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.
http://www.conquerchiari.org/subs%20only/Volume%204/Issue%204(9)/Low%20Compliance%20Trauma%204(9).asp

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,

Erica