"The truth is rarely pure and never simple" (Oscar Wilde).
This blog reflects my personal life with but not defined by MPS I- reflecting the peaks and valleys of this dx., and my life despite a rare disease while rarely falling within the standard definition of this disorder.
Sunday, March 24, 2013
Shunt settings, Boston Marathon, Genzyme team info.
I may get out tomorrow or Tues which I forgot to ask and for that matter have been meaning to ask Dr.Bragg since friday the Pharmacy here on the floor tried ordering the aldurazyme from Genzyme fri but didnt reach anyone and so said they could order it monday for Tues. I have been meaning to ask Dr.Bragg if we should go ahead and do this or what she feels as the only day I can get infused at CHW (milw) is mondays. We are stopping the PCA today (I believe) from what Dr.Bragg thought this morning so I am not sure what time that will be but she was going to leave it written so I could still do IV pain med bolus as needed if needed and of course oral med and IV zofran (although even though l feel far from perfect I atleast have not needed as much or very much of the IV zofran since we've been adjusting the valve). I just was talking to an MPS friend of mine whose son also has MPS(II) and just had a shunt revision and although doing better to is still having she believes some symptoms and I said to her I just cant help but believe that in some of our MPS shunt cases even though the drs can see the occlusion typically at the proximal (tip) of the shunt when doing revisions that we may have issues w partial blockage all throughout our shunts with these GAG (complex sugar storage) deposits (because GAG storage is clear and not visible to the eye) and so when they fix a part of the shunt it helps CSF flow somewhat better but not complete resolution of the issue? I dont know but wish I did.. The reason I wonder this is because ive always felt so much better (near 100% improvement) right away after shunt replacements vs with revisions I typically only feel partially better..
We have been slowly adjusting the Codman valve down and right now are sitting at 50 which is about 3/4's of what we can do (settings range from 10 to 200) with the tentative plan we will adjust the TPL shunt up from 0.5 to either 1.0 or perhaps a little higher. The thought behind this as I think ive written in other updates is the VP shunt which is basically the main of the 2 shunts or does more of the work (I think, I think Dr.Bragg has said) can help keep the Thoracic Pleural shunt open if the TPL shunt is set a little higher and thus drains less so collapsing less (I cant quite explain the reasoning, will have to ask Dr.Bragg). Dr.Bragg came in this morning saying she was thinking about last summer when we did the trial with the then LP and VP shunt in which we turned both shunts down as low as they could be set (.5, both where Strata programmable valves) and did serial shunt taps with the shunts set at this low setting and then also with the shunts set at (I believe thinking back) the VP, .5 and the LP at 1.0 and then VP, 1.0 and LP 1.5 (or maybe vice versa) and where able to establish with little question that the shunts both drained more effectively at slightly higher settings. Im not sure we will do the same "test" to figure this out but we are likely going to be playing around w the settings on each shunt. I do feel a little better than when I came in for this surgery and so while I wish I felt superb and near 100% like I have w the external drains or like the times we've put in complete new shunt systems I am glad to have this small step to work with.
Although I saw the Neuro-Opthalmologist just shy of 2 weeks ago (for fup, repeat visual field testing) I am seeing the local Optometrist a week from Tues to get my glasses prescription changed; this eye dr had thought last fall when I got these glasses and new rx that I really needed stronger ones but also thought I wouldnt be able to adapt if he just threw me in to the new strong rx right away so he wrote it for somewhat less. The prescription has a 1 year warranty so while I have to pay for the appt any new lense prescription will be covered under the warranty (thankfully!) from last fall. I am actually looking fwd to getting a new, stronger prescription as I think we've been dealing w these shunt issues to long and not enough of positive pressure change or over long enough period that my vision goes back to complete normal. Hopefully I will be able to read the computer screen and a book more easily again; I miss reading!!!
Ive decided I am going to go out to Boston mid-April for the Boston Marathon and to cheer on my Genzyme runner (she is part of the Genzyme 'running for rare diseases' team and running on my behalf to raise awareness of MPS I + is raising funds for research for NORD (I am fundraising for the Nat'l MPS Society and the link is on the side of my home page). I plan to fly in that Sat (13th) and stay w a friend of mine, an MPS III family (they are fantastic, run the Ben's Dream Foundation) and will meet up w Jess and the other runners + rare disease pts (representing other rare diseases Genzyme has therapeutic/drugs, programs available for) for a dinner and then on sunday we are doing a Duck tour of Boston (have been to Boston 3 times, including last summer for the Nat'l MPS Society Conf.) but never done a duck tour as I missed the one that was done for adults affected by MPS at the Nat'l Conf. last fall bc of planning/running the meeting for MPS I families regarding several researchers and the drugs they are studying that same night). Monday is a breakfast at the Allston plant for Genzyme (where some drugs are made though not Aldurazyme which is made in Novato, CA at the BioMarin headquarters and then shipped to Boston-Genzyme). Afterwards is the race in which the team meets close to where Jenn's family lives which is awesome, divine intervention! :) I plan to fly home Tues but am booking my flight once I get home this week. Below are pictures of the long sleeve t-shirt and stickers I created online (zazzle.com) for that day.
Long sleeve t-shirt designed (front) for the Boston Marathon
Back of the t-shirt
Stickers designed - Both things made on Zazzle.com
I will update when I know something more, thanks for stopping by,
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