Sunday, September 13, 2015

Dr.Bragg, Pre-op Appt., upcoming MPS Conf. (Utah)

I saw Dr.Bragg Thurs, as usual a good appt, we didn't really talk a lot about anything shunt related other than she double checked I'd set up another appt to have her take off fluid before I go out of town and made sure I had scheduled the shunt valve revision with her Secretary.
Dr.Bragg mentioned she had run in to the Anesthesiologist who did my last surgery with her so she gave him a heads up about this next revision the 22nd that he should try to get the case, I am not sure how that works at UW but at CHW Patients or Providers can request specific Anesthesia drs.
At CHW for instance i've always worked with Dr.Taylor on my Cardiac procedures/surgeries, either myself or my Cardiology Team (or both of us) would get in touch with her to request her on my cases. It sounded like he might be on the case per Dr.Bragg though which is a good thing, he was reasonable about the IV pre-surgery steroid dosing and didn't seem to go to wonky about the cardiac issues. I can totally understand (I think) their being more cautious with longer shunt revisions, like if we where to put back in the 2nd shunt say using more monitoring (prior to/leading up to the AVR surgeries with shunt revisions many of the Anesthesia drs would place Arterial lines and I believe something similar was done with the Heart Cath/EP Study this past July but for a surgery as short as a shunt valve revision it seems sort of silly. Dr.V did the normal monitoring but nothing to extreme the last time a few wks ago.
In seeing my Primary dr (Internal Medicine) today for the pre-op Appt she was asking how the steroid dosing and surgery went with the surgery a few wks ago which in turn I told her what Dr.Bragg has said about the Anesthesia dr. and that last surgery it helped having her notes re steroid dosing timing. I just made the comment that Dr.VanDyke (sp) didn't seem to get all crazy or overboard with the heart issues. Dr.Simpson) commented along the lines 'You really do have severe heart issues and are very high risk, if you where any other patient I would worry a lot more about your undergoing anesthesia and closer monitoring even with shorter surgeries but you see your Heart Team often, they are 'ok' with these surgeries and you go through these surgeries all the time'. - A somewhat funny, sobering, life of an MPS Pt. conversation, that's for sure! I probably shouldn't have laughed at the conversation we had around this but I did. =)
As far as anything else while it is not this low the majority of the time Heart rate today at the appt was 76, whoohoo! =) I'll take it! I've not noticed significant improvement in symptoms at night, nor with stairs (I generally, thankfully don't have to do a lot other than walking up the 1 flight of stairs in my Apt building to do wash or occasionally at my Parents when I go down to feed my Salamander there (I keep him there or otherwise my cats would probably try to eat him!).
With this cooler, less humid weather the past few days that helps some during the day to. Hopefully the med in addition to working more consistently on heart rate will help symptoms to in a bit more time!?! I do know my Cardiologist and Heart Rhythm dr. both said they would try upping the dose (double it) if need be at the next Appt so either way hope it helps! As I think I wrote about before, atleast the med doesn't have side effects it seems!
I have been emailing back and forth a bit with my Genetics dr. in Boston re the gene mapping testing and talked to my Primary dr also about this. A big part of why my Cardiologist was interested in the testing as was the Genetics dr. was to see if there may be an underlying, un-diagnosed connective tissue diagnosis and could that explain the prior difficulties with the 1st failed (very quickly) heart surgery and why the heart issues have continued to progress so quickly. If there is another issue could that help them plan for when we do the 3rd OHS I guess was part of the thinking.
Part of their thinking was my mutations are in the moderate-Hurler Scheie range but my features are more milder appearing and yet I have some of the most significant heart issues this Genetics Team has said they've seen in addition to the Hydrocephalus difficulties which are not altogether that common in MPS I pts. (some have it but not most and most do not have the difficulties we do albeit in my case the Hydrocephalus likely having been present since I was very young (symptoms started around 1st grade) and not having been diagnosed do make our treating it/the pressure far more difficult.
In any case my Genetics dr. emailed me to ask if I could ask Dr.Bragg how he could best get in touch with her and my Primary dr. is going to try and get in touch with Dr.Bodamer to see if she can help facilitate ordering the gene mapping test. In the case of Dr.Bodamer wanting to talk to Dr.Bragg he is interested in getting a sample of CSF (Spinal fluid) to test the Gag (MPS) levels in the spinal fluid. I think they have been having problems connecting whereas he and Dr.Earing where able to chat back in May/June so hopefully we can figure this out and they can talk.
Dr.Bragg's Secretary had called yesterday (Weds) to ask if I could have my Cardiology Team send them basically an 'ok to have surgery' from my Cardiology Team which is easy enough, usually my Cardiologist Team will just (I think) fax the most recent clinic notes and/or an letter saying they are fine with surgery. I had the pre-op for this surgery today, Fri., with my Primary dr.
On a different note, I knew the Cardiologist who is probably most familiar w MPS I pts/heart issues is speaking at our National MPS Conf. this coming week so I emailed her to ask her a question about the OHS. She in turn emailed me back this morning, she is including in her talk at the Conf. Adult issues with heart issues and MPS (Hallelujah, anyone who has been to these family conferences knows that adult topics rarely ever happen, sadly - very sad actually as there are ALOT of individuals living a lot longer w MPS these days! Anyways she also told me she has not known of anyone with MPS who has been through 3 OHS (open heart surgeries). That is a bit sobering! Something we'll worry about when needed, I think my Heart Team stresses about it enough already thus trying alternate options to buy time.
In any case, I see Dr.Bragg Tues, she'll pull off more fluid to give short term relief. I wish I could package her up and bring her to the Conf. with me! I am hopeful at minimum her pulling off fluid will help with the flight to SLC to the Conf!? If I am remember I am going to ask her if she knows of anything I could try that might calm my lower back nerves, I wasn't able to coord. with my Pain Mngmt dr (her Seretary is IMPOSSIBLE!) to see her or I'd have asked Dr.B.
This wknd we have a fundraiser for Church to help the local fire dept., my Sister, her husband and I are going out to the local raceway and Sun is our 1st day of Sunday School. Next week after ERT Monday and Dr.Bragg Tues, I leave for the Conf. Weds. I do have a couple mtngs set up there and agreed to volunteer Fri night at a Walk/Run being held for the Society. A good friend of mine is the Race Director so for her i'll help, she's awesome. =) In any case will update sometime soon,
Thanks for stopping by,

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