Friday, November 17, 2017

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,

Wednesday, November 1, 2017

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,