Sorry for the delay in writing an update; I am home (Fri) which if nothing else is nice! I hate writing these updates the most as I can imagine it must get tiring for those who read this to continually read about problems. I promise I would love to write all good updates for once to! =) To boot uncertainty makes me apprehensive (atleast in regard to the shunts) and it is a bit daunting (scary?) not having a plan.
I do atleast feel a bit better of the whole situation today than I did over the past few days. I do just want to feel better though!
Due to what I am more than certain is a valve issue and on-going, slowly worsening symptoms (VP Shunt) Dr.Bragg opted to do a shunt tap Fri morning which showed the valve is working but seemed was slow CSF flow (not entirely sure) when she was pulling off fluid. In the past this has been indicative of partial or intermittent shunt blockage; there have been numerous times we've just ended up revising that portion of the shunt (the valve).
She was able to remove with the shunt tap I think she said about 2cm (or 2cc's?) of CSF for which I can only best describe as it felt literally like a heavy cloud (cobwebs?) is partially lifted from my head. A very odd but not terrible feeling.
It's very clear the symptoms at night/morning especially had gradually gotten worse over the past few weeks. My symptoms after this shunt tap where virtually back to 'normal' by Sat morning, meaning I did not feel as good as I had headache wise after we did the shunt tan and took off some fluid.
I trust that there is a reason for not being certain what to do next but admittedly feeling like crap a good percent of the day and "just doing what I do at home" to get through it sucks (though I am happier to be here than to be in-pt). Atleast at home I control my day to day. It would be sooo amazing to just wake up and not have to feel aweful, literally feeling like your waiting as the day slowly gets better!
Even with some relief from the shunt tap (the first 24 hrs anyways) my vision is screwed up (fuzzy I guess, worse with reading) and head a day later feels like a lump of coal sitting on it just initially was not as bad as pre-tap! The symptoms do occur intermittently and worse at certain points of the day.
I think with the CSF that was pulled of with the tap she was planning to send off the CSF sample for testing though it is unlikely there would be an infection (no symptoms) and she I guess just wanted to be cautious, rule things out. I am ok with that, glad I guess she wanted to try and cover bases.
The bad thing with our relieving some pressure Fri morning is of course the headaches return and sometimes that is worse than not getting any relief till we figure out a actual plan! You can almost taste feeling better, longer with times like this where some pressure is relieved! Still I am glad even if it wasn't perfect relief to have felt a little better.
BR>
We do know the TPL Shunt is working (a very good thing!!) based off flow during the TPL Shunt revision surgery on Monday. Thankfully the pinching/irritation symptoms from the TPL Shunt rubbing is also improved following surgery which is AWESOME! One of the incision areas from Monday's surgery surprisingly is still sore. This was done via laparoscopic (tiny) incisions but perhaps it's the location that causes the discomfort. ATLEAST the pinching/discomfort along the TPL Shunt is virtually (if not completely!) gone post-surgery!
As far as what is our plan, I believe Dr.Bragg when she says there are options but it doesn't make the time while waiting to figure out what the next step is any easier. Even Dr.Bragg seems to be struggling with what to do but better she takes her time to figure out a plan I guess. One possible thing she has circled back to is doing the cranial vault
expansion surgery (where they go in a literally shave down some of the skull bone - if it sounds aweful to you believe me it sounds AWEFUL to me to!
The Peds Plastic Surgeon that helps Dr.Bragg w skull expansion surgeries is out till the 1st of August and I think it would likely be something we'd take some time to investigate (If it would work w the underlying MPS). I am more than leery of it!
In my case I think she said they would only (only!) need to shave down the skull bone and would not she believes need to reshape the actual skull. Does. Not. Sound. Awesome! I really don't know any other details about this but I think she is concerned we keep having to revise the shunts over and over and over again with limited success. It's to bad we couldn't figure out a way to replicate the TPL Shunt function (which we know works best out of the 2 shunts) in the VP Shunt!?
As far as recovery it seems if I am remembering Dr.Bragg right that is about 2 wks post-op in patient and they use plates and screws to hold the skull back together. Can you imagine? UGH!
I've talked to a couple drs in the MPS community about the potential skull bone thinning
and both feel this may not work and little precedent (if any) for it's use in MPS. The one dr must have quickly done some research to see if she could find any published data on it's use and didn't have a lot of success.
The other dr who has a ton of experience (dozen of years) with MPS I pts brought back up
the potential to look in to IT Aldurazyme via the shunt reservoir which is something I wish we could work out.
I guess i'll ask Dr.Bragg if it might be something we could look in to again and would she be willing to talk to Dr.Dickson (They had talked one other time)? I know there is 1 other MPS pt (not a shunt pt though) whose local dr was able to work w Dr.Dickson to get the IT (Aldurazyme) approved for out of study use.
Dr.Dickson has said she would be willing to try and help Dr.Bragg get it approved at UW if she wanted/was interested. I feel like we should try to look in to everything.. The idea behind IT in to a shunt is that they temporarily re-program the shunts to a 'virtual off' setting and inject the aldurazyme via a reservoir in-line w the shunt catheter.
Dr.Kakkis feels there would have to be a one way valve (not sure if the Codman Hakim is a one way valve). The thought is that Aldurazyme given directly in to a shunt reservoir may help clear our GAG storage shed from the ependymal
Dr.Kakkis also wondered if some type of reservoir could be used to flush the shunt catheter out with forced back pressure every once in awhile.. I am curious about this
and something else to fwd to Dr.Bragg. I do know the catheters clogging is not unique to MPS pts but am unsure if it is more common with our disorder or a different unique? It seems it might not be but again feel like we have to look at everything..
Something I have wondered but didn't think of till after I talked to Dr.Bragg is I wonder can Hydrocephalus pts who do well draining a lot of fluid ever get shunted
without an actual valve in place (valves control flow) on their shunt? Something to keep in mind to ask.. I would imagine a valve is needed but is worth asking.
I know as far as ideas she was going to talk to her Codman Rep about a siphon guard valve (controls amount of flow when person stands up) but she (and I for that matter) aren't sure that is a good option as I do better the more CSF I can drain.
I am doubtful she would decide to go that route and as much as I want to feel better I guess I am ok her taking her time trying to figure out a better option.
I guess I need to do better, trust whatever she decides and take these next few wks a day at a time.. I will do what I do well and that is enjoy the moments that are good while just living with the times that are not.
These periods in-between feeling good are definitely when my faith and trust are tested!
I'll update again when there is something to update,
Thanks for stopping by,
Erica
Ps Sorry I realize this update is fairly scattered and not all that well written!
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