Rheumatology, Los Angeles-Humira Study visit, Cardiology (INR)

Hopefully just a quick update.
                                        

       Humira Study - Rheumatology, long term thinking..
My Study dr for the Humira study called last week as she usually does once a month. We where talking about this study, how it is going and what comes after the study wraps up in a few months (the study was originally slated to last 52 wks but was recently shortened to roughtly 24wks (I can't remember the exact amount of wks it is now it may be a bit more but somewhere in that 24 wk range).
Anyways so I had asked Dr.Polgreen if I where able to get one of my drs to prescribe the drug either before the study ended or after could we still do the testing that she would have done in the original year long study? She actually seemed really excited by this and talked quite a bit about how this might be possible.
I messaged my Primary dr that same night to see what her thoughts where and she (Dr.Simpson) messaged me back the next day that she reached out to 1 of her colleagues, a Rheumatologist about my case to get his opinion.
This Dr raised a few potential concerns like insurance often raised a stink (Dr.Polgreen did give suggestions how to better go about getting insur., to cover the med with my diagnosis and the arthritis like joint issues MPS I causes) and as well that Humira has a known risk potential for causing congestive heart failure - an issue I/my Team have dealt with/managed before my 3 Open Heart Surgeries but he was willing to see me. That Appt is set for a few weeks from now. 
I love that my PCP is so willing to help and she's kind of amazing how fast she goes about tracking information down or talking to colleagues of hers to figure out how best to deal with an issue!
                        Cardiology - INR, Home test machine!?
INR, Up, down, up, down, will stabilize then falls again so my Cardiology Nurse when she emailed me last week with another below range level (for about the 90th time after it had stabilized for about the 90th time) suggested we try getting a home INR testing machine.

Apparently they don't always recommend these for their Pts but b/c mine is so unstable and my risk is pretty high for blood clots and I live a fair distance for either place I can test at (either my Primary drs sites in Madison, an hour drive or Cardiology's Hospital in Milw at CHW also an hour) + every time I travel and then have to check INR I have to figure out where to get it checked (have 1 of my Providers at wherever I am traveling order the lab) having a home tester would offer a lot more flexibility.

I'll still mainly test INR at my Infusion on Tues's since I am there anyways and any time I am at a drs appt anyways but to have this home tester when we need to test in-between and when I am on Lovenox will offer much more flexibility. Will kind of be the best of both (lab testing and home testing)!
                         Neurosurgery - Shunts/Headaches
Otherwise (and I seriously feel like I write this EVERY SINGLE TIME) the headaches and related symptoms have con't I just honestly can't get myself to call and reschedule the Neurosurgery Appt. I am so (I guess) burned out on that whole issue and feeling like I am not even listened to anyways when I talk to my Neurosurgeon yet I feel really, really yuck from a pressure standpt.  
  It's obvious atleast 1 shunt is working as I feel ok, I just don't feel great. I feel tired All. The. Freaking. Time. and bladder, which has been so good on the Humira has been sooooooo bad, I literally feel like it's so irritated but not in a infection kind of way.
                          Humira Study - travel to L.A
I travel to Los Angeles this week for the Humira study this wk so will ask them about this, why they think it might have helped so much and why it might seem so bad now. Is weird.

Anyways will try to update maybe after I get back from the study visit (I leave Weds get back Fri, nice and exhausting).
Thanks for stopping by,

Erica

Update long time in the writing.. Trips, Appt, Life?

I started this post about a month ago and have thought numerous times about how I should finish or delete or do something with it but being quite honest I just did not feel like it. Alas many wks later I am here writing now. It's been a busy few wks and yet in other ways at times  somewhat slow (maybe low key is the better way to put it)  - a lot going on but yet not a lot going on.
                                      

When I first started writing this post I was thinking a lot about and considering writing about the past few years and so much change. I couldn't really say why but had been thinking ALOT lately about the various people in my life I really miss - be it family or former Providers, my former Pastor (we do have a new one, he seems very nice, I think maybe it's just in part I miss all I learned from our Sunday School mtngs and that in turn helped me with SS).
 I keep thinking about 2yrs ago at this time and how incredibly hard that uncertainty was and how back then I wondered what a year out and 2 yrs out would be like.. Would I (especially) have a new Nrsgn that was atleast reasonably as good (ie caring) as Dr.Bragg had been? Would Dr.Bragg and I still have kept in touch (we do, I am thankful for her, sometimes when I want to pull out my hair in frustration she keeps me mildly more sane).
I remember wondering after the 3rd Open Heart Surgery (OHS, Mitral Valve) would that finally be the last 1 and would things settle?
                                        Time flies even when it doesn't
It's been 2 yrs this May since Dr.Bragg moved and honestly it hasn't gotten much easier. Now is a lot like it was before I had found her. In a way even I feel like she pulled my Team together, though I can't really say why that is. She was a good dr who cared and I miss her willingness to think outside the box for my not very typical headache/Hydrocephalus issues. She still helps my Neurosurgeon and as I said above I can ask her questions and we've become friends I guess you can say but I would give about anything to have her as my Nrsgn again more than anything else.
                           
It's been 2.5yrs since the 3rd OHS and things are atleast stable from valve standpt. the replaced (mechanical) Mitral valve does have the moderate stenosis (narrowing) which is not what my Team hoped for and heart/heart muscle is working to hard to some extent but overall it's not presented to many problems. Heart rate (or rhythm I should say) has been an issue and especially so the past few weeks with some really weird episodes that where occurring with some frequency but my Heart Team is on top of that, ordered a Heart monitor which showed some different rhythms and changes but overall in the big picture it looked ok. One of those confusing things - story of my life really!
                                    Coumadin / INR - Never ending
Blood thinner levels have been all over the place but that likely has much to do with the headaches and nausea and so keeping that med level stable is like trying to I keeping balls juggled and in the air (I know terrible analogy).
We spend more time to low and like this wk with INR (blood thinner level) was to high - so increased chances for bleeding but that to is manageable. Even today's level at 8.12 or something in that very close range  (can't remember right off hand) I thankfully don't seem to be having any adverse affects, no bruising, no bleeding and if not for the actual lab test I wouldn't have guessed INR was so high. Last time it was in the 8 range I felt lightheaded and other weird things so I am glad atleast that hasn't been an issue this time!
  - Atleast my Primary dr is FANTASTIC about helping with this and has a standing order for the INR (blood thinner) lab test in her system so I can get it checked either in Madison (where my Primary dr., is) or in Milw., where my Heart Team is and both Teams can see the result through the shared 'Care Everywhere' system. I also get the results within and hour or two in the MyChart system at both Providers hospitals.
Today both my Primary drs office and my Cardiology Team called concerned about the level but they communicate with each other and with me so we figure it out and I know what to watch for.

Headaches -> Shunts -> Pressure
I saw my Neuro Endocrine dr., the other week. She's been part of my team way before I started seeing Dr.Bragg (would be 7yrs this May) and knows the changes that occur not only from our time with Dr.Bragg but before that when I was pre-shunt and then 1st shunted at FMLH.
Endocrine also ran new labs (which she does every 3-6mo) to re-check thyroid level which has been an issue in the past but stabilized once we got cortisol (hypothalamus/pituitary gland issues (cortisol levels) stabilized). The thyroid level has been intermittently going awry likely due to the newest heart med I have been on (Amiodarone) so she re-ran those labs to monitor and results thankfully came back fine.
                          Boston Marathon - Running for Rare
In 1.5wks I fly out to Boston for my 6th (?) year on the Running for Rare Team - this year will be my 1st year staying in a hotel while out there for the marathon so I am a bit nervous but I think I have everything sorted and figured out for  getting to the various places I need to. The biggest being of course Monday morning to our marathon Team mtng spot in Wellesley, I am thinking i'll take the train from my hotel in Boston to Wellesley, it looks like from directions 1 of the Team runners gave me i'll have to switch trains once.
 After the race 1 of the NORD-Race Team organizers offered to give me a ride to the Team get together and then back to my hotel afterwards. My hotel also has a shuttle to/from the airport and to my Appt on Tues morning with my Genetics dr. at Boston Children's so overall shouldn't be bad. Just my need to be organized makes me a bit OCD about having all the details written down and mapped out!

Should be fun if not I get a little pent up in the leading up to making sure I have everything arranged, packed and I get to the airport on time (I am flying out of Milwaukee which I haven't done in a couple years - it's not a huge airport but more difficult to get to then is Dane County (Madison)'s airport!

Otherwise this week I cancelled and haven't yet rescheduled the Peds Neurosurgery Appt (just wasn't feeling like dealing with it) and PMR rescheduled botox which was today (I went to the Appt and my Dr. did map out a new approach she'd like to try).
      Aldurazyme (Weekly infusion) Anti-body test
The one lab test my PCP had ordered and came back is my Infusion antibody level had been climbing for a while (not a good thing) but this result came back completely normal! So unusual BUT was happy to hear this! Perhaps the pre-infusion zyrtec along with my infusing over roughly 6hrs now (at FMLH I infused over 5hrs, which is still longer than the typical recommended run time) has helped calm my bodies response down?!? Whatever the case is good news!
                                              Easter

Will try to update sooner next time, perhaps after I get back from Boston and Los Angeles (week 16 Humira study visit).
Thanks for stopping by,

Erica