Tuesday, April 8, 2014

Feeling discouraged..

This has been a semi-difficult and not altogether enjoyable past 1 1/2 wks - sure being in the hospital is never fun but being here when your trying to figure something (the shunts) out and feel you keep running in to a brick wall is down right depressing. This is definitely not anyone's fault just the way it is with MPS and complex underlying issues w the Hydrocephalus.

I can completely have patience to make adjustments and give it time but admittedly haven't felt very good since mid-late last week when we made the 1st (2nd maybe) adjustments to the VP and then TPL Shunts; the first 2 times w noticeable improvement as they where over draining but since then it feels like we neither make headway and symptoms remain un-changed ie afternoons are ok but nights and mornings I down right dread. I joke that maybe I should just not go to bed but really it wouldn't matter as mornings are difficult due changes in dynamics with how CSF flows and/or is absorbed at certain times of the day.

I don't want to be here in the hospital but I also dread the next few weeks at home; I mean at a certain point you just have to push through symptoms but certainly still doesn't make it any easier when you feel 'driven over by a semi truck' tired and vision + head are so miserable due to the pressure changes and in my opinion one or another of the shunts just not working at full capacity.

Honestly in talking to other friends both whose kids have MPS and Hydrocephalus and non-MPS Hydrocephalus parents I feel like even though our shunts typically show as 'working' once partially revised (at the proximal or valve locations) but there (again my opinion, my friends opinions) must be something playing in to the overall drainage ability of the shunts to cause the entire shunt(s) to look as if they are flowing (and they are) but isn't flowing as well as when complete shunt replacements are done?
Why this is I have no idea but several of us wondered in the case of MPS is the protein in CSF along w the GAG storage cause the shunts to get 'mucked up' but still able to flow partially? In non-MPS Hydro cases it is likely similar issues probably having to do w protein or something else in the CSF of some of us pts. along w for all of us likely compliance issues in our brains (so inability to adjust between working fully and partially working?)
I have a terrible memory for asking people like Dr.Bragg questions but am hoping I will remember to ask her what she thinks we might be able to try if another small adjustment to the TPL Shunt tmrw doesn't work? I believe she is off until after Easter (good for her, bad for me!) so the next couple wks including my trip to Boston are likely going to be exhausting and probably a little depressing..
Another MPS parent asked me the other night if I thought IT-Aldurazyme would help our shunt/compliance issues and honestly I don't know but I know Drs. Patti Dickson and Emil Kakkis both feel it might help us. I would give just about my L foot to try it and see if it helped!
This is another thing I need to try and remember to ask Dr.Bragg (if she thinks there is anyway possible we might be able to get a single pt.-off label IRB to try the Aldurazyme in to the VP Shunt? It's something I've written about here, that Dr.Bragg has expressed interest in but felt it would be really difficult to get an approval here. Drs. Dickson and Kakkis have said they would help but I don't know. I wish b/c these shunts are going to drive me to start drinking (I don't much like the taste of alcohol)! =/

Needless to say this has been a fairly difficult couple wks, again not anyone's fault just kind of depressing how very good the shunts can be when they are good and yet so frustrating when we try various things and no or little overall improvement. I did have hope at first till probably Sun/Monday the adjustments would help but I am also not holding my breath that when we adjust the TPL Shunt down tmrw it will make a significant difference
I haven't given up hope just feel discouraged I guess as well.

Anyways sorry to write such a down update,
Thanks for stopping by,

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