Neurosurgery Appt

Today's Neurosurgery Appt went ok, I have to say as much as I like and do trust Dr.Bragg I just did not want to be there asking for her help again. =/ We where discussing possibly taking out the current TPL Shunt (Codman) valve and putting in a smaller-more in-line valve (same Codman brand though) so it would be less 'feelable' with movement or sitting back as I don't have a lot of extra fat to cover the valve that is there now and sits mid-spine so not much cushion available. Dr.Bragg is talking to her Codman rep next week but is going to schedule surgery for this which it will be more minor compared to many of the shunt surgeries but still a general anesthetic which means Anesthesia and accessing airway. I asked if we could wait till the end of March and Dr.Bragg asked me to get Dr.Earing's blessing on this given the mitral valve changes (I don't think he will have a problem, if anything he may suggest they monitor more closely but that although more time consuming would just mean the A-line placement for monitoring heart more closely). I am not sure but think this all may depend on how well we manage to get heart rate down/closer to his intended target of 60bpm range.
Dr.Bragg must have saw these cardiology appt updates as she brought it up and asked me to mention the surgery plan to Dr.Earing (I fup w them the 2nd week of March and otherwise could have just let them know when we spoke regarding Heart Rate and INR Monday) Either way we'll have to stop the Coumadin for this surgery again.
She asked me if I thought where the cardiac changes due to stress which although it probably does seem like there is a lot of stress in my life I am as I've likely talked about before the type of person that I try to not stress over what I can't control to much (other than the car stuff, I have to admit that all got to me quite a bit) or I try to handle situations as best I can to control stress. In any case I just said to her that Dr.E seem to believe it was genuinely concerning changes to the mitral valve but that he also felt if we could better control heart rate we might be able to improve or prevent from worsening the pressure gradients through this valve and buy us time.
We did turn the TPL Shunt down to it's final setting (30) which means both shunts are now set as low as they can go which isn't a great thing as far as having no wiggle room but good that we had this option for now... Because her office got a different programmer they are back to having to do X-rays anytime we re-program the valve, with their old one Dr.Bragg or her NP could just re-program the valve(s) at multiple positions and we where able to avoid the need to X-ray and check setting position. Due to this re-programmer working a bit differently and being a bit more difficult (new isn't always better we've all decided!) last time and this time we had to do repeat X-rays, but we got it! - so if you see a glow it's probably just me! :)

One thing Dr.Bragg brought up was she did a talk a few weeks ago on Intra-ventricular use of baclofen and she commented how this made her think of my case and come back to thinking about/wishing there was a way to access the ventricular space with either Aldurazyme (wouldn't replace the 4 hour infusion but wouldn't it be nice if someone studied this to see if it where an option in helping Hydrocephalus in the MPS I pt?) or some other drug to better help the pressure issues. I don't think there is really an answer or way but sure would be nice! I mentioned to her how PTC Pharma has their drug 'Ataluran' which is being studied in DMD and CF pts with atleast 1 non-sense mutation and how this drug was also studied in some of us MPS I pts through skin biopsy analysis. The drug is a powder but would be given orally and some of the docs think it may cross the BBB. She mentioned sometimes drugs can be reconstituted to be used via intrathecal but isn't really something on the horizon regardless we'd be able to try and anyways not as if rare disease pts and their drs can just try random drugs. Might be nice sometimes though b/c I sure feel like as good as these 2 shunts are they are also so far from perfect!
I just cant help but wonder for the 999th+ time why no Researcher(s) take an interest in the Hydrocephalus issue in MPS I pts (or any MPS types) and want to study it more, write up about their pts, etc? I wish there was more published data or articles for all of us to access!!!! Maybe someday I can convince Dr.Bragg to share what she's learned? I don't really know what her area of interest is and she seems genuinely to care about helping w my issues but at the same time I don't think rare diseases/rare Hydro issues are her thing (I've never really asked) so who knows.

Back in Fall my PCP (primary dr) had been looking over the Genzyme recommendations for MPS I pt f/up's and wanted an Audiology fup to be done given this has been 3 or so years now and prior to when I started seeing her . I've had the referral for months now but finally scheduled this for later in March.. Wouldn't you know though I finally schedule it and other things come up! I've been working w UofMn to re-schedule the studies for their testing for some months now for this year (I've missed the past 2 yrs for them) and originally we had been looking at right before or after the World Meeting. Now we are looking at and planning for the 3rd week of March w Neuropsych testing, fMRI and Neurosurgery (Dr.Guillaume) will reset both shunts after the MRI.
Endocrine (Dr.Polgreen) at the World mtng had found me and asked if I would be interested in participating in her studies especially given they are so interested in anti-inflammatory meds and this is something I had been on in the past.
My Endocrine dr has talked to her in the past regarding some of my Endocrine-hormone issues and I know she knows quite a bit about the MPS impact on the Endocrine system so I thought it would be interesting to talk to her more. Other studies will include a gait analysis study (actually not entirely sure what that is) and I believe there may have been one other test they where considering. The Neurosurgeon who will reset both shunts is one whom I had spoken to numerous times when formerly in Dr.Dickson's IT study 5 or so years ago and when Dr.G was still out at OHSU (Dr.D had been working w/him as she suspected I was having shunt issues and was when I dropped out of the IT/didn't join the continuation study). Anyways he is now at UofMn and when a year or so ago Dr.Bragg had asked if I would ask around to see what other Neurosurgeons/drs in the MPS community thought about our issues, he was one who responded and had rave reviews of Dr.Bragg and her team (nothing I didn't know about her but nice to know that other drs think so highly!)

As far as the Audiology Appt. i'll just reschedule this which isn't that big of a deal and nothing very urgent anyways.
Otherwise mainly for appts things coming up other than Cardiology which I believe is the 9th, and UofMn which is I believe the 18th or 19th-? things include the usual ERT every Monday and INR re-check on Monday with also updating Cardiology what HR is now on the upped dose of Nadolol. Neuro-Opthalmology at UW is the same week as Cardiology.
Non-appt wise we have book club at Church Monday night, group Sunday School later in March (in addition to the sun's I teach), our Sun School Teacher's mtng the night before I leave for Mn and Zan is spending this Sat. night + I am meeting 2 friends for dinner Fri night. Fun things that I enjoy and make the medical stuff do-able. =)
Many other things will add on here and there but I should mention next week is my b-day, Happy 31 to me (and my brother!)! =)

I think really this is about all since I just updated yesterday. I'll post again when there's something else new or something worth writing about.
Thanks for stopping by,

Erica

Cardiology updates

Jacket from the National MPS Society I had embroidered last year w purple ribbon and the other designs.

Not sure how I hit the enter button to publish this but I did so it wasn't really a completely finished entry (oh well I guess), I had planned to finish Thurs after a Neurosurgery Appt.
I guess i'll just do a separate entry then, maybe..
I don't think I wrote this last week but INR (blood thinner level) was back in range at 2.2 last Thurs when measured prior to ERT (infusion) and prior to the Cardiology Appt! So dose at this points remains at 6mgs 5 days a week and 8mgs 2 days a week. I re-test next Monday while at ERT again, which gives me 1 1/2 wks between INR testing for the first time since the last surgery 5 wks ago!

I talked to Cardiology today (Monday) per their wishes and expressed my mild frustration at the Nadolol and how I feel it makes me feel tired and not that great (apart from the headaches which have also been worse on/off.) Nancy, the NP's recommendation was to try taking the Nadolol in the morning as this is a long acting med and so their thought is the med side effects may not set in till later in the day after I am home and near to getting ready for bed anyways. Today this did seem to make a difference (the first day) though I will also say heart rate has still been humming along at a pretty great clip.
The NP I work w alongside my Cardiologist called me back yesterday as she typically does (she is who monitors INR and since the 2 OHS's has took the place of Dr.E's regular Nurse for pre-appt questioning, etc.). Per Dr.Earing they want to try going up on the Nadolol but recommended I try taking it in the morning as mentioned above to see if I then wouldn't feel more tired at night (which would be ok) since this is a long acting med anyways. Heart rate (HR) today (Monday) at ERT at initial vitals check and then an hour or so later was between 88-92 so no real difference yet at the 40mgs/day dose of new beta blocker. Dr.E would like the 80mgs (new dose) taken as a one time dose vs divided up in twice daily dosing, I guess bc it is the longer acting med. I am to call them on either Friday or Monday (actually in all honesty I can't remember why she said to call her Fri instead of Monday (was some reason) and if whatever that reason was didn't occur I otherwise was to let them know Monday how it is going. INR is re-drawn on Monday anyways while at ERT so makes sense unless some issue to just talk to them then once vs twice.
I can't remember for sure but I think she said they would consider adding something else or changing meds if HR hasn't come down enough (to the 60bpm range) so that was part of why I need to let them know HR either Fri or Monday along with any symptoms update.
One thing I will say is I think the Coumadin (for preventing blood clots in new heart valve) increases appetite so even though I don't feel great I still atleast have somewhat improved of an appetite so that is an ok thing I guess..

I've experienced a few extremely intermittent episodes of what I guess is dizzy spells over the past weeks - I think literally a couple in one day BUT both times that day AND today (mon) I was sitting down so it seems pretty unlikely it was BP related. Since then I haven't experienced anymore. Heart rate cont's to hum along (as mentioned above I know). Taking the Nadolol in the morning vs at night does seem to help some with the side effects though it doesn't seem to be having a real effect on overall bringing heart rate in to a slower range. I know this rate isn't that high compared to some but it is high enough that it is affecting heart rate function and concerning to Dr.Earing/bringing on the CHF symptoms again so i'll be glad when we get it down.
I guess that is it for now, will update other things after tomorrow apt or in next few days.
Thanks for stopping by,
Erica