Being home, Zander's here, Happy Easter!

Ive been home since Tuesday and has been nice; ive been able to go to Maundy Thurs and Good Fri
services for Lent which has been especially nice. Using measurement as a gauge of how I feel headache and associated symptom wise I feel mm's better in an inches world so that's something compared to nothing! I havent been sleeping well and so when I do finally fall asleep very early in the morning I end up sleeping till 10-11 or so in the morning and the one day didnt have anything going on and didnt have an alarm set + didnt end up waking till I think it was around 1 in the afternoon (after falling asleep around 6am) which was a little crazy! Ive also been feeling really short of breah (not as in "I just ran a distance or was in a hurry short of breath" but "I cant get enough air and feels a bit like I am suffocating" a quite crappy feeling and much like I felt like before I started the bisoprolol and before we switched to that when I was on the calcium channel blocker-diltiazem and lasix (still on this). Unsure what this is about as it has been really improved since last summer. Yuck!  I also seem to have picked up a bit of a cold the past few days mainly just a stuffy nose which in turns causes a small bit of a sore throat but no fun, no less! Hopefully will be on it's way in a day or two.

Zander is spending the night tonight and sleeping in the other room (I sleep better typically on the couch so he is asleep in my bed), we dyed easter eggs (using kool aid, turned out pretty nice) earlier for at my parents tomorrow and otherwise just where looking up and watching various bridge videos on youtube and the internet + drawing as well as reading after supper and his bath. Zan loves cruising the internet and is actually very good at it so typically he will have my netbook and I have my laptop + we'll be side by side looking up videos together. Kind of fun to see what we find! I also had picked up a few bug/insect and train videos from the library the other day (his true loves) and we had those going in the background. I am actually all for quiet when it is just me so there is definitely not much of that when he is here but thats ok to! :)


This week has been pretty ok, tiring but ok as it's been nice to atleast feel a little better. My guess is to those around me (family for instance) its hard to see any difference as ive had lots of comments how quiet I am when my Mom, sister and I went out for sister w my niece the other night and when we where together for something else today but I do feel like it's a little easier to get up in the morning and though the breathing is definitely making me a little crazy it's nice to have a little break from the severe headaches - hopefully this will last! I am hoping if we where to move the codman (VP shunt) valve down a setting when I fup w Dr.Bragg in 1 1/2 wks that maybe that will improve symptoms even a little more. That would be a AWESOME result especially since I leave for Boston 2 days later!

Ive cont'd to have the Zofran delivered each week by the home health company but dropped from using it typically 4 times a day ((and some oral med!) to typically just 1 or maybe some days 2 times a day and a few days have supplemented w oral zofran to). SO NICE! :)
Zofran

Thanks for stopping by, -

Erica
ps the shirts and stickers I had made for the Genzyme-Boston Marathon (rare disease team) turned out really nice and a bonus the shirts are 100% cotton! The shirts from Genzyme are a silky material so more clingy but also pretty comfy. Nice! :)

pps I am back to teaching sunday school next sun (no class on Easter) and my co-teacher is taking the sun I am in Boston) - I am looking fwd to seeing my kids again as its been 3 weeks now, my how time flies! :)

2:30 - Dr.Bragg stopped by and asked how the shunt adjustment is going (see below) which it helped a small amount (she commented a mm in the grand scheme of shunt adjustments which is what it feels like ie helped a little but no where near enough) and then said we have other adjustments and changes we can make - we got to talking about my nephew's parents and her step-daughter and so I didnt ask her what she might be thinking but atleast it seems she might not be giving up? I know I need to trust her and I do but sometimes it just is hard I guess to fully put your faith in someone when you struggle so mightily with something so much and they help but you also know they only see you a % of the time and really can prbably only do so much? I dont really know... As ive written before I really like her so it's just a me getting past, past exeriences with former providers and believing in her + in truth learning to do a better job all of the time asking questions, getting details... Sometimes I guess I am just tired and not always the best memory either, notes dont always help either bc then those distract from the conversation at hand (hard to explain that one).   Little by little over the years ive gotten better though and I actually do think her and I make a good team. ...

Earlier in the day...
I talk about this alot and im sure it gets old reading about it (but its my blog so I guess I get to write about anything I like!?)  but I am headed home today and while you think you prepare yourself to probably only feel somewhat better after a surgery such as this one (as our hope was at the least to improve symptoms some, to buy us time)  I dont think you ever truly get used to 'another surgery, another in-complete fix' once it comes to discharge day because as much as you want to go home and look fwd to the comforts and quiet of your own space away from everyone you also wish you where going home with that elusive, magic answer feeling better, feeling more whole as if you could find the carrot dangling at the end of the stick..  Ive not talked to Dr.Bragg today yet and am unsure if she is stopping by before I get out but also am doing ERT this afternoon which will take 4 or so hours and slated to start around 12:30 so maybe she will come by this afternoon? I hope?  If nothing else its nice to be able to talk to her and talk about our fup and future plan (whatever that may or may not be) before going home after being in-patient.  I tihnk there are some left-over 'un-knowns' from this stay including what caused the incredible fluid retention and feet swelling + why do my feet still feel some tingly and why is the TPL area of my back so uncomfortable and last why is vision so ridiculous? Who knows but is very frustrating!

Dr.Bragg is on vacation next week so if I dont talk to her today I wont see her till the 11th in clinic and am guessing maybe we would adjust the new VP shunt valve down 1 further notch to 40 (it can go to 30 but we likely wont do that, but am unsure.) We have the TPL shunt set now at 1.0 which seems to help keep it open vs having it set at it's lowest at .5 and likely will keep the VP shunt set at 40 if we did go down further.  I am really unsure of any thing related to the headaches and symptoms right now. Like ive always said uncertainty is never my strong suite and MPS is a whole lot of crap uncertainty. Hate IT!


I made my reservations for Boston and am flying in Sat afternoon in time for a dinner w the Rare Disease team and fellow patients Sat night + then we are all doing a duck tour of Boston Sunday followed by a breakfast on Monday morning and the Marathon + I chose to fly home right away monday night. My friends I am staying with said I should stay till Tues and I sort of wish I would have now as it wont leave alot of time (I could maybe change my flight to Tues) but over all should be a really fun trip and I love Boston so am really excited! I need something like that to look fwd to!
                                              Shirt for the Marathon - the whole team has them.

Will update if or when I know something,
Thanks for stopping by,
Erica

Shunt settings, Boston Marathon, Genzyme team info.

I may get out tomorrow or Tues which I forgot to ask and for that matter have been meaning to ask Dr.Bragg since friday the Pharmacy here on the floor tried ordering the aldurazyme from Genzyme fri but didnt reach anyone and so said they could order it monday for Tues. I have been meaning to ask Dr.Bragg if we should go ahead and do this or what she feels as the only day I can get infused at CHW (milw) is mondays.   We are stopping the PCA today (I believe) from what Dr.Bragg thought this morning so I am not sure what time that will be but she was going to leave it written so I could still do IV pain med bolus as needed if needed and of course oral med and IV zofran (although even though l feel far from perfect I atleast have not needed as much or very much of the IV zofran since we've been adjusting the valve).  I just was talking to an MPS friend of mine whose son also has MPS(II) and just had a shunt revision and although doing better to is still having she believes some symptoms and I said to her I just cant help but believe that in some of our MPS shunt cases even though the drs can see the occlusion typically at the proximal (tip) of the shunt when doing revisions that we may have issues w partial blockage all throughout our shunts with these GAG (complex sugar storage) deposits (because GAG storage is clear and not visible to the eye) and so when they fix a part of the shunt it helps CSF flow somewhat better but not complete resolution of the issue? I dont know but wish I did.. The reason I wonder this is because ive always felt so much better (near 100% improvement) right away after shunt replacements vs with revisions I typically only feel partially better..                                                                                      

 

 

We have been slowly adjusting the Codman valve down and right now are sitting at 50 which is about 3/4's of what we can do (settings range from 10 to 200) with the tentative plan we will adjust the TPL shunt up from 0.5 to either 1.0 or perhaps a little higher. The thought behind this as I think ive written in other updates is the VP shunt which is basically the main of the 2 shunts or does more of the work (I think, I think Dr.Bragg has said) can help keep the Thoracic Pleural shunt open if the TPL shunt is set a little higher and thus drains less so collapsing less (I cant quite explain the reasoning, will have to ask Dr.Bragg). Dr.Bragg came in this morning saying she was thinking about last summer when we did the trial with the then LP and VP shunt in which we turned both shunts down as low as they could be set (.5, both where Strata programmable valves) and did serial shunt taps with the shunts set at this low setting and then also with the shunts set at (I believe thinking back) the VP, .5 and the LP at 1.0 and then VP, 1.0 and LP 1.5 (or maybe vice versa) and where able to establish with little question that the shunts both drained more effectively at slightly higher settings. Im not sure we will do the same "test" to figure this out but we are likely going to be playing around w the settings on each shunt. I do feel a little better than when I came in for this surgery and so while I wish I felt superb and near 100% like I have w the external drains or like the times we've put in complete new shunt systems I am glad to have this small step to work with.  

 

Although I saw the Neuro-Opthalmologist just shy of 2 weeks ago (for fup, repeat visual field testing) I am seeing the local Optometrist a week from Tues to get my glasses prescription changed; this eye dr had thought last fall when I got these glasses and new rx that I really needed stronger ones but also thought I wouldnt be able to adapt if he just threw me in to the new strong rx right away so he wrote it for somewhat less. The prescription has a 1 year warranty so while I have to pay for the appt any new lense prescription will be covered under the warranty (thankfully!) from last fall.  I am actually looking fwd to getting a new, stronger prescription as I think we've been dealing w these shunt issues to long and not enough of positive pressure change or over long enough period that my vision goes back to complete normal. Hopefully I will be able to read the computer screen and a book more easily again; I miss reading!!!

 

Ive decided I am going to go out to Boston mid-April for the Boston Marathon and to cheer on my Genzyme runner (she is part of the Genzyme 'running for rare diseases' team and running on my behalf to raise awareness of MPS I + is raising funds for research for NORD (I am fundraising for the Nat'l MPS Society and  the link is on the side of my home page). I plan to fly in that Sat (13th) and stay w a friend of mine, an MPS III family (they are fantastic, run the Ben's Dream Foundation) and will meet up w Jess and the other runners + rare disease pts (representing other rare diseases Genzyme has therapeutic/drugs, programs available for) for a dinner and then on sunday we are doing a Duck tour of Boston (have been to Boston 3 times, including last summer for the Nat'l MPS Society Conf.) but never done a duck tour as I missed the one that was done for adults affected by MPS at the Nat'l Conf. last fall bc of planning/running the meeting for MPS I families regarding several researchers and the drugs they are studying that same night). Monday is a breakfast at the Allston plant for Genzyme (where some drugs are made though not Aldurazyme which is made in Novato, CA at the BioMarin headquarters and then shipped to Boston-Genzyme). Afterwards is the race in which the team meets close to where Jenn's family lives which is awesome, divine intervention! :)  I plan to fly home Tues but am booking my flight once I get home this week. Below are pictures of the long sleeve t-shirt and stickers I created online (zazzle.com) for that day.

 

 

Long sleeve t-shirt designed (front) for the Boston Marathon

                                                                  Back of the t-shirt

 

                                                      Stickers designed - Both things made on Zazzle.com

I will update when I know something more, thanks for stopping by,
If you like please sign up as a follower,

Erica

Adjusting the Codman settings

The new Codman valve had been set pretty well in the middle (Highest setting = 200, lowest = 10 I believe)  for drainage during surgery Tues and tonight Dr.Bragg reset  it down by 2 increments to 70 (H20 I believe) which she explained what pressure setting that equates to but cant remember her explanation. She said at the 90 it was closer to when I had a medium pressure fixed pressure valve in the former LP shunt so I image this 70 must be low-medium pressure? Unsure really.. We will do an Xray tonight to confirm this valve setting and then reset the Strata valve to a higher setting in the near future, unsure if tomorrow or a few days or at first fup.

I am on the PCA (pain control) pump right now yet which let me say makes a huge difference for the head incision pain (bruising I guess) and headaches, hopefully this turning down the Codman will make a difference still more and when we do it turning up the Strata valve to drain less although  I know it would seem counter-intuitive will help that shunt drain more even if it where still intermittently as the thought is less continous drainage through the already very narrowed spine and already an area prone to catheter collapse will help the shunt over-all. I dont really know but it isnt hurting to try and although im fairly certain I completely butchered the proper explanation of why it should make a difference what matters is Dr.Bragg thinks it should help the TPL shunt drain more steadily and less intermittent occlusions and so if she thinks it will I believe her. :)

I gave Dr.Bragg one of the 'Hope its in our Genes' rare disease day pins (symbol is a pair of jeans twisted to look like the human gene and bi-colored. She really liked it and said she would pin it to her coat. I like to find little things like this for my providers since ones like her and my Pain Mngmt dr do so much for my care. :)

I'll update when there is something further to share or when I get discharged,
Thanks for continuing to stop by,

Erica

(Shunt) Revision #26, Codman valve

Surgery -

I cant remember which part of the surgery Dr,Bragg said she was doing but maybe taking out an old, partially occluded catheter when she said they had problems with bleeding again (much like in Dec) and so she wasnt surprised I would have brusing and there was blood on the stapled gauze they placed over the scalp incision when closing as she said it took a while to get this back under control much like back in Dec. The likely reason for this is the catheters and tubing get adhesed to the tissue so quickly perhaps as a 'side effect' (?) of the MPS storage causing an encassing of sorts?

She also said she hasnt seen the pressure this openly high other than with our lumbar external drain placement last spring when the CSF shot out and covered her from chest down to her shoes it was so elevated / high. I think sometimes she is reassured by these odd moments, not bc she doesnt beleive we have an pressure issue but because then if there is anyone second guessing her decisions sha has backup? I honestly dont kmow and doesnt really matter as she cares, goes out of her way to try and help and cont's to think of outside of the box ideas with in the box options. I always know when there is pressure so wasnt surprised  I guess when she said she got such a quick and heavy CSF spray/emptying but it does provide the reassurance that I sometimes have really lacked in my own judgement calls regarding these headaches and pressure, symptoms, etc. I am glad she shared the info about the pressure having been so increased. She did say she thinks the VP shunt was likely doing a slow or intermittent 'drip, drip' versus what would be better with the shunt if it where draining properly continually as is needed. She also said that the valve was very clearly partially or near fully occluded with (brain?) material so she was surprised it was dripping at all as she had been worried it wouldnt be and so the need to not completely replace all of the shunt component in the head.  I told ber I had also thought the shunt was working just not particulatly well. Yay, to our being right and to new things to try + Dr.Bragg's incredible gift of perseverence and just plain believing in me! I am thankful for her and for a faith that carries me through even when I am struggling - a God that is truly an AWESOME God!

She took out both the mini-nav (something like a valve but was not an actual valve) and the Miethke shunt assistant which also was not a valve and acted as a one-way device to keep CSF from coming back up the shunt. In place of both of these devices she placed a Cofman Hakim valve which gives us 20- settings to chose from for drainage starting at 20 and going as high as 200H20. She did not place the siphon guard with this valve (which I am also happy about as she said she wanted to stay with as simple as possible while also giving us a good chance for success with improving the headaches and other symptoms. A magnetic wand is also used to adjust the settings on this new Codman valve although w this it also requires an Xray to verify correct placement and w the PS Medical Strata valve only the magnetic wand is requied for pressure adjustments.

Anesthesia was with another Anesthestiologist ive had a few times before + a CRNA and the Anesthesia Resident and according to Dr.Bragg went fantastically well - she is continually amazed how difficukt this part of the surgeries is. I am glad that went well and does also then make the whole case go alot faster.

Late last week I got a friend request but wasnt sure who the person was so I sent an in-box pm and then heard back the next day. Turns out this lady (unsure how old she is) is also a patient of Dr.Bragg's (Spina Bifida, Hydrocephalus I believe where her dx's) and though she couldnt remember how she found my blog she said she has been following/reading it; very cool! :) That is the 2nd friend request in as many weeks from a patient/family of the Peds Neurosurgery dept w the 1st time being her partners patient and the Mom is volunteer director at the WI Pediartic Hydrocephalus group and she had found me through a mutual page of a little boy we both follow here in WI, unrelated to Hydro.

I ended up getting about an hour of sleep last night despite trying to go to bed early as I had to get up at 3 to shower and finish packing, round up my cats, feed the outside cats, etc and then drive over to my parents which arent to far from where I live (one city over, 15 minute drive typically)  + an hour from their house to Madison to the hospital. In trying to fall asleep I must have gotten up 5 times in the first hour alone and wasnt sure I could take the OTC bladder med I take at night alongside the Toviaz (for the nerve-spine bladder signal issues) which didnt help. I am actually almost impatiently waiting for the appt w Dr.Maskel in April (new Urologist) as I am reeealllyyy hoping she can help figure this yuckiness out!?..)

All for now, will update if anything new,

Erica

Surgery info

Surgery is at 7:30 with arrival at 5:30 - that's all there is to this post. :) As far as I know the plan is still to take out the 2 current 'one-way' (non)-valves and replace with a programmable Codman valve in the VP shunt.

Weather wasnt to bad neither to or on the way home from Milw (ERT infusion) despite the snow thankfully,
Will update tomorrow night or Weds,

Erica

Appts, Imaging, Shunt, Compensated Hydrocephalus

Window Cling - $10 (made by someone my Dad works with)

                                                   

It has been a busy week filled mostly by appts for various things but today's craziness included an "oops" MRI which was really meant to be a CT except messages got mixed up between my neurosurgeons staff. No fan of MRIs here  and was even longer than c-spine MRIs normally are as they added a few extra images since they already where doing it to look at the shunt catheter tip and something else. So the CT scan which is what was supposed to be done (for Tues surgery so she can use the stealth (endoscope) machine to guide the catheter in at a better angle)  was then scheduled for later this afternoon after my Neuro-Opthalm appt but then that to ran over so now the scan is set for Fri. This of course means another trip back to Madison, no fun! (but will do something if feeling 1/2 way decent to make it an ok trip) I saw Dr.Bragg's NP, Sue after the MRI and she re-programmed the VP shunt back to it's original setting with a bit of   difficulty but non-the-less we got it so TPL valve is back at .5, the lowest setting.

The Neuro-Opthalmology (Eye) Appt went well; the visual field studies where repeated which I must say are incredibly yuck (bc of the way your sitting and your neck is at an odd angle, a hard feat with the hardware in my upper neck)  but went fine and no progression and possible improvement so i'll take that. I dont have to go back to her for a year if no changes w any of the shunt surgeries or issues! I see my regular eye doc the beginning of April to get stronger lenses for my glasses as the shunts have done a wonder on that but I knew this already and had already scheduled this appt.

I dont talk about this alot since I do Physical Therapy almost weekly but I had PT yesterday and he was impressed with how much we've gained in just the last 2 weeks some of which he thinks has to do w the shunts regardless of their not consistently draining enough now as I wasnt retaining as much fluid all over. He also commented how he thought I was standing taller and less of the forward-caveman posture which is something I am always struggling to improve!!

Running for Rare diseases blog post from my running partner (she is running on my behalf to raise awareness of MPS):
http://runningforrarediseases.org/2013/03/13/ericas-story-living-with-mps-i/

And last but not least I meant to write about this last week as I think it has implications for MPS pts but when Dr.Bagg was talking about the possible future skull expansion (done with plastic surgery) at last Thurs's appt she commented how abnormally thickened my skull is and she feels this is atleast partially due to compensated but un-treated Hydrocephalus as a kid. This headaches, nausea and vomiting, etc that I experienced often as a kid and I think starting around 1st grade when I was sick the most often but which imaging showed no problems when that was done at one point (first grade I believe, wayyyy before the MPS dx) where likely secondary to the compensated, un-treated Hydrocephalus. She said this leads to skull expansion or thickening of the skull bone which is if I understood her correctly (not sure I am remembering it all right) then affects how well shunts work and the ability of ventricles to expand properly. Very interesting and all the more reason why kids AND adults NEED to be diagnosied EARLIER w MPS I!  And  more doctors should really study the brains of MPS I pts, the headache issues and how common is compensated increased intracranial pressure in our population and kids?

 I recently installed a app which tells me where people are from and I dont know if someone will really comment but I get a visitor from Platteville 1/2 a dozen times it says since instaling this, I am curious who is this?? I wonder if whoever this is, is actually from another nearby city as I sign on as says I am from BD but I live about 10miles away, in another city.  And for others feel free to sign up as a 'follower' help me raise my #'s! :)

Appt with Neurosurgeon, VP shunt (valve) revision

Add-in note as I was typing this Dr.Bragg's Secretary (Anna) called and the CT scan is scheduled for March 13th while I am already in their area for a Neuro-Opthalmology appt and then surgery itself is scheduled for March 19th, a Tues.

Today's appt went better in a way that I could have hoped; I thought at the very least I might have to convince Dr.Bragg why a codman valve w it's many more options for settings (they have 3 valves w 8, 18 and 20 settings a piece) might help us. Instead she fully seemed to agree saying she had been thinking of me alot over the past few days and how while it's not a bad thing I am in her thoughts often it is bc she tries to figure out a better plan. We both agree this Codman in place of the "non-valve" 'miethke shunt assistant' and 'mini-nav' "non-valve" wont be a full symptom fix nor a likely long term fix but we think having more options to turn the 2 shunts to a low-medium drainage setting can atleast help me feel somewhat better than I do now by allowing more CSF to drain while keeping the ventricles open better. This should also hopefully help us be able to stop the IV zofran (and thus not have my Port with the subsequent infection risk accessed all the time) and buy us time till either we explore the cranial expansion which would entail shaving some of the skull bone to create room or till we do the cardiac valve replacements and then look further at the cranial expansion. The 2 current "non-valves" sit at the proximal or top portion of the VP shunt and the other sits at my collar-bone level along the shunt line)  plus we will remove those and replace w the Codman programmable although I am unsure which valve specifically (certas, medos or hakim). She did say the downside of the Codman valves is they have to be reset by a programmer (like my TPL strata valve) then an Xray is done + then she has to re-check the setting. Instead of setting it, doing an xray and then my coming back to see her she would she said just go with me to Radiology and set the valve to its correct or new setting (whichever case ie after an MRI or if we where adjusting the drainage amount) right there. 

Her nurse or secretary will call me to schedule this surgery and she wanted  to try to do this in the next couple weeks; she is out of town the first week of April (which I think follows Easter) so I assume was wanting to try to do this before then. I am ok with that and ideally would be good if we could do it a week from Tues. Not that I even want to wait that long but bc then id not have to reschedule other appts and things I have next week. I do have the Hand surgery on the 20th of March but told Dr.Bragg I will push that back and when I jokingly said to my PCP yesterday after the Hand pre-op physical "lets see if 3rd times the charm" for this Hand surgery, she laughed and said well the shunt stuff takes precedent.  I'll get that hand surgery done at some point! Even Dr.Bragg joked and said something like your Hand surgeon is going to hate me for your having to reschedule his surgery so often! :) Still I am incredibly grateful Dr.Bragg thinks about my case so much and was also thinking something like a programmable valve for the VP shunt again to atleast help improve my symptoms for now! How many drs would be willing to do surgery knowing it likely isnt going to be a full-on fix? Not that the symptoms of the Hydro arent serious as they are but still we all know there are some drs who just wouldnt consider doing this.

She is doing a CT scan before the revision and using this to guide the VP proximal (tip) of the catheter to a better trajectory and location as she said she doesnt feel it is in an optimal location now. The CT acts as sort of a road map (or GPS is how ive described it before) and this + doing the surgery via scope gives a finer view point or potentially more accurate placement.

Will try to update soon,

Erica

Dr.Bragg responds; her partners thoughts,. 'Happy turning 30'...

Turning 30, a big deal in the MPS world...

Well the wait is over, I heard from Dr.Bragg this morning she sent an email with a update on what she learned from talking to her colleague (a Neurologist) who is the one that helps w their complex shunt pts. His recommendations where two-fold with his one thought she said being  our on-going repeated shunt issues and trying to take a different approach (see below for part of her email though not the complete note) + how he feels the cardiac issues may be affecting our shunt issues.

"I spoke with Dr. H  His recommendation is to consider a cranial vault expansion. At this time, there is no way to improve the compliance of the brain with the current valves we have in place. He also thought that perhaps your cardiac problems might be contributing, i.e. altering the CSF pulsations because of the altered pulse waves from the valve problems. He suggested that you have your cardiac surgery and then consider a vault expansion."

 

She asked that I schedule an appt to come in and talk further about all of this which I have since scheduled for this thurs. I wasnt to sure this other dr would have any addt'l thoughts and wasnt sure what to expect so I guess its good they have a thought but this, really this??? It is a thought Dr.Bragg has brought up before but hesitantly and never seeming to consider it to much I think maybe bc it is more invasive? I think she also (or her NP) this last in-patient stay commented they didnt know gow the storage would affect this type of surgery or would it work. From what ive read there are different approaches and it is typically used to treat an issue called craniosynostosis which I think means the cranial sutures close prematurely or not enough space in the skull and seems to (if I read it right) happen in pts with slit ventricle syndrome or where the ventricles collapese bc of not enough space. Interestingly one of the disorders listed under this treatment info was MPS I, I guess bc of the hydrocephalus that occurs in us pts and our thickened skulls w ventricles that are unable to comply with pressure and fluid changes? Again I just say yikes!    

I also cant imagine we dont even know where i'll have valve replacement surgery done yet and I dont see Cardiology till June  or July unless I move that appt up. I cc'd Dr.Earing my Cardiologist on Dr.Bragg's email so I am kind of hoping they will talk. =/?   A friend of mine who is also an (older) adult w MPS I brought up the point should I be strongly considering having valve replacement surgery where Dr.Bragg is? Will anything have to be done w the shunts during this valve surgery? I dont know but I do know the daily coumadin will make surgeries more difficult as this acts as a blood thinner and will have to be stopped before planned surgeries for a period of time (unsure how long) which then raises the risk of clots forming in the mechanical valve. =/  I do know or believe UW is doing bovine valve replacemens via catheter in atleast aortic stenosis in pts with severe underlying medical issues that would otherwise preclude the pt from getting their valves replaced. Bovine valves are rarely used anymore in MPS I pts bc the storage from MPS re-accumulates on the "new" valve causing the same stenosis and leaking within a few years of the surgery thus requiring further valve replacement surgery a few years down the road and has occured to a few of my older MPS I friends. This said would trans-catheter valve replacement and thus avoiding open heart surgery and all it's inherant risks in my case (bc of the autonomic issues, endocrine-steroid use, restrictive lung issues, the shunts, etc) be worth looking in to?  I dont know but going to ask Dr.Earing and will talk to Dr.Bragg about what if anything has to be done w the shunts during cardiac surgery, it may be that nothing is done to them.

 

I admittedly cant imagine going until we do valve replacement surgery which at the very earliest even if we say started the process now (which we are not as Dr.Earing wanted to give 3-6 mo to heal/be infection free from Dec's central nervous system (CNS) infection)  it would be I imagine another 3-4 months to get everything done before a surgery. As it is now I cant imagine we will be doing any valve replacement surgery before fall and so to live with these headaches, nausea, intermittent vomiting, autonomic symptoms and the other related symptoms  you might as well say 8 or 9 months bc of healing time from any heart valve replacement surgery seems like a long time. Even I who tries hard to live by 'one day at a time' with the medical stuff (and fails miserably I might add often at it) cant stretch my mind to imagine such a long un-ebbing period of these symptoms. =/
Please dear Lord if it is thy will allow Dr.Bragg to come up with some plan in which we might try to treat these headaches either differently or sooner than before any heart surgery. =/ I can really understand her being uncertain about how these valve issues affect the use of anesthesia and her surgeries and so  completely cant fault her on that but I still do pray we can come up with something and much sooner. This is hard, so really hard. The worst part I think is just that there are many things i'd like to be doing or more things id like to be gettng done most days and I just dont all of the time. =/

 

On a different note I am 30 today, a big deal in the MPS world. While I wasnt diagnosed as a kid so I didnt know I had something hanging over my head as far as age I do see how few of us adults there are w MPS I who are older than 30 (I think I can count on one hand or maybe some of both hands here in the US for MPS I Adults) . Ours just isnt a well studied age or issues and so I am I almost feel like entering un-charted territory, certainly atleast for most of my drs I am. Id like to think I cant top what I all had in my 20s (Alot, yikes) for medical issues (or surgeries) but I think I likely will.
  I pray for God o help guide my drs in making the best choices as we cont to try to figure some pretty complex issues out.  In some ways I am happy to be done w all that was my 20's, like entering a new phase almost. :)

 

Stay tuned for any news. Tomorrow I see my PCP for a pre-op physical for the oft rescheduled hand surgery (3rd release, and Hand surgeon doing several other things during to same hand) although I dont know if the surgery will be approved (approved by my PCP that is) bc of the cardiac issues, we planned to talk that over and figure it out at the appt. I had also asked her if she might be able to help me figure out the bladder issues (mostly an issue at night, some spasms during the day)  so PCP was going to write that down for the appt to.

 

Please say a prayer that Dr.Bragg doesnt grow frustrated and give up, that she is able to formulate some kind of idea we can try and we can come up with a plan soon.

 

Erica