It seems anytime we would/did get the shunts to a really good place some stupid thing came up that affected them (The initial surgery with Dr.Bragg where the single shunt was working better after she revised it but then fluid was also making it's own pocket in my back/spine (pseudomeningele I think is what it was called) which allowed more fluid to drain, when we'd to the external drains, when we added the 2nd shunt, times like 1.5yrs ago or so ago after a revision, before fluid started building up in lung space and wasn't absorbing I remember how happy I was at feeling so good (and how disappointed I was when I wasn't feeling as good again!).
Anyways this post isn't to vent about the past though, sorry. I can't change what I cant change but do I wish I'd magically go in to Dr.Bragg's office, before she leaves towards the end of May (around the 21st) and she'd have some idea and something she could do to help. It sounds ridiculous but at this point I wouldn't even care if it where a flipping surgery bc I feel so blessedly bad so much of the day. It is only by this time of day I finally feel better which makes for having to force myself to go to bed atleast by midnight despite knowing i'll then feel worse again. UGH!!!! Have I said I hate this? Why couldn't it be simple? I can't even say how much I've prayed for us to find an answer before Dr.Bragg moves. =/
Heart Rhythm Appt On a different note, I followed up with Dr.Kovach, Heart Rhythm / Cardiology today (he does Heart Rhythm works with my Cardiologist on my Team) and biggest thing was given Heart rate has been so high (I don't make a habit of tracking it but for information's sake I do record it 1-2 times a day on an app on my phone so we have an idea how the meds are working). Anyways despite our increasing the Ivabradine (Corlanor) to 10mgs, which is a non standard dose (not studied in the original clinical trials for this med) Heart rate has stayed high and this wk routinely much higher for longer periods (hours) average ranging from high 90s but often was 140s for wide periods this wk. Why? Who knows, that would be the answer we'd probably all love to know!? - That and a light bulb that blinks with a easy solution! Ahh but there again, that same light bulb moment could help us with the damn shunts?!!?!?!?!
Anyways because the Heart Rhythm Study/Ablation is risky Dr.Kovach would like to try a few other meds/med classes, 1 at a time which i'll take in addition to what I already take (but have been on for quite a long period, other than the Lisinopril, an Ace Inhibitor that was started right after this last OHS in Jan.). So the grand decision was to go back to a different beta blocker, this 1 being 1 Dr.Earing tried some yrs ago, though it didn't have much if any success then it also had virtually no side effects (a miracle for the BB class of heart meds). The heart rhythm issues also are likely different now than they where a few yrs ago as that if I remember right was pre-any of the heart valve surgeries and after 3 OHS the wiring in the heart can be affected.
Thinking ahead to if that doesn't work/help he (Dr.Kovach) thought he may try a Calcium Channel blocker (CCB) and gave 2 med names, 1 of which we (Dr.Earing and I) had used for some time a few yrs back. I can't remember what the difference is in CCB class of meds vs the Beta blockers (BB's) but they must each work differently on the heart? At one point I'm sure I knew! Should that all fail we'll revisit the Heart Rhythm Study/Sinus node ablation so time will tell..
Dr.Bragg always asks me about the heart stuff, Cardiology (Dr.Earing and Dr.Kovach both) always ask me about the shunt stuff so today I was kind of filling Dr.Kovach and his Nurse in on the seeming slow downward slope the shunts seem to be in, how it's slowly getting worse but like many times is in the period where it's as clear as unclear can get and basically I think it just comes down to I have to wait till 1 or both fail completely which they will (I know that sounds very negative but it's experience and history that make me know it will happen). In the interim I keep praying Dr.Bragg could have some divine idea or we could figure this out before she leaves but honestly I am losing hope, not in her but just in general that I'm going to be feeling better before she leaves. This is soo hard to think about so I try not to but not exactly easy to do whe nmuch of the day my head feel like it is in the clouds, filled with thick clouds. I guess I just keep praying in the interim I can keep dealing w it, not a lot of other choice, right? All these headaches and in turn nausea in the night and morning means dealing with very unstable INR, this in turn requiring the self injections (they suck but thankfully if I give them quickly and pinch the area of my stomach I inject it isn't very uncomfortable). INR being unstable and the injections in turn require and frequent Lab draws to watch INR level which shows if blood is where it needs to be to be most safe for the 2 valves to keep from clotting.
We'd gotten it back to a 'close enough to normal' range late last wk I was able to stop the injections but then was massively low again on Tues so back on the lovenox on top of higher Coumadin dosing. Range was up a little today so continue injections 2x day and recheck Monday at CHW prior to ERT next door.
Not to be negative but is shitty, what else is there to say? I had to stop at a drs office today to pick up scripts and in chatting the Secretary made the comment 'Youre always so positive' - honestly I appreciate that but lately I don't feel like it's all that true!? I feel pretty sarcastic lately, not at anyone but just as a coping mechanism I think while feeling so bad so often. I am always thankful I generally don't see people till after I've been up for several hours and most often if I have a meeting (Sunday School Teachers for instance) or Cardiac Rehab (which I've missed a few the past wkS) it's not till later in the day.
As I said to my Mom yesterday there are times I don't even want to be around myself, I feel like a grouch much of the day! I apparently have an ability to hide that from others which I guess is good!?! I think that same ability is what makes it fairly easy to hide from many how I truly feel as most people aren't really looking/paying attention and I often let other people do the talking and just chime in a bit vs when I feel really, really good I kind of talk a lot, lol. I sometimes wish I was better like some people are at being reallllyyy honest about how I am feeling though, does that help some people get answers sooner? I'm sure it's a question I've asked her before.. and no real answer.
But anyways going to sign off, I see Dr.Bragg late next wk. I probably wrote about it here but i've decided on the new (coming) Peds Nrsgn but really have no idea what I need to do if anything so he is aware of my history (or will she talk to him or both of us when I 1st see him could him and I conference her)? Ugh, have I ever said I hate this kind of thing? I've never really been a fan of mtng new docs, nothing against them just feel like well maybe like I am hitting new docs with a tsunami and few really, truly want a big challenge? I don't know really, but what I do know is in the past 12yrs (5yrs with Dr.Bragg in May ironically) every single time I've had to switch a Specialist no matter the reason I've hated it. I've met some of the best docs on my Team from switching and don't regret any of the switches I've made but I think it scares me in a way b/c your mtng someone you have to hope will care enough and be open enough to want to truly help. Your hoping the new doc is the type that makes their own decisions vs 'what the textbook says' (b/c MPS and especially my issues and MPS aren't in no manual be it Hydrocephalus, MPS or anywhere... -
Deep breathes, right? I'm trying, sometimes easier than others and failing miserably at not worrying about it (as I said to someone else or perhaps it was to someone's ques. about something on fb, I think I've worried more in the past 2mo than I have in 32yrs prior! Not really that awesome.
I guess I just have to hope Dr.Bragg will be amenable to us reaching out to her both this new Nrsgn when he comes Mid-Aug. and my other Specialists if need be and pray she really will be ok with that and will stay involved in some capacity. I'm sure it's obvious but I worry that someone like her who knows my oddities (MPS wise, lol) will still help us till the new guy, if god willing he turns out to be ok gets a handle on it all. Without a doubt that is my biggest worry with her leaving is going back to no one that will help the less than straightfwd issues and have patience and be willing to work with my Team at large.
Enough stressing though, right? Sorry!
See below for trip to Boston but thanks for stopping by, Erica
I did go to Boston, which despite not feeling the best was a lot of fun, was really nice weather and I had plenty of downtime so worked OK. I definitely didn't feel great all of the time but do not regret going.
I feel like MPS and how I feel already impacts so much of my life so I try to do the little things I can to make trips like that more manageable and in turn keep living despite feeling bad.
The marathon was great, the weather was finally nice (kind of bad for our runners) and a decent day in general. My flight home wasn't till 7:30 so worked out nicely being able to see Sarah, my runner after she finished the race. She did amazing in my opinion especially this being the 1st marathon she's ever run! So cool!!!
I got to meet her kids and husband with Sarah at the Running4Rare Team dinner at Harvard that Sat night and immediately feel in love with her 3 kiddos. They where awesome!
Sarah and I went to the Marathon expo Sun early afternoon which was nice, it at least allowed my headaches (pressure) to be better under control from being up quite a while! I took the train from Hopkinton to Boston (roughly an 45min-hour ride) to meet Sarah and only managed to screw up the train schedule a couple times, something of a miracle these days! Sarah and Ed (Ed is 1 of the 2 friends I was staying with, he and his wife Anne and their 2 kids are also on the RfRD Team and represent another rare disorder CHI), they all helped with planning times and mtng spots so that of course helped immensely! which was miraculous though Sarah helped make sure I got that all ok!
Sarah was my running partner this yr, which it turned out was a pretty good match as we have a lot in common despite different 'roles' in the rare disease world (Sarah as a Rare Mom, myself as Patient and each of us dealing with 2 different ultra rare disorders). I think as Sarah put it we knew we'd get along well when she saw my post after the OHS back in Jan., something to do with how gross hospital floors are and she (lol) gets the same creeps I do about them (and same desire to just get out a broom)! We've had many a laugh about that =)
Anyways so much for signing off above, if you would please say a prayer Dr.Bragg might have some idea how to help before she leaves. I hate surgery but damned I'd even take that in a heartbeat if it meant feeling better. I HATE THIS!!!!!!
Erica
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