Thursday, July 31, 2014

Neurosurgery post-surgery Appt. Looking at IT/Dr.Dickson, talking to Plastic Surgery/UW

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!

zzz 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!

Saturday, July 19, 2014

Updated: TPL Shunt site better post-surgery. VP Shunt iffy? Discharged Fri (18th)

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,
Ps Sorry I realize this update is fairly scattered and not all that well written!

Tuesday, July 15, 2014

TPL Shunt revision (#30-s/t), shorten pleural catheter

Although not bad news at all I really am forcing myself to update this, for whatever reason today I have little energy and typing this update out feels daunting!
I originally wrote out notes yesterday but then must have accidentally thrown that sheet out so am trying to remember details by memory on about 2 hrs of sleep (an hour-ish early this morning and an hour-ish around 12-1 or so. Neredless to say right about not I am feeling INCREDIBLY grouchy and irriated and just want peace with quiet with no one coming in here!
(One of the Pastors from the Hospital stopped by and honestly I asked him if he could come back in a little while, I just needed to be left alone to finish this and something else I was doing + am not really feeling like making small talk. Sometimes I just get soooo tired that I'd just like to be left alone!

Surgery which was scheduled at 1pm yesterday (arrival for labs at 9, check-in to 1st day surgery at 11) started about 1:30 (Dr.Bragg was in clinic and when she came by said she'd had an un-scheduled patient show up, not a big deal).
Really the Head Anesthesiologist and 2 Resident Anesthesiologists working w/her on my case had been standing around just chatting with my Dad and I while we waited.
I liked the whole team and could tell the head Anesthesiologist had done her homework on my past history + was well versed in the cardiac issues and what she would do differently. Her big concerns where to not cause fluid/cardiac overload and keep surgery (which was very short, intubation took much longer than the actual surgery) smooth.

Overall they did the pre-surgery cortisol replacement (there was discussion by the Anesthesia Team if it was really needed given the short length of the actual surgery but per what my Neuro-Endocrine dr has stressed to me numerous times the cortisol is as much for the induction/anesthetic drugs as it is for surgery. Cortisol deficiency with any stress of the body causes stress to the body systems and is why the cortisol has to be given 30-60mins prior to any anesthetic meds are given when possible.
It worked out fine as the Head dr said to me, along the lines of "You've been around the block w these surgeries and you know what your dr has told you regarding your case".

Surgery itself literally took about 15-20mins and if I remember correctly when they where hooking everything up, adding the anesthetic/induction meds it was about 2 hrs after this that I woke up in recovery. I am not exactly sure on any of the total time length though and doesn't much matter given surgery went good!
The Peds General Surgeon w/Dr.Bragg there basically just for "moral support for the Surgeons" (her words, lol) =) just used an endoscopic approach w 2 small incisions in order to view and to move their instruments.
They pulled out the pleural space (tip) of the TPL Shunt catheter, cut off about 1.5-2" and replaced it back in to the lung, which I believe they deflated for this surgery (a big concern the Anesthesiologist had given my airway issues and instruments she had to use).
Apparently per the Team they could visualize where the catheter was in fact sitting up against the chest wall and could see were it was causing irritation, which in turn explains my symptoms of irritation most especially when it seemed like the TPL Shunt would be trying to drain.
With the re-positioning of the catheter Dr.Bragg said it is short enough now where it should not touch any areas in the pleural space and as an added benefit we should get less resistance from the catheter which equals (we hope) more CSF flow through the catheter. That would be a good thing as we know I like to drain a lot and feel better the lower pressure is!
Thankfully the TPL catheter pinching/poking feeling has been pretty non-existent today and any discomfort there is either incisional or b/c Dr.Bragg said even with their draining excess fluid out of the pleural space during surgery there was still some that would absorb (they hope) on it's own.
Other than this positional catheter issue we've definitely had much better luck w the TPL Shunt than we have with VP Shunt functioning fully or well.

Even though the VP works less than optimally and I do have headaches/nausea/some blurred vision (intermittent) it as I've said before is still wayyyy better than when I only had 1 shunt and was endless problems.
I am sure it is at least somewhat improved now b/c the TPL Shunt takes some pressure off of the VP Shunt and they almost seem to balance each other with the TPL Shunt seeming to work more effectively and better, longer.

I feel like if we could figure out what causes the TPL Shunt to work more effectively than the VP Shunt and we could figure out a way to translate that effect to the VP Shunt we'd honestly be so much closer or perhaps as close to perfect as you could get with pressure symptoms.
Our biggest issue is Dr.Bragg is just unsure how to better improve the efficacy of the VP Shunt draining especially when I am asleep. I do sleep semi-propped up on my couch, always starting out sitting up but I think due to positioning/joints/neck I end up only partially upright.
I would LOVE it if we could figure out how to better make the VP Shunt work but hopefully we could keep brainstorming and figure something out in time? In the mean time you just do the best you can, someday's better than others with symptoms.

I will say as 'minor' as this surgery was ie very short and just 2 smaller incisions on my side and back even these smaller surgeries still kicks my butt both discomfort/pain wise and overall! =/
We are in the process of slowly weaning the PCA pump (patient controlled analgesic, I control the short acting pain med dosing based around how often Neurosurgery has it set that I can deliver med.) I am sure the difficulty with surgery recoveries in the first few days pain wise is due to so much scar tissue and so much GAG storage (from the MPS storage) which causes much tougher tissue.
Thankfully Dr.Bragg has a decent system in place for managing post-op pain w the PCA (self administered) pain pump. And she realizes for whatever reason my recoveries are just not a typical (whatever that is!) shunt patients recoveries.

I do know given we had turned up the VP Shunt setting by a notch a few wks ago it's possible if need be Dr.Bragg might also let us re-adjust that setting back down if H/A's don't improve.

Friday once I knew surgery would go ahead I called the Neurosurgery floor Pharmacists and they in-turn ordered the Aldurazyme to be given while here.
My Genzyme Insur. Nurse Case Man. was also watching for the order to make sure it go shipped out on Fri from their end.
This will likely be given on Weds morning now to give us time to fully wean off the PCA pump.
We'll also use the same ramp up rate this time that Stan (Nurse here) calculated last time. This is different than how it is ramped up at CHW, my normal clinic but something is done differently here maybe with how the drug is mixed as we otherwise where running the infusion at double the needed rate, giving it to quickly.
We'll also restart the Coumadin dosing tonight I believe and once released i'll go back to monitoring this via my Cardiology Team at CHW.

Due to my being in-patient the WI Team from Genzyme came up here to visit this morning which was actually really nice!
I'd only met Karen (Patient Care Liaison) before as she lives in the Minneapolis area and we frequently have dinner while I am there from time to time and see each other here and there at meetings.
Amy, my Insur. Case Man. at Genzyme (located Boston) as well as Carol, who is the WI Drug Rep for Genzyme (lives in Madison actually, a little bit newer to the team) where with Karen also and we had many good laughs, funny stories to share. We're all cat lovers so that was like an instant bond between Carol and I especially!
They all really liked the MPS-Aldurazyme bracelet (using the caps from the drug) bracelet I have/made and so my next project is to make one of each of these for them. I told them it would take atleast a few wks but I think it's just cool they liked the bracelet that much! =)
It's amazing the people in the Med world who really like this idea and it is neat b/c 1. they are intimately involved in MPS I pts lives everyday and 2. their company bottles the drug.
Jessi, my Boston Marathon runner also has one of these and had sent a pic of herself wearing it after she got it in the mail. =)

Going to sign off for now but if I've forgotten anything i'll re-update this and otherwise will update sometime again soon.
Hopefully I get out tomorrow or any day now; even if we still are dealing with headaches it's just nicer to be at home at a certain point!
Here are a couple pics from today with Amy, Karen and Carol this morning.

Thanks for stopping by,

Friday, July 11, 2014

Surgery details (TPL Shunt, 7/14)

Pre-op went ok, labs done the other day where fine minus one slight abnormality w RBCs but the dr figured that probably had something to do w the Coumadin. The paperwork got in on time, this dr was really great about filling it out while I was there, we needed the fax number for Dr.Bragg's ofc so I called and was able to get that. And he'd already rounded up the labs results and a prior Cardiology report to include with his paperwork to be faxed.

I talked to D6-4 (Neurosurgery) Pharmacy yesterday and they where going w the plan we laid out back in March (last surgery) regarding ordering the Aldurazyme and tentative plan is to give this Tues. morning, in-patient.
As far as surgery it looks like actual check-in for that would be 11:00 so I presume surgery is at/around 1:00 (Monday mornings are a clinic day for Dr.Bragg I believe). Per Neurosurgeries wishes I have to have labs done in the same 1st day surgery at 8am. This is if I understood correctly to look at INR and make sure it is low enough. I am not sure what i'll do in that time in-between but if I can leave maybe go to Target nearby or something.
From what Dr.Bragg's Secretary told me the other day apparently they would cancel surgery if INR where not low enough but then today when we talked she made it seem as if the time between Labs and surgery check-in was so they could get the labs back in time and have time to make a decision?
I am not entirely sure if that meant where the level slightly high they would proceed with surgery and manage it appropriately but then if the level where to high they would just cancel?
In any case my last dose of Coumadin was Weds night so even thought my INR was high when last measured and I was kept on that same dose by Cardiology (they had wanted to see if it would level out on it's own by this Monday when I'd have re-tested at CHW) the level should drop in time for Monday with 4-5 days in-between the last dose I took. I am sure praying!

On a completely different, 360 degree turn we found out yesterday one of my Cousins was killed while riding his bike.
He'd had developmental disabilities since birth but was in his mid-to-upper 20's and was struck by a Truck while riding. I really don't know any other details right now.
I am just praying for my Aunt, while we weren't particularly close as kids (we really only saw each others families on some parties or holidays) this is the 3rd child my Aunt has lost (her daughter before I was born died a few days after birth, one of her sons had severe autism and drowned in a neighbors (of theirs) pool while being baby sat and now Chad.
She also lost her husband, my Uncle several years ago. This comes after my Grandpa, her and my Dad's Dad passed away in Nov. and we've lost other cousins and family in recent years to.
I just cant even imagine what her grief is like right now. I know God has a reason behind everything he does and gives to a person but I cant help but think why does one person have to experience so much loss, as in her case?

I'll update again when there is anything new or otherwise sometime after surgery.
Thanks for stopping by,

Thursday, July 10, 2014

TPL Shunt revision, Monday 14th

Dr.Bragg's Nurse called Monday with 2 thoughts. The 1st being Dr.Bragg thinks moving the TPL Shunt catheter (the tip which sits in the pleural space) would help the pinching/poking pain that occurs quite intermittently (seems to be when the shunt may be trying to drain?)
Per the CT Scan done last wk from what Dr.Bragg told Lisa (her Nurse) the tip of the catheter appears to be sitting up against the lung space (maybe she explained it as the chest wall, not entirely sure) and so with General Surgery Dr.Bragg (along with General Surgery) will adjust the location of this catheter. It should be a relatively straight fwd surgery from what they said and we can hope! =)
In all reality I have zero desire to have another surgery but at the same time this just keeps on recurring day in and day out now and literally days like yesterday there where periods when this was occurring it almost takes your breathe away when you breathe in. It doesn't occur constantly (thankfully) which I am sure makes it seem to some like why would you want to go through a surgery for that but I can't explain nor really do I have to explain my decisions.

I am hoping as a secondary affect this adjustment might also help the leg/nerve pain (starts almost low back travels down L buttocks to the back of the leg down and begins when standing or sitting any period of time).
I don't think Dr.Bragg is planning to adjust the proximal (end) catheter which lies in the spinal canal, from the MRIs I think she had said something along the lines of that distal (end) catheter was sitting closer to one side of the spinal canal than is normal which she thought could possibly explain the leg/nerve symptoms.

I am hopeful even if it might be naively so that adjusting the tip could perhaps relieve the spine-nerve symptoms to?
As good would be if by a slim chance it helped the positional headaches to; these occur some during the day but most definitely worse at night/morning if I don't sleep staying propped up. These H/A's do improve after a few hours but reoccur some throughout the day just much milder.

This surgery was originally scheduled for next Tues but b/c Dr.Bragg needs the General Surgery Team to help her move the catheter (due to so much scar tissue, especially from the repeat OHS (2 open heart surgeries) so now surgery is actually scheduled for Mon.
Per their wishes i'll have to be at UW at 8:00am to do labwork in the Surgery check-in area. This is in order to check INR, which apparently if it where not low enough they would cancel surgery? I don't quite get that part as they can do plasma to bring INR down to a normal/surgery appropriate range if it had been to high? In any case instead of stopping Coumadin 3 days prior to surgery which is Cardiology's wishes, Dr.Bragg's Team recommended I stop today.

Cardiology called Monday afternoon after doing another INR level (while at ERT), that level was high (3.63) so their plan is (was) to keep the dose the same.
After surgery we'll re-start the Coumadin again which will likely be the same dose (8mgs, 4 days, 6mgs, 3 days) as now.
INR testing would resume 24hrs after surgery and then once I am out-pt.
The Pre-Op Appt promises to be interesting, my PCP is out on Maternity leave (I am hopeful she comes back on her regular schedule and not another PCP who goes extremely part time but we'll see and is what it is really.
It definitely does make it easier when it is one dr routinely doing pre-op's and coordinating various other aspects of care..
Anyways the pre-op is actually at a completely different Dean Clinic in Madison and with someone I've not met so I am hopeful things will get in in time, Dr.Bragg's ofc has to have the paperwork by 4pm. Anna, her Secretary asked me to call at 3 Fri to verify if they'd received the paperwork and if not she (Anna) will call and work on getting it. I think they really want to make this possible for Mon.

On a different note the 3 Genzyme Reps for WI will be in-state next week, we had originally planned to have dinner but then went to plan B. Instead as of right now, we're planning that the 3 will come to UW Tues., morning.
Karen is the Patient Liaison, Carol is the Drug Rep (and actually the only one from WI), Amy is my Drug-Insur. Case Manager at Genzyme and is from Boston. I am looking fwd to meeting Amy and Carol and seeing Karen again! I guess if nothing else this will be something to look fwd to given I am not that excited (at all) about another surgery. =/

Will update when there is anything new,
Thanks for stopping by,

Thursday, July 3, 2014

Neurosurgery Appt

Another Appt w Dr.Bragg, basically there are 2 thoughts going on at the moment.

1. Being Dr.Bragg said she really would like to figure out a way to make the VP Shunt work more efficiently as she feels the TPL Shunt does more of the work and though I am having what we think is the nerve irritation symptoms she does think the TPL Shunt is working atleast fairly well. She commented that she would really like to find a way to improve how well the VP Shunt drains as she thinks that would help us overall.
2. The other problem is the TPL Shunt irritation and figuring out where that is originating from ie is the proximal (top) of the catheter sitting at the bottom of the lung space and thus causing irritation and the intermittent (but really, reallllyyyyy uncomfortable) pinching/poking like sensation or is the distal catheter in the spinal canal the cause?
Based off last weeks MRIs she had commented the distal catheter is sitting closer to one side of the spinal canal than where they would prefer a catheter be (typically the catheter sits more centered) and this could also be the cause of the intermittent but fierce leg-nerve discomfort and sensations at the front and along the shunt catheter.

We opted to do a CT Scan to get a better view of the catheter within the lung space to see where the catheter is sitting.
She had also commented there was fluid within the pleural space where the shunt drains which would be the CSF draining, though this should typically be absorbed vs sitting within the lung. I am not sure if she saw that on the MRIs maybe.
Secondly she thought we should try turning the VP Shunt setting up, see if this might by some chance help the shunt to drain better; I typically do better at very low ICP (intracranial pressure) but this also can cause the shunt catheter to collapse intermittently and thus more likely for obstruction.
She did depress the VP Shunt reservoir and commented this was slow to refill (stayed indented) which is I think why she wanted to try adjusting the shunt setting to drain a little less, see if it might help the shunt overall drain better.
Apparently there is also a reservoir on the TPL Shunt (maybe there is a way to check flow on the shunt but it can't be tapped, I am not entirely sure) and she said this refilled normally, which is atleast good. =)

In my ever brilliance I didn't think to ask her what I should do if the VPS setting re-adjustment didn't help or for that matter even if it did but the TPL Shunt discomfort keeps occurring?
We clearly wouldn't do 2 things at once and per the NP who helped set up the CT i'll call to try and get the results Monday but it just never occurred to me am I supposed to make a f/up appt if things don't improve or would we schedule something for either shunt over the phone?
Argh sometimes I could kick myself for my lack of ability to think quickly on my feet when we are talking about things! I honestly think it is that I am processing the info we are talking about and just need time to think through everything before I can really come up w these (what would sure seem like!) basic but kind of needed questions!
I am sure I can always if need be email Dr.Bragg but would rather not so will see what her Secretary says Monday.
If we did end up doing something to the TPL Shunt due to previous scar tissue from the 2 OHS's she would use the Peds General Surgeon again to help with placement of the catheter.

I guess we'll see how things go and hope something improves!
Thanks for stopping by,