Friday, March 28, 2014

Post-VP, TPL Shunt surgery-revisions, update

Surgery started a little before 8:00 and I think ended around 11:30 but honestly I can't remember that part very well!?
I didn't hear anything adverse about intubation but Dr.Bragg when she stopped by said her revisions went pretty well with just a small hiccup but nothing that would be a big deal, just took her a little longer on the TPL Shunt valve exchange. There are 2 incisions at that site vs the original 1 she thought we'd get away with but again not to huge of an issue. The area is definitely uncomfortable and sore but that is fairly par for the course with MPS and our thickened skin, muscle manipulation and what she was doing in removing the old valve + replacing with the newer, smaller (more in-line, size of the catheter) valve, both are the same type/brand and this new one remains programmable.

The VP Shunt she said she was fairly certain had occluded, as there was no CSF flow from it or maybe she said there was very little/not spontaneous flow? I can't remember that exactly either but I know she was fairly confident the shunt wasn't working well if at all.
The TPL Shunt she felt was doing well, which I also figured as although I had bad headaches I didn't feel near as bad as I would have when I only had 1 shunt and it would stop working. I also had very little of the autonomic side effects I get when both shunts aren't working well/not draining enough. These are the times I am even more grateful Dr.Bragg thought of and decided to put in that 2nd shunt; even if we've still had our fair share of issues the 2nd shunt and the Codman program. valves have made a huge difference! I am grateful for her persistence!
As far as placement of the VP Shunt Dr.Bragg removed the proximal (top portion) catheter and valve from the R sided incision and using new hardware and the Stealth-robotic guided system created a new incision on the L side, frontal ventricle. She then took this new hardware (catheter, secured valve and the catheter was tunneled on an angle (my description, lol) to re-connect on the R side mid-skull. Total on the VP Shunt there are 3 incisions. She feels the reason this incision kept re-opening is there was likely a low-grade infection under the skin (but not in the CSF so wouldn't show up on CSF cultures) and this in-turn causes the skin to break-down in a process she explained but honestly I can't re-explain! =)

I had a newer Anesthesiologist I've not had before but everything still seemed to go well; no sore throat after and they where planning to follow the same plan as other surgeries (thankfully Dr.Bragg waits I believe to scrub until after I am out and intubated as she has said in the past there have been times she'll tell the Anesthesia Team various things they can try if they are having issues). This time they used the glidescope and for induction (going off to sleep) they almost always these days use the IV drug vs using drug through the oxygen mask as the Anesthesiologist today told me they have better control of airway intubation time (or something like that) and in my case they have concerns with any delays from starting the sedation cocktail to actually getting the airway fully secured (a somewhat lengthy process I guess).

Prior to starting surgery (after I was intubated) Dr.Bragg and her Team typically washes the pts hair (I guess) and this time b/c she was going to have multiple incisions (both sides of my head) she told me when she stopped by this afternoon that she did the small French braid in my hair (she is so sweet) this way I think my hair was mostly out of their way and she has to shave as little as possible for each of the incisions. The braid is tied with some kind of medical type ribbon (I think she had told me one other time it comes on packaging or something) and actually kind of cute. :) I feel soooo appreciative that Dr.Bragg DOESN'T shave very much not to mention ALL of my hair in her surgeries as some Surgeons do!

I'll update when there is something to update about - say a prayer this discomfort improves, OUCH! At the moment I am not sure if the VP Shunt is draining or trying to drain or what but I've never experienced a feeling/pain like what is occurring for the past 1/2hr-ish and it hurts ALOT! =/ Otherwise it is the back incisions/area and the area over the original VP Shunt valve/reservoir (now removed) that feels the worst! Ice and PCA (pain pump) are good and every day will get a little better!
Anyways, thanks for stopping by,


Thursday, March 27, 2014

Shunt revisions scheduled 5:30/7:30

Surgery is scheduled at 7:30 tomorrow with check-in at 5:30. This is the first surgery (with Dr.Bragg) I've had since the heart surgeries where I've not been pre-admitted the day before and I am not yet sure if INR was in-range (low enough) so am not sure if we'll have to do Plasma as w/2 of the last 3 of Dr.Bragg's surgeries but hopefully not! :)

Per Dr.Bragg's wishes I had INR re-drawn today for tomorrow's surgery which was ok, I guess since my infusion Nurse could then access the Port for tomorrow's surgery; the results would still be read by Cardiology and faxed to Dr.Bragg.
While walking up to the Lab to meet my Nurse Dr.Bragg's Secretary called, when I talked to her she asked if I had any way to get the Cardiology clinic note, recent EKG and Echo results faxed to them by 2 that afternoon as Anesthesia was on their case and if the records weren't there by 2:00 surgery could be cancelled. I know the clinic note is automatically sent to Dr.Bragg per the NP as she is listed on my CHW 'Care Team' so I assume the record just didn't or doesn't get where it is supposed to go? Non-the-less I was able to walk down to the Cardiology Clinic and ask the Secretary if she could have Nancy, my NP fax the records as soon as possible which she did! No doubt I was a little stressed during that period, waiting to see if it would get done in time!

Last but not least I finally booked flights for Boston and fly in on Fri late afternoon (am hoping I can make Good Fri afternoon Church Services) and fly out Monday evening. I am excited this is done!

Will post updates either tomorrow afternoon/evening or sometime Sat.
Thanks for stopping by,

Friday, March 21, 2014

Cardiology Appt, UofMn Studies

I think I perhaps shouldn't write these posts while I am quite as tired as I was writing the last ones; for anyone wondering about my math skills related to the INR I realize neither of those numbers where actually in-range (=) though the number did still climb fast compared to last time. :) If nothing else amusing the way my mind works when overly tired! =)
-I am re-editing this, actually I just looked at the INR result on CHW's MyChart and I was right the first time; INR was at the high end of 'in-range' and 2.96, standard range is 2-3 for an aortic mechanical heart valve. All I can say is tired, tired, tired and numbers still and probably never will be my friend! =)

Per Cardiology Appt (was Tues) (F/up) and the fact that I was going to be in Mn till Fri night they just will re-check the INR level again Monday when I am at ERT (same hospital) and adjust dose accordingly. If the level is really high I'll re-test next Weds, 2 days prior to Dr.Bragg's shunt surgery, in which she will be moving the VP Shunt from the R to the L side but tunneling the catheter back around to keep the catheter tunneled down my R side of body (due to the Port being on the L neck/chest).
Otherwise for this surgery I'll stop the Coumadin 72 hr's prior to surgery(so Mon) for Fri's surgery, otherwise it is usually 3 days before.
Med wise Cardiology for now due to the shunt surgeries and not wanting to mess w/to many things at once is opting to leave current meds [Nadolol, Lasix, Spironolactone) the same, watch HR and F/up in 2 no unless something changes. Their big concerns as before are trying to minimize stress on the heart, mainly keep heart rate down, (it has been somewhat lower but is not at the range they are shooting for) and hopefully by doing so buy time to needing to replace a second heart valve, (second mechanical heart valve) and thus what that entails.
Their other concern this appt especially was our preventing any shunt infections as their concern is the affect this would have on the mech. Aortic valve given I guess both shunts do drain to the same body system as the heart. Thankfully we've avoided this so far as I'm not sure what a possible infection would entail and would rather not find out!

It's very possible I am slightly crazy but I did come to Mn (flew) on Weds which entails a few studies though the study docs did cancel the MRI portion of the testing which I wasn't really complaining about! Their concern was the 2 shunts both being programmable which not that big a deal(if or when any of my drs wants an MRI Dr.Bragg orders it and then she or her team reset the shunts to their correct settings after).
Non-the-less I think it was the shunts along w the heart valve (though there is nothing about that an MRI could affect) and the other hardware that made them decide aagainst that test. Dr.Shapiro just asked me if I would ask Dr.Bragg had we done any recent brain MRI imaging as she was kind of wanting to look at recent imaging to see if there where certain changes (having especially to do with white matter I think she said) since their last imaging in 2010.
The studies I did for Elsa Shapiro's on-going longitudinal MPS I study included a gait analysis walk, which means doing various things as they monitored (with little devices attached)and recorded via computer and video. This was done on a almost run-way type set up they have made and almost comical (I doubt most of us walk exactly like we do in real life when others are watching!) but went ok.
The other study was Thurs from 9-1 (Neuro-cognitive studies) and then meeting w Dr.Ahmed after to discuss medical history and especially over the past 3 1/2 yrs since I was there last. Last but not least I met w Elsa Shapiro who runs the whole study bit and she relays the results, her thoughts or any recommendations and what they may be up to in the future. Much of what they are working on I was aware of due to my work with Gene Spotlight but is still really neat to talk about it and hear their thoughts. She (Dr.Shapiro) has been saying for the last 4 yrs she is going to retire but then in the next breathe she would talk about what was being planned for the next year if their research grant was renewed for another 5 yrs so I (many of us in the MPS I community) are hoping she will keep delaying or stay semi-retired!? =)
Results from the actual NP testing where mixed; IQ is very normal while focus is down from 3 1/2 yrs go (I had thought I did good, lol!)and memory is also down though she did say I did fairly well on remembering information when it was in the context of something (say a story) but information that was taken out of context or given on it's own (word lists for instance) did not score well.
I guess I can't say that was particularly surprising as I continually write myself notes be it on my phone, in my (2) calendars or often even on the palm of my hand in order to remember things (dates, appts, grocery list, etc).
I think given the very many surgeries-especially the literally 30+ shunt surgeries/procedures in the past almost 3 yrs gives me reason enough to not worry to much about these changes! I also don't think the VP Shunt is working optimally (I do think it is working atleast partially just not optimally) probably could play a part.
It is also not uncommon in MPS I pts to have these changes. I doubt most people in my daily life even realize the memory issues I have as I am careful to write notes, keep a calendar and keep numbers in my phone/in my calendar vs having to try to recall information I don't often use.

I had dinner w a good friend of mine from Genzyme (she is our areas Patient Care Liaison) last night (Thurs) which is always really nice; we've been doing this anytime I am in Mn for atleast 7 years now! Occasionally she comes to WI so we'll see each other here too. Anyways we also ran in to each other at the WORLD meeting in San Diego a month or so ago and she was telling me that my former Metabolic (Genetics) Neurologist was at the meeting, which would have been really neat to see him!
Because he works in Pharma now he is isolated from Patients (a huge loss to our MPS I neurologic-knowledge community) but she said next time she saw him she would see if he could reach out. I really think he could help some of my own drs!? (He is the dr that diagnosed my hydrocephalus originally and followed the C-spine compression along w my former GC + suggested I enroll in Patti Dickson's IT study and then talked to her to get that all rolling. He was the type of dr that if he was irritated or grumpy he'd just tell you as soon as he walked in the door which was nice, he didn't really mince words but he also understood the MPS/brain/neurologic issues and how the 2 intertwined secondary to the MPS storage. I would have loved to talk to him while at WORLD but is also a HUGE mtng so is not all that unusual to miss seeing someone even when you do know they are there (which we did not).
Besides coming to UofMn for these studies this week (I flew back earlier today) I had infusion Mon, Cardiology Tues (both Milw) and a Sunday School Teacher's mtng Tues night so it has been a busy! I teach Sunday School on Sunday so I had to get that lesson ready which I did most of while at ERT Monday and finished earlier tonight.

I'll update again when there is something to update or write about,
Thanks for stopping by,

Monday, March 17, 2014

Home sweet Home (again)

I got released yesterday (Sunday) afternoon, my Dad picked me up and from their house I came back to my Apt which I think was around 4:30. Pretty much the only things I did when I got home where un-pack whatever I had w/me at the hospital and vacuum (just one of those things it drives me crazy if I don't atleast every couple days w the cats) and I laid down around 6:30 I think.
As much as I'd have like to just stay in bed I did get up, shower and go to ERT (in Milw) today which the actual infusion seems like a blur but I am finally home, in my PJ's and on the couch! Between my head and being tired the day just seemed a lot like a blur though it went pretty fast. :)

On a 'this is completely opposite of last surgery but i'll take it' kind of note even after getting the 2 bags of Plasma Fri pre-surgery to reverse INR and skipping the Fri night dose of Coumadin I was still at 1.68 Sunday (well within the 2-3 range) and today when re-tested at ERT for tomorrow's Cardiology Appt (same hospital) my level was 2.98. The Cardiology Team adjusted the dose to 6mgs/7 days a week and we'll re-eval dosing tomorrow since i'll be on this antibiotic (which is the likely culprit of the quick to increase INR level) till the 28th.

The odd discomfort I was having last week (was also worse on Thurs, I think everything was Thurs!?) hasn't gotten worse, it is still there but I do wonder if it is just the CSF occasionally building up as it drains or something related to the TPL Shunt. Non-the-less I hope it completely goes away when we put in the new, smaller in profile valve the 28th!
Otherwise I think I am going to lay down with an ice pack in a little bit and nap for awhile, I am soooo tired (from basically not sleeping while I was in-patient Thurs night-Sun afternoon) other than I think an occasional hour here or there a couple times.
I am glad to be home, wish I didn't have to go to Mn this week but I would imagine if I can get somewhat caught up on sleep it will be ok.
My cats are curled up on the chair and floor around me so I don't think they will be very pleased when I leave again Weds afternoon!
Thanks for stopping by,


Friday, March 14, 2014

Revision, VP Shunt catheter, UWHC Sugery update

Surgery started about 10:30-11 this morning and I don't think took very long but to be honest I also don't really remember what time it ended or what time I woke up from anesthesia. For the surgery Dr.Bragg had come by my room up on the floor earlier, before I went down to pre-op and talked a little about what she was thinking and what her thoughts about this issue had been over the past 12 hours or so. She was wavering between a few things but ultimately decided it would be best to go in, open up the exposed area of the incision and re-tunnel the catheter further down and then post-surgery do PCA (pain) pump and antibiotics + send me home on antibiotics.
The 28th, which was our original surgery date to trade out the current bigger sized TPL Shunt (programmable) valve and replace it with the smaller (but otherwise exactly the same minus having a reservoir to tap (draw CSF off of, test shunt function) Codman valve which would be more in-line with the catheter in my back-to-side and our hope is it will be less noticeable when I move, sit back, bend down, etc. I hope!!!
I am praying ever so much God will answer this prayer and this valve will work more finely with my body type, comfort wise! I almost feel guilty admitting I have been asking in my prayers for God to guide Dr.Bragg and myself in this surgery the 28th, that the valve may be more fitting and comfortable.. That surgery b/c it would require both being on my back and (I think) side or stomach to access both incisions for each part of the surgeries will be general anesthesia (honestly makes me feel more comfortable than the very rare occasion we use sedation as I've known to many who ended up trached when sedated surgeries went awry and the Anesthestiologist then couldn't get a secure airway.

Needless worry you might say but in our community, no matter how confident a Anesthesiologist is ones airway can change drastically under a stressful situation and when ones airway is already tend towards being difficult to access... I tend not to worry as much about the things I can't control, I believe you do what you can to prepare, educate and be aware but you can't let "what could be" rule your life. I still believe that applies when it comes to Anesthesia but I do think for airway and surgeries, it's soooo important to reiterate over and over (even if your feeling blue in the face find a different way to get the point across!) as this is an issue that has caused tremendous issues for a lot of MPS I Individuals.
The reason I say any of this is b/c I felt like I was perhaps beginning to perhaps be annoying or something, it's not even that the Anesthesiol made it seem that way but I also don't think he was completely, really getting what I was saying and instead was just trying to placate me; anyone who knows me knows I HATE when people say something they think you want to here! This all said I didn't dislike this dr and have had him once or twice before, more I just wish he would have sat back and listened for a minute or two without clearly already having a response on the tip of his tongue! I did understand what he was saying but I think very few Anesthesiologists understand how from one surgery to the next and back our airways change ie one day is a relatively 'simple' access, the next day may be extremely difficult (Dr.Taylor at CHW pointed this out and explained it VERY well in her experience w/my airway and 2 OHS how different each experience was!)

The Solu Cortef was given 30mins prior to surgery and Plasma (2 bags) which acts as a quick clotting agent to counter act the Coumadin (and high INR) was given as well starting about an hour before surgery and continuing to run in via the Port/second line after I was asleep and surgery started. This is given to bring a to high blood thinner (INR) level down to a safe for surgery range so a patient doesn't bleed out on the table especially in some higher blood access surgeries.
The ice is the best at the moment actually at semi-numbing the surgery incision area, my headaches, un-related to this surgery (but I do think VP Shunt related) have been on and off all day but probably just seem worse b/c of no sleep over night and thus really tired today between not a lot of sleep Weds into Thurs and then no sleep Thurs in to Fri + the anesthesia. Unfortunately the IV steroids probably won't make for great sleeping tonight either as the next post-surgery dose is 12 hrs post-op, so roughly between 10-12pm tonight and steroids have an odd side effect of amping a person up without actually giving you a real energy boost (atleast for me!)
I almost always request not to have the pre-OR rm sedative (makes me feel really weird, makes waking up from actual anesthesia much harder) so when in the OR, on the table while everyone was doing their thing, prepping, confirming information, hooking up monitors, etc. the Anesthesiol. jokingly asked me my MR # (which I don't know), but Dr.Bragg standing to my L piped up and recited it by memory, "b/c I say it so often dictating your notes"! WOW! =) Made for a good laugh while going off to 'dream land'.

Labs look good so far, in the past CSF infection has showed up as late as 5 days on several occasions! But as long as CSF cont's to stay clear (would be a good thing on all of our parts, a reason to mini celebrate I think!?) we will be able to wait to completely move this VP Shunt valve (Dr.Bragg plans to also use a smaller profile one than is currently implanted, less prone to breaking down the skin) to the L side during the other surgery for the TPL Shunt the 28th. =) I am celebrating in a joyful way at least that so far all labs are looking promising and that part one of this un-expted surgery for the VP Shunt was a success today! =)
Visitors tonight (my Co-Sunday School Teacher, and the other is my PM Nurse, Evey)

I'll update again when or if there is any news,
Thanks for stopping by,

I occasionally post this but If you'd like sign up as a 'follower' to the R of this blog entries for automatic updates. =)

VP Shunt, Admitted to D6-4 (Neurosurg.) for catheter

Just a really quick update (i'll update when I have more information) that while twirling my hair absentmindedly (I was working and finishing up my Sunday School lesson for Sunday) =/ I realized not only did the area around my VP shunt feel damp (I admittedly hadn't even changed out of my pajamas much less gotten through the shower despite it being mid/late afternoon) but the VP Shunt felt like it was really close to the surface, similar to when my hairstylist found it exposed back in Dec.

I know this had to have happened sometime in the last week as Lindsey (hairstylist) even commented last wk that the shunt site looked really good and no catheter was visible!
I rarely use a comb much less a brush in my hair (finger comb it if anything) so that wouldn't be what caused this, I just am not sure what would. =/ If anything the skin must just be tired of having a catheter underneath it and so many surgeries over the past 2 1/2 years? I don't know? =(
I was able to see semi-well enough via a camera pic (hard to bend my neck enough fwd to see at the angle my shunt is in the mirror) that the catheter was in fact poking through the skin again in a semi-small area. Not entirely sure what to do I called Neurosurgery on-call and the Resident paged Dr.Bragg. She apparently had seen a pic I posted on fb as she knew right away when the Resident paged her about me and she told him to direct admit me + to tap the shunt, (draw off CSF) run blood labs, and start IV antibiotics as a precaution. She or the Resident even called admitting to let them and Security know I was to bi-pass the ER and to have them just send me up to D6-4 (neurosurgery) + have a room assigned to me. The Admitting ofc called me shortly after I talked to the Resident to pass along some of this information to. I guess that was a small consolation for having to come back up here? =/?

Some of the very sweet Nurses always make it a little better and one of my favorites got assigned to me, she said to me shortly after I got here "We are always happy when you are here", not that they want me here just that they like having me I guess. :) After a semi-rough wk with some really mean comments from 2 different people (but a TON of really sweet comments ro refute those other 2) Evey's and the Nurses comments really meant ALOT! I always wonder if people know how much their kind words and actions really mean?

On a different note and I don't think related (but I also don't know) for the past few days and especially today I have been having a strong discomfort (but isn't something I would describe as pain) in my mid/upper spine, actually kind of near where the valve is but not centered there) and up to my shoulder/across and goes almost feels like right through my body to my inner core and can especially feel it with breathing. Has been really odd but I have no idea what is causing it/what it would be from! Who knows sometimes!

In any case I have no idea what the plan is, ie the labs look good so far so per the Resident he said Drragg wasn't sure if she was going to do like last time treat with antibiotics for a bit and send me home (would mean I could probably go to Mn for the study as scheduled next Weds-Fri and keep the Cardiology Appt on Tues) + bring me back on the normal surgery date (to change the TPL Shunt valve) on the 28th or will she want to do the surgery sometime sooner while I am still admitted this time? It is an infection risk with the shunt exposed but antibiotics can offer some protection so I am not sure yet!
Stay tuned, will update when I hear from her,

Thanks for stopping by

Monday, March 10, 2014

Cardiology, TPL Shunt revision, Anesthesia

Per my Primary dr for surgery - (who is very familiar w the complex, pre-surgery requirements) she made a few suggestions (as she wasn't doing my pre-op) to include that the stress dose steroid dosing be confirmed, make sure the Coumadin dose (and how many days before) was taken care of/managed, and she had concerns about the Cardiology issues being stable/managed as well as to make sure I had Cardiologies approval to proceed with surgery. There may have been something else she messaged me about to make sure I brought up/made sure where taken care of but so far pre-op is taken care of, INR (blood thinner level) will be done next Monday (at ERT) or Tues right before my Cardiology Appt and then the following wk on Weds, 2 days before the TPL Shunt revision when I will also have the Port re-accessed by my Infusion Nurse (her main job is on the CHW Access Team).
I wasn't sure initially if this surgery would be pre-admit or day of (the last two have been admitted the day before) but I talked to Dr.Bragg's Nurse last wk who called and surgery will be admit the day of (hopefully first surgery) which even though means very early it is always much nicer than having to wait till the afternoon on no food and then frequently delays in getting to surgery. I suspect it may not be the 1st surgery though unless Dr.Bragg has surgery over at AFCH and wants to get any at UW done first. Is hard to say.
In reality I suspect the hardest part of this TPL Shunt revision will really be the anesthesia/anesthesiologists getting an airway and depending if Cardiology feels they need to do more intensive cardiac monitoring. Otherwise I don't think the actual surgery will be very long.
Dr.Bragg had asked that I get Cardiology's approval for surgery (mentioned in last post I think) and as well that Cardiology give their recommendation for when to stop Coumadin (actually that seems a little odd, I think Dr.Bragg's Team made this call the last 2 times but I might be wrong about that) but the INR pre-surgery check is set and when done the result will be faxed to Dr.Bragg. Cardiology if they need can make any last minute recommendations if the level is to high (I am not sure actually what day I am to stop the Coumadin that wk, I forgot to ask Nancy, the NP when she called today about this and something else). Have time though and will figure that out next wk, I think it is typically 3 days prior.
I do f/up with Nancy and Dr.Earing in ofc on Tues next week instead of Thurs this week (death in his family) so their plan is to do a repeat EKG at the appt and revisit meds as the increased Nadolol (went up to 120mgs last wk) didn't budge Heart rate (102 Fri, 104-108 at ERT today) so in the mean time Nancy dropped the dose back to 80mgs as it is at least a bit (slightly) more tolerable at 80mgs (I just altogether skipped the dose on a day like Sat where I had things going on all day). Their long term plan is still to try and get heart rate under 70 to try and give the heart more time to relax in-between each heart beat and hopefully buy time to needing to replace the mitral valve (thus a second mechanical valve, higher blood thinner level and more complicated pre/post- (any)surgery blood thinner management).

I fly to MN to see specialists at UofMn next week Weds-Fri, with all appts on Thurs and their Neurosurgeon will reset both shunts after the fMRI is done. This study follows the long term cognitive (longitudinal) function in individuals w MPS I through cognitive (neuropsych) testing and the fMRI. There is apparently 1 or 2 other things they have added on this time. Due to all the shunt issues I've not actually been there in over 3 yrs but this study really is a year to year follow up. Not exactly loads of fun but on a side note and a way to make it seem a little more bearable I am meeting up w a good friend of mine from Genzyme (saw her in San Diego actually, too) for dinner Thurs night or Fri morning depending when both our schedules work best. I am looking fwd to that part.
I am sure it may seem like why do something like this? But in our rare disease world there are soooo few pts and so few treatment options (Enzyme is it and that does nothing for neurologic and many other issues) so by participating in this kind of studies the Researchers can get a little better idea of how to help us attenuated individuals and really maybe even get better ideas how to help us Attenuated, OLDER Adults!?
The above is the precise reason why I work for Gene Spotlight, b/c I can help make in a small way research happen a little faster by helping find adults or individuals and helping get individuals involved in research studies when they are or become available. By helping collect data so that when we do get to a point of having more research studies to participate in the patient data is more readily available to enroll pts faster, being able to access research study info more easily, patient info (who have agreed to share their info) more easily, in one spot and many other reasons that before my work w GS where lacking as a resource!
A lot of what I do is connect patient A (patients) to point B (studies, researchers, Gene Spotlight or whoever needs patient info when the patients or families are amenable to being involved per whatever GS needs or ask me to work on. BR>
So on yet another un-related note =) I am another day older, another day wiser (maybe!?) =) as it was my b-day last Weds. This past Sat after spending the afternoon with my Mom, Sister and nephew shopping I went bowling with a group of friends for mine and another good friends b-day which was a lot of fun. Made for a early morning what with losing an hour of sleep and getting up for Sunday School though!
On another un-related and older note (2 wknds ago) Zander, my 7 yr old nephew was with me most of last weekend and we had been playing on the floor together close to each other. Whenever my HR is fast it is also very loud (due to the mechanical valve).. Zan sitting close to me stops suddenly, looks over to me and without missing a beat says "Your heart is REALLY loud!" That cracked me up as I don't think I've ever had another adult or kid just know what the sound was coming from and yet Zan with high functioning autism figured out quite quickly what the sound was! =)

Tomorrow (Tues) is Neuro Opthalmology f/up (1 year) which I think the only issues may be perhaps glasses needing stronger (but actually not really certain on this) and sometimes it does seem like peripheral (side) vision is worse which is an issue they and others have been following for some years. We'll see what the appt brings!...

After Sunday School and Church one of my fellow SS Teachers (who was also bowling w us Sat night) and I went out for lunch which has become a semi-common place thing for us as we'll often go to Church together after SS and then if neither of us has kids (her grandkids, me w/Zander) we'll go out for lunch together. Tonight, after getting home from ERT and a few stops on the way home I finally made it to a Ladies Aid mtng at Church which was nice. Just another thing to be involved in, fellow Women (older than myself but some are friends of mine, others I don't yet really know) who are involved in different aspects of the Congregation. My fellow SS C-Teacher and another friend who is on the Church Committee I am on with me had been telling me I should come for months so I finaly got back in time and had some energy left (amazingly) so I decided to go once. It was nice. :) I am not the most out-going person and honestly don't mind being by myself, doing my own thing but this and Book Club are nice, something for a couple times a month or when there is something going on that the group needs help with.
In any case, i'll update either after the Neuro-Opthalmology Appt or next week after the Cardiology Appt.

Thanks for stopping by,


Monday, March 3, 2014

TPL Shunt Surgery date (to replace valve), Cardiology, etc.

Surgery with Dr.Bragg to take out the current TPL Codman programmable valve and replace it with a smaller (more in-line with the cathether) valve is scheduled for March 28th. I could have likely done sooner but have an assortment of other things in the next few weeks which I wanted to keep and not miss. Pre-op atleast at this point is this Fri (Madison), and I see Cardiology next Thurs which was someone Dr.Bragg wanted their ok to go ahead with surgery (is just a fup from last month, see more below).
Dr.Bragg's Secretary wasn't sure and I didn't think to ask Dr.Bragg last week if I will be admitted the morning of surgery the 28th or is Dr.Bragg doing a pre-admit the day before as she has these last 2 surgeries (due to INR, blood thinner level mngmt). She was going to call me back on Weds with that info and not a big deal either way. I might be able to skip the pre-op appt if it is a pre-admit which I guess each has it's pro's and cons.. The pre-admit con being it's typically a long-ish afternoon of testing but then there is wide free time to in-between. I don't know, don't really want to be there any longer than necessary so we'll see what Dr.Bragg has to say.
I talked to Cardiology per their wishes regarding what Heart rate was a week after upping the Nadolol (mid-90's, upper 80's at ERT today) and did the repeat INR which was within range again at 2.6 (range is 2.0-3.0). Nancy (NP) said she talked to Dr.Earing to let him know the HR hadn't really improved much and he told her he needed to think about it some. She seemed a little baffled by his initial response per what she told me but he also can go onto the computer and look at the inputted infusion vitals to see HR if he so desired, she was going to talk to him more in the morning on patient rounds and call me after with whatever they decide. I am truly hoping it isn't increase the Nadolol (beta blocker) dose AGAIN! =/?
Otherwise I see Neuro-Opthalmology for 1 yr fup next Tues and the following week Weds-Fri I fly to Mn for a few short days for the continuing MPS I cognitive studies (NP Testing, fMRI, and 1 or 2 other things they have added on related to their studies.) Their Neurosurgeon then will reset both shunts after. It looks like i'll have lunch w a good friend of mine from Genzyme during that last day though so that always makes it worthwhile and 'better'! =)

Fri night I had dinner w friends and Sat Zan spent all day and part of Sunday with me which was a lot of fun, I really enjoy having him here and he is so fun to listen to talk about various things he has learned or is interested in! A bundle of knowledge!!
Tonight at Church we had book club (long day after ERT in Milw!) and later in the week 4 of us are going to a fundraiser Fri night (for local beekeepers, not entirely sure what it is for) and then the same group of us bowling Sat night for Joan and I birthdays. Very fun! =)
I teach Sunday School this Sun and the following Sunday so turning the clocks ahead this wk though nice in reality will be a bummer losing that hour of sleep!

Stay tuned for updates, may be till next week unless something changes or worth updating.
Thanks for stopping by,