Tuesday, March 5, 2013

Dr.Bragg responds; her partners thoughts,. 'Happy turning 30'...

Turning 30, a big deal in the MPS world...

Well the wait is over, I heard from Dr.Bragg this morning she sent an email with a update on what she learned from talking to her colleague (a Neurologist) who is the one that helps w their complex shunt pts. His recommendations where two-fold with his one thought she said being  our on-going repeated shunt issues and trying to take a different approach (see below for part of her email though not the complete note) + how he feels the cardiac issues may be affecting our shunt issues.

"I spoke with Dr. H  His recommendation is to consider a cranial vault expansion. At this time, there is no way to improve the compliance of the brain with the current valves we have in place. He also thought that perhaps your cardiac problems might be contributing, i.e. altering the CSF pulsations because of the altered pulse waves from the valve problems. He suggested that you have your cardiac surgery and then consider a vault expansion."
She asked that I schedule an appt to come in and talk further about all of this which I have since scheduled for this thurs. I wasnt to sure this other dr would have any addt'l thoughts and wasnt sure what to expect so I guess its good they have a thought but this, really this??? It is a thought Dr.Bragg has brought up before but hesitantly and never seeming to consider it to much I think maybe bc it is more invasive? I think she also (or her NP) this last in-patient stay commented they didnt know gow the storage would affect this type of surgery or would it work. From what ive read there are different approaches and it is typically used to treat an issue called craniosynostosis which I think means the cranial sutures close prematurely or not enough space in the skull and seems to (if I read it right) happen in pts with slit ventricle syndrome or where the ventricles collapese bc of not enough space. Interestingly one of the disorders listed under this treatment info was MPS I, I guess bc of the hydrocephalus that occurs in us pts and our thickened skulls w ventricles that are unable to comply with pressure and fluid changes? Again I just say yikes!    
I also cant imagine we dont even know where i'll have valve replacement surgery done yet and I dont see Cardiology till June  or July unless I move that appt up. I cc'd Dr.Earing my Cardiologist on Dr.Bragg's email so I am kind of hoping they will talk. =/?   A friend of mine who is also an (older) adult w MPS I brought up the point should I be strongly considering having valve replacement surgery where Dr.Bragg is? Will anything have to be done w the shunts during this valve surgery? I dont know but I do know the daily coumadin will make surgeries more difficult as this acts as a blood thinner and will have to be stopped before planned surgeries for a period of time (unsure how long) which then raises the risk of clots forming in the mechanical valve. =/  I do know or believe UW is doing bovine valve replacemens via catheter in atleast aortic stenosis in pts with severe underlying medical issues that would otherwise preclude the pt from getting their valves replaced. Bovine valves are rarely used anymore in MPS I pts bc the storage from MPS re-accumulates on the "new" valve causing the same stenosis and leaking within a few years of the surgery thus requiring further valve replacement surgery a few years down the road and has occured to a few of my older MPS I friends. This said would trans-catheter valve replacement and thus avoiding open heart surgery and all it's inherant risks in my case (bc of the autonomic issues, endocrine-steroid use, restrictive lung issues, the shunts, etc) be worth looking in to?  I dont know but going to ask Dr.Earing and will talk to Dr.Bragg about what if anything has to be done w the shunts during cardiac surgery, it may be that nothing is done to them.
I admittedly cant imagine going until we do valve replacement surgery which at the very earliest even if we say started the process now (which we are not as Dr.Earing wanted to give 3-6 mo to heal/be infection free from Dec's central nervous system (CNS) infection)  it would be I imagine another 3-4 months to get everything done before a surgery. As it is now I cant imagine we will be doing any valve replacement surgery before fall and so to live with these headaches, nausea, intermittent vomiting, autonomic symptoms and the other related symptoms  you might as well say 8 or 9 months bc of healing time from any heart valve replacement surgery seems like a long time. Even I who tries hard to live by 'one day at a time' with the medical stuff (and fails miserably I might add often at it) cant stretch my mind to imagine such a long un-ebbing period of these symptoms. =/
Please dear Lord if it is thy will allow Dr.Bragg to come up with some plan in which we might try to treat these headaches either differently or sooner than before any heart surgery. =/ I can really understand her being uncertain about how these valve issues affect the use of anesthesia and her surgeries and so  completely cant fault her on that but I still do pray we can come up with something and much sooner. This is hard, so really hard. The worst part I think is just that there are many things i'd like to be doing or more things id like to be gettng done most days and I just dont all of the time. =/
On a different note I am 30 today, a big deal in the MPS world. While I wasnt diagnosed as a kid so I didnt know I had something hanging over my head as far as age I do see how few of us adults there are w MPS I who are older than 30 (I think I can count on one hand or maybe some of both hands here in the US for MPS I Adults) . Ours just isnt a well studied age or issues and so I am I almost feel like entering un-charted territory, certainly atleast for most of my drs I am. Id like to think I cant top what I all had in my 20s (Alot, yikes) for medical issues (or surgeries) but I think I likely will.
  I pray for God o help guide my drs in making the best choices as we cont to try to figure some pretty complex issues out.  In some ways I am happy to be done w all that was my 20's, like entering a new phase almost. :)
Stay tuned for any news. Tomorrow I see my PCP for a pre-op physical for the oft rescheduled hand surgery (3rd release, and Hand surgeon doing several other things during to same hand) although I dont know if the surgery will be approved (approved by my PCP that is) bc of the cardiac issues, we planned to talk that over and figure it out at the appt. I had also asked her if she might be able to help me figure out the bladder issues (mostly an issue at night, some spasms during the day)  so PCP was going to write that down for the appt to.
Please say a prayer that Dr.Bragg doesnt grow frustrated and give up, that she is able to formulate some kind of idea we can try and we can come up with a plan soon.

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