Thursday, January 30, 2014

Neurosurgery (VP Shunt) change #30, PMR update,

Oddly enough a 1/2 hour after seeing Dr.Bragg and the NP having re-adjusted the VP Shunt literally while walking in to the grocery store still in Madison I could feel the shunt begin to drain differently-an odd but not bad feeling. We didn't do anything with the TPL Shunt which is still set at I believe 50 or it might be 40, in any case we have I believe slight wiggle room with that shunt and how much it drains. I think Dr.Bragg is more leery of messing with that (TPL) Shunt as out of the 2 I believe that is the one she believes most likely to fail and also the (my impression) most difficult to revise in a sense given the scar tissue in my pleural space from so many previous attempts to access there and the previous open heart surgery-chest tubes (3 from first surgery, 4 the 2nd surgery and we've revised the Thoracic shunt several times).
Dr.Bragg after coming in to the exam room said to me "your ears have probably been ringing lately" as she said she was talking about me/my case to someone talking about the difficulty we've had with keeping the shunts working and her cont'd, possible interest in actually working at what she believes is the (compliance) problem. In my case she believes the MPS, storage and bone thickness affect the shunts although honestly I can't remember how other than not enough space for CSF due from what I understand the Hydrocephalus in her opinion having gone un-treated for so many years and the skull becoming thicker over the years and thus non-compliant.
I don't know would thinning the bone and creating space mean we could take out the shunts? I didn't think to ask her this question and she said she honestly didn't know if thinning the bone (if I was even open to it) was something that would even be a possibility in my case due to the MPS (storage) but would be something she would have to look in to if we ever got to that point (of wanting to really explore the idea).
She said the idea would be they would literally remove part of the skull, thin off cm's of the bone and this in turn in theory (as they have done in some other of her non-MPS pts. who've had on-going, virtually un-relenting issues w their shunts) gives a bit more space that the CSF has room to circulate.
The 2 worst things con't to be 1. the Pleural incision (where the distal end of the Thoracic shunt goes in to the pleural space) is literally right at or under the bra line and the TPL valve sits mid-back yet (as I've written about) and the valve is very noticeable w/movement.
For now we'll just see how this shunt adjustment goes, hope it lasts but also being realistic know the shunts rarely stay working long but longer would be a good thing and who knows? One day at a time, right!?
I just have to say I am thankful that Dr.Bragg is one of the few providers who is able to look at me and just knows (she always says she can tell by my eyes) whether I am feeling well or not. She doesn't look at if I am dressed reasonably well or well put together, she just knows I've gotten to the point where the shunts when atleast working semi-well I can function and thus make myself look presentable. I am VERY grateful that to her appearance isn't mean her pts. do or don't automatically feel good!
A view of the Codman valve implanted in both of my shunts

On a different note, INR when discharged last Weds was 1.1, today per my infusion Nurse who looked it up (ERT is at the same hospital as my Cardiologist and thus labs are done) was 1.2. Nancy, the Cardiology NP called w their recommendation the next day and I test again on Monday. If level is not yet in range they will up the Coumadin dose (oh joy). She was curious if Vitamin K was given prior to or during surgery to bring INR in to surgery appropriate range though I did forget to ask Dr.Bragg this. It wasn't a big deal to the Cardiology Team, they where more or less just curious and said when Vit. K is given to bring INR down it often explains why INR comes up more slowly.

Quinn, one of my nephews was sitting across the table from me last Sat playing Sequence (and other card games before) and says to me "What is that noise?" to which I replied "I don't hear anything, what is it?" He listens for another few seconds, leans over the table and says to me: "Is that you?" Lol, to funny, I forget that when my heart rate is very fast you can both see my heart beating wildly now on (thin) chest and you can apparently to other people hear it pretty well as I've had probably 1/2 a dozen comments since either of the OHS's about my heart sound! =) He seemed to think that was pretty funny!

I think I wrote about it a while back but I am partnering w the same Genzyme-Running for Rare Diseases runner as last year for the Boston Marathon but turns out the Marathon is Easter wknd. =/ Not only am I unsure I want to give up our families small tradition (easter is my favorite holiday) but the girl running on my behalf just found out from Genyzme they wouldn't allow her and I to spend Easter together. She had asked if I would want to go to Church w her and to her Cousins but Genzyme has a policy against this, against their employees spending time in their homes w patients who get Genzyme's drugs. Odd! I am not sure now if i'll go to Boston or not as the activities are mainly Sat night and then Monday, the day of the race. =/ I am sure I could still stay w my friends but don't really want to intrude on their Easter day celebration. =/ Have some thinking to do to do and talk to my own family. It really was a great time last year and would be really special this year given I was there during the bombings last year and for Jessi and Genzyme's Team running/completing. Just not sure. I do wish Genzyme and other Pharma's realized that patients and their employees spending time together outside of organized activities isn't hardly going to cause the world to end!

Weds's PMR appt for botox was cancelled the morning of, although I admit I wasn't to sad as I was tired and not feeling entirely like going there and then later to Milw. Dr.Caldera, the PMR dr did later that morning call herself, which was unexpected but clearly shows why she comes so highly recommended! She just wanted to ask a few ques's and let me know she was really trying to work out the insur-out of network aspect of my seeing her and if I wanted she thought maybe she could get it worked out within a couple weeks. I am just floored she in what I am very sure is a busy schedule and having met me only one other time would take the time to call me personally!

Will update when I know something or something worth updating,
Thanks for stopping by,

Thursday, January 23, 2014

Discharge (Weds), Shunt revisions.

I am soo incredibly tired, I just can't even explain it other than the pressure in my head, my vision being so messy (improves some in the afternoon or the longer I am up) and overall how tired I am altogether is making me feel like I am in a zone. =/ Although it seemed like Dr.Bragg and Sue (her NP) where hesitant when the Resident offered my going home and I wanted to Dr.Bragg did let me out.
The Resident had said this morning it was up to me, how I was feeling if I wanted to go and I figured why not? I wasn't really doing anything here (in-pt) I that I couldn't do at home.. I do feel like crap and so tired (slept roughly 13 hrs over night); I think I may also lay down for a nap this afternoon all that sleep aside.

Dr.Bragg tapped the VP Shunt on Tues and appeared to be flowing ok, a good thing albeit no idea why I feel so crappy. She does still feel time may be our best bet in this case given how much she did in surgery last week and I often tend to have difficult recoveries (slow) after these shunt surgeries. I think this may be b/c Endocrine hormones tend to be all over the place and even w our treating the cortisol deficiency this still has an big impact on post-surgeries.
Dr.Bragg commented how she was thinking of this surgery and how I've been feeling when she was at home last night (Tues night) and things we've done in the past (especially the pseudomeningoceles or CSF filled pocket which is something we always marvel at how well I did when I had those. Pseudomeningoceles are fluid (CSF) filled pockets under the skin that can expand, acting almost like a shunt), many people do poorly with these as they can only drain a small amount of fluid (I guess) but it has always seemed like the more fluid I could drain the better I felt!
She also reinforced as she typically does that there will be other things we can try if I don't improve (symptoms, how I feel) but that she (we both!!) hope a little time will give me better days ahead. She just said she wouldn't let me feel like this for a long period and we'd re-assess at f/up next week. I know (as I've likely written about in the past!) I shouldn't doubt her or that she's going to be there to help me for the long run but I do sometimes (then I feel guilty) though not intentionally. I think a big part of my self doubting stems from all the issues I had w other Neurosurgeon's in the past wanting to help and then bailing out. I am lucky that she is persistent and not easily scared off as it has been 2 1/2 yrs now of our working together. I know it has been an up and down 2 yrs and not perfect but I am very grateful she's hung on. I think it's a feeling only someone else who has had a lot of specific issues and had to work w/a Specialist often ( Very often!) could understand? I don't think I ever feel totally at ease but I do trust her, even if I doubt a lot!

I am back on Coumadin (blood thinner) after having been off of this for nearly 2 wks and having been bridged w heparin injections for several days while Dr.Bragg decided whether she wanted/needed to go back to surgery for my shunts or was she going to keep waiting and watching. The dose just starts back up where I left off almost 2 wks ago.
I talked to Cardiology (NP) earlier Weds regarding being discharged and when to do another INR check for them. They advised Monday, since I will be at ERT (infusion) at their hospital anyways and they kept the Coumadin dose at the prior 6mgs. My level this morning was virtually normal (for someone not taking blood thinners) but the NP felt since we've had a fairly steady pattern on how my levels rise (usually from her tracking record takes 5-7 weekly or bi-weekly lab checks) and she didn't think this time would be any different as I am finished w the IV antibiotics (which can affect a blood thinner level) that where given for the past week in the hospital.

If not before, I will update after the Fup w her next week. This was originally on Monday but due to my receiving ERT every mon (which she/they where able to arrange both weeks while I was in-pt!) in Milw (Children's Hospital) she was ok moving the apt back to next Thurs.
I see the Rehab dr next week for Botox injections which are done mid-spine (around the shunt but not in the immediate area of), the c-spine (neck) and across the shoulders. This is with the new Physical Med Rehab dr. whom I first saw last month and who Dr.Bragg/her Nurse recommended.
Will be a busy week so I am praying I feel some better!
Thanks for stopping by,


Sunday, January 19, 2014

Post Surgery, in-patient update.

Friday Dr.Bragg did another Chest Xray (to look at or monitor the inflammation around the pleural space and make sure nothing had collapsed (lung specifically) which inflammation I think was stable and thankfully (though I didn't expect it had/would) the lung itself looks good.
She re-programmed the VP Shunt down to 40, which is also what the TPL Shunt is set at, I haven't noticed a big change from this but I do think it may have helped a little. As is almost always the case mornings are the worst and have been a little hard. I can always tell when I feel particularly bad as I actually get some (restless) sleep albeit I wake up very often (sometimes like last night according to the Nurse every 15-20mins, which I then have this weird habit of while apparently still sleeping (I never remember doing this but do it quite often in a night) sit up and on the side of the bed. From that I must rock back and forth as the Nurses are constantly walking past and waking me up fully to get me to lay back down (I sleep on my side semi-upright.) Out of all of these waking up/sitting on the side of the bed and being awoken by the Nurses I only first fell out of bed (fwd, landed on my knees) early this morning. Needless to say days like today I ended up falling back asleep after being up and to the bathroom, brushing my teeth and restlessly sleeping for another hour or so including when the Neurosurgeon and Resident (separately) came by on each of their rounds.

After surgery last week when Dr.Bragg stopped by she did comment that when she tested both shunts in surgery they both appeared to be flowing which she was somewhat surprised by as she said to me "I would have honestly thought one of the shunts was not working and I would have guessed the Thoracic Shunt", but non-the-less they looked "ok". =) We both said it figures we would have had to mess w the VP shunt given it was working!
Hopefully now we can figure out whatever is going on ie is it just "brain irritation" from Neurosurgery having had to do so much in this surgery or is there different pressure dynamics w the 1 shunt now draining back to the pleural space?
We've never had both shunts have this Codman Hakim programmable valves (the best we've had as far as how well a valve or valves have worked for my symptoms) and both drain to the separate locations so hopefully this will smooth out! I am glad atleast the shunts are draining separately; so nice for stomach spasms sake!
When the shunts where both draining separately last Spring I had gotten the Codman Hakim (I think) in March and we didn't place the 2nd Codman (TPL Shunt) till this last June (leading up to the heart surgeries) so we didn't know as is the case now would symptoms change?
Although both Shunts worked pretty well together I did have some symptoms (and the stomach spasms) after we placed the 2nd (same) valve on the Thoracic Shunt last summer and we where messing w the draining location, so this changes last week is basically trying to sort which of the many things caused these current symptoms? We will figure it out.

I am not sure if it's the Lovenox and suddenly stopping the Coumadin last week or what but I am shedding hair the past 2 days and especially after the lovenox shots like it's a new business. I literally can run my fingers through my hair and pull out 15-20 strands from one small section (and yes I know that is ridiculous to even estimate how much you lost!).
It is a few days later as I am adding more to this post (before it saving for all to see) and though I hate to jinx myself I asked to not do the Lovenox injection this morning and I haven't dealt w very much shedding hair yet! I hope it is done!?!? I highly doubt it's coincidence my having been on the Lovenox and given this worsened shedding only started after I started the (lovenox) injections but you never know. I am just hoping the worst is over! Whatever the case I hope the loose hair stops for good! =/ I have more than enough hair right now you cant tell I've shed like crazy but even w a lot of hair you still only have so much to lose over time! (I know that's incredibly vain but I feel like hair is one thing I can have a little control over despite everything else and I don't need any extra help w my hair being crazy!..
The thin hair seems to happen for far to many people from reading about Coumadin and this issue online but yet few seem to know the likely connection and few seem to be aware sometimes taking replacement minerals like the B-complex (biotin for instance and then Zinc/copper, fish oil) can help.
I know the Cardiology NP last summer/fall after the OHS commented that they recommend to pts to start a Multi-Vit as Coumadin messes w the body a lot. I hadn't started anything till recently, I guess mostly bc I wasn't sure what impact she meant. From reading online/asking around to friends those who do experience it many seem unaware that this hair issues is something that the Coumadin can cause due to how it messes w the bodies mineral/vitamin balance. many seem interested in this and want to start something but often don't know where to start. Others who've been around the block w this issue frequently recommend 2 supplements.
According to a friend she much the same as the NP commented that Coumadin messes with the bodies balance; =/ Rat poison will do that I guess! This same friend (whose son has MPS III) gave me some specific recommendations to look for that wouldn't be so hard on my body (upset stomach wise especially) and she felt would help balance out what Coumadin un-balances in the body.
The reason Dr.Bragg has been doing the Lovenox she told me Sat morning vs re-starting the Coumadin is she doesn't want to have to wait for INR to drop if she where to decide to go back in to surgery. I don't think she will but she mentioned she'd probably tap both shunts before releasing me and she worried doing either (anything invasive) to soon would stir up any possible infection that was waiting around in the CSF.
She has been doing twice daily IV antibiotics since surgery too. She wasn't here this morning (her and her partner switch Sunday's they round and on days like today her partner sees her pts.) so I didn't get to see what her thinking is but hopefully tomorrow we can figure something out!

I know it is semi-ridiculous to worry and talking to my parents the other day about my feelings (regarding would Dr.Bragg ever just get tired of trying to find answers or the best optio, a topic I've written about here before) they wisely felt like if Dr.Bragg has been through this much with me already why would she stop now? Like I said isn't always make sense but I still sometimes worry a bit about her giving up as she seems to have a down to earth-look at it practically view of my shunts and overall issues. When you have something like MPS I think it is fairly normal to worry a little about the good drs who invest time in your care suddenly leaving or giving up!

I should probably worry less about this type of thing and more about what I can influence (I don't worry about a lot of things including very much about the actual surgeries I go through but I do mull over things that really aren't worth worrying over!

Anyways, I think this is about all I know for what's going on since surgery; i'll update again when I know something more!
Thanks for stopping by,

Wednesday, January 15, 2014

#29 (I think) VP and Thoracic Spine Shunt revisions.

All's I can say is OUCH (but to be expected I know) w where incisions all are- 7 total, (2 on back, 1 side, 1 abdomen/stomach, 1 neck area, 2 on head (back and frontal-side area).
Dr Bragg ended up sticking w the 2 valves I had (new ones, not re-used) and together with General Surgery assist they where able to get the Thoracic Shunt disconnected from the VP shunt at the 'Y' connector (stomach/abdomen) and re-convert this TP shunt to the Thoracic Pleural Shunt (she'd had some concerns there might be to much scar tissue from the repeat chest tubes during/post the 2 open heart surgeries in July and Sept.) I am very happy they could get the catheter back in! (yay!!).
The only downside of it all is she said due to the difficulty they have had w the ProSA valve and it being so difficult to re-program (lower/raise the drainage amount or after some types of imaging)and the Codman Rep plus others she talked to didn't have a great suggestion for something smaller profile she could try that would fit the tubing I have so she stayed w the same Codman valve I had before. She did say she'll keep an eye out or ask around to see about if other companies maybe have a smaller profile valve for the TPL Shunt we could try as she is still willing to try something else here if she could come up with something she felt had a reasonable chance of success while also not being irritating to the skin. Regarding the ProSA valve she said it being a metal disc she did also feel this might be just as uncomfortable to place in the mid-spine area given I have so little far there and would likely move when I move or sit back too.
The way the current valve is the valve is big enough to feel anytime I move, sit back or bend down but it is the best working (imperfect but still the best working) valve we've had yet out of any of the many types of valve we had used in the past 2 1/2 yrs. A bit bummed about still having the Codman valve BUT I know Dr.Bragg wouldnt have used the same valve if she didnt think it was our best bet overall. She did promise she'd keep an eye out for something smaller and similar as there are some valves she and her partner dont typically use. Hopefully for now maybe it will heal and be a little different, I can hope atleast! =)

For The VP Shunt portion of surgery, the ventricle Dr.Bragg was trying to use was just to small (smaller than a pencil width around, she said even with zooming in the Stealth assist machine she was using (this is where Monday's CT Scan was loaded in to) she really wasn't able to get a clear view of the inside of the ventricle and she did have to cut out a bit more bone in addition to what they had drilled out last year when she was also using this ventricle to get re-access. In that case, last year she had a lot of difficulty maneuvering the catheter in to place but was able to do so, just at a less than optimal angle from what I remember her telling me. She did end up revising that particular shunt location a couple times shortly after it was placed last year if I remember correctly.
Dr.Bragg decided to disconnect, take out and remove this whole set up (the same ventricle she was trying for again yesterday) + last year after multiple attempts to revise the catheter angle and once the infection was treated + moved the whole shunt back up to a frontal placement. The same basic situation this surgery, she had already re-routed some of the ventricular catheter to place the shunt back in to the parietal ventricle but she had to abandon that, remove what she'd just placed and move the valve and catheter back up in the frontal location again where it had been since last year and the area she had operated on a few wks ago when the catheter was found to be exposed.
Dr.Bragg did say she placed something either under or over the newly placed catheter (she took the old catheter and valve out and replaced w new parts to atleast minimize overall infection risk) to keep the catheter from pushing up and eroding the skin again.

Per Anesthesia who stopped by in post-op recovery (was a Anesthesiol. familiar w my history, who has had me before.) the fiberoptic intubation (which is always used whether intra-nasal or throat intubation due to in my case an excessively small airway) was used and the actual intubation effort went well w their using the scope and assist machine. Always nice to hear considering how many times I hear from either the Anesthesiologist or even Dr.Bragg that they had a lot of difficulty getting a stable airway!

Yesterday I was super uncomfortable after surgery and although I had IV pain med and the PCA it was pretty bad pain levels. Thankfully Dr.Bragg is great about adjusting meds to try and better get on top of overall pain and thus manage my post-surgical pain as that better management does often mean faster or better healing (able to move around a little more easily). I did get a little sleep overnight, on and off for an hour or so at a time but atleast with the pain being managed by a cocktail of med opions and PCA (pain) pump I wasn't waking up to aweful pain just discomfort which pushing the PCA button helped. Right now I'd say the worst of the incisions is my side (where TPL shunt distal end was inserted in to the Pleural Space) and the Thoracic valve incision which is in the old site (same incision) but the spine of course has a lot of muscles and nerves which make pain a lot worse.
I guess the 3rd worst area is head and I feel wiped out despite a little sleep so keeping awake is an interesting feat and I keep coming close to nodding off and startling myself awake every few mins. when I do nod off. Hopefully, maybe I will be able to sleep a little more easily tonight and for longer periods in between each time I wake up. Probably affecting last night some was the stress dose cortisol (100mgs) had to be given very close to bed time as it is givne 8-12 hours after surgery.

One of my Pastor's from Church was just here and we where talking about everything including the committee I am on which he helps oversee and guide. We where talking about the various things I am doing for this and one of the Ladies (who I am friends with) not really realizing how much else I have going on between my work w Gene Spotlight (right now for instance researching information regarding 4 types of Gene Therapy (which I otherwise know basicaly nothing about!) and putting this in to writing so the Foundation can make a decision regarding what studies they might like to fund-for MPS I. I know this doesn't sound like much but believe me when you know nothing about Gene Therapy it is like reading the alphabet all mixed up in a pile of soup (I don't know, hard to explain but not always the easiest reading!) :) That all said I have enjoyed learning about the various types of Gene Therapy and am learning a lot but also honestly not sure I could re-explain it in a way that would make sense in a general conversation!

Anyways in addition to that for GSF I am helping with the Committee fb page and other project planning while also trying to get a little rest (imagine such a thing!) here at the hospital. I am tired!! There isn't alot to do here anyways but am trying to not over-do to much here but also move atleast a little. I said to my Pastor I don't think this friend really gets at all what I all have going on especially when outside the hospital between ERT pretty well all day Mondays and the drive to/from, appts typically 1-2 other days a week in Madison or Milw., my work w GSF which although part time and varies in how much time I spend on it depending what they ask me to do still does take up time!
Additionally on my wks to teach teach Sun School I do spend time putting that together. I don't always feel the best so sometimes something that might take most people a short amount of time takes me alot longer. Although I am not a very exciting person I do have a life outside of the things I am involved in to! Like most people doing my own thing be it errands, going to my parents or sister's or having lunch w another friend or two is great (like most people like to do!) =) Sometimes just decompressing by reading a good book are nice, mindless really-things you do while not really having to think while doing them! =)
! I honestly don't think this friend/leader of our Committee could really get how complex the Med stuff is and thus how much time it takes as she apparently said something (not in a mean way) to Pastor about my having so much time she was glad I could do all these things she and our other member (one of my close friends) didn't have time to do!?
I said to the Pastor I don't think people realize just how much time it all takes or even most people realize that I DO work part time outside of the things I am involved in! This lady also doesn't work (was a lawyer till she moved) so I am not entirely sure what she has going on and it doesn't bother me as we all just need to focus on out own tasks and not worry about any one of us having to do more than another!
Anyways I just needed to write about this a little, kind of bothered me a little but I do know it's just a matter of she's truly pretty unaware of the overall medical issues and thus time it all takes. Can't blame someone to much for that I guess as Connie and Joan have a little bit better of an idea as they've picked me up from numerous hospital in-patient stays as well as Joan bringing me this week to be re-admitted for another surgery.

I don't think you can every truly get how much time medical stuff takes unless you are doing it yourself as someone talking about it is completely different than being in the situation over and over again yourself.

I think I got the basics of the surgery and today they re-started the blood thinning though (and I'm not sure why this route) they re-started it with Lovenox injections vs going starting to the Coumadin 24 hrs after surgery. The injection actually wasn't as uncomfortable as I thought it would be based off what others have said to me-I guess not much injection wise can compare to the SoluCortef (stress dose) injection I have to do from time to time in my upper thigh-that goes directly in to the muscle!

Thanks for stopping by,
I'll update again when there is something worth writing about or any changes,
Take care,

Not the best pic but from this stay - there is one other incision at the back of my head also covered by Telfa and then the 5 others ranging from shoulder/clavicle on down. A lot in other words! Per Dr.Bragg "I think we may have even broke our previous record in # of incisions in one surgery with you, this time." LOL, probably so!

Monday, January 13, 2014

Pre-admitted; Shunt revision (#29) surgeries (tmrw)

The Aldurazyme (enzyme replacement) is infusing right now, not sure how the NP got it changed but originally Pharmacy had told the Neurosurgery Team they couldn't order this drug until I was admitted and so I likely wouldn't get it till Weds (wouldn't have been to huge a deal). - Glad it was sorted out and able to be given today though! :) Any little "help" ahead of surgery is good!

The CT Scan is also done and was earlier, not horrible albeit laying flat was not awesome but also thankfully quick other than the 3 mins it took seemed quite long and honestly by the end throwing up seemed like a very real possibility! Thankfully it was quick and within 20-30mins after I was mostly feeling fine again. I've been sitting on the side of the bed since working on a blanket for my niece (her 3rd birthday is the 21st!) and watching last weeks episode of Parenthood. This CT scan as I've written about several times before is used in the robotic type computer machine (not sure if that's really how best to explain it) Dr.Bragg will use in surgery in order to try and best place the new location of the VP shunt in the lateral (parietal I think it may be called) ventricle. -I should really know it by now but can never remember the names of the 4 ventricles! She is planning to try and use the previous incision and area we had used last year after the shunt infection and after which she placed that new VP shunt in the back ventricle.

Accessing the Port was a joy today, I actually really get letting other nurses who've not ever accessed a Port before try to learn but sometimes I just wish I wasn't always the guineau pig! =) It took 3 tries today, the 1st two over-seen by 2 experienced Port-access Nurses and the 3rd one was actually done and accessed by one of these nurses.-The final Nurse to get it is actually who accessed it (over-saw accessing it) last time too. Anyways all is well now, Dr.Bragg walked in the first time the Port was being accessed (and failed attempt) and I think her face wavered between disbelief or "I cant believe you have to deal w that" and it was almost like she wished she could just do it herself. Once the head-overseeing Nurse deciced they would wait and come back I was bleeding from the Port and Dr.Bragg was quick to try and find something to cover it as she didn't want it getting all over my clothes. I like that she cares and notices these things. =)
Once we had the Port access on hold for a bit Dr.Bragg and I started talking again and I asked her if she had changed her mind on anything or if we where still going to go w the same basic plan. We talked for a good 15mins about her plan and one part (the Thoracic valve) she is likely going to use a different type on as well as she was planning to talk some more to her Codman Rep (who I think she said would be in the surgery tomorrow) to see about a smaller profile but similar Codman valve as she wasn't comfortable going ahead and placing the ProSA valve (due to it being a metal profile which she felt had a strong potential to still be irritating to the muscles and back tissue as is the issue now) and she said they have a lot of difficulty getting this valve reset/re-programmed.

She did say this surgery would be on the longer side given what she all will be doing (5 or 6 incisions, back/stomach/side/2 on head and maybe if I understood correctly one at the neck/shoulder area to pull the catheter through). She is hoping if she is able to just pull the catheter from the shoulder down (or maybe it was from the abdomen up, honestly that part is a little confusing) she thought this may be a little less painful than having to completely tunnel a new catheter/new shunt trac (given how tough our MPS skin is "like going through shoe leather" and the bruising that tends to leave all along the abdomen/chest/upper body. My guess is the back incision(s) and back of head incision (due to laying back) will be the most uncomfortable - the back incision because there are more nerves there and I gues to, probably the side because there is less fat to protect.
The surgery will be her first of the day "as she wanted everyone to be fresh and on their toes" (or something similar to that) and she said originally mine was scheduled as second but she had it changed as her second one is simpler and will be the shorter of the 2 she has. She just said something like this will be a longer surgery, you'll be on the table longer. General Surgery will help disconnect the 'T' connector holding both shunt distal ends in to the peritoneum and they will be the ones to tunnel the Thoracic distal (draining) end back in to the pleural space which she said they would be using a side incision vs the incision we used last time. I think that means they help her with the new valve placement in the Thoracic spine but I am not sure of that part. Otherwise she will do the VP shunt and revisions/changes there. She planned to start w the Thoracic shunt so as not to make General Surgery wait around for her and then she will move on and complete her portions of the total surgery.

Overall I feel confident (no reason not to, she is great) as usual in her and her decisions and feel she is or will make what she feels is the best decisions for my care. Hopefully this will go wonderfully but I also always know from experience she hasn't walked away yet (thankfully!) should we have any issues after.
I'll try to update when I feel up to it, some of you are also on fb and can see shorter updates there to.
Thanks for stopping by,


Sunday, January 12, 2014

Monday Re-Admit (UW) for Tues's Shunt surgeries

Hi All,
Labs for Monday's Admit ahead of Tues's surgery looked "fine" so those shouldn't have to be re-done when I am admitted Monday.
Admit time Monday is 1:00 for which Dr.Bragg has wanted the CT Scan ( Stealth, for robotic guidance in surgery) and Port Access for INR and Lab. Joan (Co-Sunday School Teacher, I've written of her in the past) and her son who I've somewhat gotten to know are meeting me and going w to UW tomorrow. Should be a good time as we plan to grab lunch on the way and generally I don't think we ever don't have a good time when together. =) I don't know what time surgery is scheduled for Tues but will update here or on fb when I know any details.

As far as how long will this in-patient stay be, I don't really have a clue. If Dr.Bragg hasn't changed her plans then she will be disconnecting the 'T' connector connecting the VP and Thoracic shunt distal catheters to 1 draining end (both currently end in the peritoneum)and moving the Thoracic shunt distal end to the Pleural space (pre-heart surgery location). 1 or 2 incisions. In addition on this shunt she had planned to change the Thoracic shunt valve (currently a Codman Hakim) to the ProSA programmable valve. (Incision #2 although clearly I have no idea what order she'd do all these changes).
After the Thoracic shunt she is additionally changing the VP shunt proximal (top) catheter from the current frontal location (due to the catheter having been exposed, the surgery a few weeks ago to re-burry this temporarily) to the location at the back of the skull-I can't think of what that area is called or the name of the ventricle she'll use. I guess that would be incisions #3 and 4. (again no certainty what order any of this would be done in ). She had as of the last time we spoke a couple weeks ago planned to keep this VP shunt valve as the current Codman Hakim valve. The re-programmer for this valve is a bit different than any of the others according to Dr.Bragg but doesn't seem to difficult.

This weekend and past week have been busy w several Appts, teaching Sunday School, Zan spent Sat night (and then goes to Sun School and Church w me) and Sat my Mom, Sister, Zan and I went shopping in Madison. This past Thurs night and then Fri for several hours I had Church committee meeting related things going on both at the Pastor's house and then a meeting at the library in town. Thurs I also had an Appt in Milw with Pain Mngmt-in her case she asked me to keep her updated and if Dr.Bragg needed to have her (Dr.Bragg) call Dr.B. Today after my sister picked up my nephew I also met up w Connie (another good friend, fellow Sun School teacher) and Lois (the other on our Committee) and had launch, hashed out some more of our Church related things and in general was nice.
I guess that is about all I know, really had just wanted to update a bit about the re-admit tomorrow (Monday) for Tues's surgery.
Thanks for stopping by,


Monday, January 6, 2014

INR's, Next weeks Shunt Surgeries; related Info

Nancy, the Cardiology NP called with the INR results today (last Thurs) and as was to pretty much be expected (given I had been off of Coumadin for a week prior to the surgery 12/27) my INR was not in-range and fairly low. She and Dr.Earing re-upped the dose to 6mgs and I re-test on Monday 1/6.
1/6 Today's INR result was still low at 1.68 (goal range 2.0-3.0) though the low INR level wasn't that surprising again given the time off of Coumadin.
Dr.Earing told Jane whom I was talking to to keep the dose at 6mgs considering I take the last dose of this on Thurs and then stop until 24hrs after next Tues's double shunt surgery. I am to then let the Cardiology Team know once I am released from Dr.Bragg/being in-patient and we'll re-start INR draws again. The goal INR range for surgery is below 1.2 so our hope is by stopping Coumadin 72 hrs prior to surgery this level will fall to the 'magic' (low enough) range where we don't need to give Plasma replacement again (we had to do the 2 bags last time right before surgery to bring it down enough).

I talked to Dr.Bragg's Secretary and NP several times today regarding next weeks surgery and pre-admit, the CT scan, INR, etc. There was initially some confusion which day I was to be admitted (Monday ahead of Tues's surgery or Tues the day of surgery) but this was easily enough sorted out and just a few phone calls between them and myself and their having to get ahold of Dr.Bragg who is on vacation till I believe Monday.
Dr.Bragg had mentioned to me while I was still admitted a couple wks ago that she would want a pre-admit the day before for the INR management and in order to get a Stealth CT scan (used for a guidance system in VP shunt/brain surgery) but I guess hadn't left this in her notes. All was confirmed and worked out though and i'll be admitted at 1:00 on Monday. I'll go to admission, from which i'll go up to my assigned room on D6-4 and then all in-putted orders will be processed (CT Scan, Port access/INR draw) and the Aldurazyme is in the process of being worked out between the floor Pharmacists and Dr.Bragg's team. They are fantastic help!

The weather here has been CRAZY cold! My Dad had to come this morning to help me get my car started which he was easily able to do (first try, lol) but it turns out he just went with me anyways and went shopping most of the time I was infusing and then came and spent the last of the 4.5 hrs hanging out/talking while my Nurse and I played Yahtzee. I was able to cancel what I have for Tues and although I have things Weds/Thurs these days are supposed to start to warm up. I get stir crazy being home all day but I guess I can take one day of hanging around my Apt in my PJ's catching up on stuff. :)
Otherwise I just need to do labs later this week and a Pain Mngmt fup on Thurs (Milw.) I think next Monday Joan, (came with me when I was sent by Dr.Bragg to the ER at UW for the exposed shunt) is coming with me to Madison; our tentative plan is to get lunch before I am admitted. I enjoy spending time with Joan so am happy she'll be coming with. :)

Yesterday I had Church book club after our Sunday School 'Group' Class and after Church. The book club finished the book they had been reading and are starting a new one (by Max Lucado). This Thurs after going to Milw. one of our Pastor's asked myself and the 2 others I co-run our Young Adult committee with if we could/would come over to his house for supper (after he is done with Church) and to go over our Young Adult Committee plans. I don't think i'll be back in time for Church but plan to meet everyone at his house and then just go to Church per normal on Sun after Sunday School. One of the others on the Committee is coming from work (Connie and I teach Sun School and typically sit together on Sunday's in Church) so she also is coming after the Church service and meeting at Pastor's house.
Friday I am mtng with 2 of the Young Adults that where at our meeting last Sunday (for the committee) and who've offered to help set up a fb page for our St.John's Young Adult outreach efforts. Both of these things should be good and interesting.

I don't think I wrote about it in my last post but in Febr there is a big meeting related to all of the Lysosomal disorders (the disease category my diagnosis, MPS I falls under) being held in San Diego and I am attending on behalf of Gene Spotlight, the Foundation I work for. I'll both be there to attend meetings/sessions and to work at the GSF booth regarding GSF's outreach/funding of research efforts. I am really excited as it has been 4 or 5 years since I've been to a WORLD meeting! That was last held (when I was last there) in Las Vegas! I am very grateful for these experiences and to get to see Allison and Chip again who have become good friends. :)
The week after that I am tentatively scheduled to fly to Mn to catch up on the longitudinal cognitive study for MPS I (neuropsych testing and fMRI (for which they have a Neurosurgeon (who knows my Neurosurgeons AND highly recommends my Team!) who will reset both my shunts post-the MRI. I believe I may also be doing a Endocrine related study then.

I am sure there are somethings I am forgetting but I can't think of much else in which case I will sign off and update when or if I have anything else to add. Otherwise I will update again either after being admitted Monday or sometime after surgery on Tues.
Thanks for stopping by,

Wednesday, January 1, 2014

Happy 2014!

I did get released Sunday early afternoon which was nice and home in time to run to the store, from there I had time to stop at my Apt. Although I might have been a little crazy to do so given how tired I was then went to a Church Committee meeting which we where holding at one of the Restaurants here in town.

Today (written Mon) as much as I'd have liked to sleep in I went to ERT (Infusion) at CHW in Milw. and was atleast able to get caught up on most phone calls and paperwork. I guess I had noticed it already a little last night but I was retaining fluid quite a bit worse this morning, socks and pants that normally are loose and comfortable I couldn't even fasten or get on. I had one pair of slip on shoes I could wear-on one of the coldest days of the year! Per Dr.Bragg and what Cardiology had told her I had to call Cardiology regarding INR mngmt and so let them know about the fluid retention. The initial Nurse I talked to wasn't one of my normal Team's so she just took down the info (she was familiar with my case) and said to let them know if it got worse. My Infusion Nurse saw the fluid retention later and told me I should call them back and she was concerned in which case I called my Cardiologist's Nurse's # and talked to her. She told me a few things to do and not do and said she would plan to call me in the morning to see if it was any improvement, would alert my Cardiologist and if it hadn't improved or got worse I should plan to see them on Thurs when I am already at CHW for Labs. - I am hoping the fluid overload (more retention I guess since we didn't use that much fluid when I was in-pt) just improves on it's own! I already pee a freakin enough and not really wanting to take more diuretic than I do already! The Nurses did all say they would likely have the Holter monitor results on Thurs to (they thought maybe Dr.Earing would decide what if anything he wanted to do differently).
Dec 31, 2013
Jane, Dr.Earing's Nurse did call today and bc fluid seems a little better she just said I should wear the ted stockings (compression stockings) and if this got any worse page Dr.Earing who is on-call on New Year's Day. Otherwise they will call me with the INR results after I go to the Lab (at their hospital) Thurs and will fup on how symptoms are. She mentioned again they would hopefully have results on the Holter and an idea on the heart rate then. I really think it is just overly high which isn't un-common after heart surgery but Dr.Earing had also said he didn't want this to be occurring for long periods of time which it does tend to do (a mix of long and shorter periods of rapid heart rate). Anyways we'll see I guess.

The (shunt) head incision site continues to be a pain, literally. I am just simply amazed by how uncomfortable this area has been since surgery! - Adding to this post today (Tues), New Year Eve (wow!) I can say the site has been a little more comfortable, isn't as uncomfortable thankfully. The big things that make it painful include washing it (barely touching the areas around the incision!), dressing/un-dressing, simple things like the wind outside and as well simple things like bending down or exaggerated facial movements. So odd and so different than most other head surgeries. As I think I wrote in my post-surgery update though Dr.Bragg wasn't surprised by this and said given what she had to do it didn't surprise her.
I hadn't thought of it till now, but with Dr.Bragg doing the Thoracic shunt surgery revision as well as the VP shunt surgery on the 14th I wonder if she is still planning to use a Peds General Surgeon? I guess I will find out closer to then! Not really something that matters much either way to those of you reading this either I am sure! =)
I did not hear back from the Nurse (her partners Nurse) regarding the Fup Dr.Bragg wanted later this week so i'll have to call her Secretary on Thurs and see if this can be done on Fri as Thurs I have to go to Milw for Labs and other things. I might also see about temporarily starting back to Cardiac Rehab as they had said to just let them know when I was out of the hospital and felt up to coming back; I had figured I would maybe be able come back temporarily for a week or two between this last incision-closure surgery and the actual 2 shunts being revised surgery the 14th. I'll see how I feel Thurs though and then talk to the clinic.
I also emailed with Dr.Bragg's one NP Sue and she is going to check in to my receiving the Aldurazyme infusion when I am re-admitted the 13th for surgery on the 14th. Since I normally get infused on Monday's I would otherwise miss that week. She is out of the office till next Monday so said she would talk to Pharmacy then and get back to me with what they have to say. I am glad Dr.Bragg's office is always so willing to atleast try to get this figured out, chances are if we can't get it for that Monday they will order it for say Weds of that week.
Otherwise it seems hard to believe as I literally feel like I was just writing this type of post a year ago but it's a New Year (16minutes in to be exact at this moment!) and I can't help but wonder what this year will bring? Clearly one shunt surgery and I am going to the World-Lysosomal Meeting in San Diego (Febr) to help the Foundation I work with (Awesome!!!), as well as partnering w Jessi Colund of Genzyme for the Boston Marathon again but other than that I am not venturing any guesses (on med stuff) as I made that mistake last year and although I am not superstitious I cant help but feel like sometimes maybe thinking one way means the opposite is more likely to occur (ie I honestly thought I'd con't to be fine from a heart stand-pt, I don't think I thought id have that much issue w shunts although in general we did find some answers on that front) and just overall many things I wondered about at this time last year where the opposite!
In any case this is what I wrote/shared on facebook:
I remember writing last year that I thought 2013 couldn't hardly trump 2012 (as far as medical stuff) which this yr pretty much did.. (Hard to believe) so instead this year I am just going to say:
1. I hope to Con't to have my Faith.
2. I hope I don't ever take for granted the Providers I have; that the good ones con't to help w my Care.
3. I'll con't to learn to accept the things and people I can't change.
4. Adversely that the Family and Friends who mean so much know despite my sometimes opinionated self they mean the world to me! The Family and Friends who support me in all the surgeries can't possibly know how much that means!

Thanks for stopping by,
Happy New Year!