"I don't..."

"I don't agree with the plan and theory". Those are the words included in a message from my Pain Mngmt dr this morning. She is good so I know she wouldn't say it lightly. She knows me well and knew me before I began seeing Dr.Bragg (just over 3 yrs now) so she (Dr.B) has seen the pre-2 shunts ups and downs. Prior to Dr.Bragg Dr.B tried many, many different medications (medication classes) over a long-ish period of time to try and improve the headaches. She was always a firm believer the symptoms I was having where headaches/symptoms from the then LP Shunt and tried many, many times to get others to help. Some of my Specialists at the time tried also to help her but others where of little help.
Thankfully through a friend (someone I worked w a bit at APF who formerly worked at UW) I was able to find a Neurosurgeon willing to see me and in turn through that dr. found Dr.Bragg. I am forever grateful to her (Dr.B., and Dr.Bragg) for having believed me when I am sure there where many reasons why she did not have to see me (including her partner who had not believed me nor believed drs. of mine who where communicating with him).
Although I don't think she has given up (I hope not, it scares me actually) I do see how different it is then in the beginning, everything takes so much longer. In the past if I'd have called with a question (regarding changes in symptoms) they would have called back but now I just feel like a pain in the arsh pt. (whether true or not on my part I don't know). I am sure part of it is that she is just much busier given likely she has more things to do having been in practice longer. That said as any patient knows it doesn't make feeling worse and feeling like everyday is a big question mark any easier.
For as long as I've been dealing with a lot of Specialists I've still just never gotten used to having to rely on someone else to feel better or having to ask for help! I admire pts. (or families) who have no qualms about calling their Providers or pushing for answers (I think I am pushy but I am not sure if it's a good thing).
Honestly to I think I could do better to have better patience, which has never been a particular strength of mine when dealing w medical stuff, waiting for answers (solutions).
I wrote on fb earlier "I can honestly understand why pts just go to the ER, atleast your likelier to get answers, though I don't think it's the right thing for me to do right now". After not hearing back from Dr.Bragg's office today (called her Secretary regarding my PM drs. thoughts and regarding vision changes w the headaches) I really can understand why pts just get to a point where they feel bad enough or feel bad even if intermittently and you feel like it's just not going anywhere fast that going to the ER might get a solution or at least feeling a little better, faster. I'm not going but boy do I wish I felt better. =/

Honestly b/c I don't always come up w questions to things we talk about right away after appts. I miss being able to email and she would respond. I miss us coming up with solutions faster, even if it didn't always work we atleast got relief intermittently and sometimes just having a small bit of hope, that relief might happen or even when we'd get temporary relief it was enough to re-charge me to keep on dealing w this, to keep on pushing.
I realize it may sound like I am giving up, or depressed and I am not but I am discouraged and that is hard. I just want to be able to have hope. I just want to be able to do things without thinking about (praying) i'll feel ok or at least ok enough and almost most of all I just want to be able to read without feeling "the dizzies" (as a friend whose daughter who also experiences the same vision issues when having shunt issues calls it). I also want to not feel like an annoying, high maintenance pt!
As I am sure is more than obvious from this I am feeling more than a little discouraged by these symptoms, sure they may be intermittent but it frustrates me that reading, most of the time is so difficult (a problem when headaches are a problem but these have been occurring more often). And as I wrote above that everyday, especially mornings and for that matter nights are so crappy. I get up hours before I have to do anything so at least the worst H/A's are somewhat improved (though they repeatedly recur, just to a milder degree) and honestly I might sell my R foot to sleep through the night once!?!

Most of all I just wish I felt like I had hope, that I would never have to ask Dr.Bragg for help again with headaches/shunt issues and that it was easier to communicate with Providers (as well to getting them to communicate).
I think I feel most discouraged b/c while Dr.Bragg always said something to the effect of 'we have options, I'm not going to give up' I admittedly still feel like we don't have as many options and it is sometimes really hard spending so much time, feeling so bad. I know (as I've written before) I look fine when people see me so that to while a good thing in a way doesn't exactly help getting answers.
I guess i'll just keep praying we can come up with something sooner. I honestly am not sure I can deal w these symptoms long enough to find out if we could get IT approved in an IRB (I kind of have my doubts but trying to be a little hopeful).
I can wish, right?
Anyways, sorry if this is awfully discouraging. I guess really I don't have to apologize since it is my own blog but it would be nicer (and is) when I am posting about something positive or even better when I am posting that something we tried helped!!!!

In any case, thanks for stopping by,
Erica
P.S: I've come to the conclusion maybe I need to go to Church everyday (we have service 1x weekly or the same service on Th night and on Sun). I feel restored in a way after Church but then my doubts resurface even though I know I should put my faith, hurts and pains to God.

Bay Beach (Amusement Park) - mostly

Let me say, I like the outdoors but I am not an outdoors person in particular! That said other than the mud and wet our little overnight camping adventure (after the Amusement Park) Fri and today was fun. My parents have a tow behind-full size (all amenities I guess you'd say) camper so my Sister and I had drove up separately yesterday, met my parents at Bay Beach and afterwards followed them to the Campground.
My Sister, the kids and I did tents - although my Mom had gotten a fold out/reclining chair so I was still able to sleep upright and off the ground. Honestly other than bathrooms I like the campfires, playing at the park w the kids and we had a good time exploring the (small) downtown last night in Algoma, which is on the tip of Door County and right on Lake Michigan.
I definitely was happy to be home this afternoon though, it wasn't a ridiculously long drive (about 2.5 hrs) but I was tired, couldn't wait to shower in my own comforts and I like being un-packed! =)
I have to say I had been praying all week to feel atleast reasonably ok while at the Amusement Park Fri and overnight which despite waking up no-less than 4-5-6 times Thurs night my low back and leg didn't hurt to bad and I couldn't complain to bad how I felt at the Bay Beach! I honestly think sometimes God just knows when we need a break and will always believe he listens even if we don't always get our answer!

As far as Bay Beach (the Amusement Park) we got there by 9:30, the rides open at 10 and Zan, Quinn and I where in the first bunch to get on the Ferris Wheel which otherwise the line gets ridiculously long. I'm not sure there is any ride you have to wait more than 20mins and typically more like 5-15min wait but the Ferris Wheel gets sooooo backed up!

From there Zan and I did the kiddie helicopters, I went on the tilt-a-whirl, (no affect whatsoever on the lightheaded/weird feeling I've been having intermittently!) - My Dad, Quinn and I did this one. Another of my favorites and ironically a ride I detested as a kid!

After that we did a wide variety from the Carousel, 100ft slide (you ride down on potato sacks though the slide could seriously use some new grease!), Giant swings, the kids did some kids rides. My Dad, Mom and Quinn did the scrambler (that line also gets pretty long or I'd do it!) and we did some of the other rides + some rides multiple times.
In the past few years Bay Beach purchased a old wooden roller coaster that used to be owned by Elvis Presley at his park - (the Zippen Pippen) so I decided to try that this year. YIKES! Probably NEVER again! Wowza on your body is all I can say, perhaps worse to me b/c I don't have full range of motion in my neck!? I've already had somewhat of a stiff neck for a week or so so today my neck and body feel like they've added about 40yrs on and feel like creeky stairs whenever I move! It was Fun (for the most part!) but wouldn't do it again!
The Park also purchased a new wooden rocking boat (like at carnivals where it tips way up in the air one way then comes rocking back and goes up in the air the opposite way) though surprisingly none in our bunch did this.
Really the best part about Bay Beach is even when it's busy the lines aren't anywhere near Six Flags or Disney wait times and the tickets which you need at most 3 but typically 1-2 per ride, per person are 25c a piece.
Consider at a fair the tickets are typically $3/piece that's crazy! This park is owned by the City of Green Bay but has a trust set up by some Lady when she passed away so her on-going endowment runs most of the costs, pays for upkeep/new rides apparently and she wrote in to her will that park costs could never increase beyond a certain point. When we where kids and went there every year, roughly 20 yrs ago the tickets where 10c a piece so the cost haven't increased much!
Typically we'll have a stack of tickets left at the end of the day (when we are done anyways) so we just give them to someone at the park or as they are coming in to the ride area. It's neat!
I am glad overall it was a good day and my stiff/sore/tired body will recover. I know I could not do all that craziness and thus in turn not pay for it the days after but what is life if we don't do the things we enjoy? Honestly I need days like yesterday to re-charge as I had been feeling a little discouraged, I've been feeling not that great so to spend a day almost (almost!) forgetting it all is just what I needed! =)

On an un-related note I was reading some med records from PMR and Neurosurgery and just wanted to share this thought Dr.Bragg had in one of her recent clinic post-visit notes.
..."The other potential would be cranial vault expansion, as Erica has significant thickness to her skull." (no smart arsh comments anyone) =) "She said she discussed this with other physicians who have significant experience with Mucopolysaccharidoses who felt that the reason that she has poor CSF flow is related to her underlying Mucopolysaccharidoses.
"The 1 thing is that Erica does feel better when she has had externalized shunts, so I do feel there is a compliance issue related to her CSF and her inability to be headache-free when her shunt(s) is internalized." -
Prior to this comment she talks about the concern with potentially adding an anti-siphon device both in would it slow CSF down to much (she comments regarding this especially b/c I sleep upright and have almost without fail since beginning to see her minus the short varied periods we've had H/A's under good control) or on the other hand she wonders are the H/A's due to over drainage and partial intermittent occlusion of the shunt catheter. <-- In the case of if it where intermittent over drainage a anti-siphon device may be helpful. Last wk when she had talked to her Codman Rep that person also was not clear on what we should do in this regards.
I just share these sentiments b/c while Dr.Bragg and I of course talked about these options (she also mentions getting more info about the potential for IT) I know I don't always likely remember everything we talk about fully or atleast completely correctly and is interesting to read her thoughts in print.
I often think it would be helpful to record appts with some Specialists but I don't want to put them on edge and would never do it without disclosing I was recording!
I'll update if I hear any news from either Dr.Bragg or anything going on in general. My Pain Mngmt dr was texting w me a little yesterday and mentioned she would probably try calling Dr.Bragg to get more info on what her thoughts are regarding the low back issues.
In any case that is about all,
Thanks for stopping by,
Erica

Neurosurgery Appt., alot going on, few immediate solutions

Earlier today I saw Dr.Bragg which I guess went ok. I say this because we have ideas but no for sure plan which is hard when you feel bad so much of the time; not having a concrete plan of action and timeline for things to occur in place is difficult to wrap your head around. It is much easier to be positive (hopeful) for better days when you know there is a sure plan and timeline.
There are so many issues going on so it's often what do we treat 1st? I said to Dr.Bragg today or really I apologized that so much of the time as in this case many of my Providers will defer back to her and her expertise how to deal with an issue. She commented something along the lines "No worries it's what I do or know" (not those exact words) and that she was happy to manage the various issues going on and not just help with the headaches.

I am grateful she does offer to manage the overall neurologic picture/imaging and seems ok when others of my drs (in this case PMR, PT, Pain Mngmt) defer to what she thinks of an issue/issues.
Right now b/c I swear it's never just one issue symptoms overall include the low back/L leg issues (leg spasms/intermittent discomfort), Intermittent episodes lightheaded, intermittent H/A's, vision which goes along w headaches. Nausea periodically and waking up frequently at night from low back/leg.
I don't know if I am wrong or if Dr.Bragg is just trying to figure out a better solution given we've had so many shunt issues but I admittedly feel discouraged and a little frustrated (not at her really) about spending so much of most days feeling crappy and yet there is no easy (easier?) answer what to do.

I think she is pretty good about trying her best but I feel like even she might be unsure what is the best solution though I could be wrong about this. Maybe I am just so worn out from it all that I feel discouraged and thus think she feels so - hopefully she isn't and instead is just trying to find better solutions.
I completely forgot to ask if she'd talked to her colleague about the bone thinning, which is not something I am eager to jump in to anyways but I am a little curious what that dr thinks ie would she think it would help and would it be as big a procedure/surgery as I feel like it might be? At this pt we've kind of put that as our last ditch option I think but i'll still try to find out if Dr.Bragg talked to this other dr at all.

As far as the Intrathecal Aldurazyme which was something i'd asked her about at the last appt (if it might be an option) about a month ago it sounded like she had gotten a little info about how setting ↑ IRB's works at UW.
She hadn't yet talked to Dr.Dickson (kind of a bummer but given I hadn't heard anything I kind of wondered and I understand she is busy) but I think from what Dr.Bragg said today she was going to talk to their Research Coord. regarding this person reaching out to Dr.Dickson (potentially try IT-Aldurazyme in to shunt/brain) for info. The Research Coord. would then in-turn be the one to try and get IRB approval.
As far as H/A's her Codman Rep also was on the fence if we should try adding a anti-siphon valve to the VP Shunt. This valve would prevent over draining but in-turn would mean less CSF pressure released which we know isn't ideal. We've kind of left that as a non-decision at this pt.
Regarding low back/leg on-going issues I am a little confused on this (but honestly one of those things I need to mull over before having questions) as previously she'd mentioned she thought it might be due to the TPL Shunt catheter.
Today her thoughts seemed more towards lumbar/Sacral disc bulging/disc changes.
Before we did anything drastic there she'd like to see if injections might pin pt if the lumbar/sacral area is in fact the cause. If the injections helped I guess then she'd consider doing something more in-depth like decompressing the area? This again one of those things that is a little confusing and a lot to process.
I'll talk to my Pain Mngnt dr about this as she is likely who would do it if she agrees (Dr.B may decide she'd want to talk to Dr.Bragg). Coumadin weights in to that ie if we did injection(s), but could be managed fairly easily and is out-pt.
I personally am not a big believer in efficacy of facet injections but maybe and if I understood it all correctly Dr.Bragg thought this on-going symptoms/changes in symptoms could be due to the previous LP Shunt and the many previous Lumbar surgeries. (We've had issues in the past w nerve roots from several levels becoming scarred together and have done decompressions).
Today's appt was definitely a lot in a semi-short period (though not rushed, Dr.Bragg is good about explaining her thoughts). Really it's more that we have no clear road (plan) which is what makes it difficult.
Atleast when you know there is potential relief in sight you have hope.
Honestly I am just praying for something soon, I don't (as I'm sure I've said a million times before) want to keep asking Dr.Bragg for help but I would also give so much to feel better.

I see Optometry next week to see if vision (glasses) prescription has changed, it seems like it has and seems like this secondary to the intermittent headaches has gotten worse. I really, really like to read and thankfully there are large print books but even many times I end up just putting whatever I am reading down as the print just begins to swim-not in an I am just tired way but just b/c vision gets so fuzzy.
I am going away tmrw, staying overnight which should atleast be fun. Ever since i've been a kid we've been going to Bay Beach which is a small Amusement Park near Green Bay. These days we take some of the nieces and nephews - it is beautiful, on the shore of Lake Michigan! Kind of like being a kid in a grown ups body for a day! =)
This year we are staying near Bay Beach over night. I so, sooo badly just want to feel good all day and night and just want my body to not hurt, to just be able to stand in lines and sleep normally!?!
Will post pics sometime soon.

If you believe in prayer, please pray Dr.Bragg and I are able to come up w a good solution soon to one or more of these issues. I feel a little CrAzY given it's always something! =/

Erica
Written earlier in the wk
I've been thinking alot lately but especially this past week about my care, on-going issues and honestly feeling some stress about people (who I really don't know) in the MPS 'community' that feel it necessary to spread untrue crap about me. Why I don't know but I have a hard time not letting it get to me. I've definitely been spending less time on fb and not sharing much on there.
I've always used fb mainly as a source to share my own experiences w MPS and to learn from others but lately I often wonder about any of this and can't help but feel fb just causes a lot of unnecessary drama and honestly pain.
These last few days have not been the best (the usual nights and mornings to mid-afternoon) being the worse and then starting to feel somewhat better by late afternoon and only to start again by night. I've not felt really great and not entirely sure what it is from. I've been having episodes of what I guess is lightheadedness (maybe related to reading), having the headaches worse, feel really tired the past couple days (Monday and Tues being the worst despite sleeping near 12 hrs Mon. night) and probably doesn't help (maybe is influenced by?) that INR has been high/low/high though I only seem to feel really tired when it is high.
I don't think the headaches are related to INR and instead in all honesty think it is something shunt related as it improves a little the longer I am up though never going away yesterday or Tues. Today, Weds has atleast been a little better.
It seemed as if symptoms progressively got worse Monday, (the headaches, nausea) which Monday night I finally just took a 1/4 dose of the valium after trying Zofran with no luck and went to bed. The lightheaded feeling comes and goes and seems only positional in all actuality very similar to past specific shunt issues.
Either way even though I pray Dr.Bragg can help sooner I will always be grateful to her for the help and time she has given over the past years. I've definitely learned those Providers who want to or try to help and have learned who will and won't help in your care and who will and who won't necessarily listen.
It seems hard to believe but Tues night we had our first Sunday School meeting of the year - this starts Sept 7th. I'll con't to teach grades 3 and 4.

Exciting news for PTC's 'Ataluran', PMR/other news

Additional indication: Based on an evaluation process and in discussion with outside experts, PTC has selected mucopolysaccharidosis type I (MPS I) as the next indication to pursue for Translarna. It is PTC's goal to initiate a Phase 2 proof-of-concept study for MPS I in the second half of 2014.
It is estimated that 60-80% of MPS I patients have their disease as a result of a nonsense mutation.
There is no cure for MPS I and enzyme replacement therapies do not sufficiently address the central nervous system, skeletal or cardiac symptoms associated with the disorder. Prognosis of patients with MPS I is poor and there is an urgent need for the development of new treatments targeting the underlying cause of MPS

At PMR today I saw my docs partner (she is on maternity leave) and asked him as we where talking pre-botox if he thought botox might help the low spine/hamstring nerve like symptoms.
He after doing a quick assessment did not feel it would and also, like my PT has concerns it is caused by a spine nerve being irritated. See below about last weeks PT appt. but I guess this means I should call Dr.Bragg.
I see my Pain Mngmt dr on Thurs and perhaps going to 1st see what she thinks, she has been on my 'Team' since pre-any of the spine surgeries so she may have a good vantage pt. I suspect I know what she'll also say but still I am in no hurry to ask Dr.Bragg for help on still another (potential) TPL Shunt issue!
Earlier last week at Physical Therapy, (which I've been doing on and off for years but in this case since March or April) my PT had some concerns with the sciatic nerve (we think) related symptoms occurring down the back of my L buttocks, L back of leg (hamstring/related muscle groups).
Because the symptoms have not improved at all and seem only to be getting worse-occurring more frequently he was concerned it may be a spine nerve issue, given my history. Beucase the symptoms have begun occurring at night I wake up often having to move/get up/stretch. His concern is whenever I've had other issues w muscles/nerve we've atleast been able to decrease symptoms and everything will at least improve. This time, going on for the past couple months and symptoms only worsening, with no response from our efforts in PT (again soooo unusual) it is often miserable when symptoms occur.
We know it is full spine MRI's looked ok minus more typical MPS spine issues (narrowing for instance) that it isn't actual compression or something like a herniated disc but Dr.Bragg when reviewing the imaged had mentioned the TPL Shunt catheter, sitting in the spinal canal around T-10 (I believe) was closer to one side of the canal than they prefer and she thought it was possible this could be the cause of the symptoms.
Tom, mentioned at the end of that PT Appt how at the end of each session he is able to make a small amount of improvement in this particular muscle group but then each wk I'd come back he could clearly tell the muscles where back to if not worse then the wk before, as if he'd never done anything.
Per his experience this is not normal and he asked if I'd had MRIs recently (I have). Being Tom is incredibly conservative and not a big believer in a lot of the interventions/tests that are done for some pts (he seems to get the need for more in MPS) I was a little surprised by his concern.
He asked that I talk to Dr.Bragg to make sure she is aware the symptoms are only worsening and to make sure it is not the TPL Shunt causing these nerve irritation symptoms.

I've not heard what's going on regarding if she was able to talk to Dr.Dickson about potentially setting up to do IT (if it is possible it will take time) or if she was able to talk to the Plastic Surgeon about the other bone thinning she was interested in pursuing (I am more than hesitant about that one but willing to get information on it).
I guess really I need to call her Nurse or Secretary about all of these things or probably just make an appt.

Otherwise things have been really buys but not altogether in a bad way. INR for the mechanical heart valve has swung high and low repeatedly so we con't to adjust that - last wk Monday it was 5.0. Thurs after a slight dose adjustment it was 2.89 and I re-test again either Thurs or Mon (my choice, they wanted Thurs but said I could do Monday since I am at that hospital every Mon.).

Last week, early in the week I had lunch w the WI drug rep from Genzyme (which was nice) and she + the whole team (Carol, Karen, Amy) where in-turn in WI Weds-Sat.
They where hosting a Fabry mtng in Milw. Fri night and invited me to attend "since we don't have MPS I mtngs".
I knew many of the families at the dinner though not their significant others and as well was a really lovely surprise when one of my former and 1st drs. from UofMn was the invited speaker! Our Genzyme Team must have told her I was also attending b/c I am not sure who was happier when I walked in - it was soooo freakin lovely to see her (she is in private practice in Mn now but had left for some yrs to practice at Denver Children's)!
We honest to goodness spent most of the night catching up, talking and just talking about all of the changed in my 'Team', my care and overall the MPS community. She is so fantastically smart but exceptionally down to earth! We've exchanged contact info and more than likely now that she is back in the Midwest we'll run in to each other again from time-to-time.
On a kind of cool note each of the 3 Genzyme reps had asked for one of the Aldurazyme 'flip off' bracelets which I sent to them and where wearing them + KB also liked + asked for one! Very cool the little ways we can raise awareness as many people ask about my bracelet!

On a completely un-related note our family (my parents, myself, my sister and her family) all go to an amusement park near Green Bay each year along w my brother's son and are doing this next week.
Bay Beach is like a mix of a giant (but uber cheap) carnival, near scenic Lake Michigan view, giant (REALLY GIANT) slide you ride down on potato sacks. There is a train ride that goes around the park, an old wooden roller coaster from Elvis Presley/down south, a pool and this year a new ride was being added similar to some that are at carnivals on top of many, many, MANY other awesome rides big and small!
It's such a perfect place for any age! I'll be sure and post pics in one of the future blogs. =)
Thanks for stopping by,
Erica