Friday, August 31, 2018

UofMn visit, Peds Neurosurgery visit - 2nd opinion. Post Humira Study - Rheumatology visit, etc..

It has been a long while since I've update here mostly b/c I just haven't felt like updating. I genuinely like writing and doing this is a bit cathartic but well I just haven't b/c I hoped I suppose maybe if I waited long enough I'd have some good update. Alas somewhere in-between but still nothing really on the shunt front - see below.
UofMn  - Dr.Guillaume, Peds Neurosurgery.
Because I like to torture myself I was in Minneapolis (flew there vs driving) for the longitudinal neuropsych study-testing I'd been a part of for yrs (but missed the past 4 or so yrs. I was apparently last there for this study 4yrs ago).
Anyways since I was going to be up there my PCP had suggested I see the Peds Neurosurgeon I know/have seen last year at UofMn and get his opinion on what's been going on with the shunts or more specifically with my headaches (as likely the shunts are working atleast partially if not fully but other than when I have spinal fluid leaks along w/the shunts I rarely feel overly good. This being b/c the shunts drain fluid from the ventricles in the brain but not the fluid that is outside the ventricles in the skull and what likely causes my headaches but also why spinal fluid leaks cause my headaches to be relieved (if that makes sense).

That appt went well though how it will tranlate to my Neurosurgeon remains to be seen. Doesn't seem very promising and that honestly is really, hard but I am trying to be positive though I'd be lying to say dealing with this over and over is easy. I just want to feel better and knowing it's basically as simple as needing to drain more even if keeping draining more is difficult (I get that) is difficult.

UofMn Neurosurgery Appt - 2nd opinion
Dr,Guillaume the neurosurgeon I saw again mentioned a few options he felt might be good options (see below for more on this following the appt) but this included it might be better to re-locate the 1 (LP Shunt I believe) shunt to drain from cisterna magna (base of skull), where more CSF (cerebral spinal fluid) is. He wondered/thought it may be  a better spot to relocate the 1 ( current LP) shunt to  get closer to the  negative pressures I feel best at.
The other was a different programmable valve made by Sophysa that drains lower than my Codman Hakim valves do and he felt may give better drainage/relief.

1 of the other things Dr.Guillaume mentioned was b/c  I have the 2 shunts, 1 in the brain and 1 in lower spine that it may do better to switch the current programmable valves I have (Codman Hakim programmable valves) to a valve that drains at much lower CSF (again spinal fluid)  pressures than do my current valves. So the big thing we talked about was
1. trying the Sophysa lower draining valve to get more CSF off and get closer to the negative (CSF, brain pressure) we know I feel best at.
As mentioned above try moving the LP Shunt to cisterna magna where there is more CSF (a bigger CSF space then where my current Lumbar Peritoneal Shunt sits/drains. I wish and no I don't want surgery but I sure do want to feel better!

Dr.Iskandar asked that I make an appt with him which I have for a couple weeks from now (I have other stuff next wk and try to find a balance between life and not tooo many appts in any given wk. Often the balance is skewed already but I really try to do no more than Infusion 1 day (1 trip, hour drive 1 way) and 2 other appts which more often than not are on 2 different days so already 3 days of appts/driving).
 I am praying Dr.Iskandar will atleast reconsider 1 of the 2 things Dr.Guillaume suggested. That he will keep in mind what other of my Drs have said and the affects the not draining enough is having on other of my MPS issues. I just want to feel better, it's not like I (anyone!?!?) would willingly want to have surgery!? I guess time shall tell. I am praying.

The original plan was he, Dr.Iskandar and Dr.Bragg where all going to have a conference call but I received emails from Dr.G and Dr.Iskandar earlier this wk and the two of them had spoken.
The reason I felt it was important Dr.Bragg be part of their conversation even if she's not been my Nrsgn for 2yrs now is b/c she had such a good understanding of and vast experience with my shunts and skull compliance issues (her near 40 surgeries and 5yrs on my case vs Dr.Iskandar and I have been seeing each other for 2yrs and I believe he's done 4 surgeries). I will never say he isn't a good surgeon, he clearly is. He's brilliant in fact but our ability to communicate effectively with each other has not been great. I hope at some point it gets better but it's hard in the process.
International MPS Conf - San Diego
Otherwise a few weeks ago I was in San Diego for the International MPS Conf which was actually really good! SOOO BUSY and jam packed as I had so many mtngs and when I wasn't in mtngs I'd be on my way to a talk only to run in to someone I know or knew but hadn't yet met but it really was neat! This International mtng meant there where families, researchers, drs, and biotech from literally all over the world. I also sat on the adult panel and gave my talk on 'transition to adult care' which seemed to go over well.

Last Humira Study Visit - Los Angeles
Last wk as if flying to San Diego 3 wks ago and Minneapolis 2 wks ago wasn't enough I flew to Los Angeles to wrap up and finish the Humira study. I am glad it's done and the traveling to LA is done but also glad I did this study! I in fact saw my Rheumatologist today for the post study f/up (2nd time I'd met him) which went well. He unfortunately told me he is moving to Guam for atleast the next yr so is handing me off to 1 of his colleagues (ironically a Rheumatologist I saw back in HS, 1 of the 1st Specialists I was sent to from my then Peds Cardiologist and a local Neurologist who sent me to a Hand specialist and that Hand specialist sent me to this Rheumatologist (who then sent me to a Peds Neurologist among a few other drs).

 This Rheumatologist is 1 of the drs who suspected there might be something underlying going on. She had tested for Peds RA (rheumatoid arthritis which many of us MPS I pts get tested for and are suspected of having before the right diagnosis (MPS I) is eventually found amongst a few other inflammatory disorders she had done blood work for. It is often funny how the circle of care works sometimes. Many years ago (10 or so yrs ago, before my current PCP)  I had been sent back to a Peds Neurologist I also saw in HS though that dr then referred me to or suggested someone else she felt was better though I don't remember who that was (this was before I'd found Dr.Bragg, my former Neurosurgeon).

Anyways so my current Rheumatologist did put in a new order for the current humira and just continued to emphasize the heart failure/infection risk with humira and for now benefits outweight risks. I am a little sad to see him go. I really liked this guy! =)

Pulmonary - Pulmonary Function Testing:
I saw my Pulmonary dr about a month ago and then had pulmonary function testing a few wks ago. I don't know the actual pulmonary testing results yet but the results sent to me seem to show some significant changes. I asked my Cardiology Nurse if she'd ask my Heart dr what his opinion is. I may ask my PCP, I actually kind of just forgot about asking her this (I feel like I am always asking her questions although she is awesome and doesn't seem mind).  Next yr the Lung function testing and pulmonary appt will be coordinated together so I can get results right after from my Lung (aka pulmonary) dr. I may just email and ask her. She also is a really good dr.

Sunday School, etc.
Otherwise besides whatever I am forgetting Sunday School starts back up next wk. It's always a little amazing actually how fast the time between when SS sends in May to when it starts back up in Sept goes but other than getting up that extra hour or so earlier I am looking fwd to seeing our kids again and to another year. Hopefully I have everything organized and our teachers are ready! God is with us so who can be against us as we start a new year!?
Please say a prayer if  you will that something is able to be sorted re the shunt/headache stuff. It's been soooo nice having the spine-nerve bladder stuff being calmer at night (thanks to the humira) so if only to get the shunts (pressure) better!
Thanks for stopping by,