A bit earlier I was talking to one of the former D6-4 (Neurosurgery) fl Nurses on fb about a book I gave her and we got to talking about Dr.Bragg/today's appt.
I said to her jokingly in talking about today's appt and how we had a tentative plan how grateful I am still that Dr.Bragg keeps trying and made the comment 'She's a good one Mr.Grinch'. I think the real saying is "She's a mean one Mr.Grinch" but really I con't to be amazed how willing Dr.Bragg is to con't to try to help make my shunts work better and my symptoms overall better. AND that she sees me mostly at good pts of the day but she still believes me! To be honest I am continually (but so grateful) amazed!
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I brought up to Dr.Bragg about how the 2 drs I had talked weren't real confident a cranial vault expansion (thinning the skull bone, create a bit more room for the brain to expand) would help w our issues w seeming not enough fluid being drained/headaches/keeping the shunts working. I also brought up how these same 2 drs have suggested to me I ask again about trying to do IT Aldurazyme and I wondered if she thought this might be something we could try to get approved at UW as an outside study?
Dr.Bragg made the comment when I asked her that she didn't know a lot about IT as I am her only MPS pt and that we would have to try to get an IRB (internal review board) approval at UW but she was open to talking to Dr.Dickson and I should send her the contact info.
Dr.D and Dr.Bragg had talked a year or more ago about the actual IT Study in CA but I wouldn't likely qualify, most especially due to my shunts, repeated spine surgeries and unlikely ability to have LP's which is their means of injecting the Aldurayzme intrathecally.
When I was originally in Dr.Dickson's first Cord Compression IT Study we had had to get an IRB exception to do the injections in to the cisterna magna (base of the skull) due to my then LP Shunt.
Dr.Bragg made the comment we would have to likely insert a reservoir to inject the drug via likely. I think per these 2 drs their recommendation had been to me they thought a way to virtually turn the shunts off for a few hrs (or turn as high as they can, to drain as little as shunts can) would be a good idea (to allow the drug to bathe as long as possible as Aldurazyme doesn't otherwise cross the BBB during the weekly IV (Port-a-cath) Enzyme Repl. Infusions I get.
Dr.Bragg is going to talk to Dr.Dickson (Patti replied almost immediately to my email) and see what Dr.Dickson (Patti) suggests as far as going about trying to get approval and if approved how to set it up.
Additionally Dr.Bragg and I talked about the skull bone thinning (cranial vault expansion) and agreed we'll keep looking in to this, she'll talk to her Peds Plastic Surgery colleague who returns next wk. and see what that dr (Dr.Mount) thinks.. ie does
Dr.Mount think w the underlying MPS it could help, is it feasible, etc. If need be we'll meet the 3 of us and discuss that. For now it's sort of our least desired option which I am more than ok with!
I could tell Dr.Bragg really wished she had some way to help immediately today. She asked if there was anything I wanted (thought) she could do to help? She had the shunt re-programmer with her but we opted to leave things are as at least while very imperfect
I can take Zofran and it helps w nausea. If it gets very bad (some nights) I can take a exceedingly small amount of the valium to get back to sleep (this is like a last resort to me).
Via pumping the VP Shunt reservoir the refill time was ok. Our suspicion is always that this is the shunt that works the least effectively more due to overdrainage (even though I do best draining a lot) and thus the ventricles collapse, sucking brain debris in to the catheters. Our wish is really to find a way to make the shunts work tip top.
We discussed the TPL Shunt fluid seeming to accumulate but I didn't think to ask her about the strong discomfort that keeps occurring (and PT is really trying to help) down the back of my L butt and L/hamstring area.
Honestly I am impressed with myself that I at least remembered to ask her all the things I did though (!!) so I think it was a reasonably successful and accomplished appt!
One thing I did ask her was why she might think I (and others I've talked to have commented the same thing) would notice a greater difference in symptoms when we do a full shunt replacement vs when we do a partial revision (just a small part of the shunt)? She wasn't really sure though one thing she thought was possible
was that with a full revision you are under anesthesia for longer and this impacts CSF pressure longer, to a greater degree thus you may feel like you feel better (or something like that).
I honestly am not sure I think that is true, nor has it been my experience but maybe for some it is. In my own case I think anesthesia no matter what seems to impact my body for days but not necessarily in a positive way.
In my own case though I think that has a lot to do with the endocrine hormone issues, the affect anesthesia and pressure has on these hormones and although it makes a significant, positive difference (the steroid use for cortisol stress dosing) my body still seems to go through some stress response. I might be wrong though.
She also commented to the Resident and Student that where with her when we where talking
about the possibility of IT, about her talking to the Plastic Surgeon next wk and about
the shunts in general that our best that she has seen cont's to be with either the External Drains (EVD's) or when we had the external (but under the skin) CSF fluid leaks. I would have to agree with her on those thoughts! While having the 2nd shunt as I've written about before atleast helps b/c there is always atleast some pressure being drained having the 2 shunts is no the same as how good I felt w the CSF leak or the EVD's!
We did discus the possibility of adding the siphon guard further down on the VP Shunt to prevent over-drainage which in turn leads to the shunts occluding. The thought behind this is preventing over drainage would in theory or they've been able to establish help avoid the pressure having to build significantly in order to force the catheters back open after over-draining.
Her (and my) concern with this is I do best the more I can drain and anti-siphon guard valves allow only limited amounts of flow and I believe are positional. It's something we've only semi-ruled out I think but also not entirely leaning towards as out best option.
After the appt I went up and visited some friends who are in-patient. Their little girl
has a lot of shunt issues like I do though sees Dr.Bragg's partner.
Shannon often comes to visit me while I am in-pt and I genuinely enjoy talking to her and her husband. I wish I honestly I knew better how to help their little girl feel better though (but I am sure they do to!
You would think I guess given we have the same shunt problems it'd be so easy to know but it's not! I am really glad I could visit though.
Shannon is who will often access my Port these days when surgery is on a Monday or if I can't get to Milw to get it re-accessed (Noodle, her little girl has a Port and Shannon is a Lab Tech).
Honestly, I kind of think she and her hubby are awesome (fairly close in age to me), all of their 4 kids are adopted with 2 having special needs (their little boy is similar needs to Zan and also high functioning though I don't know if he is technically on the spectrum) while Noodle has the shunt and I believe CP. They are also in the midst of finalizing adoption of another little girl from Krygzstan (sp) and Shannon flies there in
less than 1 1/2 wks for the Court appearance!
Thanks for stopping by,
If you believe in prayer, please pray we are able to sort out the above things in a not to lengthy amount of time and also find a solution to the shunt issues!
I am so very grateful to have Dr.Bragg on my 'Team' and that she never seems to give up!
Erica
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