mine. I swear between the shunts and Heart which could drive a person nuttier!? I suppose that depends on the day! The Amiodarone which I've been on for I want to say about  a month now but I honestly can't remember has been going okay but doesn't seem to be working any to well. Some times heart rhythm and rate seem okay then other times it's just pure nuttiness and I am in now way being dramatic about this. I mean literally excess-long runs of skipped/extra beats (PVCs or whatever, has been different than even my normal, not normal) beats. so less than awesome.
I know Dr.Earing (Cardiology) is/was concerned about these and if the Amiodarone doesn't work there not being another option (other than going back to Cath lab to re-look at heart and rhythm more closely but I know Dr.Kovach is really reluctant to do that b/c of the 2 heart valves and (if I remember right) this rhythm is coming from an area where the new mechanical heart valves are or it may have been that this area + scar tissue would complicate any procedure. I can't remember for sure just that he is less than enthused to go that route. I completely get that! Hopefully Dr.Kovach has some idea or we could figure something out.
These rhythms are downright exhausting not to mention secondary symptoms it causes. Can only pray - pray for God to give my Providers guidance and for myself con't to ride it all out! God is good so as frustrating as it all can be (and oh it is at times I'd be lying if I said otherwise) I know ultimately 1 step at a time... AND will enjoy this shunt-headache relief however long it decides it is going to be good! Atleast 1 issue is being nice!!!!

                                                           Re-starting  Coumadin

I restarted the Coumadin today (oral blood thinner) and per Cardiology test INR (lab test to measure how thin blood is) Tues at Infusion (I LOVE that my PCP works with my Cardiology Team and will order the test for them + has said she'll be sure the result gets faxed to them). So i'll be able to have this drawn right at infusion similar to how I used to at CHW/FMLH. As in the past although Dr.Simpson will fax Cardiology the results they've asked I call and let them know so they can call back the same day or soon after with any changes they may want to make or when to test again. Due to our issues with this in the past and the concern of the TIA (mini stroke) that occurred a few months back + Cardiology wanting to prevent that recurring as much as possible (or atleast minimize risk from heart-blood clot risk). Never to dull but should be ok and thankfully my Team is all working together! I also have to say the 'Care Everywhere' system which allows my Providers at 1 health site to access records for Providers at another site is really, really nice!
Thanks for stopping by,

Erica

  A bit late but Happy Thanksgiving! I will admit I've struggled a bit this holiday season to get excited for the Holidays (I am looking fwd to some parts just struggling with other things) but no less I am grateful for all I have and all who are part of my life.

Things con't to go pretty well with the headaches - I honestly think this is 1 of the longest periods I've had in a long while (perhaps some yrs) where I've not had pretty bad headaches. I am so so glad Dr.Bragg has stayed part of my care and my life and that she con't to work with Dr.Iskandar (my Neurosurgeon since she moved, her former Partner) - together they have made a difference especially this last surgery though really I think this relief is almost an unintended benefit of surgery (no doubt moving the shunt catheter helped significantly, that radiating pain is 90% better and only occurs now on much rare occasion and to a much lesser degree).
Best perhaps of all (though don't get me wrong that relief is fantastic to!) But the CSF (spinal fluid) leak that occurred after this last surgery which continues to a small (or varying really) degree is what truly relieves my headaches. The 2 shunts alone make a significant difference in my headaches but I rarely feel truly great. With the additional CSF leak it is truly like a whole different situation and WONDERFUL!!!!

Heart rate wise oh this heart rate of mine. I swear between the shunts and Heart which could drive a person nuttier!? I suppose that depends on the day! The Amiodarone which

Nothing major.. Nothing to new.. AZ trip (my favorite Nrsgn), Heart F/up, etc.

I've been particularly bad about writing here lately so will try to do a atleast semi update of a few things I've been up to. - I still con't to feel pretty good headache wise which is some kind of miracle given the LP Shunt surgery was the end of Sept and it's now late Nov and I still feel pretty good. A small CSF leak cont's to play a HUGE part in that but I am not complaining!!!

I have to admit I had not entirely understood this/why it makes such a difference in the past but a few wks back was talking to Dr.Bragg (my former Nrsgn, she cont's to stay in touch and involved in my care). Anyways she explained to me that with my skull thickness/non-compliant skull even small increases in pressure which most people are able to adapt to with my body (my skull) does not have the extra capacity (space) to expand nor can it expand due to stiffness and so even very small increases in spinal fluid (CSF) creates headaches, nausea and can lead to throwing up.
Interesting and completely made sense + I now get why Dr.Bragg advocated so hard I consider the cranial vault expansion (bone thinning of skull bone) albeit still sounds freakish to me and since it wouldn't be with her I'd be even more hesitant. Still interesting and good to understand it better though!
                                                                    

Cardiology 6mo Appt:
This went okay, certainly not a bad appt. He cont's to support my trying the Humira study (asked if I was still doing this, what was going on with it). I go out next mo to do actual enrollment and start the drug (whether actual Humira or placebo initially) but I had opted to put off the actual enrollment visit after the TIA/Mini-stroke I had about 2mo ago till next month. I am glad I waited honestly mostly b/c I  ended up going to San Fran, CA to speak at Sangamo and then the week after that speaking trip I made the trip to AZ (for which I got to see Dr.Bragg) -> That trip seeing her alone was worth the exhausting nature of it though!
Anyways so Cardiology Appt we are leaving things the way they are. I have been fiddling with my Lasix dose, many days just doing 20mgs 1-2 times a day other than infusion days wheres before I was on 60mgs 2x a day except on the occasion I'd skip a dose.
I am having some intermittent breathing issues but this is intermittent and I go back up on the Lasix as I see fit. I HATE that med (oh heck I hate the Spironolactone to which is a longer acting version of the Lasix!)
Blood Thinner re-trial - PCP
We are going to trial re-starting the Coumadin which is a safer blood thinner than is the Lovenox injection I give myself 2x a day (safer only in the context it's easier to monitor levels) but while we tweak and monitor the dosing initially for a while i'll also have to stay on the Lovenox injections. Initially my Cardiologist wants lab work done every 4days after I start and expects we'll have to go up on the dosing (they'll start at 5mgs) but I am semi confident we'll have a little easier time compared to 13mo ago when we where dealing with shunt issues.
This said just have to pray the CSF leak cont's and the shunts keep doing their thing so we don't have to start the INR mess all over again! Dr.E said he would just go back to Lovenox alone again if we ended up having issues at any point as he doesn't want dosing to get as high as the level of Coumadin I was on just over a year ago. CRAZY STUFF!

As far as testing goes we are waiting to start the actual med till next Fri (day after Thanksgiving) so there are Providers around at Cardiology to monitor the blood levels and give feedback. I am ok with that as it gives a little time to get a few things squared away.  My PCP has already put in a standing order at Dean for lab draws and i'll be able to get some of the lab draws while at my weekly infusion + she offered to make sure results get faxed to my Heart. Because of electronic med records i'll also leave a msg for Cardiology Nurses and they can access the results remotely via EMR. They in turn call back later the same day with if any changes in dosing are needed, when to test next, etc.

B/c this week wasn't busy enough I also had knee MRI and Neurosurgery f/up after (to have shunts re-programmed) Monday + Ortho Appt today.  The Orthopedic dr. opted to do a knee injection (wasn't as bad as I've heard some people say they are). Hopefully this might finalllyyyy calm the irritation that's been going on since the end of Sept!?!?! I am hoping!

Will try to update some time soon. Attached are a few pics from when I was in AZ, including Dr.Bragg and I. Her daughter (she's about 6mo younger than Z) who took the pics. Was sooo nice to see Dr.Bragg and to see her daughter again!!!! So grateful she took the time to come pick me up and have supper + we had a funny candy store run.  I don't remember why we went there but was pretty funny!. Couldn't ask for a nicer Neurosurgeon! I will always be grateful for her and certainly always miss her!!!
Thanks for stopping by,
Erica

Speaking @ Sangamo, Headed to Phoenix, Hydro Walk

The trip to Sangamo last week was good, a little stressful on my part although that having nothing to do with the actual trip there. I think my talk overall went really well and seemed to raise many interesting questions and interesting points. Afterwards 1 of the Sangamo MD-Researchers came up to me to ask a question and asked if anyone on my Team had ever done further testing to see why I have such moderate MPS presentation but such mild outward appearance. It was actually a really interesting conversation and I wish I could remember who it was that had asked me!

Needless as I've written here my Metabolic Genetics dr. has done the whole exome sequencing and just taking a long, long, loooong (nearly 2yrs now) time to get the results. This delay in results is due to the testing was done under a study or some special way so it doesn't cost my insur. anything but in turn the results take a lot longer due to being done when time permitted I believe (it was something along that line).
In reality whether there is or is not something else going on thankfully I've made it through the worst of surgeries i'll likely face (the Open Heart Surgeries!?) and when we do get results I think many on my Team are very curious to see what the sequencing shows.
Simply out of curiosity if I remember I plan to ask my Cardiologist about the testing overall or what his feelings are in general (he also felt there may be something else in addition to the MPS I going on) when I see him coming up.

                                        Sangamo - (Photos hanging at their headquarters)

Otherwise the CSF leak is pretty well healing up, it seems there are still some periods oddly enough where fluid forms and the only way I know is I feel better and so then I (lol, I know maybe weird) feel to see if there is a CSF bubble on my back and there usually is a small 1. Thankfully this has been occurring quite a bit so I have more periods where I feel good than periods where I don't and it seems if I get up and move around aggravate my back a little it aggravates the area causing the leak to open up or increase or something. Whatever it is I'm not complaining as I am flying out to AZ (Phoeniz, to Hydrocephalus Walk) Fri this week and volunteering at that most of Sat. Will be GREAT to feel better for it!!! Also makes flying much more tolerable!

                                Speaking at Sangamo, Sangamo Reps, Halloween and new Car 

                                                             
On a completely separate note my car has had some issues lately and have had to do quite a bit of repairs on it (my car has over 200,000 miles, I drive a lot) + this last wk was really being weird. That amongst a few other things was a nice source of stress while I was in CA! Anyways I've never really known what my credit score was and honestly thought it was probably pretty bad (I can't really say why I would think this I do pay all my bills on time, have 2 store credit cards that anytime I use them I pay the charges immediately after I use the cards (so I can get the discounts! =D ) and often just pay the charges in full right away. Well anyways so I couldn't get there so my Dad went to the bank and talked to Loan guy he works with and that guy called me + I apparently have really good (excellent apparently!) credit rating. So I went searching for a new(er) car. I didn't want to buy brand spanking new - I like buying a different car every 4 or so yrs but found a 2011 Chevy Aveo with really good mileage, very small, great MPG (what I ALWAYS look for b/c I drive a insane amount) and is cute. I go tmrw (Thurs) to sign the papers for the loan but have about a 1/3 of the total cost I am paying down so my loan payment every mo won't be bad (and the quicker I pay it off the quicker I can drop full coverage insur!). I like my car now but I guess will be fun to get something new?

Will update soon, maybe sometime next wk after I get back from AZ, Dr.Bragg is slated to speak at this Walk I am going to although she messaged to let me know she is on call so she's hoping she'll still be able to come. We are going to try and see each other 1 way or another. I am hoping! I will always be grateful to her, probably especially so b/c not only she took on my care and never gave up but now she's remained steadfast in helping me this past year despite she's in AZ now. She's amazing and I am grateful to count her on my Team yet even if from a distance!
There are other symptoms going on but another day, another update and it isn't pressure so as I wrote above I am enjoying that however long it lasts!
Thanks for stopping by,

Erica

Spinal Fluid leak cont's, F/up with Team. Speaking at SF, CA 26th

I've had a pretty big CSF leak now for about 2 or 3 wks after this last shunt revision though the leak is slowly decreasing in size but initially it was really good  which started about a wk after surgery which in and of itself was a little unusual (the late onsent of the leak) but I sure wasn't complaining! I really started to feel good and that is always such a huge blessing!!! I think the leak is slowly getting smaller which makes me sad (very sad!) but for now this extra fluid sure is a night and day difference in how I feel! I feel like a actual human being! For the most part I don't have to think much about things that usually these days make my brain feel all fuzzy and mornings are just like normal and I just don't generally feel as tired!! SOOOO nice!!! I know I say this every time I have a spinal fluid leak in my spine but I wish so bad this would last or there where a permanent way to recreate! I can wish, right!?

With the CSF Leak, the Shunts may be working but my body still wants to drain more CSF; and I guess will find any way it can which it did in this case about a wk after the last shunt revision as mentioned above which really the surgery  wasn't a major surgery (the lumbar shunt catheter was pulled down from thoracic (mid) spine to sit back in lumbar area. Perhaps b/c of all the scar tissue and because my body wants to drain excess CSF it doesn't care and will find a way when there's even a slight breach (of sorts) in the skin/dura (I think that's the area but I am not sure, where they go through for surgery) in spine that finds a way to create a temporary Cerebral Spinal Fluid (CSF) Leak.
I was talking to Dr.Bragg some about this all, asking her questions to better understand why the Spinal fluid leaks and External drains work so much better than do just the shunts alone and she explained it that with just the shunts they go directly in to the ventricle in the brain and can only drain what spinal fluid is in the ventricles (which in my case are very small so not a lot of CSF available to drain).
 With CSF leaks these are likely draining spinal fluid from around my brain/skull, my body finds alternate pathways to get fluid out and thus my to thick of a skull/not enough room in my head for my brain (essentially) causes headaches and symptoms except when the fluid finds a alternate way to drain! These in turn act a lot  like with the CSF (Spinal fluid) leaks and External drains. Weirdly cool! So wish there where a way to permanently recreate this! It does make me better understand why Dr.Bragg really advocated doing the skull-bone  thinning surgery (in this surgery they thin the bone of the skull so it can in theory expand with even slight changes in pressure like people without Hydrocephalus/Complex Hydrocephalus are able to do. That said there's no guarantee a surgery like this would work and has risks and my Nrsgn now is much more conservative so he wants more data first.

I met with  my Peds Neurosurgeon today to evaluate having the spine (low back/ lumbar) stitches removed (they left them in last wk b/c of the CSF leak, concern if the incision would break open under the pressure) and to discuss surgery/further plans.
As off now he is pleased (I think) surgery went well and we're looking at further options. The surgery did 75% help the radiating pain down my R side and that also went up my back. I get it some but no where near what it was and THAT is soooo nice!!!!
As well he is looking in to a device that's approved overseas but not yet approved here in the U.S so he is looking in to it for myself and a few other of his complex Pts to get a better (but blinded) idea of what my pressures are (ya, I know b/c we don't have a lot of data on this already).
I am not sure and I don't think there is but would be so great if there where a way to permanently recreate thee Spinal fluid leaks so the extra CSF to get out around the 2 shunts (which drain from ventricle in my brain to pleural (lung) space and from lumbar (low back) to peritoneal (abdomen essentially) to drain CSF (Spinal fluid).
If I understand It correctly with these CSF leaks the spinal fluid probably isn't even coming from my ventricles (b/c they are so small and there is only limited fluid that can drain) but instead the fluid is likely draining from around my skull/brain and I feel so good b/c this frees up space in my very tight, very narrowed space in my head and thus really good headache relief. B/c I have such a thickened and non-compliant skull ( can't adjust for even small changes in pressure like a normal person can even many people who are shunted) these CSF leaks offer  a alternate way for CSF (Spinal fluid) to leak out and create room in my head. It's AWESOME while they last!
I have to admit this extra room between skull and brain when the CSF leaks is a concept I didn't totally get up till this time and 1 of my very awesome drs took the time to really explain it to me.

Now if only there where a way, even if a unconventional way to recreate this! Alas we'll try to get the device I mentioned above approved and we'll go from there.

 I guess i'll really enjoy the pressure relief while it lasts and pray that by some miracle my Neurosurgeon would really get it this time that I'm not just crazy, that I'm not making this up that  even his own Team could see the difference not to mention 3 of my own Providers last wk made comments they could see a noticeable difference b/c the pressure was off. The same comments I hear everytime the shunts are either working really well or I have these CSF leaks.

Heart Rhythm Appt - Symptoms -
 I saw my Heart Rhythm dr last week and he and my Cardiologist agreed it's reasonable to start a trial of the med Amiodarone which is a Heart Rhythm med (can have plenty of side effects) so we are doing this with some monitoring. I see my Cardiologist anyways next months so we'll trial it and monitor as we go along. Initially I start at a higher dose and then after a week I back down to just once a day.
   
                           Speaking at this Pharma Oct 26th, San Fran, CA

Sangamo Biotech/Pharma (MPS I Research Co.)

I go next week to San Francisco based Sangamo Pharma (Biotech Company) to give a talk on living with MPS I. I am currently putting a powerpoint together for this. My intended topic is 'How did I get here and where do I go from here
 w/a focus on training more drs (especially Peds/Adult drs), arming Pts w/their care (where possible) and team effort (Patients, Providers, Pharma). I am hoping to finish my powerpoint tomorrow at ERT (Infusion) but we'll see!

Knee:
This R knee issue is still going on, maybe getting a little better? Sometimes I think so then other times doesn't seem much like it. It's been since around labor day I think (or when I first went out to CA for the initial enrollment portion for the Humira study). As  a side note they are holding my spot and I am planning to go out for the last part of enrollment the end of Nov., we're figuring dates out yet.
Thanks for stopping by, will try to update if nothing else after the SF trip mid-next wk.

Erica

#45 ? - LP Shunt revised; is it good? Hard to say

I got out of UW Fri - a relatively short stay is always nice albeit even nicer when your leaving and your head feels good. Mine does not and have been super tired (I did not sleep well while in-pt although thankfully I did have a recliner to sit/sleep in which helped a little).
Dr.Iskandar (Neurosurgeon) when he came by Fri asked why I was sleeping in the recliner and do I always do that (kind of goes to show how little what we say as Patients gets through to some drs, I don't think he does it on purpose but I've told him several times I sleep in my recliner at home b/c of the headaches and shunts drain better when not laying down). Anyways it seemed to really occur to him that it might be an issue for me (imagine that, I not be lying or something?) I doubt still it will change anything though, he just doesn't get it.

Symptoms since surgery have been really frustratingly not great. The headaches (pressure) is still there, vision issues with certain things still there - that improves as the day goes on though doesn't seem to entirely go away - I am hoping with a few more days maybe this will be better. It seems today as bad as this morning was (it's bad when I have to take triple dose of pain med for actual pain (wish it touched the headaches/pressure!) - I am hoping my Physical Therapist can work some of his magic on my shoulder/back stuff (surgery related, I'm not sure it's actually from the shunt but don't really know yet).
As the shunt seems to drain it helps the headache as is good but then I get this very intermittent, very sudden wave of lightheaded feeling - super odd and hopefully goes away to! It literally makes me feel like I am sitting on shifty sand vs on a solid surface (lol, not sure if that makes sense) and then my head just feels buzzy/off for a bit till it settles again after awhile, as if the pressure drains and then adjusts. VERY, very weird!
 It stinks though how with certain shunt placements you feel fantastic from day 1, particularly when brand new systems are placed (not that I want a whole big surgery its just that its a common sentiment I hear especially in us MPS Pts. we often feel so much better with new shunt systems then we do when parts of shunt are replaced). Just another odd thing some of us have I guess.

  Low-to-mid back pain is pretty intense though moving the catheter atleast so far has seemed to help the pain related to that down my R side  some. I truly think and probably am always going to think that the bigger issue is with the valve and reservoir placement on my back and frankly will probably always be mad at myself for backing down about this last Jan (I think that's when it was). I don't blame anyone else but I should not have let myself be talked in to this. I knew and know my body but sometimes you just feel so frustrated and worn down and that is what I felt that morning in pre-op after Dr.Iskandar and I had already agreed to move these back.
I really don't care what anyone says I know my body and for that matter there must be a reason none of the Neurosurgeons before him placed these devices like this. Frustrating to go from someone you felt like listened to someone that thinks they are listening but they really aren't.

As far as surgery they did Neuro monitoring during (someone from that Team had stopped by in pre-op Tues morning to introduce themselves and say what they would be doing). B/c of TIA (mini stroke) a few weeks ago and a heart med I take for heart muscle function (Lisinopril) they  also placed a Arterial line (An A-line gives more direct, better measurements of pressures between heart and body I guess)  which I really don't remember. Biggest thing is everything seems to have went smooth during surgery. And even if I don't feel great yet I am glad for that (it having went smooth)! I know there was a fair amount of concern in pre-op that morning between the Anesthesia doc and my Neurosurgeon although I did not feel the same concern they did.

Surgery wise Dr.Iskandar said he didn't have to do as much as he thought he might as the LP Shunt was draining and contrary to what he had thought the catheter actually was already started in (led in) to my Lumbar Spine (low back) so he just had to pull it down some from thoracic spine. He wasn't or wouldn't have been able to do anything with low back scarring otherwise which is a bummer (that it's gotten this bad) but I am glad atleast the catheter could be adjusted.  He had thought the catheter started in my thoracic spine though that to because of the incisions and work he did last Fall I was almost certain it started in lumbar (low back) area and just tunneled up to thoracic spine.

Headaches have sucked actually since surgery, they where bad enough before the surgery but now are just plain crappy. I JUST WANT TO FEEL NORMAL!!!! And to not wake up everyday and it take hours and hours to feel more normal. Like I said above today seems to be 1 of the best days so far so hopefully maybe will get better. I am sure hoping!
I when talking to Dr.Iskandar on the day of discharge once again explained why I need his help and no I don't actually want to be there repeatedly asking him to believe me and that I get it I look normal to him but he sees me for 5-10mins every so often while I have to live in my body every single day and try to function. I told him how I really didn't think it was the LP Shunt that was the cause of my headaches to begin with but was glad he atleast tried adjusting the positioning, see if that would help the leg stuff.
For whatever reason this morning and I can't really explain it but sincerely hope it's just a passing thing but my L arm is sort of numb (not like numb in I can't use it but just a painful numb) - seems to go along with as the shunt is draining. It is so freaking frustrating that these shunts can't just be good and not painful and I can't just feel good. =/ - Thankfully finishing this up later on that has passed, whether it was the extra pain meds or who knows what I'm not sure but hopefully stays gone!

I  realize I sound pesimistic or something but I hope Dr.Iskandar really takes to mind what he and I talked about and does have the conference call he talked about with Dr.Bragg and Dr.Guillaume to try and come up with an alternative idea for treatment/management.  Do I think something will change or he'd actually do something with either A. the LP Shunt valve reservoir location (these sit on my very low back, whereas they used to sit on my side connected to the LP Shunt) or the VPL Shunt without another long drawn out fight? Probably not. He just doesn't get it and doesn't get that for someone like my case, some of us with MPS we present very differently and he doesn't see it b/c again he sees me for like 5-10mins at a time. I wish it didn't have to be this frustrating though! No one should have to fight to feel better and to be believed.

Will try to update soon. Please, please say a prayer things improve each day and I can start to feel like a more human and my Neurosurgeon really consider that I'm not just making this crap up!
Thank you,

Erica

Neuro Stroke Appt; Cleared for Surgery (benefits outweight risks)

Neuro Stroke Appt today went well, I will admit I was a little nervous although not bc of the actual appt or anything they might say (what could they say?) but b/c surgery tomorrow hinged on approval from the Neuro-stroke Team.
The 2 Providers I saw (2 NPs, I really liked them) seemed to have actually done quite a bit of homework on my background, what my MPS diagnosis is and how that might play in to the TIA episode.  When in the ER/In-pt overnight the actual Neuro-Stroke MD had seen me and the 1 NP works with him + the other I believe she said was training. Anyways they feel the episode likely was a TIA which is what the ER at BD and then at UW had felt as well. This NP just said with TIA there is no real way they can "prove" this but based on symptoms that is what they all feel it was and not a stroke or something else. Likely cause from my heart valves / cardiac risk factors.
As a side note (and I read this to when learning more about these 2 things after the episode) did you know a TIA is an episode with stroke like symptoms but temporary/transient in nature - so usually or likely in my case caused by a blood clot or something from heart valves breaking off I believe she said and goes to the brain + temporarily blocks blood flow. A stroke simply (or not simply) means it's an episode that causes same symptoms but permanent damage evident on imaging (usually MRIs).

                                          

 
 Ultimately after much exams on their part and all the different Neuro tests they do, talking, sharing information, their asking a million questions (not a bad thing) they said they had been in touch with my Neurosurgeon as he had questions if surgery would be ok/safe. The consensus is that in a elective surgery they'd want to wait 9mo (wow! having something to do with risks and healing and may have been something else I'm forgetting) but b/c mine isn't really an elective surgery and benefits to this surgery out weight the risks they gave their "okay" and surgery can proceed. I do not know if there will have to be extra monitoring during surgery but am just glad surgery can go fwd!

                                                                     

The LP Shunt revision is scheduled for 10:00 arrival (pre-op check in and all that jazz) then 12:00 surgery if all runs on time. My infusion Nurse (ah I LOVE her!!!) accessed my Port today after I was done at UW so that is good to go for tmrw. I think I am packed (I bring my own comfy shirts and pants) and hopefully get a little sleep tonight - I haven't gotten much the last few nights and I don't generally sleep very good in hospitals! And to hopefully good Nurses, a bunch of my favorites took clinic jobs (I see them just won't see them on the floor and Traci, my favorite will come up to visit b/c she's kinda AWESOME! =D)
                                                                  
Will try to update in next day or so and facebook for sure.


Thanks for stopping by,

Erica

Various Appts., LP Shunt revision (Tues) 9/26

A few days till the LP Shunt revision (exploration) surgery which is set for Tues. God willing... Am praying the Neuro-Stroke Provider on Monday signs off for surgery (I don't see why they wouldn't but I suppose i'll be a bit nervous right up till I hear from that Providers mouth that she sees no problem with it!).
After the Neuro Appt i'll call my Infusion center (a few blocks away so pretty close to UW Hospital), my primary infusion Nurse was fine accessing my Port for Tues (so much easier going in with my Port accessed then trying to get the Pre-op Team or Access Team down to do it! the morning of surgery).

Otherwise a few wks ago, actually while in CA yet I had tripped/fallen (I am not the most graceful) while going up the shuttle steps to go to the Airport and bruised my R knee though at the time was uncomfortable but certainly not the worst thing I've done. That for whatever reason over the 3 or 4 wks since has only seemed to get more sore and uncomfortable and the bruising/slight swelling hasn't gone away, This has has made walking a slight annoyance!
I finally went to the local Urgent care yesterday as my Primary dr is out on maternity leave and no one else in her office could see me till next wk (which would have been fine except the surgery is Tues and the appt they offered was Thurs).
Per Urgent Care doc and Xrays he thinks it's likely just bruising, try to rest ( easier said than done!) and get it rechecked in a few days if it's still so uncomfortable. They wrapped it in an ace banadage and said to keep that on other than showering so hopefully will improve in 3-5days!?!?! Would be nice!

 Neuro Opthalmology

This past wk I seen Neuro-Opthalmology (having to do with the eyes and brain) which I suppose shouldn't really be that big a surprise but the visual field testing was pretty bad, some pretty significant issues with it. I hate these tests almost like I hate no other test (that's saying something!!!)! Mostly I hate the visual field test b/c of how you have to sit, leaning kind of fwd, neck and upper back leaning fwd (super hard, I don't have normal motion in my neck and my low to mid-back is fiercely unconfortable in almost any position these days due to the LP shunt. which is the shunt being looked at next wk).
Anyways so I apparently bombed this visual field test (my Opthalmol. showed a copy of the test vs the last ones I did a few yrs ago (I am supposed to see this dr. yearly but kept cancelling the past few yrs); even I could see the stark difference. It was pretty obvious.
Given we are having shunt issues I asked my Eye doc if that couldn't be the cause for the changes and so she agreed and instead of moving on to other tests to look more in to this that she'd first want to do she asked me to let her know in a month or so when I am healed from next wks surgery and she'll want to repeat the test before deciding if we'll need to do further investigating (testing) in to this.

 Hopefully it is in fact an issue that heals after the shunt issues get fixed. I talked to a few of my docs and they seem to think with time it could very well straighten out. Not high on my list of things to be to concerned about.
Dr.Bragg (my former Nrsgn, though she stays involved) just said I needed to mention it to Dr.Iskandar and when I emailed him he said he would look at the results. I suppose maybe bc we are only looking at the 1 shunt and say that is working and we aren't planning to look at the VPL Shunt (plan is to just explore the LP Shunt and spine on Tues) maybe there is an issue with the VPL causing these vision changes? I'm not sure and really don't know. Maybe just pressure in general can cause the changes. Either way isn't affecting anything much at the moment that I can tell anyways.

 Pre-op (for surgery) Appt:
Separate from this I had pre-op last wk which was with a Provider I'd never met (mine is out on maternity leave through next month, I've always known she's great but there are so many small ways I have missed her in regards to my care these past couple months!) - anyways the Internal Med dr. I saw seemed to have familiarized herself with my case and the whole appt went really smooth. I had made this for an hour and a 1/2 time frame before my Infusion (made for a long day, was in another part of Madison) but went well and quick! My Neuro-Endocrine dr has sent their pre/post surgery stress dosing recommendations for surgery and Cardiology's lonox/blood thinner recommendations are also on file so wasn't to hard.
Cardiology did their Echo last wk as well, as well as the Lovenox (injection blood thinner) labs and I've not heard anything re the Echo result but did see the result on MyChart and not unexpectedly (given wasn't more than 4wks before that or so we'd just done this test!) results looked pretty well the same.
The mechanical aortic valve just mild leaking or narrowing and the mechanical mitral valve mild-to-moderate narrowing. The lovenox level was within normal (range it should be for this med) level as well.
                                                       
                                    
Otherwise I feel like i'm forgetting something but I really am not sure what.
Will update when there is something to update.
Thanks for stopping by,

Erica  

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!

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

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