Friday, June 1, 2018

Cardiology Appt., Mitral valve changes - unusually, unusual

I had 6 month Cardiology Appt yesterday  with Echo for testing (basically ultrasound of the heart). Overall a good Appt., the big (I guess?) thing is mitral valve which has shown changes since we 1st did the mitral valve replacement 2.5yrs ago has increased some more from 6mmhg to 9-10mmhg which is not a great change nor expected with mechanical valves in the setting of my MPS (we chose mechanical valve despite the increased risk with blood thinners for the express purpose further valve issues do not typically occur).
 It's well known valves usually worsen in my MPS if tissue valve is used so that's definitely unusual and concerning to my Cardiologist but we just monitor.  - I don't remember for sure but think it was double digits when we replaced this valve 2.5yrs ago although I think there was also leaking then. Now it is at 9-10mmhg so high moderate range I guess for stenosis. Thankfully although concerned why it would keep worsening Dr.Earing also said there wasn't anything we'd do now anyways and we'd just keep monitoring. The good thing (and big reason why we can just keep monitoring) is there's no signs of recurrent CHF and PA pressures look ok.

 I guess he'd monitor most closely for recurrence of the CHF and I do currently (STILL, ugh! Yup I might be whining just a bit =D ) still take medium dose Lasix (20-60mgs, 2x a day) and Spironolactone (25mgs 2x a day) and then 3 or 4 other heart meds for heart function, heart rate and to help heart muscle. CRAZY though!
 If someone would have asked me before my 1st OHS if  I ever thought I'd go through more than 1 Open Heart Surgery much less the 3 I've already been through and then, lol heart overall and valve still be so imperfect I'd have thought they where CrAzY! Is what it is though I guess!? God knows the plan and I walk with him (thankfully and even when I falter he's still by my side)..
I take meds to help heart muscle function (low end of normal), heart beat/rhythm (was lowest it's been in a long time at the Appt (70s-80s, Dr.Earing was pleased with this and feels the Amiodarone likely is benefiting) and lastly I also take Ivabradine for heart rhythm, Amiodarone, Lisinopril (heart muscle), and the 2 meds for keeping fluid off +  the Coumadin and then Lovenox when INR is to low (to bridge till we normalize blood thinner level with Coumadin).
 In his note and we talked about it yesterday Dr.Earing did raise concern about how often my INR has been out of range and apparently that can have an affect on heart function - maybe b/c it's greater (thicker? lol I honestly am not sure) blood flow when we need really thin flow through the mechanical valves to decrease risk?
So we just monitor and watch and I keep working in my MPS Community to hope something comes along that might help my heart issues. Can hope!
I go to Boston Sun., to see my Genetics dr., I know he's working on a re-formulated supplement (Resveratrol) that the hope is it will act like a chaperone therapy, cross the BBB and get in to the heart + help clear out GAG where my weekly Aldurazyme infusions do not. The feeling is these therapies whether the Aldurazyme, the potential Resveratrol re-formulation or the Gene editing/Gene therapies studies (or all taken together) will all be complimentary therapies with no one likely to be a stand alone therapy. Whatever the case I just need something better - heck something that could calm my crazy heart rhythm down would be AWESOME! Is so darn ANNOYING!!!!

Dr.Earing also asked about the Humira and what the plan was with this. How would we know if I should stay on it. At 1st I wasn't entirely sure what he meant but explained that the biggest thing I've noticed is the spine-bladder irritation is so much improved (which was/is enough for me to want to stay on it). Dr.Earing was pleased with this and like my PCP and the Rheumatology Dr. just reiterated we'd have to con't to monitor for CHF (Heart failure, a relatively common side effect of this med I guess) and infection risk, He is okay with my continuing it though as there aren't signs of CHF at the moment.
Rheumatology messaged (MyChart) and called the other day they have started the Prior Auth process with my insur., for trying to get approval of the drug. They seem to have done their research and really listened to me/Dr.Simpson as in his note Dr.Pruhs not only lists my MPS I but then secondary arthritis (information more often needed to get the med approved per Dr.Polgreen).

The big take away really and it's not new is that versus any dramatic change on any of the bigger surgeries or meds I take I made the comment to Dr.Earing I tend to get smaller changes from a lot of the stuff we do and I take but taken together all of these surgeries and meds and therapies make a much bigger difference for my life and my care. Subtle alone but much bigger differences together if that makes any sense!

Will try to update sometime soon after I get home from Boston next wk,
Thanks for stopping by,

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         

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!