Wednesday, May 16, 2018

Hand Surgery (Appt, new Dr., 4th surgery scheduled). Rheumatology, Neurosurgery, etc.

This is another of those posts i've been writing over a week or two so bear with me. Sorry! -
                             Yesterday was MPS AWARENESS DAY                     
                                        My Infusion Team and I
Hand Surgery Appt - 4th time around
I saw a new Hand Surgeon today which I have to admit I was nervous. I kind of hate meeting new drs and especially new Drs when it's an issue where they will be dealing with an issue you've had numerous other Specialists (or in this case Surgeons) for. In the case of my Hands I've had 3 surgeries on each of my Hands since 2002 with each consecutive one being more complex then the one before it and each one (1st set, 2nd set, 3rd set) done by different Surgeons. The 1st ones where done in BD by an Ortho Surgeon (actually he's 1 of the docs that sent me to Madison to Dean to a Hand Specialist as Dr.Callahan felt there had to be some other reason why the muscle loss at the base of my thumbs was so severe and my joint restrictions). From there was several other Specialists and well the long-long journey to my true and final-real diagnosis (many others where considered in the process).

After the 1st two hand surgeries it was some years before I had the 2nd hand surgeries which included the tendon transfer on the R side and then I think about 4 yrs between the R hand surgeries (which where about 1-2yrs apart) and the L hand surgeries which where done by Dr.Bliss at Dean and included the fat pad grafts on each hand and also about a year or 2 apart if I remember right. With the 3rd surgeries the recoveries where pretty easy even with the splinting in total being about 4wks.

This time a few wks after I had scheduled the Appt with my former Hand Surgeon the office had called (last fall) and said that Surgeon had left (was kind of weird! Seemed super sudden and you know most Surgeons or Drs send a letter or something?) well anyways so the original Appt was rescheduled to earlier this Winter and then there was bad weather or maybe I wasn't feeling better, I can't quite remember so I had to reschedule. So ya, lol 6mo later I finally saw the new Surgeon today. =) Thankfully he did seem like he had read my history some and best of all when he'd suggest something or ask a question he genuinely seemed like he was listening (repeating an EMG --> I brought up would it give a true result?) He agreed and so decided not to order 1. He asked if I do splinting (I do just not all the time, mostly at night. The splint helps but also inhibits my being able to use my hand easily) and he asked a few other questions.
Was REALLY nice having  a Surgeon that actually LISTENED!

So needless surgery is scheduled further out, for Sept which I am ok with. I am glad he agreed to do it and listened to me and although I can't wait to have symptom relief again I also am never in a huge hurry to go back to the OR (Surgery)!
There is always much to be sorted for any surgery - bit of small so time gives us that. My PCP is fantastic at working w/each of my Providers who plays a part in background surgery stuff (blood thinners, solu cortef-stress dosing steroids, etc) so also better when there's time for that to all be sorted more carefully.
                 Neurosurgery - written last wk
Several things have been on my mind lately but I saw Peds Neurosurgeon the other day. It had been something like 4.5mo since I was last there and headaches + other symptoms (vision, so frustrating b/c this makes reading which is something I really enjoy doing) much harder as my vision gets messy. I had talked to my PCP (via MyChart, I love that she rarely makes me come in) about the various issues going on lately and she to thought b/c of past similar issues that where related to the shunts each time that this time is also likely shunt related.
Neurosurgery Appt? It doesn't surprise me I guess but I won't lie it is extremely discouraging and hard to swallow that I don't have a Neurosurgeon who believes me or will help. When even other Providers see it's likely shunt related?
Needless was a pretty heated Appt and not for the 1st time I walked away extremely discouraged. I get that my case isn't straight fwd and is complicated but that makes it ok to let a Patients issues con't and symptoms con't? At the very least JUST LISTEN to Patients, all we ask is pretend to listen to us. Don't have an 'answer' before we've even finished a sentence. Don't assume you know when what you say doesn't even remotely match up to the situation. Gets so frustrating!

Long odds, MPS decisions.. Life choices..
Not related to above but have been thinking about the long odds i''ve faced with some of my surgeries (the Open Heart Surgeries especially) and yet despite great uncertainty how I made it out each time much better than my Drs expected and in quicker fashion to. I know it may seem odd to even think about this (is it odd? Do others think about this? Maybe just others in my Rare disease community?) I mean it's actually not something I think about much and really didn't even to much before my OHS's but lately I have been a little.
 All I've gotten out of this thinking though is really that despite risks I'd rather take the risk at times with various surgeries I go through (and there is always risk, with airway and heart especially) and I guess better quality of life after. I absolutely get each of us makes different decisions and there is no right or wrong but I am so grateful for a Team who has supported me and helped me through each of my (many) surgeries. And been honest about the incredible risk (especially the 3 OHS, the risk that death was a real possibility, that they may not get me off the table and especially that the recovery would likely be long).
I think honestly each of those times albeit maybe it was just my incredible stubbornness w/a large (incredibly large?) dose of  'it's my job to prove my Drs wrong, to prove MPS wrong) and I had things I still wanted to do so I took the leap. I think about this I think lately b/c friends of mine are going through a decision none of us with MPS (or any chronic, life limiting disorder) should ever have to make and it's made me think a lot about this life we live.

Are there (many I'm guessing) times I feel frustrated and unsure and question my care? Sure, I'm very sure there is. In fact there has been episodes of that this wk, especially about the shunt stuff but honestly I just second guess myself to much (way to much, I make a art of it and wish I trusted myself more!).

Rheumatology Appt - new Specialist
I am skipping around quite a bit on issues and subjects and things so I apologize basically just whatevers on my mind or been going on and finishing what I'd started last wk. So last but I guess not least I see Rheumatology tmrw, also a new Specialists (I saw one years ago, shortly after my MPS diagnosis). This a Specialist my Primary Dr knows or talked to on my behalf when I talked to my (Humira) Study dr about possibly using the drug long term after the study. I talked to my Primary dr., and she in her kind of awesome nature and in quick order after I'd talked to her (also over MyChart) reached out to one of her colleagues to see what he thought about possibly prescribing Humira for my case.
      That Dr. per my PCP concern would be Humira can cause Congestive Heart Failure which is an issue I/my Heart Team have dealt with but it's also something that resolved after my 3 OHS/valve replacements and we monitor heart function every 3-6mo with Echo and other testing + meds I take seem to keep heart muscle function in a reasonably good place. Hopefully the Appt goes well, honestly the biggest thing the Humira has helped and even if it where the only thing it helped is the neurogenic bladder irritation (waking up ALOT at night to pee) and honestly I just realized it the other day but even with the low back discomfort and R leg is irritated (I have a weird limp at times) I don't actually have as much of the radiating pain I normally do. That IS awesome! Yay to the Humira!

I'll update about that Appt in the next post. Thanks for stopping by,

                                            Love my SS Kids         

Erica
A last thought:
Sunday School (I take pics each wk, as most who know me know I love pics but these I give to my kids so they (hopefully) remember their time in my class w/good memories.
Hard to believe but my (I believe it is) 5th year teaching Sunday School wraps up this coming Sunday! Hard to believe another year will be over. I love the kids I teach and truly enjoy Sundays (even though honestly class being that early I rarely feel super great) but these kids are well they are just a joy to teach. I'll work over the summer to get things coordinated for next fall and soon enough a new year will start. Summer always goes fast!
                                         

          

Saturday, April 21, 2018

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