Wednesday, June 27, 2012

Ooh this crazy, crazy shunt and MPS world...

A day late but perhaps not a dollar short writing this and updating on yesterdays neurosurgery appt. The appt was actually moved from mon which when Dr.Bragg's secretary called and asked if I could do Tues instead of mon I was more than willing! Driving an hour to ERT, sitting there for 5 1/2-6 hrs and then driving another 1-  1 1/2 hrs to Madison for a shunt tap, etc and then another hour home did not seem all that appealing! By the end of ERT I was pretty restless (a ton of phone calls that day and paperwork) and just wanted to go home irregardless of how my head felt!

So Dr.Bragg did another shunt tap which worked ok - the shunt valve is set at higher setting so is a double edged sword; we turn down the shunt to drain more and headaches improve temporarily but then the shunt tubing/catheters clog. We turn the valve up  2 settings higher and helps keep the catheter open but we end up with worse headaches due to not enough CSF draining. Neither is awesome... She drained about 20cc and then the discomfort along the shunt catheter began so she stopped. I actually felt aweful last night between chills and sweating (fever of 102)  and horrible utterly horrible stiff neck so I pretty well just tried to sleep the majority of the rest of the evening and night on and off. Im not sure what that was about as headaches where better it was just that my neck was soooo stiff everything and everything hurt. By the middle of the night that was improved and this morning completely gone so I think had or has something to do with the flow dynamics and perhaps the narrowing in my neck and rate of which we drained the CSF. I havent ever experienced that before so was a little unnerving!

Some of the things she talked about (while also admitting she needed more time to think about the situation) was to potentially take out the valve but we would have the same problem of keeping the catheter open with the increased flow so  she wanted to think about that some. I wondered also thinking about this on the same home what if we turned up the VP shunt to a higher setting and then turned down the LP shunt ie different balance of pressure though I think that would still be the same headaches are only well when both shunts are at lowest settings. =/ Ironically we do know that when both shunts are set at .5 my headaches are completely gone but that is what then leads to the shunt catheter occluding and so then of course headache issues again due to not enough drainage. Its a little humerous really if I didnt feel like such crap given it seems like such a simple answer (turning the 2nd shunt down to .5 again) but we know from our serial shunt tap 'testing' that the catheter then just re-occludes and when turned up to 1.5 the catheter stays open better but not enough CSF draining.

Something we talked about 2 shunt taps ago and which I keep forgetting to ask Dr.Bragg was if she ever found any info on if the external drain catheter sizes are bigger than the actual shunt sizes as she wondered if they where could we use that in place of the shunt kit catheter. I tried search engining (sp?) several different terms regarding that and also pubmed and didnt find any info.. Something to ask. She asked me to email her and let her know how I was over next day or 2 so will maybe ask that.
She was going to look over the last MRIs and see if there might be a better place to put the catheter or if there would be a way to place a 3rd catheter (but not a 3rd shunt) and have it connect to one of the current shunt systems to take some pressure off the 2 shunt catheters (the VP appears to be working well which is good - the LP shunt has definitely helped that last longer!)  She wasnt sure about this and wasnt sure where she could even place a 3rd catheter if it where possible but was another thing she was going to ask around about.

Another potential idea was to put the distal (end) catheter in the pleural/lung space (thinking about this on the way home im unsure if that would work due to the restrictive lung disease??) as a different drainage source as it does appear in MPS pts that the peritoneal area doesnt work well in alot of pts.

Another thing she also wondered about was if there wasnt enough CSF in the 2 different compartments where the 2 shunts are and so each shunt helps but if we could figure out a way to create a altogether outside the CSF space area much like with the pseduomeningocele's (CSF fluid filled pockets we dealt with last summer) as that was the best we had as far as symptoms besides the external drains.  We know with my cervical (neck) area that  it is pretty narrowed and not alot of spinal fluid (CSF) gets through at several levels + there is the area I already have plates and screws in above this and that could be affecting this all though she doesnt believe that would be affecting the headaches. (I dont either as we've gotten complete headache relief is just a matter of keeping the shunts working).
In reality though with this LP shunt I did have a far better appetite (had gained like 9 pds in a period of a few weeks and have since lost same general amount )123 down to 114) in the past 1 1/2 weeks. I had much better appetite with the LP and VP shunts together so while headaches may not have been perfect they where definitely better and that is frustrating!!!! I happen to really like food. = /

She jokingly (im fairly certain she was joiking!) talked about which was kind of funny as I had sort of laughed about the idea to myself before this was putting in a 3rd shunt to trick the 2nd shunt in to working as the 2nd shunt has done a good job of keeping the 1st shunt working.  (was that just a mouthful?)I do think she is considering if there would be a way to put in a 3rd catheter (not a shunt) and connect it to 1 or both of the other systems to take off some of the pressure and thus we'd have the headache relief we are having a problem finding the balance for. This seems like the ideal option to me but I also know she didnt really know where to put one if we did and so I dont know what will happen.  She asked me to call her nurse early next week and we'll figure something out then. Who knows, who just knows?

On a different note my work with GSF has been going awesomely well and this week in particular has been really busy and putting in quite a few hours. I really like what I do though so not a bad thing! :) Right now we are working on and out details for a meeting at the Boston Conference related to MPS I and pretty exciting times in this crazy, crazy MPS I world. :)
About all for now - waiting for the weekend for something my Mom, sis, nephew and I are doing Sat and then Zan's B-day party on sun which also means seeing my sister-in-law and niece to! I am hoping to feel semi-reasonably human by Sat night (one can wish and hope, right?!) as id like to have Zan spend the night.
Thanks for stopping by,


Thursday, June 21, 2012

Neurosurgery take 3, shunt tap x's 2

Hi All,
I saw Dr.Bragg today and because the symptoms where so quick to come back yet again on tues after mondays tap in which we took off about 20cc's of CSF and turned the shunt down to the lowest setting she did another shunt tap and actually ended up tapping the VP shunt as well to drain CSF. The LP shunt tap was slooowwww and just to get off a couple cc's it took us close to 15+ mins I think  which compared to when we had the external lumbar drain in pre-this LP shunt we could drain that much CSF in a matter of a few very short mins - typically under 2 mins I belive it was w the drain vs this amount of time now. She tried both letting the tap free flow/drip as well as applying pressure/pulling back on the needle to get fluid off and many times commented how slow it was, sluggish and just abnormal. After she removed the needle the first time and the re-tapped the lumbar drain the CSF was again the sandy color although no debris this time so we arent sure what that is about. She had me sit up and we tried to see if the reservoir/shunt would drain better that way and no real change + both laying down and sitting up cont's to re-produce the pain from my L side back to the shunt line and around right to the valve and reservoir. She thinks maybe this catheter in spine is sitting up against a nerve root (not a great thing I gathered) and causing the discomfort - without even watching what she was doing I could completely tell when she was draining and when she was applying pressure to the needle vs taking a break - YEOUCH! Think of a dull knife being shoved in to your side and then someone kicking you in the back at the same time and applying pressure and that my friends is exactly what this shunt tubing feels like. Holy batman I say let me off! After she couldnt get much CSF off the LP shunt she tapped the VP shunt which was functioning fine and I could hear the bubbling but almost reassuring sound of the CSF draining (it's not a bad sound, just interesting) and she drained around 20cc's total till that to wouldnt drain anymore (but the reservoir wasnt depressed so we think may have just gotten air in) - I feel awesome tonight and could tell almost right away. I cant say how nice it is to feel good and not have symptoms even temporarily even though I know by either tonight or tomorrow i'll be back to feeling crummy again for the weekend. She did turn the LP shunt  valve back up to 1.5 to see if it will help force open the catheter again.

One of the things she mentioned if we end up re-revising this shunt is possibly taking out the valve on the lumbar shunt altogether  so there would be free flow of CSF but she also mentioned wondering if this would still prevent the ends of the catheter where there are tiny slits that drain the cerebral spinal fluid from clogging once again or not wbich is our cont'd problem.

Her other thing she brought up was possibly threading the spinal catheter up even higher in to the upper thoracic region (currently sits lower thoracic around T7) or even past that to the mid or upper c-spine area although with this which we talked about just briefly at the end of the appt she said she had concerns with there being so little CSF (spinal fluid) in the area already and the space being so congenitally narrowed (she showed me the MRIs of thoracic and c-spine the other day and the c-spine area was slimmer than a pencil or straw I think she had said and  is very little fluid there.)  There of course would be the concern of the previous c-spine surgery at C2-4 and hardware although that I believe sits outside of the actual spinal fluid canal (??)

She also mentioned possibly threading the catheter lower still than it is or has been but didnt seem to like this idea, I think bc there is so much scar tissue in my lumbar spine she wants to try and create a completely new incision and go above all the previous lumbar surgeries. She was going to cont to think some more on this over the weekend and we'd re-convene monday and see how the shunt tap goes with the valve reset at 1.5 again and if CSF flow is easier. I like that she is taking her time and trying to figure out the best solution and has all along as we clearly have no simple or easy answer for what to do with this shunt,
I see her for one last (we hope!) shunt tap on monday for which she is seeing me outside of her normal clinic as I first have to go to milw for ERT which takes about 5 hours total there and then about an hour drive to Dr.Bragg's clinic later in the afternoon. I figure i'll go in to ERT earlier than normal on monday. At this appt we'll also figure out what we want to or rather should do with the shunt issue ie if revision or what.

Otherwise its been a chaotic week working on some things for the advocacy-foundation position and gathering info which is time consuming (but not bad) and some tentatively good news with research there which is about all I can say so far.

I also have been caught up gathering info (thankfully with the help of my insurance CM) on a few providers I am tentatively going to switch as well as making sure paperwork is in before July 1 and also taking notes and numbers regarding some potential PCP's, My CM was given good reviews by a Pediatrician she knows through Dean of a Family Practice primary doc and also good reviews by the clinic staff of another Internal Med Primary dr who is younger and new to Dean/practice but apparently interested in my kind of cases.  I plan to spend part of Mondays ERT calling these places as well as the 2 other potential new specialists (a Hand surgeon though I like mine and so highly recommended!) and a PMR doc of which there are 2 potential options. Dean has so far approved Neurosurgery (hallelujah-amazing!) as in-network which my CM advocated for not having to change due to Dr.Bragg's knowledge and our on-going issues w the shunts, spine. The other 3 that are approved so far and easily where Neuro-Endocrine, Genetics and weekly ERT/infusion as the UW Genetics-metabolic doc told my CM I was better off staying at CHW which I figured. The 2 other big ones to me are Cardiology (bc he's adult congenital and the issues go beyond just the heart issues but having experience with the associated issues like restrictive lung disease and autonomic issues as well as understanding the Endocrine impact. The other is my Pain Mngmt dr and i'll fight that one tooth and nail - I very highly doubt the PMR doctor (who I otherwise seen at FMLH for botox) really does good long term Pain Management.  It's definitely at times been a little stressful with this and completely not going to be awesome having to start over with yet another new PCP since I just did this 3 yrs ago when my Family Practice doc went to be a hospitalist and we're in a decent place now. =/ Not perfect but decent! I think i'll probably start by emailing the 2 names ive been given personally as it cant hurt.

Stay tuned is about all I can say - never dull. .... Hopefully this weekend will be semi-ok it sure would be nice to have a little energy and a little less headache and associated symptoms to go swimming, potentially going down to my sister-in-laws on sun and other things. I can hope atleast! :)
More soon, thanks for stopping by.
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Tuesday, June 19, 2012

Shunt tap, Neurosurgery Appt. (mon)

Yesterdays shunt tap was a little better than fridays although towards the end caused the same side and back discomfort which to me means there's definitely some connection between that pain along the shunt line and the shunt issue.. (??) It took about 10-15mins for Dr.Bragg to get 20cc's of CSF (spinal fluid) off which compared to when I had the external lumbar drain we could get that amount off in a matter of minutes as the flow was so great (fast) due to pressure. While draining during the tap she also commented that it cont'd to be slow and sluggish and then there was spine/CSF debris and blood tinged at one point both of which arent good things but she also thought maybe that was what was intermittently clogging the catheter - we also turned down the shunt to .5 so both the VP and now the LP shunt are at the lowest settings as we thought it couldnt hurt and maybe help.

She was looking at the most recent thoracic and c-spine MRIs and commented how little spinal fluid there was at many levels and in one area was less CSF than a thin straw or maybe it was that it was equivalent to that in size. I asked if she thought the headaches could be related to that but she didnt think so mainly bc we've gotten headache relief with these shunts. The LP shunt catheter I believe she said sits at T7 which is the mid-upper back of ones spine and pretty well exactly where I would get the discomfort before (havent gotten it now in well over a week). Thankfully the VP shunt cont's to work and so picking up some of the slack.

They have an appt set up for monday to fup with her of which I probably will have to change if they want me to keep it as I missed ERT this week already and there isnt a nurse who can come in and do it at CHW due to their schedules. Missing a single week isnt to big a deal. I think Dr.Bragg just had wantd to possibly tap the shunt again see how it flows and she mentioned having talked to her partner and some things they thought about if we did end up back in surgery to revise this shunt. She was going to look up to see if the external lumbar drain catheters are the same size as the catheters used in LP shunts as she was curious if one was bigger would that work better.  She also said that if they did have to revise this LP shunt she would avoid the current Lumbar incision (although I didnt think to ask if they would have to go in to the old incision to remove the catheter or could it be done through a new incision). She also commented again how they would try to insert a new catheter more superficially and higher up (again not sure what that means).

Last night im unsure if it was because we took off that 20cc's of spinal fluid and turned down the LP shunt valve to the lowest setting (figuring even if it wasnt working right maybe more fluid would get through temporarily I think and no harm at best) on top of our having pumped the VP shunt numerous times (she showed me how to do it and said when the headaches get bad I could go ahead and try pumping the reservoir to see if it would help more fluid drain and thus reduce headaches to make up for the sluggish LP shunt) but I was so aweful feeling last night. (during the day I felt great after all this!) I ended up throwing up 3 or 4 times, had a fever (highest 102) and finally took some diazepam I had left over from a surgery months ago as I knew that would help the vomiting and maybe help me sleep. This morning I feel somewhat better though not great and fever seems to have went away (99 vs 100.4 and 102). If it gets worse i'll call them back but hopefully doesnt and can just wait till Weds or Thurs to call and give them an update.

Stay tuned, never seems to be dull - although it really could be exciting in a better way..


Friday, June 15, 2012

it's a shunted iissue, go figure.

Well yesterday I saw my PCP because of the pain in my L side (towards the back) and she wasnt sure what it was but recommended I touch base with Dr.Bragg's office. After I had the botox injections done at PMR I did call and talk to Lisa, Dr.Bragg's nurse who in turn talked to Dr.Bragg. They asked me to come in this morning.

I saw Dr.Bragg and the NP (Sue) and first Dr.Bragg did the simplest think by checking the settings on both shunt valves which where still accurately set at .5 and 1.0, Given this and given the VP shunt reservoir refilled fine (ironic that the LP shunt has done wonders to keep the VP shunt working well!) she (Dr.Bragg) tapped he LP shunt (which just means inserting a needle in the lumbar shunt reservoir on my side and seeing if she can pull off fluid easily) and wasnt able to get very much spinal fluid off and at the same time she was doing it, it did re-produce the L sided pain I had been getting although we dont know what that is about. She also said that either the mid-spine pain could be because the arachnoiditis (inflammation of the arachnoid or one of the layers that covers the spinal cord) could have just suddenly improved or the side pain could now be appearing as the lower back discomfort that also comes and goes - she isnt sure. Because of not being able to pull off enough CSF and being very sloowww (sluggish) as well as the reservoir then appearing caved in for a short while after the needle was removed she thinks part of the catheter is likely occluded. Initially she re-set the valve to a slightly higher number for the weekend (kind of miserable even more so really already) to see if this reduced CSF trying to drain will help force open the spine catheter and I see her back in clinic on monday ro re-tap the shunt as she is also looking at my last imaging to see where she could best place a new catheter. She said she likely would disconnect the lumbar-thoracic catheter that is in place now and tie that off as it would be to risky (I think I got that right) to try and remove it due to the irritation we've been dealing with, the lack of CSF in the area, and the anchor that is holding it in place in the upper thoracic region. Instead of going through the current several inches long incision we've used many times in my back she at this point thinks she would go either higher or lower and re-create a smaller incision to tunnel a new catheter in and she said something about thinking she'd probably end up having to do another laminectomy (remember last weeks analogy of the ladder and how the steps on a ladder represent essentially the same as the lamina in our spine - the supporting bone for the rest of our bodies spine and weight.) as every time we've had to replace the spine portion of the catheters we've had to laminectomies. (3 levels at this point I think?) .

She also said something about needing to keep the catheter away from the nerve roots so the catheter doesnt act as a suction and re-create that issue w the nerve roots clumping together and further leg pain that occured with my previous LP shunt and surgeon and since we're unsure what is causing the current numbness on and off in my feet from time to time. - There where a few things I know she was just thinking aloud on and I asked questions about that she answered well but being I wasnt feeling awesome sometimes the best explanations can still go over my head! :) Given I see her mon I knew I would have time to think about this all and she would have time to think about it all more and we'd come up with the master plan. Monday she just wants to re-tap the shunt to see if by chance temporarily resetting the valve will have helped and then so we can figure out final options/plan. I am hoping the VP shunt will pick up some of the slack from this higher setting LP shunt as it kind of is completely affecting my head right now pressure wise and started within an hour-ish of our resetting the valve although at the same time it's clear the VP shunt is draining bc while the headache is bad its not yet horrible. (please dear God stay that way).

When Dr.Braggs NP came in we where talking as we always do and joking around (taking things at lighter value makes it easier to deal with for me) and Sue commented how it had been a while (not long enough! But she also wasnt there when I saw Dr.Bragg and Lisa last week) and said  "your like part of our family we like seeing you" - as ive commented before it's so nice that these providers like me and that we get along so well but I do hope we still get to a point of maybe seeing each other a little less! Even an occassionaly email would be ok but this every week or every other week stuff, yah I dont love being in their hair (rven if im not)!

I was also supposed to have lunch with a good friend of mine who works in Madison/loved in SP and I guess I had so much on my mind I completely spaced out on it and forgot to ever send her a message when I was done! Oops!! We talked earlier today and going to try for monday to meet for lunch since i'll be in the area again.  I am hopingto feel semi-human (please!) this weekend as it's a busy one with Father's day car show sun and im sure swimming afterwards at my parents and then my cousins graduation tomorrow + id really like for my nephew to spend the night. I hope I hope. I am meeting my Mom for supper tonightt although food sounds atrocious really.
More soon - if nothing else will update monday,


Tuesday, June 12, 2012

Sorry, I meant to publish the writings below sooner but kind of forgot as I was writing it while watching my nephew sleep beside me and while trying to get myself to go to sleep as well and then sun was busy, hectic and tiring at my parents with all the kids and most of us siblings there!

So the real reason for this particular writing is 2-fold. First last week from my Mom no less (given my Dad is the school district support staff union president I am more than surprised he didnt tell me as he knows how important any insurance changes are for me to know ahead of time!) I learned that we are changing from a Point of Service plan with Deam to an HMO - for anyone who doesnt know an POS plan pretty well means you can see providers anywhere with no referrals and you dont have to designate a Primary dr 'to run the show' ie give referrals or in other words if I decided I wanted to walk out the door (theoretically) right now and see specialist X, Y or Z I could and no one would have to approve it ahead of time. Not the case with an HMO. Believe me I am disapointed and frustrated but at the same time realistic and I know as far as in-patient stays providers always still had to call and get the authorization and Dean was good about approvals even with the 4 week stay last month and the 16+ surgeries in the past year. My Case Manager whom I talked to yesterday (have I ever said how much I appreciate her???) is putting in over-rides for the next 3 months so I wont have to start a-new now and get referrals from an in-network PCP  and start over by first seeing in-network specialists in order for them to tell me they dont have experience with my issues (Dean is a super small network and only one of my specialists whom I am first going to be seeing next week sees patients at Dean as well as at UW where he is primarily based). This over-ride will mean I can stay with my current slate of providers in Milw and at UW (all out of Deans network) and in the next 3 months we will take their reports or if need be get letters from them stating why they are the doctor needed for my care/why they have the specialization to treat me and why they feel I need to stay with them and not an in-network provider (and my Case Manager has said in the past she has stepped in and said to Dean drs who said they could treat one or 2 of her super-specialzied (rare disease) patients and asked the doctor(s) if they realized what they where really going to be getting in to and then those doctors in turn agreed they probably didnt have the resources and expertise to care for her patients. It will be extra work but what am I going to do?

Dean is continuing to work to set up their own 'specialty pharmacy' designation with Genzyme so they can receive and ship the Aldurazyme from their office in Madison to CHW in Milw (continuing to pay for it up-front before it is ever shipped which is many moons more cost effective and saves more paperwork in cases like having it shipped to the hospital when in-patient.) but in the mean time the drug will cont to ship from Walgreens Specialty till about Sept-Oct to CHW.

The other stumbling block with this change we are trying to figure out is if I can stay with my current PCP and get an 'approved out of network' PCP designation with them to - my Insur Case Manager had thought I had to have a in-network PCP so ive asked around a little for any suggestions but at the same time the middle company that negotiates policy changes between BDUSD (school-Dad and Dad's union) and Dean Health Plan each year said something in 2 separate emails back and forth this morning I may be able to get a approved out of network PCP meaning my current PCP who is Internal medicine can stay on my plan/care and just be designated out of network but have authorization as needed to do PA's/referrals for any specialists (which in the past year we've only had 2 or 3 new (and in several cases temporary) drs reffered by my other specialists due to surgery issues or other issues so I dont think it would be alot of extra (??) work for them then it is now? Granted the past year has been pretty crazy with all the surgeries but at the same time thats pretty well why id like to stay with my current PCP especially bc her NP is so familair with my care, my current specialists (especially Dr.Bragg's team) and they get stuff done quickly especially all of these pre-ops we've essentially needed at the last minute just days before shunt surgeries... Since Dr.Bragg came on my team its like any PCP issues have really worked out I think because any Neuro issues where taken off of their slate and taken over by someone who knew what to do with it...

Otherwise the headaches and other symptoms have really been so-so, last night I threw up clear out of the blue and before I had time to take zofran with enough time for it to work (about 2 mins is all thats needed!) so that was pretty not awesome and the first time thats happened since the first couple weeks I was in the hosptial (when the shunt reset there one of several times). Ive been waking up now in the mornings with the headaches which also wasnt happening and then they tend to slowly improve throughout the morning after a couple hours and afternoons and pretty better though I do get milder pressure type headache. Nights I wake up, fall asleep, wake up even despite laying up-right for the most part (I move around as I sleep so dont always end up up-right).  Vision is on and off - last night ironically it was pretty fine, this morning it's fairly fuzzy/blurry. Rather than call and bother (not that I am really I dont think) Dr.Bragg's team since it's only been a few days and the shunt(s) are atleast partially working or atleast one of them is picking up slack i'll wait and see - maybe it will get better. The one ominous sign is that the mid-back pain is pretty much gone other than here and there symptoms (positional)  vs being almost non-stop on and off before when I had very little headache and very little other symptoms. Appetite is another thing that is always a good sign of how the shunt(s) are working as I am always wanting something to eat when they are atleast pretty good and now I eat a little and not really hungry again.
This is so frustrating, I am frustrated with having just saw Dr.Bragg on thurs, (with my body)frustrated with these symptoms and how we get complete symptoms resolution (as ive said previously) with the external drains and how it seems like we get atleast fairly good results for a while but then go back to having problems over and over. I know its the shunt(s) bc its the same symptoms repeatedly that go away repeatedly its just a matter of what really causes this intermittent (I assume again) occlusions?? Have I said I hate shunts? Its nobody's fault but I still dont have  to like it!

6/-/12 - Sat night-Sun night
It's definitely been an up and down few days which is a little (but I guess not really after this past year?) unusual and definitely frustrating... Ironically mornings are again worse with the headaches but then the mid-back pain is better (something I also often experienced when I had the former LP shunt - when it worked well the nerve type pain was bad (although nothing compared to how this can be) and when it wasnt working well it was next to nothing if any pain at all and that is exactly how it has been now to - no headache then definite ups in mid-back discomfort but if headaches then zero to very little mid-back pain.) Mornings cont as has been the pattern over the past year of these up and down shunt issues the worst and typically as the day wears on the headaches get better although they do ebb and flow just nothing like the mornings and nights are. I spent the day with my Mom and sister and nephew (who is now sleeping next to me) and then had lunch with my Mom, Dad and sister + her family + went swimming at my parents (they swam, I took pics just wasnt willing to get in the water with it being in the 70's!) :)  The morning was was the same yesterday was needlessly so roller-coaster-ish but I wanted to do what we where doing so I just tried to play around with the pain management protocol I have and bear with it (and as my sister said last night(fri)  when we all went out for supper "tended toward being quiet as a church mouse" which is a usual give away of when I dont feel great - I dont say alot and instead listen more. I would feel fine, then the headache would come on and then I wouldnt have a headache and feel fine, then the back discomfort/nerve type pain would come on and was over and over - it was pretty sucky actually but what was I  going to do? I cant exactly let this dominate my life more than it already does - I want to do things with my family and so force myself out of bed, to get in the shower and to drive over to wherever we are meeting to do whatever we are doing bc it makes me atleast happy and a good distraction. Besides usually by the end of these days then my joints hurt so I think less about how the other stuff is! :0)! Tomorrow (sun) we are meeting at my parents (yup a very family weekend) as my brother, sister-in-law and niece are also coming up as is my sister and her family again and my nephew and I after church are going over to spend the afternoon swimming and have supper with my parents, pretty fun really. I probably will even get in the pool tomorrow after taking more pictures as it really does get pretty warm in the water and in all actuality is pretty fun with all of us messing around! :) Distraction as they say. :) I figure as long as the headaches stay the same I am just going to let it be for now, as its bad but not horrible yet and maybe its just that  one of the 2 shunts has reset or one of the shunts has occluded again, who knows but I really have no desire to visit Dr.Bragg so soon even if she and her staff are so super sweet.  I am just hoping this will be an intermittent thing and works itself out. It could happen, optimism is key and as I always say 'one day at a time!' :)

I talked to my Physical Med-Rehab drs office (separate from my Pain Management dr and my Neurosurgeon although referred by my Pain Mngmt dr last year)  and we've scheduled botox injections for my neck/shoulders, down cervical spine and thoracic spine for next thurs which if nothing else will/should help (as it did last year) the muscles type spasms I get in these areas with almost any fwd type movement either while sitting (for instance typing) or when sitting on the floor or even when trying to sleep and with little things like washing hair, etc. These where always worse after the previous LP shunt (first time around but prior to first botox) and then we thought of trying botox last year previous to seeing my new (not new anymore but then) neurosurgeon  and it helped a great deal. I did it 2 different times 3-4 months appart about 30-some injections and then after we removed the non-working LP shunt I didnt have the same or as bad symptoms so didnt have it done again but with this 2nd shunt (the added back in LP) the symptoms mimic muscle type spasms and so going to try it again. I sent a message to all relevant drs to make sure it was ok given the LP shunt catheter sits up in the thoracic spine but I cant imagine the botox could affect the catheter/shunt function in anyway.

All for now - sorry this tended towards long!


Thursday, June 7, 2012

Neurosurgery Fup

I saw Dr.Bragg today for 2 week fup from getting out of the hospital (about 6 weeks since surgery to place the 2nd shunt and laminectomy/decompression in lumbar spine) and all was fine or as fine as we expect it to be as the spine nerves cont to heal. She did say interestingly she has been listening for anything from others about LP shunts causing/affecting mid-back nerve irritation and since knowing about my issues and our dealing with this she has heard from 2 of her partners patients who also have LP shunta that they also have similar type of pain/discomfort/irritation. She said it is very likely from internittent shunt catheter rubbing on the nerves and arachnoid space (one of 3 protective layers of the spine) and we just cont to wait and hope this will improve. Right now from being a definite 10 at re-admit it is a 4-5 which in my book isnt awesome but also isnt anywhere near as bad as it was and thankfully still just cont to be worse with things like this (sitting, driving, typing) which are the ironies since typing and driving are 2 of the things I do most. Otherwise the headaches we didnt really talk about but there is always the option to turn the 2nd shunt down the final setting if we so desired at fup (in a month) and if the mid-spine nerves would improve we had previously talked about we could even take the programmable valve out all-together so the CSF (spinal fluid) could free flow. I think Dr.Bragg's concern is in that the nerves are so irritated already at the low pressure setting on the programmable valve and so little fluid around the thoracic spine would it be even worse pain/discomfort wise? - such a trade off.. Hardly seems fair but hardly can sit around feeling sorry for myself simply bc I wish I didnt have one trade off for the other (nerve irritation and preventing us from draining even further fluid) - atleast I do feel better now with the 2 shunts than I did with just the 1 shunt and the 2 shunts also atleast appear to better balance each other and keep each other working thus preventing the occlusions we where dealing with every couple weeks. Dr.Bragg said to me if she could she would take some of my pain as she felt so bad about trading one issues for another and wanting to just make me feel better headache wise similar as we have with the external lumbar drains. I told her that I wouldnt want her to take some of the pain away as she then wouldnt be good to us her patients and we need good doctors like her who listen, believe us and care which it is true.

Balance, balance, balance and we will figure it all out I do beleive since we have already figured this much out. It is what I like about Dr.Bragg she's persistant and she and her team havent given up. She was sweet in saying I could come in anytime as I always make her laugh but in reality her team has quickly become one of my favorite providers and they to with their easy-going, optimistic (never
give up) attitudes make me laugh as well.

I asked her about my feet going numb and lower lumbar discomfort which is also something my Pain mngmt dr wanted me to ask her about and she thought it could be a result of the  multiple  laminectomies we've had to do in the past year,
 less support (removal of the protective bone covering the spine or in the case of the lumbar spine the nerves as the spinal cord terminates just below the thoracic area) and less 'structure' to basically hold everything up. (think of a ladder, if you take out  a link or step on a ladder you basically have the same weight but  more precaurious bc of less support for the same weight). I fup with her in a month and since we've had problems with nerve issues in my L leg we are going to take a 'wait and see' approach to the numbness symptoms in my feet (since it is intermittent anyways) and see if it doesnt heal with the spine surgery healing.  Hope so!

The one thing I didnt think to ask her (remembering 2 of multiple questions is still something!) :) was about potential botox with her Rehab dr which is ironic bc we where talking about this Dr.Ward and his opinion to her that he had told her about giving the thoracic/spine nerves time to heal from the assault of the surgery vs fiddling with the catheter and causing potentially more irritatiom. I have a message in with my current PMnR dr at FMLH about doing further botox (it's been a little over a year) but think it would help. I know she works with her dr and wonder if it wouldnt be good to have a dr like this at the same hosptial as a specialty like hers - she herself said to email her with any questions so I can always do that if I decide to. I wondered if botox couldnt help prevent leg muscles from becoming so tight so quickly so will probably email/call her and her nurse at some point and ask their opinion as I know it really helped my upper spine/neck (which gets so stiff) when I did that. areas.
I saw Cardiology on tues (a crazy week) and as I figured we are leaving the new beta blocker dose and lasix dose alone (blood pressure is now super low but feel ok with it) and fup with new AEcho in 6 mo. We cont to just teeter on the edge of everything being very moderate but not severe minus the atrial enlargement which is severe and waiting and watching. We dont want to do valve replacement bc of the harshness this would be with the restrictive lung disease, requiring long term coumadin (mechanical valves) and would likely be double valve replacements. I am defintiely in no hurry for any heart surgery and cracked chest - yuck!

Otherwise I typed this part yesterday but was feeling like clearly this was the week for everything to stack up and weight on my mind; usually I am exceptionally good at putting things out of mind nd just dealing one by one but not this week as everything seemed to have been weighting down. I think partially it was learning info about changes to our insur July1st, the recall election and Walker not actually getting recalled (though we did get control of the senate which should prevent any special sessions till the actual elections in Nov when we can hope we keep senate/assembly control again). and a few other things. I am back at my apt atleast and that is defintiely nice! One by one I think I just had to deal with and sort things out in my mind and in some cases on paper.

Will update again soon, - it is my youngest nephew Zander's 6th b-day today and he is spending sat night here plus headed to see my sister in law, brother and niece sun.


Friday, June 1, 2012

Day to day to day.. Almost 5 weeks.

Sorry, this is another of my many (it seems?) recent multi-day updates. Will try to keep this part shorter.
I went and saw my Pain Mngmt dr yesterday whom I really like and is good at what she does, she is also who got me interested in advocacy with APF and so we have always had Pain outreach as a common interest to talk about and catch up on outside of actual care issues. Yesterday was definitely no different what with APF closing and so much cont'd turmoil in chronic pain mngmt. Anyways, as far as actual pain goes while in the hospital she gave me a few suggestions to give to my Neurosurgeon (Dr.Bragg) of which Dr.Bragg used/tried the suggestioins. Dr.B my PM dr suggested we make a Pain reccomendation plan for future surgeries or hospitalizations like this (bc as much as we might have wishful thinking that there will not be anymore there will be for some issue or another be it the shunt or something else.) On the docket for instance is carpal tunnel (a 3rd time)  whenever I decide to go back as I was scheduled to fup this past week but then with getting out of the hosptial and the holiday ERT got switched to Tues when the appt was supposed to be. So anyways Dr.B and I are going to meet again in 2 weeks and in the mean time she thought she would also print out what meds we have tried in the past with success or failure in the different med classes. The pain plan is something we did with the post-op/in-patient cervical spine decompression and my Ortho-spine dr liked it and used it then - obviously in that 3 years alot has changed so we will just update or change it overall. When I knew the adult pain coord at UW it worked fine her just making suggestions as she had gotten to know me through our combined interest in APF/pain outreach but since she has since moved to WA state and given we struggled some to figure out what to use while I was in the hospital this last time I think my drs idea is a good one. Ive also had this drs cell phone and home number since almost when I began seeing her (although other than the beginning and occassional text updates I cant say the last time we've talked to each other via phone) and she asked me for if there are future shunt surgeries to give her cell # to Dr.Bragg so she (Dr.Bragg) knows she can reach out and they both not feel uncertain about contacting each other since each is at different hospitals/academic centers as Dr.B said she felt a bit intimidated about just calling Dr.Bragg herself (Dr.B and I would email and I in turn would fwd it to Dr.Bragg and her team) as she didnt want to step on toes/decisions which I told her with Dr.Bragg I really could tell they appreciated Dr.B's advice and appreciated having it to try.  I think both things should help as both drs are realllyy very good and 2 of my best.

As far as changes to help the overall pain issues since the 2nd shunt placement (lumbar-thoracic to peritoneal)  surgery (which the pain is alot better since that week I was re-admitted but when it does flare up, Ugh!)  Dr.B is bumping up by a small bit the pain patch (butrans) and then adding a slightly incrementally bigger dose of lyrica which is a med that helps calms the nerve signals and one of the best that can be used for this issue. It's a med actually my former PCP put me on 3+ years ago and we've played around with the dose many times over surgeries. This time I am taking it morning and night and then I also take a immediate release pain med 3-4 times a day.

On a different note, Zander graduated 4K last night and given his noise intolerance I am SUPER proud of my kiddo - he is growing so big and will be 6 next week, 6! Which is crazy bc that on yet another differnt note means ive had my shunt (in general) for 6 years as Zander was not yet born when I first saw my then neurosurgeon and just botn when  it was first put in. That kiddo is my HERO! (for many reasons).

Last, to update below, yes today was somewhat better than the rest of the week has been, with the stomach binder although I dont understand it, it does make things a little more bearable and I was able to go and be on and off my feet fairly well most of the day. - Tues and Weds night where just bad nights so I was feeling a lot tired and a little depressed at the sitation when  I woke up and was writing that Thurs morning before I got up and going for the day. Thankfully the day got better and ive semi-figured out a better way to stay on top of the overall pain.

                                                          Zander's 4K Graduation -

As far as advocacy and work with the new foundation it is going really well and we cont to work on the database for MPS I attenuated families for future research - I like the work and is interested plus in the end also benefits me which is a nice perk. :)
See below for earleir updates but otherwise thanks for stopping by!
Well, this has definitely been hard, one of the hardest surgeries really overall as  I hurt alot and I dont feel great alot and I am frustrated by the nerve pain. I wake up in the morning and throughout the night dreading the day because I know how uncomfortable it will be alot of the day. That said not to minimize this all but I know I cant let it stop me from living a life so I have been marching on doing the things I do which means a family get-together on mon,. infusion on tues., and seeing my nephew and niece yesterday (they give me energy in some ways and I love just following Zander around as he talks about his bugs and butterflies and catching bugs, that kiddo is just to cute not to smile at and his sister is so like him it's very funny as already they compete. I often wonder how I could have gotten so lucky as an aunt to have these nephews and nieces and the time I get with them? I dont know and I know sometimes its a little over-whelming as I do have some of them alot but id rather it be that than not enough as it is with the older ones during the school year - I know next year when Zander is in school full time I am going to miss him like crazyyyyyy....

Anyways, so back to this healing, wow, just wow, ooh wow is about all I can say! I wake up alot at night and on a bad night like tues I sleep less than I do more as I am up so often trying to figure out how to get comfortable (wouldnt matter in some ways if I was laying on a bed of cotton molded to my body). Do I still think this was the right thing to do, with placing a second shunt? Yes, simply because with the external lumbar drain was so sweetly, serenely, amazingly good.. I just wonder why are the external drains be it ventricular or lumbar so good and yet we have such an incredibly hard time with these internal drains? Is it because with the external drains we control them and the flow? Is it because with internal drains it isnt being absorbed enough and thus isnt in turn flowing enough? I dont know but wish I did! Yesterday afternoon I called Dr.Bragg's office; her nurse isnt there on Weds so I talked to her partners nurse and just told her how miserable I am with this nerve pain in my mid-spine and how it affects nearly everything (including sittring here typing this which isnt awesome as my work and alot of what I do is computer based.)  :o/ and is so uncomfortable. In reality in some ways it reminds me of the first LP shunt so I wonder if it is less about nerve irritation in general and more about nerve irritation due to not enough CSF to cushion the spine, I dont really know. I wouldnt mind if I did know!   I fup with Dr.Bragg next week thurs in clinic as today I see my Pain mngmt dr (I am hoping the 2 of them maybe can think of something individually or together to help this as it heals..)  and the nurse offered a clinic appt today but bc the one med I get from my Pain dr is a patch every 7 days and have to fill it this weekened I kind of need to see her anyways + not much I figured Dr.Bragg could do in clinic. Her actual nurse is back today and going to call me though. I super hate calling them (or any dr) regardless of how nice they are simply bc I hate bothering people and I just talked to Lisa on tue (just to schedule the fup) and feel like (not bc they make me feel this way!) I am forever asking them for help with these dang shunt issues. I defintiely in many ways was not made for having health issues atleast as far as having to ask others for help.