Monday, February 27, 2012

Hydro info, update, news

Below are a couple responses from my neurosurgeon as well as from Emil Kakkis, MD regarding shunts and MPS - both are interesting and I think could be informative to MPS families. Ive taken out any names and inserted just initials plus the emails where edited to just contain the part I think would be informative so keep in mind there was more to both of these messages.

Received - 10/11
Sorry for the delay in response.  CSF pressures in general are not compartmentalized, but pressures can build in one area and cause a generalized increase in the brain pressure.  Pseudotumor is likely more of a problem with absorption of CSF, whereas hydrocephalus can be that or it can be an obstructive problem.  Problems with absorption of CSF are also seen in other syndromes, like glycogen storage diseases.  The problem isn't so much the definition, but defining what the problem is and working at solving it.  Sometimes addressing one or both still doesn't solve the problem because it doesn't necessarily deal with brain compliance (how stiff the brain is).  That is what Dr. H helps us understand as well. 

Received - 2/12
In MPS, the hydroscephalus is caused by obstruction or blockage to absorption of the CSF at the top of brain in special structures called arachnoid granulations.  This is the place CSF is absorbed back into the bloodstream.  But the blockage and slowness of the absorption leads to increased intracranial pressure, so the shunt is supposed to help over come the block by pushing CSF back into the body using a catheter.  The valve helps you control how fast it goes.  I think your shunt should be slow so it will be less likely to plug and so the slow release of pressure will help reduce pressure on your brain enough to feel better, but not too fast to cause the filter in the catheter to plug.  I hope it continues too.
Brain compliance has little to do with it.  The brain may be full of GAG and that may change the compliance but it does not matter how soft or hard the brain is, but more importantly how much fluid goes in and how much goes out.  Hopefully you have the balanced now.
I had a really good weekend headache wise - there was some headache but within an hour or so of turning down the shunt friday afternoon I was feeling pretty good and although I woke up with that same 'hung over' like feeling (that typically wears off a few hours after being up) I had a great day sat andon  sun waking up was improved (though i didnt sleep much then either as both Quinn and Zander spent the weekend and woke up REALLY early) + the day was also pretty good. Vision remains an issue but otherwise I was THRILLED to finally not have a nagging pressure sensation and nausea, etc! Today, hopefully maybe only because of the ERT fluid  load has been somewhat bad on and off - ive been getting these almost lightheaded like sensations although I dont have low pressure headache which is what id normaly associate light-headedness with! Instead its a pressure feeling and vision has been attrocious plus tired, really tired. I had a meeting after ERT over at FMLH (which is next door to CHW)  for the project I agreed to help with, with the new Adult Congenital Heart group; basically a meeting of the minds to bounce ideas off the 4 of us that met to discuss our website, fb page and overall ACHD group. Maybe because of not alot of sleep last night and then ERT all morning/early afternoon I felt like I was trying to focus from a million miles away which drives me insane!!

Tomorrow I have a sleep study (yuck) at FMLH for my Cardiologist and Pulmonologist and then see neurosugeon thurs at AFCH-UW. Im hoping and remain optimistic that today is jsut a fluke with the shunt!
I also got an email from the Genzyme person ive been working with on the Adult-MPS I project and we have a conference call tomorrow to go over the final survey and will go from there. I am excited we may be close to having this ready to send ou!

On a last note - Weds febr 29th is World Rare Disease Day - wear your 'genes' (jeans) and share your story!
Take care,


Saturday, February 25, 2012

New setting, new change - good things.

So ive been working on this update on and off for the past few days and have been quite slow about it. I typed the entry below the break before what I am about to write happened so forgive that this is a bit all over the place. I guess if nothing else it is a good idea into how my week has been. Below I wrote about the headaches and vision and frustration and all was written mostly weds and thurs but earlier this afternoon I called my neurosurgeons office and talked to her secretary and then to one of the nurses about an hour later. The nurse Catie talked to my neurosurgeon as I had asked if it might be possible to have the shunt turned down the final notch and when Catie called back she said Dr.Bragg was willing to meet me in the hosptial lobby at 4 (it was about 2:30 and I originally called about 1:30) and was this possible for me? I live about an hour from the hospital but it worked out ok. So I did end up meeting Dr.Bragg  a little after 4 (when I got to the hosptial I called her secretary who paged her and then she came down and met me) and she reset the shunt valve (with 2 different small washer like devices and another one that looks alot like a compass and  smaller than the size of my palm - all of which are held over the valve) down to .5 which is the lowest setting that the shunt valve will go to. She said to call or let her know over the weekend if I had any issues or call on mon but I am hoping this wont be necessary and would only do it as a last means as I am just extremely grateful she took that time to meet me today basically at the last minute! I fup with her anyways on thurs next week so would be great to have good news! She said she didnt want me to have to deal with getting an appt so thats why she met me in her hospitals lobby and we did the re-dial there.  It was kind of funny to watch a family that was walking past as I could tell they where curious as heck what we where doing but it took all of about 2mins to re-set it so not a big deal.  Ive said it before but Dr.Bragg is pretty amazing!

I usually request copies of providers records (some providers send me their notes and some dont - I really should ask Dr.Bragg if she can automatically send her reports when she dictates them but just never think of it). and so I requested a few weeks ago the records from last fall up till now which included the meeting with Dr.Bragg's Neurologist the day after I was admitted by ambulance from the local ER and day before the EVD/ICP drain/monitors where placed. Anyways I knew Dr.Bragg always had the question of was 'low intracranial compliance' secondary to brain stiffness (from the MPS) a cause of my pressure issues and this Neurologist also raised that in his notes from our meeting. Ive been reading about this more online via google searches and has been enlightening reading - L-ICC may explain why I am so sensitive to intracranial pressures and seem to do better draining at such a low intracranial pressure as the brain (if I understand it correctly) otherwise doesnt react like a normal persons brain to pressure changes and at lower pressure it has something to do with more room? (dont quote me on that one!) This Neurologists note really was just very interesting - he had floated several ideas and it is obvious he to knew a thing or to about MPS/storage disorders. He'd make a good replacement for Dr.Charnas ive a feeling. (and Dr.Bragg has said as she is managing both my neurosurgery and neurologic care now that if there is ever anything she isnt sure of she will ask him. He helps her with their complex shunt patients and seems to have a vast array of knowledge.

Here is an article that talks about low intracranial compliance (it uses the analogy of a balloon when air is forced in to it the balloon can expand - a brain is similar to this because it is elastic it also typically can expand as needed. In most MPS I pts the brain has storage in it causing a stiffness which causes what it seems is thought to be low compliance (vs high compliance - more elasticity)  so my neurosurgeon and her neurologist are thinking perhaps the brain cannot react to injury, trauma or increases in intracranial pressure or increased intracranial cerebrospinal fluid production in my case because of the storage. So the lower the cerebrospinal fluid pressure (CSF) the higher the actual compliance would be of the brain tissue. (more room? - not sure of this answer...)  Im not sure I explained this well nor have I explained their thinking well (??) but the article below does a pretty good job of explaining high vs low compliance in brain tissue.

I agreed to take my 9 yr old nephew for the weekend so im hoping like crazy I wake up feeling good on sat!! I could have had Quinn last weekend if not for the surgery so was disapointed about that and so when I found out he was free all this weekend I was pretty excited. :) Im hoping also to have my 5 yr old nephew spend the night so we will see!

----- 2/22/12 - 2/24/12 ......
Ive been meanng to update for a few days but havent felt much like it so my apologies for not doing so. I was released from the hospital on late monday afternoon and was nice to get home. I ended up sleeping most of the drive while my Dad drove. The shunt has been set at 1.0 which is the 2nd lowest setting since monday morning and has been a bit of a roller coaster as far as symptoms. I honestly dont know what to make of this all and is slightly disapointing. When it is good it really is pretty good and my parents when I went over there weds night + my PT when I went there thurs night both said they thought I had 'a sparkle' back in my eye and when I feel good I do think I have that! Mornings are definitely the worst followed by trying to fall asleep at night which is why I am up writing this instead of in bed. I am unsure if the shunt needs to be turned down (there is one more setting we can try) or if my body is just doing the build, dump, drain, collapse routine and so thats why it feels like there is pressure some of the time. Today atleast was better than yesterday in which was quite aweful other than the evening - it literally felt like someone was pinching from the top portion of the shunt down to my ear on and off 1/2 a dozen times an hour. Not nice! I dont know how to feel about all of this, I guess it's frustrating because I want to have energy and want to feel better - I dont even expect a 100% resolution just wish we could figure out a somewhat resolution I suppose. I follow up with Dr.Bragg next thurs and just hope this improves before then as the thought of giving her more bad shunt news is unappealing (not that she isnt great and always trying to think of ideas) and I just would love, love, love it to be able to tell her it is working great!! I honestly think if we are faced with having to do something else with this shunt im going to have to if she would still recommend it do the ICP/EVD monitor and drain and let her try adding different things to the drain like a 3rd valve + also if she still recommended it do the dye-MRI study. I also wonder if maybe the 2nd valve where it is right now might be getting kinked at night given I sleep somewhat upright and the valve sits at my collarbone so anytime I would sleep in a funny position could it be not draining as well - I dont know the answer to that. The other question is does it have something to do with where the CSF drains in peritoneal cavity and is it not absorbing right and does this thus affect the top portion of the shunt (she had said that can and does happen), Just some things to ask more about.  In the mean time I do hope this is just my body recovering from the surgery and will work well with no further surgery and maybe adjusting it down to the lower setting will do the trick. Not sure. I dislike and have a hard time with relying on others with all this shunt issues - I am thankful to have good providers in that aspect but have never been great at having to rely on someone else especially repeatedly. Please just say a prayer that this works out and adjusts on its own. 

I honestly am  also wondering if some of the headaches feeling worse isnt related to vision - when the pressure increases, vision is affected and definitely seems worse when trying to do things like read (I love to read but havent been much simply bc it is that much more difficult) or read things on the computer, etc. I have been wearing my glasses more but hope that when the pressure equals out vision will also equal out. I talked to Dr.Bragg about seeing an Opthalmologist (s/t she brought up a few weeks ago) and was there anyone she recommended at her hospital and so she and her nurse are going to help me figure out who is best to see. I go back to UofMn a week from tues and could see Dr.Bothun there who is very educated on MPS I but I tihnk it makes sense to have a local Opthalmol. and someone who is at Dr.Bragg's hospital so communication can happen more easily.

The endocrine results came back and im not actually sure what the result means but the growth hormone marker/pituitary hormone was low - it didnt seem crazy low and I dont think this is the same one I take meds for (maybe it is as the cortisol issue is secondary to pituitary deficiency but there is another test endocrine dr originaly did to confirm the low cortisol issue which was done over a series of hours and countless blood draws). Im sure my Endocrine dr will call when she gets a chance or if it is anything important.

Earlier this week was my 7 years post-diagnosis (MPS) mark - I didnt even realize it till the day after. In all actuality I knew I probaby had MPS back in Nov of 04' thanks to my Cardiologist but the official results came from Genetics in Febr 05' it was a monday the 21st and I remember I was taking a nap after a day of classes when my then GC called. A week later we met with that GC and the new GC (who I had up till last spring) and the new metabolic Geneticist. 3 mo later in May I started ERT though approval was had by my insur within 1 1/2 weeks of that Febr appt/meeting. It seems crazy to think it has been 7 yrs and yet im very glad its this point and not back then. I have a pretty good group of providers and feel like the best drs really believe me now when I say something which wasnt the case in the beginning. I also feel like though im still not that comfortable talking about myself or bringing up questions to providers I atleast know which providers I think I can reallly trust and who really seems to want to help vs back then I didnt have alot of providers I knew well or that I felt that comfortable around/with. I also feel like I know which providers I dont need to see as often as may be recommended and I know who I can go to if I need help with something. Its also nice to not have to basically fight over my ERT infusion rate which is something I had to do for the entire first 6months to 3/4's year I was on aldurazyme. Ive lost some really good drs since 04-05' but ive also gained some equally good ones and ive also found even better ones for others that where formerly on the team but  that I didnt really trust that much. Is my care perfect? No, but I dont think medicine is ever perfect and I dont expect my care ever to be. Non-the-less it is nice to be at a place where I feel more comfortable and dont have to feel like someone is questioning my every thought. Even compared to a year ago I feel so much better about where my care is and that is an awesome feeling!

I am working on a new project (I know how do I get myself in to these! - but it should be interesting and fun) for the WI-ACHD group which is formed between my Cardiologist and one of his patients. I will be heading up the blog and fb page and have 3 other people working with me. I will manage both and the 4 of us will meet on mon to brainstorm more ideas. For now I have added a few changes modeled after mine and other blogs and have a few more changes in mind and also added a few things to the fb page. In between when the Genzyme project should be coming (to edit/review) soon  and APF I definitely keep busy but it's a nice busy and all things I enjoy with good people + flexible.

Otherwise symptom wise the only bigger things have been cardiac wise the shortess of breath has been pretty good on the increased lasix dose while heart rate has been pretty erratic as far as rate and rhythm which can definitely get annoying! Nerve pain wise my legs have been falling asleep/hurting pretty fierce when I sit again for any period of time but it isnt as bad in some chairs as it is in other sitting positions and chair designs. Im sure it is just the nerves irritated for whatever reason again but hopefully with my cont'ing hands on PT that will help. I do that (PT) typically once a week.

Stay tuned - all for now,


Monday, February 20, 2012

Day 3 post-op, Revision 12?? (since May)

Hi Again, I am here on the neurosurg floor yet and last night the resident on call got permission from Dr.Bragg to re-adjust the shunt valve down a notch. They went from a 2 down to 1.5 and then again this morning when Dr.Bragg came by since it had been about 12 hrs she adjusted the pressure down again to 1.0. She said it takes about 12 hrs to get an accurate assessment of how well a new setting will work but gave me the option of going home today if I felt like it. I talked to the nurse after taking a shower as I figured there wasnt anything I couldnt do at home that isnt being done here.. He thought it would be better to wait atleast till mid-afternoon or so to make sure I handle this new pressure well or to see if the pressure is still to high in which case he would recommend staying and seeing about having it set down to the lowest .5 setting. Since it's only a few hours I am ok with this and will probably take a nap. I slept about 4-5 hrs last night all while sitting propped up with the head of the bed sat up and this really made a difference  I wish I could figure out a way to sleep like this at home! I was able to adjust to get pressure of one hip or the other when needed but still be upright and was well. In-fact I was still 1/2 sleeping when Dr.Bragg came by this morning and so didnt ask her about the IT or anything else. That doesnt much bother me as I can ask her at fup appt. Really the only problem is discomfort/searing type pain directly over the incision/valve area that occurs every 1-2 hrs and last about 15mins otherwise headaches do seem alot better which is fantastic!

My primary insurance case manager called mid-morning to see how I was doing and to give me an update on a change Dean is trying to make with how/where my aldurazyme is shipped from. Right now Dean is contracted through July 1 with AWalgreens Specialty Pharm but they are working to get a specialty phamacy designation themselves so they could receive and ship the aldurazyme diectly from their own Dean-East Pharmacy. This I think would cut down on confusion with shipping as well as I think might make it easier to get aldurazyme here at UW when I am in-pt. They have till July 1 so we will see. Will be nice if they do get it!

I am working to try and get my infusion from today set up for sometime this week but havent heard from my GC tet. My former NorthShore infusion nurse (a CHW employee) can do it tomorrow if I can get approval but I have yet to hear anything so we will see. Otherwise I emailed one of the other infusion nurses to see if she might be able to later this week but it all depends on if CHW will give us permission for a 2nd day just for my infusion.

All for now, nap time,


Sunday, February 19, 2012

Neurosurgery day 3 In-patient

The valve I have now is set at a high pressure; high resistance setting and so while saturday morning and afternoon where very good (I suspect because CSF would drain off to some extent during the surgery so my body would have that to make up for the difference in the high pressure setting - the fixed pressure valve was medium pressure before). My neurosurgeons partner was seeing her patients today and so I asked him about lowering the valve setting some as last night I was miserably bad feeling with a high pressure headache and nausea, etc + very drowsy/sleepy till the pressure wore off some. He thought we should wait and watch for a few days so I am still in-patient on the neurosurg floor until atleast tomorrow and will see what my own neurosurgeon says. I know sat she said she was hoping we would be able to tolerate the shunt at the high pressure-high resistance setting (less likely for ventricles to collapse and then less likely for intermittent and eventual permanent occlusion as has been occuring almsot every month to date.) She did say if my headaches got bad though she was fine with lowering the shunt and we would see what I could handle as we knew doing this I may even need to drain more CSF than I was with the previous medium pressure valve. Hopefully having the second high resistance-high pressure valve in place at my collar bone will provide back up pressure if we do have to lower it to lowest settings in order to help prevent future occlusions.

Dr.Bragg and I talked about seeing an Opthalmologist yesterday (I have one at UofMn but would like to have one locally and at Dr.Bragg's hospital since to some extent the 2 will go hand in hand.) I also need a Opthalm to follow the corneal clouding which so far is just at the back and top of my cornea.  Dr.Bragg was going to talk to her nurse Lisa who has worked here longer than Dr.Bragg and they where going to set me up with whomever of the docs they work with and think is best.

Right now pressure is building up at the valve and is alot like a piercing feeling down in to my eye but not outright painful just makes you take notice and somewhat uncomfortable. This will eventually lead to headache and then draining.

I think I am finding amazingly it easier to talk about all that I have going on face to face with people - there are plenty of times I dont feel like talking about any of this MPS stuff and so will change the subject or turn around the conversation but I do think I am getting better at explaining whats going on and not feeling so self-conscious talking about myself. I have no problem talking to large groups of people as that can be so informal and I can basically let myself get lost in the crowd while talking but 1 on 1 it is different and has always been really uncomfortable for me im not sure why.  Especially providers I dont know well or people I dont know well and even sometimes family bc often I just need a break from thinkng about all this MPS stuff and when by family just want to think about other things. There are a few people ive gotten to know really well including a couple providers and I have no issue for the most part talking to them but in their cases some of them have been so open about their own lives and what they or their family are dealing with and this almost makes a mutual connection. I dont think im ever going to be one of those people who has as easy of a time talking person to person about my life as it is easy for me to say write here or write about what im dealing with on fb or the MPS Forum. A work in progress I guess.

The nurse and I tried to see if we could get the aldurazyme infusion done here at UW while I am in-patient on the floor but UW does not carry Aldurazyme (as far as I know there is only 1 other little boy in this area who has MPS I and he is post-BMT so no longer on ERT thus UW would have no reason to carry the drug). I am going to call my Insurance Case Manager tomorrow see if she has any ideas otherwise will try to get the infusion done later this week at CHW if one of my infusion nurses is able to come and do it. Hopefully.

I have fwd'd all the IT communications from Drs. Dickson, Kakkis and Elsa Shapiro to Dr.Bragg and will talk to her about her thoughts. In the mean time Dr.Shapiro and her colleague Kate who does the actual testing and scheduling for UofMn's part of the cognitive study is looking in to my having the fMRI done at UCSF and then UofMn will do the neuro-cognitive testing. From there I will have to make a decision if I am interested in doing the IT and doing the pre-study testing to see if i'd qualify. I havent asked Dr.Dickson but assume this is the study she would want me in but then get IRB approval to use my shunt valve/reservoir for the actual injection to see if it helped slow down or prevent the occlusions and clear the MPS-GAG storage from choroid plexus and brain matter. I plan to talk to Dr.Bragg this week and get her thoughts on this all as I dont think  I would try for the study if she didnt think it would be of benefit or if she where against it for some reason. We will see - stay tuned.

Please keep my good friend Lea in your thoughts, she has been one of my closest friends in the MPS community and yet we are comfortable enough we can go what seems like eons without talking and still know whar the other has going on/is dealing with. Her son Gabe is having his funeral today and tomorrow and Gabe had MPS II another form of my disorder. Gabe was very young and is incredibly sad. I cannot wait to hug my nieces and nephews extra hard.

God bless,


My life's but a weaving between my Lord & me,
I cannot choose the colours...
Oft times He weaveth sorrow
And I in foolish pride forget
He sees the upper but I the under side.

Not till the loom is silent & the shuttles cease to fly
Shall God unroll the canvas & explain the reason why
The dark threads are as needed in the Weaver's skillful hand
As threads of gold & silver in the pattern life has planned. -B M Franklin

Saturday, February 18, 2012

Neurosurgery revision update: 12th or 15th (since May) and 6th or 7th for VP shunt (since Oct)

Hi All, Just updating for yesterdays surgery. I drove while  my Dad came along (I like driving I guess bc I drive so much the rest of the weekI otherwse dont know what to do and even if it's jsut 45mins get bored just sitting there! Dad will drive when I get dsharged. Pre-op went well and suprisingly on-time for the surgery being at 2:00. Dr.Bragg had other surgeries at her Peds hospital next door and mus have finished in good time as she was over by 1:30. I had 1 of the same anesthesiologists ive had in the past (not surprising I usppose given how many revisions/shunt surgeries we've had.) - this was one of the anesthesiologists that did realy well and I liked so that was good. I like the ones that have no problem with letting me stay completely awake right up until they are ready to intubate and start surgery. (versus given midazolam in pre-op before heading over to surgery). They as per usual used the fiberoptic scope due to airway issues and anesthesiologist was really happy afterward when he stopped by. I had my Port accessed since this past tues from my infusion nurse and so we used that initialy heading in to surgery and then anesthesia placed a 2nd peripheral IV once I was asleep. (my neurosurgeon is such a god send in this respect as when any anesthesia folk give a hard time about starting a 2nd peripheral IV she tells them to do it after I am asleep. She has walked in, in the past when I was being accessed and even the access team took 4 or 5times before they went to using the Port so since then she has been a champion for me for using the Port. Both the Port and peripheral IV where used im sure for the clindamycin and cortisol stress dose (solucortef) and then for the anesthesia drugs and both where running when I woke up. After about a 30min period anesthesia gave the ok to remove the peripheral IV. All I have now running is fluids and the colucortef by IV tapering every 8 hours.

To borrow my neurosurgeons analogies (she always apologies for using these but in all reality they make it so easy to explain what she finds and what causes the problem! Im unsure where she comes up with these but they are actually kind of funny to. So when she got in to the top incision she initially apparently didnt see anything material wise (brain material from choroid plexus and another area that frequently apparently sheds) but then when she looked further she said the valve and catheter where more than 3/4's occluded (in a 3 weeks period - yikes) and then using the endocscope she saw another area further down that had material scattered further around. Her way of explaining this was much like a toilet; in my case with the fixed pressure valve I essentialy was like a flushing toilet - you flush and all the water is dumped and then you are unable to flush the toilet again until it completely refills. Instead she wants the shunt to work the opposite like a broken, running toilet where the CSF continually is draining so that there isnt the build-up, drain, collpase, build-up, drain, collapse which causes the ventricles as described to collapse in on the shunt (she thinks because my ventricles do not expand normally like many non-MPS hydro pts I may already have the ventricle and shunt catheter resting on each other which isnt what they'd normally like.)  Because of this on-going collapsing it acts like a straw and wet bread where if you where to suck through a straw and apply that back force on the wet bread it would cause dimples in the bread and small pieces to break away and clog the straw. The shunt essentialy in other words acts like a straw.  With this new programmable valve they have 5 settings they can choose from and this is done by a small device resting on the shunt valve (on the head) and a 2nd device checks the setting and this then is removed and a 3rd device - the magnet acts to reset the dial on the valve. The valve is longer and a bit taller so there has been more discomfort with this incision and surgery with many movements but my headaches seem to be doing well and I havent had much nausea and no needed zofran. I am taking oral and IV pain med which helps the discomfort and Dr.Bragg thought i'd be able to get out tomorrow.  Right now she has the shunt set at 2 which is the resistance setting and i the best setting for preventing the dump, drain, collapse problem. If needed we can adjust the shunt down to 1.5 or lower though and will just monitor to see how it does.    Being the so down to earth and definite Peds dr she is instead of stapling telfa to my head over the stitches (1 big incision and 1 smaller incision at the end of the valve) and  she said they combed out my hair at the beginning of surgery and then when it was finished she tied that side of my hair (see below) back in a ribbon to keep the telfa dressing in place. - Very cute and I might always do this just because at home as I do think it is cute. She said she thought the Adult hospital OR staff probably thinks she's silly for the care she takes with peoples hair but I completely appreciate that she doesnt shave all of the hair on that side of head and then tries to make it look cute to boot! :)

I am still on Isolation pre-cautions which means Dr.Bragg and the nurses have to gown up before they come in to my room and for pre-op everyone had to as well as in surgery before they scrubbed. She is going to try to get this removed through Infectious Disease who placed it on my file after the injfectious UTI bug came back after the last surgery (XDR bug - antibiotic resistant). Since ive finished the course of both antibiotics (initially cipro then nitrofurontoin)  she thinks we'll probably just have to get 1 or 2 more clean cultures. My PCP's office should have faxed over their result but Dr.Bragg hadnt seen it so was going to look for that. It isnt a big deal but I tihnk everyone would love to have the isolation measures taken down! I wouldnt mind either. :)

                                                            Dr.Bragg's post-op ribbon
                                               My silly Dad taking pics in pre-op

Because I now have the programmable valve Dr. Kakkis (dr who created aldurazyme) and DrDickson really think I should consider the IT Study as they think we could use the VP shunt for the injections (given my lumbar laminectomy/nerve root surgery and nerve injury Dr.D wasnt sure it would be an option to do it via LP). Because I have a reservoir directly within the shunt they feel the programmable valve could probably be turned off for a few hrs for the IT injection (using aldurazyme) and then re-programmed after a couple hrs to the original setting. The IT injection would be given via the reservoir. Dr.Dickson didnt say but I assume maybe she would have to get IRB approval for this as when I did the decompression initial phase IT study 3+ years ago we had to do the injections at the base of my neck vs giving via an LP and she got approval for that. Im unsure if the study would be done at her hospital or at Oakland. I need to gather much more info and weight it against my other on-going medical MPS issues but their thought it maybe tbat could aid in preventing these on-going occlusions or help minimize the occlusios as they feel it is happening so much because MPS storage is likely causing the choroid plexus and ependymal tissue from the top of the brain to be thicker and thus harder to get through the shunt without clogging. A big downfall for me would be 1. flying every month for 4 1/2 hrs (lay overs help but still a long time) and I need to talk to Dr.Bragg about this and get her feeings. Since she is acting as both my neurosurgeon and my neurologist essentially I feel it wouldnt be right to make a decision like this without talking to her and getting her opinion. She has atleast a little MPS experence so I would trust whatever she said. We will see - at this point I am not leaning yes and I am not leaning no, I am just squarely indifferent I guess till I talk to Dr.Bragg and weight options. In the mean time since this would take some time and there isnt even a guarantee id get in to the study (or could be randomized for first year to placebo which is essentially 'wait and see' but you still have to go every month for other testing. I also would need to find out if id still be eligible for Paige Kaplan's  chaperone drug study (ataluran for use in people with atleast one non-sense mutation, they expect this may be in trials by the end of the year and may cross BBB and is an oral medication.)  Ive spoken with Elsa Shapiro at UofMn about this and her feeling was if we could do it she also thought I should really consider giving the IT another try for teh shunt occlusins specificaly.  So we will see.

Thanks for all the kind messages and prayers on my fb page yesterday - this means alot and I know means jsut as much to my Mom.
Thanks - go in peace and may you be blessed with good health but if not then appreciate the little things and find light where sometimes it might seem light doesnt shine.


Thursday, February 16, 2012

Surgery time

We have to be at the hospital (UW-Madison) at 12:30 and surgery is at 2:30 tomorrow (fri).

This is an unusual change with my neurosurgeon as she tries to do her shunt patients first thing in the morning and before her peds patients. I wonder though if it isnt because I am an 'add on' case so she may have had to take whatever time the OR schedulers gave. Will try to update tomorrow night.

Please also keep my friends Lea Bauman and Phil Creese in your prayers as their sweet son Gabe lost his battle to MPS II today. Lea has been one of my best friends, though we dont talk often I never felt like she judged me nor did she try to tell me how to be. She just listened and we just meshed. Godspeed sweet Gabe, heaven gained a very special little one today. This makes me want to hug my nephews and nieces all that much harder.

Between the lines, where there are no lines.

Wow, I had to go back and look at blog posts on here from 2011 to see what number this surgery would be for the VP shunt. Total shunt revision since ive had a shunt it will be 15 since 2006 with 3 of those in 06-07' (6 mo frame) and the other 12 of these now in the past 8-9 months since the end of last May with 1 in May, and I think the rest since august till now. 6 of these have been for the VP shunt and apprently 5-6 where for the LP shunt and 1 for the shunt infection/shunt removal/replacement. I know some may ask well when do you stop and say enough with these issues but the difference is my neurosurgeon feels she can help and that we have options and I see and feel  (and others including family and providers tell me) how improved it is when we do get to that good, sweet spot physically with a working-well shunt. Shunts are finicky and especially seem so in chronic disorders with MPS being no different. We will get to a place where we have a shunt that works well for long it's jsut a matter of finding the right fit essentially as terrible as that may sound. Because the pressure issues have done such havoc to my endocrine system (central hypo-pituitarism, affecting female hormones,  cortisol, thyroid, etc) and we see how these improve (although perhaps now the cortisol may not improve fully any longer) and then improve my autonomic issues (temp regulation like decreased and increased sweating, GI system functions much more normally with functioning shunt, and headaches go away completely, vision improves, nausea and vomiting go away it isnt a  hard decision to try and find a better fix to the shunt.- As they say you either have a shunt that works well or you have a shunt that never works until you just get to a good spot.

I get asked alot how I stay positive and what are my emotions really like underneath as I avoid talking about how I really feel about things. I dont really have an answer to this for people other than what sounds very cliched im sure (it isnt fair to take it our on someone else (though I am not perfect and do have moments) and that it could be worse.)  but I do believe in living 'one day at a time' as an old provider once told me not long after I was dx'd and I believe in asking for help when I need it but that I also dont need to tell everyone everything how I feel deep down. Thats just my personal feelings and it is bound to change day to day sometimes to. And honestly if I want to talk about how I feel i'll tell you but mostly id rather listen to someone else and as I told my psychologist the other day (whom  I hadnt seen since early last fall) if I could/when I can I much prefer to turn it around and ask other people how they are. In truth people read to much in to what I write or even say sometimes, for instance a small group of my providers have asked to be sent updates after bigger surgeries so I do this and certainly not a big deal.  A while back a provider said to me I seemed anxious in what I was updating after one of these surgeries and I knew exactly what she was talking about but was a little surprised/amused really that this person was reading so far in to a simple update. Believe me if im going to tell you how I feel about something it definitely isnt going to be in a group email to all of my core providers or ina group email to anyone. (4 or 5 providers get the update) I will tell you if I am feeling nervous or scared or whatever about something. It drives me a little crazy when people think that everyone has to get nervous or anxious about what is going on. I have a firm faith in God and I have a good group of providers that I work with and as well have a reasonably strong support network - my parents are amazing, I am close with my sister and my sister-in-law and I have friends who rally with support both in-person and online often and honestly my biggest inspiration is my 5 yr old autistic nephew. - His is a hard life between his autism classes, his split family and not the best relationship with his step-dad, adjusting to his little sister (whom he loves but get nervours by) and 2 different other classes 4 and 2 days a week and he still has a smile most every day and is funny as can be so why shouldnt I also try to have a smile everyday?  It is jsut me but for surgeries  unless it is a major surgery the one thing I will likely be a bit on edge (and rightfully so) is anesthesia and making sure that the pre/post cortisol replacement is given as I know how dangerous this is if not given and I know since you rarely have the same anesthesiologist everytime I have to be educated about why this is needed (although my nrsgn is great about it to). Like my Neuro-Endocrine dr said the other day I might sail through surgery with little problem and only have a tougher time recovering if the stress doses of cortisol werent given or my body in the one instance we dont do the stress-dose replacement cortisol may not be able to take it and my body may not recover and that can (another sounds to dramatic but is very true) lead to death. Does this sound dramatic? Sure! Should it? Yes! Between the airway issues and present cardiac issues and neuro-endocrine issues we have reason to be concerned but my drs have cleared me for surgeries and when they are fine (with pre-cautions in place) then I am fine as well.  So please people stop reading more in to what I write then is there, again if I am stressed or need to vent I will vent but otherwise I just am writing or updating pure and simple and for providers if you read this I know you've asked to be updated and I know some of you do read the messages post-op/post-big issues as you reference these but I also will not be heartbroken if there are some who do not, it doesnt hurt my feelings it simply is a mechanism to keep the core providers 'in the loop' and 'on the same page' as alot goes on with my care with very many different providers and not everyone gets all providers reports. I do request copies of all reports but it is still impossible to share these with everyone. If providers do read this then great, although I would hope like with the 'not reading more in to my words then is there' that you are not offended when I vent as  I am human and more than just a patient. Thank you!
**I was thinking about this post this morning (I wrote it last night) and do feel I should add a addt'l comment  - I met with one of my providers after ERT (at another clinic) as well as a girl who is also a Psychologist (newly so, congrats J!)  and if she is here reading this (we have become friends of sorts on fb and she also blogs about her own health issues) please dont think my comments about not talking about my emotions was aimed at you as it wasnt!! There is a big difference between your asking and someone in another specalty asking although in that other providers case I know to they just want to make sure their patietns are ok and thats a good thing I just wish people didnt try to lump everyone in to one category. **

Thanks for reading this - I know it's a bit off the beaten path but seemed like I needed to write it as it is something that like I said I do get asked about alot! (how do I stay positive) - Now if you where to ask my family they might tell you just to hang aroun a bit more and I wouldnt disagree I am stubborn and I do have a temper at times so sometimes my frustration gets taken out (not on purpose) on innocent people I love in my life. I do know to apologize though as apologies mean lots!
I'll update with surgery times here or on facebook and will try to update sometime fri after surgery.


Friday, February 10, 2012

Mindless thoughts, good info to?

Things on my mind today:

Some providers are high on my list of ones I prefer to work with while on the other hand  one or two of their secretaries are low on my list of support staff I enjoy talking to/working with. (and over the years ive gotten to be close friends with very many support staff.) My infusion nurses and I text. email and share facebook comments so I dotry hard to be kind, be patient and not be annoying! The reason I write this is because when you have a dozen + different specialists and you get ones who never return your calls or you have to keep calling them despite leaving VM's in hopes of catching them in does get old fast - I completely get that we all at times forget things but with some secretaries it is EVERYTHING  and EVERY call it seems like they never return. Yuck!

-Thinking of things i'll need to do before the next time I travel (get a letter from my PCP stating I can take drink on to plane - most often security will jsut let me when I explain my situation but on occassion harkening back to "you dont look sick" some security will simply refuse and I end up paying for drink after security. Not always a big deal but sometimes it is.
- My neurosurgeon has given me shunt cards for each of my shunt valves (one is PS Medical-medtronic and the other is Paedi-gav) but both are fixed pressure so no concern with airports. I'll have to doubly make sure to get a new updated shunt card with the programmable info and keep the card on hand when traveling , although I normally do just get them laminated and keep in my purse with her info.This is because airport screening can reset the magnets in shunt valves and so pts typicaly get waned instead.

- Talked to Dr.Kaplan at CHOP and she expects skin biopsies to be done in a few weeks (the testing) and if a significant amount of ours turn out/react positive to the ataluran (study drug) then they will file for a IRB for human trials. This is a drug already in trials for CF and DMD so should be less hard to get a trial approved but she thought this could potentially happen before the end of the year, (again if biopsies are positive results).

- Very looking fwd to vision improving and hoping this programmable shunt is the trick - going to ask about who she recommends for Opthalmology at her hospital. Pain Mngmt dr today asked if I had saw the Neuro-Opthalm she recommended at Medical College-Eye Institute but I havent simply bc makes sense to see a Opthalm at Dr.Bragg's hosp so communication can better occur.

- Lisa from Dr.Bragg's office called late this afternoon they are trying to schedule surgery for next friday but Dr.Bragg's schedule is full so scheduler is working to try and re-arrange her surgeries to get me on. My thought? Clearly spend to much time with a doctor when they will rearrange their schedule on many occassions to try and accomodate a patient of theirs so as to not let pain go on for long, and as little as possible. I appreciate their kindness as she did this with her tight schedule also on weds when she doesnt otherwise have clinic. She took an hour of her time to see us anyways. **The other nurse called and confirmed that surgery will be this fri and the NP called fri night to go over UA pre-op questions as we need this to be clear so Infectious Disease docs can clear isolation measures from my file. She faxed over the previous UA 'bug' results to my PCP and will do the test on monday before ERT. No pre-op required as we beat the 'every 30 day' requirement by 3 days with surgery being on friday.
- See Neuro-Endocrine dr on tues - will be ineresting as certain hormones really seem to have been affected over the pre-ceding months secondary to the shunt issues; some improve when the shunts improive, some things didnt and some things are jsut plain new. I plan to ask her if she might be able to her me rgaph my hormone levels over th past few years pos-initial shunt and revisions and  give this to my Nrsgn just because. Since this dr works with the Neurosurgery docs at FMLH she knows issues with hormones secondatry to pressure issues inside and out and has been an invaluable provider on my team. I hate having surgery, period but especially hate having it on friday's so am dreading next week and the weekend. It also basically ruins my parents weekend off from work which sucks, even though they dont complain I still feel bad. :/

Pain Mngt dr in few mins we did talk today would like me to have a EMG (nerve conduction) done on my very low back and legs to see where numbess in my leg is coming from - it could be form the spinal nerve injury although it is in hte opposite leg more often than not over the past 2-3 weeks so I am unsure whats causing it. I will talk to Dr.Bragg (as she acts as both neurosurgeon and neurologist for me now) to see if she thinks the test would be helpful to her/would it change any outcomes. Definitely not that I dont trust PM dr just that isnt fair to not run it past Dr.Bragg since it s an area she has been managing.

Saw my nephew today (my niece was sleeping) and felt badly for him - I love that kid to the moon and back and in many ways I get him bc my life isnt a normal persons life and neither is his but he constantly reminds me that even in the middle of bad moments we can stop, re-assess and be ok. As he always says "It's no big deal" - this has become something of my own mantra lately along with my long held 'one day at a time, one week at a time' as that is the best I can do and it allows me to not worry and just enjoy what I have now. Like I always say doesnt mean you dont stop planning for say 2 weeks from now or the future in general just that when it comes to every day you take whats here now and try to enjoy it as it is. Like Zander with his 'It's no big deal; and fully knowing he doesnt always believe himself when he says this or sometimes he says it like he's asking permission for something to just be ok I also have times where I fail at my 'one day at a time' but we're all human and you do the best you can do!
More to follow,

Thursday, February 9, 2012

Neurosurgery Appt Good; Good Doctors Great!

My parents went with me and we met with Dr.Bragg, my neurosurgeon for close to an hour (she had graciously re-arranged her schedule to meet with us as this wasnt her regular clinic time).  Ive detailed some of the conversation and thoughts + decisons below as well as a few other of my 'ramblings' inter-mixed throughout. Essentially though before trying the ICP and EVD study again which I talk about below we are going to try placing a programmable valve which yes, does mean another surgery. Once again Dr.Bragg and her team have re-arranged her schedule and surgery will be next friday I believe the 18th.

"The goal is to find a setting that provides a tolerable upright flow during the daytime, yet flows well enough at night to accommodate ICP spikes during REM sleep (from too high of a valve setting" - I pulled this from an article I was reading and it is one of my issues where my headaches are much worse at night (I havent fallen asleep since before 3 or 4 in the morning in weeks and wake up with aweful symptoms. -- Headaches are worse at night because there isnt as much gravity pulling over-riding the shunt valves so pressure builds up and affects vision and head and all these other symptoms and then when I get up in the morning my body re-adjusts to over-riding the valves again due to gravity which is why the pressure is better then but I may need to be draining more at say a lower presure valve/setting. This constant up and down is why even though the H/A's improve during the day alot of the other symptoms havent this time (vision especially as it takes time for the optic nerves to heal). Other things that havent improved as much are endocine hormones (period is a day  and spotting whereas it should be longer and cortisol is affected so I feel tired even despite the med replacemet as well as GI isnt as good as it has been sometimes (constipation) and autonomic symptoms are so-so bur have improved greatly each time we've had the shunt in a really good place.

I see my Neuro-Endocrine dr tues and so am going to have her help me graph out a time line of my endocrine hormones over the beginning years of the shunts in 06 up till now although we only have measurements from 2 weeks after surgery back in June and non since then as I was supposed to see her in 3 months and re-check labs (everything from female hormones to thyroid, cortisol and growth hormone for isntance) so we are doing them now at 6 mo roughly which is better than nothing although we wont see upward trends (if we will at all now with the pressure having gone on (and off) for so long  - I dont know nit if hormones will improve it wont be till we get the shunt in a good place for longer.

My Mom asked couldnt I just stay like I am, would that be so bad (ie would it hurt anything) and Dr.Bragg was sweet to jump in and "she feels like crap' and "we can get her feeling better more of the time".  My Mom wasnt saying it like she wants me to feel bad but more because she sees me more when I am feeling better and we werent sure of a plan at that point. I appreciate my Mom's concern and appreciate Dr.Bragg because so has gotten to know me so well on many levels.

So Dr.bragg thinks the cause of the proxima (top) shunt occlusions have been due to pressures ebbing and flowing up and down like a roller-coaster  with ICP (intracranial pressure) goes up and ventricle opens and then the coaster goes down and ventricles collapse and then back up slowly and ventircles open and close over and over. Or like a clogged drainage pipe - pressure builds up for so long that it eventually gets past atleast some but then re-clogs because of the defect and then is forced open again by pressure.

One of many things we talked about  was switching the distal catheter from the peritoneal area to another site but no decision rhere and we are going to leave the proximal - beginning end in the frontal ventricle. The reason this was brought up is 1. sometimes shunt occlusions are because of CSF not being absorbed properly when drianed in to the peritoneal cavity and 2. this appears to be common in MPS Hydro pts.

Dr.Bragg and my parents and I talked  extensively about why she thought repeating the ICP monitor would be helpful including she could leave my shunt open and place the ICP/EVD on the other side and watch pressures and then adjust to see where I sit when I have no headaches which they could also 'experiment' essentially by placing outside valves on the EVD (external drain) to see if these had any effect - she talked about a patient she's worked with of her partners who they had to put 6 valves on the persons EVD to get to a good place. She also would probably bring Opthalmology by for a consult regarding the vision stuff and talked about doing a brain dye study to see if she could locate any clear area where either  a obstruction is happening or where the different parts of the brain arent communicating and this could be done while the drains where in place. We are first going to try doing a programmable valve and see if this helps - the valve will have 5 settings and can be adjusted by a simple magnet up or down to see how headaches respond to varied pressures as she does wonder if I need to drain more fluid. (with a programmable shunt would need to avoid scanners at airports and have it reset after any MRIs/CT scans)

She talked about my airway issues and how the anesthesiologist though they always (and I think they do) read my previous anesthesia notes and are aware of the  difficulties (when they either call the night before or come in to pre-op they have been well versed) mutter away when they are trying to get me intubated and how she hasnt ever looked while they are intubating me but that it clearly is very difficult for anesthesia and she woulnt want their job. She mentioned kind of laughing but yet serious how these drs know my airway is bad but yet bc I look so good they clearly dont realize just how bad it is bc they apparently assume if I (again) 'look' normal then I really mustnt have that bad of airway regardless of all the previous anesthesiologists notes but then  they are actually in the process of doing the actual intubation and fully get aware in a crash course of sorts of what they are dealing with. Many of these anesthesia docs have stopped by in post-op to comment on my airway and a few have admitted they where frankly blown away by just how much more difficult it was then they thought it would be. Kind of funny really - the reason we had gotten to talking about this was I brought up how I had met her colleague a few years ago and he clearly then didnt believe me I had pressue/needed a shunt and that I had a accurate diagnosis and how it IS frustrating that some drs cant get past how I present 'nomral' even when I feel like crap bc I beleive in taking care of myself and tryng to look 1/2 way presentable. I do little things to look a little better and it is only me so I handle 100% of my own care even if I dont feel good and dont feel like getting out of my PJ's. She mentioned how she can tell when I dont feel good and when I do but it took her a while to pick this up and if drs arent looking they wont realize bc I geneally wont admit to feeling bad unless it's really, really, reallllyyyy baaddd.
I talked to her how I appreciated she believes me despite all this and that she has never once questioned my MPS dx just because I "dont look like I have it" (ie my fingers are very long and my face is coarse but not as same features as some)  and  that it is frustrating much like the above trying to look decent even when I feel like shit. Well just bc I dont 'look like MPS' doesnt mean it hasnt done it's crap on my body from hydro to endocrine, cornea clouding to multiple repeat carpal tunnel surgeries and tendon transfer in hand to severe cardiac (all accumulated together)  issues and on and on and on to hips, back surgeries, etc in addition to multiple hernias and a few surgeries as a kid. (its pretty clear by looking at past records I have it but some drs want to beleive what they want on initial consults and they are just arrogant know-it-alls). Anyways this wasnt meant as a ramble more as a vent but also as a 'thankful for a dr who really does believe me' and thinks independently regardless of what other comment to her.

I know there are things we talked about ive missed as we sat and talked for close to 45mins so if  I do think of anythin bigger I can always edit this.
Thanks for stopping by,


Tuesday, February 7, 2012

Shunt decisions or in-decisions

Just a quick note - I called and spoke with my neurosurgeon nurse for quite a while this morning, I like her and know she wouldnt mince words but I also know she wouldnt just say something just to say it to me and is honest. I was really upset about all of this and it bothers me that this would be the 3rd ICP monitor and feeling like my neurosurgeons colleagues are almost convincing her that im not really presenting with symptoms though she herself has said to me so many times over the past 6 or so months that while the effects have never been long standing she has seen how good it can be/I feel when the shunt was working well. Im just not comfortable doing another 'blind' ICP study and not really having a clear answer what we hope to gain. Sure I would love to not have this shunt and that has nothing to do with why I feel like I need to gain more information before just jumping in to that. I think my neurosurgeon belives me but I think also her colleagues  because I dont present clear cut have never met me and non of them where on my original team back when the shunt was first done and none saw the improvements after each of the LP's. Like I think I said before it is hard not having my former PCP to vouch for me (now a hospitalist) nor my former metabolic-neurologist who also saw me and had seen more cases of high pressure/hydro in MPS pts than any other neurologist or neurosurgeon anywhere so he knew what to look for and how it presented. They didnt see how it used to get so bad I literally would be driving somewhere and would pull off to the side of the road, open my car door to throw up and then keep going because it was everyday and I still was working + had many other MPS things going on. My sister called this morning and was just blown away by all this and frustrated that once again because I dont 'look' (in appearance) like the average MPS pt and I dont always present the same that someone, especially she said drs who had never met me would assume I just didnt have anything and was mis-diagnosed from the start.  At one appt several years before my neurosurgeon came to this practice  her colleague when I was referred to him accused me of being a drug seeker even though I had not once asked anything about medication AND I had a Pain mngmt dr who was so upset when she heard this as she knew how bad I was feeling but also knew I wasnt the type to go behind her back. She trusted me and was upset that this other dr had never bothered to talk to her first before jumping to an assumption about me. Thus im sure is why you, the reader can see why I trust this other colleague of hers not an ounce and why it all bothers me. Sure he may be a good dr and many may like him but I dont and I dont trust him. Brains matter little to me if the dr has no respect of the patient and cant get past their perceived indifference of a patient to actually get the story.  Some may wonder what happened to my former neurosurgeon and honestly thats a 'no easy answer' line of question. My Neuro-Endocrine dr told me she works with him and his colleagues (at a different hospital) and that he had dropped another of her difficult patients to basically because that patient also had multiple revisions and he didnt want to deal with it though I honestly think it had far more to do with problems with his NP than with him. My former GC and former PCP both refused to talk to her (the NP) as she was so blatantly rude (I happened to be listening to a conference call my Geneticist had with this NP before my first revision when I had a golf ball sized and growing pocket of fluid on my low back and the NP flat out told my Geneticist he was wrong. He was irate as clearly he could see it and knew it hadnt been there at the previous weeks ERT infusion. I apparently have sucky luck with male neurosurgeons or their staff although I do remember his nurse and secretary where very nice. I dont want this to seem like I dont think my neurosurgeon believes me I just think she isnt sure what to do and is being told things by her colleagues and imsure it is hard.

So anyways in talking to her nurse today I asked instead of jumping in to the ICP and resulting issues could I think about it? We agreed id do this and call her back this afternoon. I was really upset by this time by it all because I know others have seen how good this is from my parents who I see a couple times a week, to my sister, my Physical Therapist (who again last week just commented on how much different the systemic change is to my body even despite all our issues with the shunts because we've had it working on and off and the difference this had on tissue swelling, muscles, etc is stark to him. I cant quite explain it like he did but it completely made sense how when there is pressure it affects your whole body. Other providers have seen the increase in energy, the endocrine improvements over time (and then associated decreases with pressur re-building up and improvements over and over) as well as the actually quite amazing positive affect on GI system (constipation improves completely) to the autonomic improvements  such as temp regulation/sweating issues going away and blood sugar issues stabilizing among other things.  I called and talked to my Mom as I  and we decded we'd like to meet with my neurosurgeon and talk about all this more (parents dont usually go to appts because of work and sheer number of issues beyond just neurologic I see drs for and I am a capable adult who can manage it on my own and prefer to for my own sanity most of time. (thats just me) They've set up an appt tomorrow afternoon so I wrote down a few things to bring up like I did here and also a few questions such as wouldnt I be getting low pressure headaches if the shunt where doing harm/werent pressure and a few other things such as why all these other symptoms always improve amongst a couple other things.

I'll update when I can tomorrow night or thurs.


Monday, February 6, 2012

Shunt take ??

Ive been apprehensive since last weeks appt with my neurosurgeon but tried to block it out as much as possible over the weekend but when feeling well I wanted to have a good weekend which I did. Thanks to taking zofran pre-emtively in the morning and right away at night I only threw up early sunday morning (awoke out of a dead sleep and thanikfully have a garbage can next to my bed). Otherwise days have been pretty good with just occassional milder headaches and nights so-so.

So anyways Dr.Bragg (my neurolsurgeon) called while I was on my way home from Children's Hospital (Milw) with some information and questions. There's not even a slight chance i'll remember everything we talked about as I think we talked for well over a 1/4 of my drive and covered many things but also kind of went over the same things multiple times trying to iron the possibilities out. The jist is that they are wanting to do ICP monitoring again with the VP shunt in place to see what pressures are and then probably tie off the VP shunt (a relatively simple knot) and watch to see what pressures are. If it goes 25-30's (intracranial pressure) then it's a matter of figuring out the shunt still but otherwise possibilities of potential other options several of which she somewhat touched on like 3rd ventriculostomy (hole created so ventricles communicate I think) this I dont quite get how would work in MPS given the storage material continously but will cross that bridge if needed. Other things she mentioned was having Opthalmology come by to see if they can weight in (I get vision affected with the headaches but papiledema (pressure showing up on the optic nerve) doesnt show up often in MPS pts. She said it's possible it is more of an obstruction problem and wondered if we had done any imaging with dye although im not sure now if she meant of the brain to see if there is a place that is causing the problem or if she meant through the shunt to see if the dye gets through.  She and her colleagues wondered if they may be able to find a non-shunt way to manage the pressure issues so we will see. She talked about 2 areas especially in MPS that present different in MPS and could they isolate a specific area that pressure/problem is coming from and find a solution to it. She didnt know but apparently dealt with this in MPS pts while she trained in Utah, I kind of figured she had to have saw atleast a couple MPS pts there (Primary Childrens Hospital). I had asked her if she thought the location of my 2nd valve could be affecting the shunt (sits at my collarbone was originally below breast) and she said when she was training they where able to demonstrate if 2 valves where put next to each other the back pressures where not regulated at all compared to when the 2 valves are spaced further apart so it may be a possibility she isnt sure.

  I dislike very much not knowing solid plans and dont feel great about this (not that I dont trust her) and hate that once again have to re-schedule and re-try to figure out everything I have going on. I wish this damned shunt would just work and I wish it would just be done with so I could do other things. I feel bad for Dr.Bragg because clearly it has to suck when you struggle to help a patient and answers arent clear but I also just pray and hope and wish we figure something out soon. Please just say a prayer this monitor shows something and we can move on with an solution quickly. In the mean time tonight ive emailed one of the drs who created the Enzyme replacement drug I receive each week as he is as familiar with MPS as anyone I know to see if he had any take on all of this. I have seen a few other MPS I pts have similar issues and suddenly they get to a place their shunts just work well so thought maybe this dr would have an idea.

Dr.Bragg's nurse is working out the details of all this so if ive forgotten anything will edit this and will update when I know more.


Thursday, February 2, 2012

Post-Op Appt, options, ideas, insight.

So where to start? I doubt im going to get all this info correct but saw my Neurosurgeon for fup late this morning regarding post-op from last weeks shunt revision. Turns out the UTI from last week grew back a bug that is resistant to many antibiotics including what I was on (it did help some interestingly) and Dr.Bragg plus her staff had to do isolation precautions. Interesting.  So she dd a shunt tap to see how the shunt is working as although my headaches improved since last weeks surgery they arent completely gone and she is concerned about this. The shunt tap took longer than normal but eventually they where able to draw back a couple cc's of fluid although the valve appears to be sluggish again. She talked about some options we have but is going to present my case on monday at their Peds-Neurosurgery weekly meeting and bounce ideas off of her colleague. (always makes me a bit nervous as he is the one who did not believe me severeal years ago) but she has promised again she isnt going to give up and thinks we can get the headaches better under control. Some options include (and this is where I should have taken notes so will have to update after mon) either moving the shunt to a different ventrice where less tissue (choroid plexus) to get in the way of the draining, adding a 3rd valve to slow down flow (though she is concerned my ventricles want to drain more fluid but when we didnt have the second valve I occluded and now when I have the second valve am still occluding because pressures appear to fluctuate variably.)  or adding a programmable shunt which we could then adjust the pressures as needed. Other options might include optic nerve fenestrations (if it is more of a PTC phenomena) which might either help the shunt or allow us to get rid of the shunt though this seemed less on her radar but I could be wrong. Im not sure which she is leaning more towards as she talked about all fairly equally. Other option may be that we need to put the distal (end) portion of the shunt tubing in a different location such as a vein to aid in better drainage and absorption as she said it is possible my body isnt absorbing the CSF well and this is causing the spikes and then lull's in pressre.  She joked that my body is allergic to these shunts which I think I might have to agree with.

Honestly I suck at waiting and so while I dont want the weekend to be done I do want it to be monday (either Dr.Bragg or Lisa will call and let me know if they came up with any ideas or what the plan is. I am at ERT infusions every mon but typically use that 5-6 hours to schedule/cancel/rearrange any appts I have or need to make, take care of endless paperwork, deal with any insurance stuff (thankfully I dont usually,typically have alot of this as my primary insur Case Manager is very good but I do end up with some things inevtiably to take care off. Compared to other days I usually have my phone nearby  so I sjouldnt miss any call from them  on what happens next and yet with me I seem to do a great job at missing call! :). I dont know actually what I hope for answers wise - I think it all freaks me out slightly (like ive said before I suck at uncertainty) but I also know Dr.Bragg is very good at what she does and I know she cares about my best outcome which is why she isnt rushing in to anything. She had asked me if I had ever been on anything before for the headaches and I have been on diamox pre-shunt although it worked some but not great. We didnt delve in to that further after talking about the affect it had on my labs (went a little crazy so former neurologist was having to adjust the dose some) but on the way home I was thinking about this all and wondered in my usual after appt insight (I so need to be one of those quick on my feet to come up with questions kind of  people!) if diamox could help now/buy time while we do try to figure out what to do. I left a message for Lisa (her nurse and whom is fantastic) asking her this very question and apologizing for not thinking of it then - I dont think Lisa minds my questions but still its not as if I am their only patient! **Add in: Lisa did call me back and talked to Dr.Bragg who didnt want to add in the diamox just because she'd really like to have almost a clean slate (so to speak) to work off of and make a plan with her team at mondays team meeting so it doesnt get messy/confusing on what is affecting what. I love that Dr.Bragg sort of thinks aloud and shares any and every possible idea and goes over each thing but then will admit it's alot to process and like I said im not that quick of a person to come up with immediate, un-planned questions!

 Also going to try sleeping upright in the recliner with a airline pillow (those u shaped pillows)  to see if it causes less neck pain (plates and screws so not as normal of movement)  and thus less headaches in morning because headaches for me are worst at night and in the morning.
If I think of anything else ive forgotten will add it later.
Say a prayer - may be a long weekend (though good things planned even if when the headaches are there and the resulting symptoms it really does stink). I really do think one has to try to make do with the good times though when possible and speaking off off to get some zofran!
Take care, God Bless,