Home sweet Home (Mon)

I got out Monday, haven't really updated much throughout this surgery but it went ok minus the need for the 2nd un-expected revision/surgery Thurs (2 days after the 1st).

I had rescheduled my weekly ERT Infusion to today (Tues) which was the only day the Infusion center had open. As it turns out I got about 1/2 way there, stopped to get a soda (have to have something to look fwd to on the drive ) and my car seemed to be overheating. I love my car but it sure has it's issues! Thankfully after letting it sit for about 30mins and talking to my Dad and Brother separately both felt as long as no dash lights where on/came on and as long as the thermostat (temp gauge I guess) stayed below 1/2 they both thought it would be ok to drive home. So much for infusion I guess? Thankfully though my car was fine, I went over to my brothers (Beaver Dam, about 15mins from where I live, 35ish mins from Hartford) and he looked at it and thinks maybe water just got up in the engine on a certain part (can't remember what he said exactly) but it was raining some this morning so is possible. Needless to say I am just glad nothing seems to be wrong - believe me I am praying it stays that way! Definite perks of having a Dad and Brother who are mechanically inclined but I'd still like to avoid buying new car parts!
Yesterday when I talked to Dr.Bragg before getting out we talked some about this recent surgeries and then about f/up and timing to put back in the 2nd shunt. I see her Monday so am not entirely sure when she would be thinking but I get the impression per what she said sometime in the next few wks after Monday's Appt.
I definitely do not really want to go through another surgery and especially so soon but for the sake of feeling better headache wise may be worth it. In this surgery we'll replace the VP Shunt we had taken out in May's infection/surgeries and I think the plan is to use the same Codman Hakim valve. I am hopeful we can put the regular size one in, which I know is weird, why wouldn't I want the smaller one but it just seemed to work better than the smaller profile one we have in the LP Shunt. Either way will be nice to feel better after that is done!
I knew going in last week I likely wasn't going to leave feeling to great, albeit I WILL say the back discomfort/nerve discomfort is like 50-75% better which is AWESOME! My former Infusion Nurse/Access Team Nurse who drew my INR last week (day prior to surgery) said to me something to the effect of "You know your going in and still likely going to leave not feeling really good" and that was a little unnerving (the thought not her comment) but I also knew there was the good chance Dr.Bragg was open to putting back in the VP Shunt in the not that distant future after last wks surgery.
That thought/hope made it seem more acceptable (which I know is likely an odd thing to say), basically it came down to there was hope for feeling better down the road a bit. It was like the "consistently shitty" was going to be helped and that is hope enough. =)

It isn't horrible and if I had to guess is just when the shunt is draining/working to drain but there is still the very odd almost sharp twinge in the LP Shunt side but all things considered, while annoying it isn't the worst I've dealt w. The side and back incisions do get puffy as CSF seems to accumulate so perhaps it's related to that.

As far as setting goes in last weeks surgery she did set the LP Shunt valve at the bottom (drains the most, setting of 30) which I kind of thought she might do but had forgotten to ask till yesterday.
EP (Heart Rhythm) Appt f/up -
Next wk I see Dr.Kovach, the EP (Heart Rhythm) dr., to review the (approx.) 1 mo point of being on Ivabradine. The thought was if heart rates weren't consistently below a certain bench mark they would double the dose.
Heart rates have been more up than down but atleast they are some improved I think. =) I think the best thing of the med is sitting down I don't get the 'hard to get enough air' feeling as much although stairs often seem harder these days. Hopefully upping the med if that's what my Team decides to do will help that.

Not a lot else going on medical wise which is ok. I see my Pain Mngmt dr. tmrw but nothing to big there, just has been a few mo's and that's when the app worked out for.

Stay tuned :)

Erica

Shunt revision #40-or? 2nd un-expected revision 2 days later.

Tues surgery went well, another quick one lasting around 1- 1.5hrs. This time a 5:30 arrival and 7:30 surgery time. I am not completely sure but I think the Anesthesia dr. was a new one to my Team. Either way he listened to what Dr.Bragg suggested and all went off without a hitch it seems.
They used an LMA for airway access which is something recommended in MPS pts when possible, due to our severe airway issues. I've heard more times than I can shake a stick at that "Your airway was a lot more severe than I really thought from looking at you, you need to emphasize how severe it is to new airway drs. So needless I get a little nervous w Anesthesia drs that seem a little cocky!
That said, initially this dr. said to me and the Resident working with him "I don't see how her airway could be that bad" and something like "it doesn't look like we would have to use any extra equipment". I commented to Dr.Bragg to please, please make sure he wasn't going to try anything new. These are the moments I am sooo grateful Dr.Bragg is so up on my airway issues and what works!
In this surgery Dr.Bragg was just taking out the current fixed pressure valve and exchanging it with a programmable valve we'd used before. Instead of the regular size Codman Hakim valve we've previously used there must be a new(er) version of the Codman Hakim programmable valve that is smaller in profile which is what Dr.Bragg placed. Although headaches where not as good or atleast I didn't think so as we've had with the regular size valve surgery did seem to go fine.
I honestly don't know when symptoms started up so bad but I think it may have been Weds I started feeling really, really bad again, I think throwing up, nausea and severe headaches. Because of this other tests where done Weds (Xray) and Thurs morning a shunt tap was done by Dr.Bragg. I do sort of remembering telling the Nurse Weds night I wasn't feeling good and thought something was wrong. She must have talked to the Resident as I remember that dr. came by and said he'd talked to Dr.Bragg, she wanted to wait till the next day to do a shunt tap which she did do herself Thurs.
Either way I am VERY glad that this issue w the shunt was found fairly quickly (Dr.Bragg hadn't wanted the Resident to tap the shunt Weds night so when she came in Thurs morning she did a shunt tap right away) this along with the prior Xrays showed the catheter was kinked and I think the valve was flipped?
honestly I can't remember for sure which occurred 1st of the tests but this led us to a 2nd surgery Thurs afternoon and fairly quick answers to why I was feeling so much worse vs better after the 1st revision.
In the shunt tap that Dr.Bragg she was able to get 30ml of CSF (spinal fluid) off in a relatively short period. This amount of fluid is really unusual for us as a normal tap Dr.Bragg gets from 8-10ml per tap off. I don't remember for sure but think that atleast gave a little relief as it was sorted with what was going on.
The Neuro floor Pharmacists had ordered my weekly Enzyme Replacement drug so this was given Thurs. The floor Pharmacists and my Nurse ran the Enzyme Replacement IV Infusion which was good; a Nurse of mine who has ran it before was my Nurse for that day.
Dr.Bragg said the Radiologist had initially read the Xray as normal but once she looked at it she could see the problem. They booked an OR for an 'e-case' which turned out to be Thurs early evening.
I think we where about 1/2-3/4 of the way through the drug being infused when the OR called and I had to go down to pre-op.
Somewhat surprisingly my Team and the OR folks (Anesthesia, which was the same one as Tues) didn't ask that the ERT Infusion be stopped apparently so this ran during everything they did. I am not sure how that works with giving drugs for surgery during the infusion, (perhaps they stopped it temporarily) though the Nurses made it sound like they just let it go and once I was back to the floor my Nurses adjusted the final part. Not entirely sure. In any case just glad I had gotten it!
I should get released tomorrow, Monday from here which is good albeit I don't know if i'll be able to get my Infusion this wk, will have to call FMLH and see if there's by any chance any openings. Mine runs over a fairly long period so is not to easy to get an infusion spot the same week.
I don't feel great, I am hoping this resolves atleast a little in the next weeks while we sort when to place the 2nd shunt back. I am glad atleast Dr.Bragg is open to putting the shunt back in in a few wks but hoping between now and then the 'above and beyond' symptoms I have will resolve or calm a little! I am really hoping my body will just adapt to this smaller profile valve and work like the regular sized Codman Hakim valve did..
I had also switched my Infusion next week to Weds thinking timing for when I got out of the hospital original would fall in place to f/up next Monday but now I am unsure if that will work either! I know I see Dr.Kovach the 9th in place of the f/up I was supposed to do last Thurs so I am hoping it works out I can keep my Infusion to next Weds which I changed it to and see Dr.Kovach that Thurs (next Thurs, 9th) and hopefully follow up with Dr.Bragg this coming Monday. We'll see, right? It is amazing how 1 little extra surgery can change the best laid plans!
Needless it was a bit of a odd week, between the shunt revision and sudden 2nd revision on Thurs. On top of this all occurring last wk my Port worked fine on Monday when I got labs but then when we went to run fluids through it for surgery the Port wasn't flushing; turns out we where dealing with a clogged Port during Tues surgery and in to Weds where we got TPA (Altyplase) after numerous tries to open up and un-clog the cathethers. This has worked fine since then.
Thankfully by Weds night I believe it was we had the Port flowing again and could switch the Peripheral IV back to running through the Port. Always a 'Yippee' moment, honestly!
It has been a hard week symptom wise, I honestly am just beginning to feel like we don't have luck with the smaller shunt valves as this Codman Hakim is a mini version of the regular size. I am hopeful my body may adjust or when we put the 2nd shunt back that will make the difference.
On the note of the 2nd shunt I am not sure if my Mom asked Dr.Bragg about it or how the conversation came about but they did talk about it and Dr.Bragg figured (from my having talked to her to, the next day) we'll likely re-replace the 2nd shunt (VP location) in a few wks. She just said something like she'd want to give my body a little time, get through this stay and f/up with her in clinic and we could talk about timing then. It seems she is thinking give it a few weeks before we did anything.
Honestly I just cant wait for it to be done but I also get I think giving my body a break, it is all the longer the new heart med will have to work and we'll have an idea how my body does with this surger(ies). I am mostly just relieved to know she's going to put it back in! And to even though I don't think this mini-Hakim valve works as well as the regular size 1 I can atleast say the back discomfort/nerve discomfort is much improved from a week ago! I do have some unusual side pinching type pain (ouch!) that is intermittent and these weird sort of swimmy/light headed like episodes. These occur mainly with certain things and hopefully will resolve to.
There are a few little things I need to talk to others of my Providers about but overall I think we're on a good path and so, so thankful as always to Dr.Bragg!
Will try to update soon,
Thanks for stopping by,
Erica
BTW: Sorry if this may be a little all over the place, not enough sleep does that. =)

My prayer, my thoughts, my uncertainties.. Dr.Bragg took fluid off, headed to Utah

A good article, the last 1/2 of which describes somewhat how I've felt today..
"When life is shitty, Show up for Yourself" - it's a good, poignant article
http://www.elephantjournal.com/2015/09/when-life-is-shtty-show-up-for-yourself/?utm_content=buffer8816f&utm_medium=social&utm_source=facebook.com&utm_campaign=buffer
Saw Dr.Bragg this morning (to have shunt tapped, fluid removed before I go to Utah), it was only just as I was sitting at my desk a bit ago to quickly respond to another drs. office that I realized what I forgot to ask Dr.Bragg this morning. I swear it doesn't matter if I put reminders on my phone to myself to ask, I STILL forget to ask quest ions!
In this case my Genetics dr. at BCH in Boston has been trying to connect with Dr.Bragg and apparently not having luck (he was/is wanting to see if he can work with her to get a sample of my CSF to test the MPS (gag) levels in the CSF.
Given we pretty routinely draw off CSF I cant see why this would be an issue but I'm not sure how to connect these two and admittedly a little uncertain why it has been so difficult. It sounds like he (his Secretary) has talked to Dr.Bragg's Secretary (who is great) several times and I know they have each others email info.
Unsure!? I do think it is information/a test that could be helpful overall so I wouldn't mind his getting a sample to be able to test...
Anyone have thoughts? I absolutely get their schedules are busy, I just am a little uncertain as Dr.Bodamer asked me if I might know how he could get ahold of her/could I ask her the best way..
I am not to big a fan of playing Secretary given I already feel like I spend hours and hours of my own care coordinating, doing insur. (which isn't bad), pharmacy stuff, paperwork, paperwork sortment/getting various records (I get copies) sent to appropriate Providers, etc..
Will keep working at it and try to remember to ask her next wk when I see her for surgery. Dr.Bodamer has talked with Dr.Earing, my Cardiologist so I know it can happen and they can all help each other..
On that note, I did obviously see her this morning, mornings are not at all my thing but I'd gotten up several hours before I had to leave as the longer I am up the better the headaches get (never thinking to look at my phone messages/email, I rarely ever look at anything besides to check if anyone has called while in the shower). - Thankfully was just a short msg from her and the appt was still on, I initially had a little 'oh shit' moment thinking perhaps it had been cancelled.
Anyways I should have but I didn't ask her what her thoughts are re: if she's continuing to consider replacing the 2nd shunt, timing for that if she is, etc. I really wanted to (in the worst possible way, actually) but I hesitate to keep asking her as I don't want her to get annoyed! Sometimes I am my own worst enemy when it comes to bringing things up..
She's really great about answering questions I have but that still doesn't mean I want to ask her or any Provider the same thing over and over and over again... Believe me you don't even know how bad I wish we could just replace that stupid 2nd shunt. I am SOOOOOOOO sick of feeling like shit 1/2 the day!
I know our replacing the LP Shunt valve will help that shunt drain more consistently but I also know it isn't going to be enough, as it wasn't enough after only the LP Shunt was placed in May.

Whether right or wrong just about every day in my prayers I pray and ask God if he can give me wisdom on this issue and patience to con't to deal with this as sometimes (not to sound whiny) it is really, really hard. I pray he can guide us and above all if it be his wish we can replace that shunt. I absolutely don't want more surgery but I DO want to feel atleast better. =/
When I've woken up for the 3rd, 4th or 5th (sometimes more) time at night, days I have to get up early despite and even though I know it wouldn't likely be perfect I know without a day it would be a lot better than it is now. Being perfectly honest, probably b/c I am so over tired I am sitting her now in tears typing this. I am not a crier and try not to whine but when tired and frustrated it gets to me.
I cant say why but it has been a struggle the past week especially as I feel worse headache and symptom wise (despite the shunt is working) and yet I know I look very fine to people around me. I know I have an abitlity when I do feel bad to smile and laugh and in all honestly although I doubt people realize it I am quieter.
I listen more so than I participate in those times I am tired and my body aches, (low back? Holy hell it feels like it's on fire or something when I walk especially) and my head hurts though that atleast gets better throughout the day whereas the other stuff doesn't as much.
I apologize if I sound like I am whining? Maybe I am =) / =/ Sorry, I think it's because I am going to Utah tmrw, to the National MPS Conf. and although it will likely be fun once there I am not really excited now. It just sounds daunting and tiring and to be perfectly honest I just want it to be a week from now so atleast I can feel a tad better, more consistently? My ultimate wish would be to have that 2nd shunt back but in that regard i'll keep praying.

4yrs ago, 1st VP Shunt (pre-2 working shunts, LP Shunt was tied off)

As far as the CSF (spinal fluid, what drains through the shunt, the source of my headache grief) Dr.Bragg pulled some off again this morning as I mentioned above and opted to send a sample to be tested "just to be safe" before next wk's surgery.
I doubt there's anything wrong with it, infection wise (but I am good with cautious).
She commented the fluid had some blood tinge to it though I don't think that's a huge issue.(it doesn't surprise me, the blood tinge, I can feel when she is pulling fluid off), it's not very comfortable as it's being removed (but to me not something that would make me kick and scream albeit I guess that does take a lot for me to complain) but at the same time worth the short term symptom relief in headaches and amazingly my appetite is pretty quick to come back for short term! That is always awesome!

Med wise for surgery I stopped the Baby Aspirin already and stop the Coumadin after Thurs. (5 days before). Then i'll re-test INR Monday at CHW prior to my ERT Infusion next door at FMLH and that result gets sent to Dr.Bragg's office.

Traci, one of my favorite Nurses from D6-4 (Neurosurgery at UW) sent me this pic earlier today. She is at a Conf. in IA for Neurosurgery Nurses and on the flyer saw Genzyme has a informational booth! WHOOHOO they are finally reaching out to other Specialties (last yr one of my other former Infusion Nurses Son's sent her a pic he'd seen my pic at Genzyme's table at a Opthalmology (eye) Conf he was at).
This is especially exciting for Neuro though as atleast for MPS I these issues are so misunderstood and not well treated. I thankfully, FINALLY by the grace of God found Dr.Bragg but for yrs prior to her had issues w/my shunt that no one would touch after my 1st Neurosurgeon abandoned ship. In my case as I suspect is more common than realized the Hydro is suspected to have been un-diagnosed for many years since symptoms began as a kid.
So good to see this kind of outreach! Traci was going to stop at the table and check it out. =)


In any case I fly out of Madison early tmrw afternoon for Utah (a connecting flight) but if nothing else the Foundation I work for has it booked in 1st class (can't hardly complain about that). =)
The week is filled w/sessions, meetings, a Walk/run I agreed to volunteer at Fri night and then I fly home Sun.
I then turn around go to ERT Monday and surgery if nothing changes (I think I may cry if anything does!) on Tues.
Thank for stopping by,
Erica

Dr.Bragg, Pre-op Appt., upcoming MPS Conf. (Utah)

I saw Dr.Bragg Thurs, as usual a good appt, we didn't really talk a lot about anything shunt related other than she double checked I'd set up another appt to have her take off fluid before I go out of town and made sure I had scheduled the shunt valve revision with her Secretary.
Dr.Bragg mentioned she had run in to the Anesthesiologist who did my last surgery with her so she gave him a heads up about this next revision the 22nd that he should try to get the case, I am not sure how that works at UW but at CHW Patients or Providers can request specific Anesthesia drs.
At CHW for instance i've always worked with Dr.Taylor on my Cardiac procedures/surgeries, either myself or my Cardiology Team (or both of us) would get in touch with her to request her on my cases. It sounded like he might be on the case per Dr.Bragg though which is a good thing, he was reasonable about the IV pre-surgery steroid dosing and didn't seem to go to wonky about the cardiac issues. I can totally understand (I think) their being more cautious with longer shunt revisions, like if we where to put back in the 2nd shunt say using more monitoring (prior to/leading up to the AVR surgeries with shunt revisions many of the Anesthesia drs would place Arterial lines and I believe something similar was done with the Heart Cath/EP Study this past July but for a surgery as short as a shunt valve revision it seems sort of silly. Dr.V did the normal monitoring but nothing to extreme the last time a few wks ago.
In seeing my Primary dr (Internal Medicine) today for the pre-op Appt she was asking how the steroid dosing and surgery went with the surgery a few wks ago which in turn I told her what Dr.Bragg has said about the Anesthesia dr. and that last surgery it helped having her notes re steroid dosing timing. I just made the comment that Dr.VanDyke (sp) didn't seem to get all crazy or overboard with the heart issues. Dr.Simpson) commented along the lines 'You really do have severe heart issues and are very high risk, if you where any other patient I would worry a lot more about your undergoing anesthesia and closer monitoring even with shorter surgeries but you see your Heart Team often, they are 'ok' with these surgeries and you go through these surgeries all the time'. - A somewhat funny, sobering, life of an MPS Pt. conversation, that's for sure! I probably shouldn't have laughed at the conversation we had around this but I did. =)
As far as anything else while it is not this low the majority of the time Heart rate today at the appt was 76, whoohoo! =) I'll take it! I've not noticed significant improvement in symptoms at night, nor with stairs (I generally, thankfully don't have to do a lot other than walking up the 1 flight of stairs in my Apt building to do wash or occasionally at my Parents when I go down to feed my Salamander there (I keep him there or otherwise my cats would probably try to eat him!).
With this cooler, less humid weather the past few days that helps some during the day to. Hopefully the med in addition to working more consistently on heart rate will help symptoms to in a bit more time!?! I do know my Cardiologist and Heart Rhythm dr. both said they would try upping the dose (double it) if need be at the next Appt so either way hope it helps! As I think I wrote about before, atleast the med doesn't have side effects it seems!
I have been emailing back and forth a bit with my Genetics dr. in Boston re the gene mapping testing and talked to my Primary dr also about this. A big part of why my Cardiologist was interested in the testing as was the Genetics dr. was to see if there may be an underlying, un-diagnosed connective tissue diagnosis and could that explain the prior difficulties with the 1st failed (very quickly) heart surgery and why the heart issues have continued to progress so quickly. If there is another issue could that help them plan for when we do the 3rd OHS I guess was part of the thinking.
Part of their thinking was my mutations are in the moderate-Hurler Scheie range but my features are more milder appearing and yet I have some of the most significant heart issues this Genetics Team has said they've seen in addition to the Hydrocephalus difficulties which are not altogether that common in MPS I pts. (some have it but not most and most do not have the difficulties we do albeit in my case the Hydrocephalus likely having been present since I was very young (symptoms started around 1st grade) and not having been diagnosed do make our treating it/the pressure far more difficult.
In any case my Genetics dr. emailed me to ask if I could ask Dr.Bragg how he could best get in touch with her and my Primary dr. is going to try and get in touch with Dr.Bodamer to see if she can help facilitate ordering the gene mapping test. In the case of Dr.Bodamer wanting to talk to Dr.Bragg he is interested in getting a sample of CSF (Spinal fluid) to test the Gag (MPS) levels in the spinal fluid. I think they have been having problems connecting whereas he and Dr.Earing where able to chat back in May/June so hopefully we can figure this out and they can talk.
Dr.Bragg's Secretary had called yesterday (Weds) to ask if I could have my Cardiology Team send them basically an 'ok to have surgery' from my Cardiology Team which is easy enough, usually my Cardiologist Team will just (I think) fax the most recent clinic notes and/or an letter saying they are fine with surgery. I had the pre-op for this surgery today, Fri., with my Primary dr.
On a different note, I knew the Cardiologist who is probably most familiar w MPS I pts/heart issues is speaking at our National MPS Conf. this coming week so I emailed her to ask her a question about the OHS. She in turn emailed me back this morning, she is including in her talk at the Conf. Adult issues with heart issues and MPS (Hallelujah, anyone who has been to these family conferences knows that adult topics rarely ever happen, sadly - very sad actually as there are ALOT of individuals living a lot longer w MPS these days! Anyways she also told me she has not known of anyone with MPS who has been through 3 OHS (open heart surgeries). That is a bit sobering! Something we'll worry about when needed, I think my Heart Team stresses about it enough already thus trying alternate options to buy time.
In any case, I see Dr.Bragg Tues, she'll pull off more fluid to give short term relief. I wish I could package her up and bring her to the Conf. with me! I am hopeful at minimum her pulling off fluid will help with the flight to SLC to the Conf!? If I am remember I am going to ask her if she knows of anything I could try that might calm my lower back nerves, I wasn't able to coord. with my Pain Mngmt dr (her Seretary is IMPOSSIBLE!) to see her or I'd have asked Dr.B.
This wknd we have a fundraiser for Church to help the local fire dept., my Sister, her husband and I are going out to the local raceway and Sun is our 1st day of Sunday School. Next week after ERT Monday and Dr.Bragg Tues, I leave for the Conf. Weds. I do have a couple mtngs set up there and agreed to volunteer Fri night at a Walk/Run being held for the Society. A good friend of mine is the Race Director so for her i'll help, she's awesome. =) In any case will update sometime soon,
Thanks for stopping by,
Erica

Neurosurgery Appt, We have a plan (#37, I think?) coming up

To continue a long week of Appts., I saw Dr.Bragg yesterday (Thurs, 3rd). The Appt went well I think it'd have to say.. these appts usually do, she is so down to earth, explains things using her funny analogies (which I fully think everyone should have to hear atleast one, they always make me smile) and she just seems to get that albeit I like her, I don't really want to be there asking for her help (if that makes sense)..
I think I wrote about this in my last post but she always asks about the heart stuff, honestly I think maybe it makes her a little nervous (which there's nothing wrong with that if it is the case), better a cautious, thoughtful Provider then 1 who thinks only of their particular treating issue OR just plain doesn't care about the whole picture!
We talked about the current shunt valve and the irritation this is causing + how the Codman Hakim valve even though it is imperfect as far as draining wise it is the best I feel like as far as shunt valves we've tried and consistent drainage vs this current valve seems very inconsistent and a lot of spine/nerve irritation.. That may not make a lot of sense but while it doesn't drain enough CSF (spinal fluid) when we've had that valve in the various shunts it seems to drain pretty constantly so atleast there isn't the nerve irritation and isn't intermittent issue..
I am unsure how exactly to describe what it is about the Codman Hakim valve that the other 3 or 4 or so valves we've tried over the past 4 yrs including the 1 I currently have in place on my shunt haven't done well.
She had looked in to see if there was a Codman valve (this is the brand, not a type of shunt, for instance Nike is a brand, 'Air Jordan' is the product.. if that makes sense?) that drains lower than the current valve I have but was unable to fine one. Not surprising I guess, and we did talk about would that have caused worse irritation to spine nerves, but is hard to know.
We've decided initially to take out this current valve given it is causing so much issue and to re-replace a Codman Hakim valve in the LP Shunt.
This will occur Sept 22nd, in the interim b/c I have a few things going on and wanted to try and postpone the surgery(Sun School starts a wk from Sun, Conf. in Utah 2wks from this past Weds) Dr.Bragg is going to do shunt taps to take off CSF next Thurs and the following Tues, before I fly out to Utah on Weds. I am beyond grateful to her she will do these taps! While the relief is short term headache wise the other symptoms seem to last longer (it is weird), a few days sometimes and it is awesome! It makes dealing with the headaches more tolerable.
It never ceases to amaze me but Dr.Bragg had told me when we where walking out of the Appt together if I wanted I could go ahead and call her Secretary that afternoon to get the surgery scheduled (her and I had settled on a general timeframe after looking at our respective calendars) as well as schedule the appts to have her tap the LP Shunt and take off fluid in the interim. She said something to me about "if you don't mind the drive i'll be happy to take off fluid, I know it gives you relief for a short while' (my wording) which albeit sure the drive is a bit of a pain BUT to feel better it is worth it!
I was able to talk to her Secretary that same afternoon of the Appt and Anna without even having seen the inbox message Dr.Bragg was going to send her yet scheduled the 2 appts (next Thurs and the following Tues) and scheduled the surgery (2 wks from this coming Tues) which amazes me but this is what makes Dr.Bragg's Team kind of amazing, they all communicate really well, Anna trusts if I am giving her info then it is what Dr.Bragg has ok'd to me and same goes when I am talking to Dr.Bragg's Nurse for that matter.
I definitely was relieved to have that all scheduled and sorted before the long wknd which also meant in turn I could schedule the pre-op and sort that with other things I have going on next wk. Given I have the Conf. the following I needed to get the pre-op next wk and it did work out and with my own Primary dr. thankfully which makes that appt along easier and more smooth!
I did ask her about if she was against putting in the 2nd shunt after she and I talked about the LP Shunt and changing the LP Shunt valve 1st. She isn't entirely opposed to replacing the 2nd shunt, albeit if we do that she does not want to do it at the same time as the LP Shunt valve due to infection risk and I think she wanted to give the new heart med a bit more time to have it's hopeful affect on the heart before putting my body through a tougher surgery like a VP Shunt replacement/insertion surgery.
She commented how she didn't really want to put me through 2 surgeries but overall she would feel that was safer, which I can understand. It would suck but to feel better I'd probably jump through burning hoops, so...
It seems like as I mentioned above she is willing to put the 2nd shunt back, she made a comment along the lines about giving this shunt valve change once it is done some time, maybe do the 2nd shunt later in fall? I am not entirely sure on that. Atleast she seems fairly open to the idea? She asked me what my thoughts where re where a 2nd shunt would drain ie what where my thoughts on both shunts draining to the peritoneal cavity (abdomen kind of) giving we know heart surgery is going to happen at some pt...
Basically regarding heart surgery related to the above and any shunt stuff and just what my Heart Team's thinking is, how they kind of manage decisions regarding any of it, it is a 3-6mo at a time call, I see Dr.Earing every 3mo and Echo's are done every 6mo where we re-visit a 3rd Heart surgery and 'can we postpone it some more' each time..
I see my Heart Rhythm dr in-between those visits as well and he talks with Dr.E so all are on the same page.
Given the unfortunate knowledge of heart surgery in my future and given pleural spaces (area around the lungs) are needed during heart surgeries we know it wouldn't be ideal to put a 2nd shunt if we put it back in to drain to a pleural location. That said we agreed, should we put the 2nd shunt back, both would drain to same location in the peritoneal location.
Perhaps at some pt. further down the road, after a heart surgery where over with we could move 1 of the shunt distal ends to pleural space.. While it isn't ideal having both drain to same location at this point I'd take that and probable, potential to feel some better pressure wise!
And if we're lucky this new heart med will work for a good long while and we wont have to face heart surgery for awhile..! I am hoping, I think (I know) everyone on my Team is hoping for this!

On an un-related, related note I saw my Physical Therapist today, he was impressed w how little edema (fluid?) there was today in my face and joints - joints especially and felt like he was really able to make a impact today on getting my shoulders to move better (a significant source of discomfort for me). He has also commented how in general areas like my legs, face and upper body just seem much less "puffy" (I've lost around 4pds but I don't think it is this weight loss (unintentional) that affects the less puffiness he notices. Even the heart meds (that make me pee like a sive) don't seem to touch the puffiness he has commented on from time to time.
His counterstrain/myofascial work is AMAZING though! He is like a god send when it comes to helping the muscle/nerve pain and in turn I am able to take less pain meds. We've never found a good way to effectively help the headaches but that I truly think i more b/c it is from the storage issue/MPS.
I DO think the shunt being back in LP location even if it doesn't drain enough and the new heart med even if the heart med is not yet having a significant impact on heart rate/breathing is seem to be impacting this overall puffiness. I asked Tom about it and he thought both of these (shunt and new heart med) could definitely be why he's seeing this change. Whatever the cause, if it's helping him help me i'll take the improvement!
In any case, thanks for stopping by,
Stay tuned,
Erica

Cardiology F/up, post Heart Studies. Neurosurgery f/up Thurs.

Follow up today with Dr.Earing (Cardiologist) - both the usual reminders of what's ahead and all that's going on (as if I can forget, living it day to day) and some funny moments, good laughs I have to share. =)
Re the Mitral valve/heart stuff, per the Heart Cath/Heart Rhythm Study back in July and the Echo back in June we know the mitral valve is in the severe range for leaking/narrowing and we know pulmonary pressures (having to do with oxygen between the heart and lungs I believe) is elevated, (which isn't a good thing) and we know there is signs of heart failure despite all the meds we've tried.
We've been on the Ivabradine for 2wks now, with heart rate today at 92, per the Fellow (a graduated MD done with her Residency training to be an Adult Congenital Heart dr.) Obviously the heart rate isn't ideal as the goal of the Ivabradine is to bring heart down to I believe below 70.
This said, studies done in Europe show it can take up to a month to show significant improvements so we're all (Dr.Kovach, Dr.Earing and myself) kind of hoping for that.. No one on my Heart Team is ready to take the significant risk of a 3rd open heart surgery yet. As Dr.Kovach says "we're pulling things out of our pocket to try and help you, to try and avoid surgery as long as possible".
Dr.Earing just said again today what Dr.K said last wk, should HR not be consistently where they'd like or symptoms not be a lot improved they'd try doubling the Ivabradine dose (10mgs 2x's/day). Given it doesn't seem to have side effects (anyone who knows/takes heart meds knows this IS kind of AMAZING!) I would be ok with that, if needed. Better than rushing to OHS!
Dr.E's exact words (or close) regarding heart surgery, which might I add, ironically it is 2yrs ago Thurs when the 2nd re-do AVR/Konno procedure was done where: "It's bad but i'm not ready to push the button yet, we just wait as long as possible and hope this med works. You know I'm dragging my feet as long as I can on doing this".
Elmiron
Separately Dr.Earing brought up about the Elmiron, the drug my Metabolic Genetics dr. at Boston Children's had called him about. Dr.E is interested in the drug and thinks we should give it a try albeit he cont's to feel now is not a good time given the many other complicating issues going on we're sorting out (heart issues, shunt issues) and worries that would make an already complicated picture even more so. I don't disagree with him and am fine waiting, I don't think right now would be a great time to start it. The Ivabradine also appears to affect the Coumadin dosing so they've adjusted that dose upwards and i'll test again next wk.
Insurance (funny stuff)
I don't quite remember how we got to talking about Insur., other than maybe Dr.E, when he came in made a comment along the lines: "I don't know how you managed to get the Ivabradine covered but I think you need to come work for me, then when my other pts. can't get their stuff covered you can just do it for them, for me". - LOL!
I know I said to him, laughing, you don't really question how I got it covered do you? =) His response, something like, no, I know you, but it's still amazing and you do a great job on this".
Sometimes my stubborn, sometimes pushy and persistent nature comes in handy BUT it is nice that he realizes it's not simple stuff to deal w the insur. stuff and appreciates what it takes as a patient to be doing what Providers normally do. I don't mind, doing this sort of thing, it makes me feel like I am helping with my care but I really appreciate that Dr.E and Jake, my Pharmacist (Skywalk) are kind enough to want my help. =)
Dr.E made the additional comment "I don't know how you do it" as he knows exactly how difficult some of my Insur. company stuff can be, he has other patients w the same/similar Dean coverage. albeit in my case I work directly with a Nurse Case Manager and have in place 'out of network approvals for a yr at a time for Echo's and Appts with him. I do not believe that is the case with other of his pts. w the same insurer.
His Fellow said something along the line after that comment by Dr.E. about "I needing to work for him", "I need to take you with me". LOL, I honestly don't think anything I do re getting Prior Auth's in place for Dr.Earing, Dr.Bragg and some of my other Providers is all that special nor do I think working on the med stuff on my own to help them is anything special but I guess it's something few pts. must do? Must be part of having an independent, I want to get this taken care of streak? In any case.. =)

And on a last note, just as Dr.Bragg always asks me about the heart stuff, Dr.Earing always asks me about the shunt stuff. Before updating him he says: 'I know you just had something done, dumb it down for me, I'm not a Neurosurgeon, I know you know more about this stuff than I do". LOL, that made me laugh! I kind of doubt that's true but non-the-less, pretty funny =)

Afterwards he said to me as we (Dr.E, Fellow and myself) where figuring out the new Coumadin dosing and f/up timing (3mo Echo/Appt unless anything changes, Dr.Kovach keeps him updated and I see him the 24th) he says regarding the shunt stuff:
"I just wish this Neurosurgery stuff where easier for you". Obviously I do to, but most of all I so appreciate he doesn't question Dr.Bragg, he gets that she's doesn't make hasty decisions. He gets that it's complicated and he appreciates she hasn't given up or abandoned my care (he saw all the issues with the 1st Nrsgn and the subsequent issues finding 1 after that who would not only follow my care but treat me and 1 who would believe me/believe those of my drs. like him who did believe I was having a shunt/pressure issue before I found Dr.Bragg).

I absolutely know he is protective and at times questions some aspects of my overall care but I am not sure he has ever had reservations about Dr.Bragg despite how complex of times we've had. I appreciate that b/c I really like him. =)

Neurosurgery F/up, Dr.Bragg, Thurs.
On a side note, I am sitting here, having taken to much zofran already, my stomach is super on edge, in all honesty I have a feeling unless I take some ativan (yuck) it is going to end up with my throwing up which super sucks. I am trying really, really hard to ignore it. Ugh, I wish my stomach would calm down!
Anyways, I see Dr.Bragg Thurs., f/up from the surgery. I am a little nervous, not b/c of actually seeing Dr.Bragg but b/c I am hopeful she will have an idea how to help when I see her for f/up. I am still hopeful she'll agree to put back the 2nd shunt but I know I have to trust or listen to whatever she might propose or if she has a different thought.
I am trying to not get my hopes up but honestly I am so, so miserable the past week-ish I just want to feel better.

I honestly think the current valve works but I don't think it works as consistently as the Codman Hakim valve did, perhaps b/c it is smaller (?) and it seems to cause extreme nerve irritation in my mid-back + my low back has literally felt like the muscles are well on fire or something (the muscles down closest to my butt actually).
It is almost funny to me, the Codman Hakim valve didn't cause the nerve irritation and did atleast drain some fluid, not enough and seemed to work consistently, just again not enough.

This valve seems to work very inconsistently and when it does seems to help headaches some but causes the nerve irritation and low back stuff and well it just is a pain overall! Ugh! Out of any shunt valve we've ever tried I'd have to say the Codman Hakim's where the best even if not the best re how much drainage. To bad they cant make them so they drain just a bit more! I honestly just have wondered if that valve where in say the LP Shunt and another fixed valve where in a VP Shunt would it be really good or a lot closer to good? I think so but that's just me..
Anyways, from time to time I am getting what I can best describe as a 'zingy' sensation along the shunt tract so I feel like a bit of a mess! The pain between my shoulder blades makes things like showering, especially washing my hair which I do every morning harken back to the 1st LP Shunt I had. I am so hopeful maybe my Physical Therapist can help with this again but I do know without a doubt some of it has to do w the valve. It started some days after the new valve was placed and I think I just didn't notice it when in-pt as much b/c of sitting so much and I didn't shower everyday there. Who knows?
In any case the week cont's to be busy, I see the Neurology doc tmrw for botox (I'd have rescheduled given this week is ridiculous with Appts but had to reschedule in July and in Aug. and I think it could possibly help the muscles to. Tmrw night we have our last planning mtng before Sunday School starts in a couple wknds and then Dr.Bragg Thurs + PT (thankfully that is local) on Fri.
Thanks for stopping by, stay tuned,
Erica
I attached some screenshots from a heart rate app I have on my phone, this is not actually a significantly high heart rate for most people, it is high but not abnormally so I guess you'd say. In the case of myself per Dr.E b/c there is already the other heart meds including/especially the Digoxin (works on heart muscle, to help the heart relax) heart rates should not be as high as they are. Even rates in the 80s are uncomfortably high to him, in my situation due to the mitral valve mixed disease (stenosis/narrowing) and the increased pulmonary pressures/Heart failure.