Friday, March 21, 2014

Cardiology Appt, UofMn Studies

I think I perhaps shouldn't write these posts while I am quite as tired as I was writing the last ones; for anyone wondering about my math skills related to the INR I realize neither of those numbers where actually in-range (=) though the number did still climb fast compared to last time. :) If nothing else amusing the way my mind works when overly tired! =)
-I am re-editing this, actually I just looked at the INR result on CHW's MyChart and I was right the first time; INR was at the high end of 'in-range' and 2.96, standard range is 2-3 for an aortic mechanical heart valve. All I can say is tired, tired, tired and numbers still and probably never will be my friend! =)

Per Cardiology Appt (was Tues) (F/up) and the fact that I was going to be in Mn till Fri night they just will re-check the INR level again Monday when I am at ERT (same hospital) and adjust dose accordingly. If the level is really high I'll re-test next Weds, 2 days prior to Dr.Bragg's shunt surgery, in which she will be moving the VP Shunt from the R to the L side but tunneling the catheter back around to keep the catheter tunneled down my R side of body (due to the Port being on the L neck/chest).
Otherwise for this surgery I'll stop the Coumadin 72 hr's prior to surgery(so Mon) for Fri's surgery, otherwise it is usually 3 days before.
Med wise Cardiology for now due to the shunt surgeries and not wanting to mess w/to many things at once is opting to leave current meds [Nadolol, Lasix, Spironolactone) the same, watch HR and F/up in 2 no unless something changes. Their big concerns as before are trying to minimize stress on the heart, mainly keep heart rate down, (it has been somewhat lower but is not at the range they are shooting for) and hopefully by doing so buy time to needing to replace a second heart valve, (second mechanical heart valve) and thus what that entails.
Their other concern this appt especially was our preventing any shunt infections as their concern is the affect this would have on the mech. Aortic valve given I guess both shunts do drain to the same body system as the heart. Thankfully we've avoided this so far as I'm not sure what a possible infection would entail and would rather not find out!

It's very possible I am slightly crazy but I did come to Mn (flew) on Weds which entails a few studies though the study docs did cancel the MRI portion of the testing which I wasn't really complaining about! Their concern was the 2 shunts both being programmable which not that big a deal(if or when any of my drs wants an MRI Dr.Bragg orders it and then she or her team reset the shunts to their correct settings after).
Non-the-less I think it was the shunts along w the heart valve (though there is nothing about that an MRI could affect) and the other hardware that made them decide aagainst that test. Dr.Shapiro just asked me if I would ask Dr.Bragg had we done any recent brain MRI imaging as she was kind of wanting to look at recent imaging to see if there where certain changes (having especially to do with white matter I think she said) since their last imaging in 2010.
The studies I did for Elsa Shapiro's on-going longitudinal MPS I study included a gait analysis walk, which means doing various things as they monitored (with little devices attached)and recorded via computer and video. This was done on a almost run-way type set up they have made and almost comical (I doubt most of us walk exactly like we do in real life when others are watching!) but went ok.
The other study was Thurs from 9-1 (Neuro-cognitive studies) and then meeting w Dr.Ahmed after to discuss medical history and especially over the past 3 1/2 yrs since I was there last. Last but not least I met w Elsa Shapiro who runs the whole study bit and she relays the results, her thoughts or any recommendations and what they may be up to in the future. Much of what they are working on I was aware of due to my work with Gene Spotlight but is still really neat to talk about it and hear their thoughts. She (Dr.Shapiro) has been saying for the last 4 yrs she is going to retire but then in the next breathe she would talk about what was being planned for the next year if their research grant was renewed for another 5 yrs so I (many of us in the MPS I community) are hoping she will keep delaying or stay semi-retired!? =)
Results from the actual NP testing where mixed; IQ is very normal while focus is down from 3 1/2 yrs go (I had thought I did good, lol!)and memory is also down though she did say I did fairly well on remembering information when it was in the context of something (say a story) but information that was taken out of context or given on it's own (word lists for instance) did not score well.
I guess I can't say that was particularly surprising as I continually write myself notes be it on my phone, in my (2) calendars or often even on the palm of my hand in order to remember things (dates, appts, grocery list, etc).
I think given the very many surgeries-especially the literally 30+ shunt surgeries/procedures in the past almost 3 yrs gives me reason enough to not worry to much about these changes! I also don't think the VP Shunt is working optimally (I do think it is working atleast partially just not optimally) probably could play a part.
It is also not uncommon in MPS I pts to have these changes. I doubt most people in my daily life even realize the memory issues I have as I am careful to write notes, keep a calendar and keep numbers in my phone/in my calendar vs having to try to recall information I don't often use.

I had dinner w a good friend of mine from Genzyme (she is our areas Patient Care Liaison) last night (Thurs) which is always really nice; we've been doing this anytime I am in Mn for atleast 7 years now! Occasionally she comes to WI so we'll see each other here too. Anyways we also ran in to each other at the WORLD meeting in San Diego a month or so ago and she was telling me that my former Metabolic (Genetics) Neurologist was at the meeting, which would have been really neat to see him!
Because he works in Pharma now he is isolated from Patients (a huge loss to our MPS I neurologic-knowledge community) but she said next time she saw him she would see if he could reach out. I really think he could help some of my own drs!? (He is the dr that diagnosed my hydrocephalus originally and followed the C-spine compression along w my former GC + suggested I enroll in Patti Dickson's IT study and then talked to her to get that all rolling. He was the type of dr that if he was irritated or grumpy he'd just tell you as soon as he walked in the door which was nice, he didn't really mince words but he also understood the MPS/brain/neurologic issues and how the 2 intertwined secondary to the MPS storage. I would have loved to talk to him while at WORLD but is also a HUGE mtng so is not all that unusual to miss seeing someone even when you do know they are there (which we did not).
Besides coming to UofMn for these studies this week (I flew back earlier today) I had infusion Mon, Cardiology Tues (both Milw) and a Sunday School Teacher's mtng Tues night so it has been a busy! I teach Sunday School on Sunday so I had to get that lesson ready which I did most of while at ERT Monday and finished earlier tonight.

I'll update again when there is something to update or write about,
Thanks for stopping by,

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