Thursday, March 28, 2019

Many updates, lots going on.. (MPS never stops but thankful)

It's been a long while again since I've written here so sort of a long update (sometimes fb makes it to easy to just do short updates there!). =0)

3mo f/up, Cardiology,  Febr.
 My PCP reached out to my Cardiologist after the Cardiology (heart) appt back in Febr about the meds I am on and the fluid issues  + my Cardiologist had mentioned at the appt back in Febr possibly adding a 3rd med on short term basis (3 day cycles I think which is what my PCP has been doing with larger Lasix doses intermittently where she has me increase the dose from 40mgs 2x daily up to 60mgs 2x daily and then back down to 40mgs twice daily. We are having to do this quite a bit but my PCP just asks that I let her know when I am adding the increased doses which has been generally once a wk lately give or take.

Anyways I was a little confused about the med after the appt but was also a lot we had talked about but after PCP spoke to Cardiology instead of adding this 3rd med on intermittent basis for now he told my PCP its something he'd consider down the road.
Honestly I am ok not adding another med, I am tired of meds (how's that for perhaps sounding a little whiny and brutally honest but it is true). Some actually make a difference others I just feel like its just another thing to take.
So for now we've just been doing the additional added dose of Lasix as needed (which is enough). I f/up in May. -
I suppose one of the most frustrating things of the appt is the standard fall back about how symptoms are probably heart and lung related and "progression of your MPS".  Anyone who knows me knows I despise when drs use that (what seems to me) cop out answer as to me "progression of your MPS" is not really an acceptable answer but what can you really do?

Neuro Opthalm/Optometrist: 
Seriously between headaches/pressure and all those secondary symptoms (and then the new glasses script I got earlier in Winter was not correct so I ended up with my Neuro-Opthalm., suggestion just getting a re-exam at my local Optometrist and a new eye glass script (which has been better than the 1st). For whatever reason the 1st script was changed to near sighted and I most definetly have more issue with far distances though my glasses are bifocal.
 Vision in general is not all that great with reading (stinks because I LOVE reading books but this won't improve till the shunt/pressure headaches are back under better control. My Peds Nrsgn says the implanted pressure monitor he's been trying to get approved is through the FDA process here in WI and now (I guess) is just getting it all set up at UW. My PCP (LOVE HER!) spoke to him twice last wk and in one of her messages told me what she spoke to him about including asking him if this device was in fact ever going to be approved or where we just being strung along (not exact words).

I said on a fb post if it is possible to have a provider who was a God send then my Primary dr is in fact I consider my gift from God. She seems endlessly patient and to never give up.
Not only has she repeatedly spoken with my Neurosurgeon and my Cardiologist and other of my drs., but she's spoken with the CEO of the company whose (reformulated Aldurazyme) drug we are trying to get under compassionate use and if this in fact becomes a option (they will speak again in 6wks after some changes are finalized at the company) then she will go to bat on my behalf at her med center/my infusion site that I be allowed to receive this drug there.
She's already taken steps to confirm a few areas related to this drug and has laid out who on my team she'll speak to, to confirm they are ok with the plan if we can get access (there is a lot of if's on this access yet).

I like my team of providers but my PCP truly gets a gold star for SO THANKFUL FOR HER! I at times worry a little I must be a pain in her *** b/c it literally seems like there is 1 thing after another with my case that I have to ask her help for but she never seems to mind (or she hides it well, lol =) )  as she continually responds to messages and is open to what I think.

 In my rare world we all need a dr. like her on our team to help manage complex care and for a dr. who admitted she knew nothing about my disorder when I was 1st given her name and 1st reached out to her she most definitely stepped up and kept her promise she would learn!

WORLD Mtng - Orlando, FL
I was in Orlando for the WORLD Symposium back in Febr., which is 1 of my favorite mtngs throughout the yr. - there was not a lot of talks related to my MPS this yr but I had quite a few mtngs and lunches with various Biotech Co's I do work with and all of those where interesting and at minimum insightful. It is 1 awesome mtng I am always grateful the foundation I work for is willing to send me to!

Headaches -
Headaches oh the headaches - I have been having some pretty fierce radiating pain along my LP Shunt which goes from the valve/reservoir on my low back around the side to the front and super painful whether sitting back or standing although thankfully does ebb and flow some. In addition it's also started (although thankfully this much more intermittent than the other pain) getting a very odd, really uncomfortable pain like a shooting pain that's very clearly from the shunt (b/c I've had the same before in the past). Oy!
I pray about this virtually every day. I can only keep praying something will happen soon but honestly it's hard to even try to be optimistic anymore about the shunts (doesn't mean I'm being negative just realistic). Sometimes I still can't believe even 2.5yrs later I'm back to not really having a great Neurosurgery team. That isn't to say the Nurses/NPs and Secretary aren't great, they are but unfortunately they aren't who fix shunts. I can only pray my Nrsgn really does get his device approved SOON and it gives us information for which he doesn't just SIT ON! =/

Neurology Appt.
 I saw Neurology at Dean earlier this wk - my PCP wanted to get their opinion see if they had any insight or at the very least show my Nrsgn that they to feel it is shunt related (which this dr did based off what I all told her). Not that this will likely do any good but atleast it's 1 more piece supporting me I guess which is odd to even say.

Labs -
Last but  not least I guess due to some other issues my primary dr had run some general labs which came back with a few off results (most where normal, a few where off); thyroid we'll check again in a mo., although given the long standing-on-going headaches I'm not surprised it was low.
 My Neuro Endocrine dr has been able to establish when I have longer standing pressure issues it over time affects my endocrine hormones and especially thyroid level. PCP added a short course of prescription strength potassium to the daily potassium I already take and iron is on the low end of normal if I remember right but I do already take that daily to.

GI System has been kinda a mess lately (a life long struggle for me way before the MPS was even dx'd) but with a friends help I've added in a few new things including a strong probiotic, digestive enzymes anytime I eat and (sliighhttlllyyy expensive) Aloe vera to help calm down inflammation (but it seriously is helping something crazy I think!) which in turn is helping stomach and bowels slowly get back to a more normal. It is always crazy to me how many different areas MPS affects! Literally like a sh** show sometimes trying to figure stuff out but grateful so many people and providers are there to help.
 New car..                                          
Last wk I finally gave up on my car and bought a (almost new) one - a 2016 Chevy Cruz.
I am no fan of having a car payment and my car insur apparently in turn now also went up to b/c of the newer car (my other car was a 2011) but this all aside I LOVE THE CAR! I swore I'd never buy this new a car but at the same time I really do love it!
Thankfully I had a 1/3 of the total cost to put down so while my car loan is still enough atleast I have very good credit so it overall is very doable. I'll still celebrate when I get it paid off though! =D)
Thanks for stopping by,