Thursday, October 29, 2015

VP Shunt plan; 2wks to surgery. Trying an old approach..

I saw Dr.Bragg today (how many posts start with/like that?) which was fine, I learned some more info about the upcoming VP Shunt surgery-install.
I have been wondering if the LP Shunt is working optimally, I don't think it's not working just more that based off how i've felt I wonder if the LP Shunt is just not working as well as it should. I am not entirely sure but based off the CT Scan which Dr.Bragg pulled up today the ventricle size is normal (which is a good thing) and should help the VP Shunt placement be more smooth/precise placement but at the same time my ventricles are never not small except when there's a shunt issue? I definitely don't think it's that the LP Shunt isn't working b/c clearly I'd be a lot sicker and my headaches wouldn't just be intermittent but I think the ventricle size could explain why it seems like my headaches and symptoms are a lot worse? I am not entirely sure, just my feeling..

As far as surgery, my Primary dr. messaged earlier today via MyChart and had not heard back from either of my Cardiology drs. (Cardiologist and Heart Rhythm dr.) yet so she planned to call them again if she'd still not heard anything by the end of today. I honestly am not sure what Dr.Bragg would do if they said either no to surgery or wanted some extra precaution? I pray she'd talk to them to make it a go! Hopefully there just won't be an issue.
In the mean time at today's Appt while looking at the CT Scan results Dr.Bragg talked about what her plan was with this VP Shunt re-replacement. Last time we really talked in depth about this surgery and placement of the distal (draining) end we had agreed b/c of the likely potential an OHS is going to happen to put the 2nd shunt to also drain in to the peritoneum (where the LP Shunt drains).
Today she asked my opinion instead of tunneling this VP Shunt when she places it to drain to the pleural (area around lung) space. She commented if/when we do end up doing the Open heart surgery she realized we'd likely have to go back in and move the shunt distal end but she strongly felt we should maximize the best potential for the most success we could for now and have the 2 shunts drain to separate locations. I was and am happy she thought this and wants to place it in pleural space for now! For all we know a 3rd OHS could be a ways down the road, it seems unlikely given the way my 2 Heart drs talk at each appt. but I would rather have to go back in later and move the distal end temporarily and have better chance of success now with better chance for relief now.
B/c of all the previous pleural space surgeries/procedures and scar tissue she'll get General Surgery involved to do that part of the VP Shunt surgery.. I didn't think to ask her but I wonder how that works, does the shunt get tunneled from the skull, behind the ear, down the chest and then kind of around the side to the pleural space or is there a way they can tunnel it to pleural space from the front? I'll have to ask her at Monday's appt. I am kind of curious.

As far as what ventricle she is going to try to place the distal (top portion of the cathether) in in the brain she said b/c ventricle size is bigger than normal (normal size but bigger than my normal I guess?) she was going to try to place it at the back of my head in the lateral ventricle (I don't know if that's what that ventricle is actually called) as she felt she'd have better success this time then we've had in the past. She also said in prior surgeries they've used a magnetic guidance type system, it's something where there's some sort of sticker (or something) on the forehead and that with the computer I guess helps guide their placement of the shunt catheter?
Anyways instead this time she is going to use a system they use when removing brain tumors, it must be something like they use in c-spine surgeries where your head is held in place by a fixture so it gives her the best chance of best catheter placement within the ventricle (more precise placement, better chance of best outcome I think) while also minimizing/preventing any movement of head during surgery which can throw their computer guided system off.

In any case as long as no hold ups of which I'm still praying every day for there not to be surgery is 2 wks from today. I have to admit this sometimes seems like the surgery that's been a long time coming, a long wait though I am glad we have it scheduled and I know the plan. Dr.Bragg is so awesome about it all even about placing the shunt catheter in to the pleural space for now, knowing it may mean another surgery for that down the road.

I was talking to an MPS Mom just a while ago tonight asking if she might know where I could potentially find the 'MPS I recommended assesments/testing' sheet Genzyme published some yrs ago (it is an elusive fact sheet it seems!) I've asked multiple different Medical People in the MPS Community and at Genzyme and none are entirely aware where to find it but several are helping to try to locate it, so I can send to my Primary dr.
In any case this Mom and I where talking about this sheet and how it would be good to make it available to families again as the information is probably needed - it is important information to know (once I find it/get a copy i'll post it to fb for families) and then she commented something about my having a Team that works together and talks to each other and how she wished she had that for her girls (they are each early 20's), Providers that where willing to work together and communicate as needed.
Honestly I remember just exactly how bad it was in the beginning and all the advice many families gave me. I know how many Specialists I saw and didn't really connect with so I found a different one over time in that Specialty and sometimes it took several different ones before I found one I felt like was a good fit for my care/and team. I also know although it can be a pain how much time I spend driving between where I live and Madison and where I live and Milw. b/c 1/2 my Providers are in Madison including my PCP and the other 1/2 are in Milw. including my weekly ERT Infusion but with this set up my Providers actually communicate with each other.
My PCP does an amazing job talking to other of my Providers when needed and overall my care is really pretty good and especially compared to many in the MPS Community. I get comments frequently from other families on my care Team and how they wish they had Providers like I do. I don't know what the answer to that is but I can say I am grateful for the drs I have albeit it took a lot of time and years to put together who I have on my Team now!
My care now with Providers scattered at 4 different health systems and in Boston (CHW, FMLH, UW-AFCH, UW, and Dean + Dr.Bodamer at BCH in Boston) is amazingly good and frankly much better than it was when all my Specialists where right at CHW and FMLH where each could literally much more easily reach each other. I can't complain to much and I love my Team. =)
I think beyond grateful would be appropriate words to use! God is Good and b/c of him I have great Providers!
Thanks for stopping by,

This is not my meme - contrary to some of my typing I can sp 'Operation' =)

Wednesday, October 28, 2015

Pre-op Appt (check), checking with Cardiology for 'ok'..

One more step checked off towards having the VP Shunt re-replaced Nov 12th. Today was the pre-op Appt with my Primary dr. She has cleared all aspects other than she wants to talk to my Cardiology Team and run a couple things by them make sure they are ok to sign off and do not want to see me before hand or have any concerns with symptoms. To use Dr.Simpson's words indirectly "I'll clear you for surgery if your Cardiology Team will but if they have any hesitations we'll have to put the shunt replacement on the backburner till these heart issues are sorted out". Yay?
I said to Dr.Simpson re this that I hope my Team will either say we can go ahead with surgery and keep the f/up appts I already have scheduled in the wks after surgery (both w/Dr.Kovach and with Dr.Earing + 6mo Echo) or if anything, if they have any concerns maybe request closer monitoring by Anesthesia but give us the go ahead for surgery. I am hoping we can get this surgery done, it seems like it's the 'surgery that's never gonna happen in some ways'!?
I swear if this surgery gets cancelled I am going to lose my mind!? I CANNOT even explain how bad I have felt lately, during nights/mornings to mid-afternoon and then I finally start to feel some better. - It is both shunt/headache pressure and heart symptoms BUT I absolutely know from a headache standpt we're doing the right thing putting a 2nd shunt back in. Mornings, nights and some parts of the day are beyond ridiculously aweful - sure I keep functioning but literally I need that 2nd shunt to take some of the pressure of the LP Shunt, my abdomen, very low back and neck are so uncomfortable (in 3 different ways). I'm gonna for real cry if this surgery where to be rescheduled!!!!
I do see Dr.Bragg on Thurs so will talk to her about all of this, it is really, really bad often lately so if you believe in it say a prayer! I want to say my Cardiology Team will clear me but who even knows, I don't know why they wouldn't b/c I think they realize it's not as if we're just doing this surgery for the sake of doing surgery but in fact to help symptoms overall.
Between the heart and shunt stuff Dr.Simpson and I where talking today, she (as do all of my Providers besides Dr.Bragg) was asking about the recent shunt surgeries we'd done, trying to sort what shunt we'd just did revisions on. Honestly the conversation was kind of funny, I think we where both laughing by the end and my teasing her (PCP) a little, she couldn't keep it all straight (teasing)? ;)
I don't fault Dr.Simpson or my Providers at all but today she was asking me about what was done in the 1st of those 2 last shunt revisions (about a month ago, a couple days apart) and what was done in the 2nd revision + what is it exactly we are doing in this next surgery.
Honestly I have to laugh a little b/c who could keep all these shunt revision/surgeries straight much less the overall issues? I am not sure there is any one of my Specialists/drs who can really keep straight what shunt I have vs what I've had in the past and what each of these surgeries are we do. =) It is a lot for sure!
My Primary dr. does a really great job managing everything and though she's still relatively new to my Team (3yrs I think) and had never heard of MPS prior to that (she told me as much) but she's learned a lot, really tried to work with my Providers and to stay on top of what is recommended for testing, even testing that others of my Team (Genetics) usually does as is the case now. She also never hesitates if she thinks it's needed to reach out to any 1 of my Providers such as Cardiology this time and last month she contacted my Genetics dr. in Boston to get better details re the gene mapping study.
She ordered a chest Xray today, is calling my Cardiology Team and is going to try to order the urine/blood test that is done for MPS. (all came back normal, we're sorting out how to go about ordering the urine/blood MPS test as it's pretty specialized and just a few labs our E. that run the testing.
We where talking about her caring for me and learning about my MPS, about how ideally a Patient with a complex diagnosis like my MPS would be followed by a Provider in a newer specialty like Med/Peds. This is what Dr.Earing / Adult Congenital Cardiologists train in before doing Cardiology residency/fellowship as these Providers train in both Pediatric Medicine and in Adult-Internal Med. so would see Patients from Pediatrics on through Adult.
In any case my Primary dr. now is Internal Medicine trained and has been really good for my Team, she may not know everything about MPS but she learns, reads and talks to my Providers and she isn't afraid to make suggestions where she thinks something might be better.
As we where talking about everything going on I made the comment to her 'wouldn't dull and boring just be good' (her MA comments pretty much every time I am there how frequent they see me for pre-ops and as she's leaving each time she'll say 'I hope this is the time we don't see you for a surgery visit for a good long time'. Funny, funny sometimes these visits. We definitely laugh a little at the insanity of it all.
After I made the 'dull and boring' comment, Dr.Simpson said to me something like 'In healthcare that is often the case but that just wouldn't be you". LOL, sadly so true?
As far as knowing if surgery is a go from Dr.Simpson and Dr.Earing/Dr.Kovach's perspective Dr.Simpson was going to reach out to them and thought she'd know by Fri. I am praying for a 'YES, She can go ahead' (with surgery)!
Dr.Simpson said something along the lines 'I know your not going to be excited if the surgery is postponed (an slight understatement, I don't want any surgery but I do want these headaches/symptoms better controlled) but technically your heart comes before anything else'. I think she also know I wouldn't be wanting this surgery or going for it though if we didn't think it would really help so I think she'll do her best to get surgery approved.
Sometimes I think it is just it sucks how tied in each issue is to 1 another and for that matter I commented to her these days, sometimes I feel like we'd be better off just to do the 3rd OHS and manage the risks, get it over with so I'd feel atleast some better on the heart front!? Ugh!! As I've written about before my Cardiology Team has tossed around doing the 3rd OHS at just about every visit so it's something we've all considered and just a matter of when is the right (best/better?) time I guess.

I think in some ways it's just hit me the past few wks how truly terrible I feel many days, it kind of stinks actually. My Mom, Sister, nephew and I went bumming this past Sat and the 1 before that which is something I'd have normally looked fwd to but instead I sort of just 1/2 dreaded it (it was fun, I just stay in the car or wait at the front of stores when done) b/c even little amounts of going from 1 store to the next or garage saling flat our wears me out which I might add stinks!.
On a fun note Zan spent the past 2 Sat nights, he's easy enough so that was fun. =)
Often 1/4-1/2 way through anything we do I end up staying in the van which Zan often then will chose to hang out w me. I don't mind just waiting but I truly look fwd to the day, perhaps post a 3rd Heart surgery as kind of daunting as that tends to seem (b/c of the unknown, knowing it's never been done before in an MPS pt. 3x's) I hope I get more energy again after!
These days some days are definitely better than others and maybe it will be a little better after we put the 2nd shunt back in.

I have 2 more appts set up w Dr.Bragg, for her to take off fluid before the surgery the 12th as long as it goes ahead. The 1st is this Thurs, the 29th and Mon the 2nd I believe which is this Thurs and next Mon, (ERT switched to Tues). Otherwise I did the Stealth CT Scan last Tues, 20th which is what she uses to place the ventricular (brain) portion of the VP Shunt catheter.
As long as no issues come up with the surgery i'll test INR per usual a day or so before the surgery. Given surgery is now on a Thurs, I may have to make a separate trip to Milw. but have to think about this some.
Lastly I did the Holter (Zio) monitor last wk for just under 2 days which turned out to be a tad on the interesting side as I kept bleeding under the monitor on/off and making a huge mess especially at night. My Team will have the results in 1.5wks or so.
Un-related to surgery I talked to the Genetics Counselor-Research person at my Genetics drs. ofc. in Boston and they mailed out the consent form for the gene mapping study. This in turn she said can take a while to get the test results, up to 6mo once I sign and mail back the consent form and complete the actual test which they use blood or saliva.
This literally maps the entire body-looking at any and all abnormalities in all genes and chromosomes both to see if there is any other underlying, un-diagnosed issue to explain the (especially) unusual heart issues my Team has dealt with.
Per the UofMn drs and my Genetics dr., both of whom see a lot of MPS pts they have never seen the issues apparently my Team dealt w in surgery and the issues we deal with now/the unusual progression I guess.
In addition to the moderate-Hurler Scheie like mutations but very mild outer features. My Cardiology Team here and Genetics Team in Boston wonder if a separate/secondary issue could potential explain the issues we have/or help them when we do go forth w the 3rd OHS planning.
In any case will update here when I know anything,
Thanks for stopping by,
Erica Pre-op, pre-surgery check list

Part of Z's Christmas list - to funny =)
Just b/c I love him =)

Recent Heart monitor (oh what a mess I made)
New jacket, a little (Team) MPS Awareness

Thursday, October 15, 2015

Shunt tap (is so good), Surgery date change. Musings as a Rare and complex pt.

"I think they fail often b/c it is a such "one size fits all" solution" for a condition that is anything but "one size fits all (hydrocephalus)".
I started writing this entry the other day, before today's Appt with Dr.Bragg, to have some CSF (spinal fluid, what the shunt drains) taken off. I saw the above quote on a page I sometimes visit and thought it was pretty appropriate (AND true)!
Anyways so I did see Dr.Bragg today; after chatting some about the dates she'll be out, about setting up a few more appts to have her take off some more CSF (intermittently, gives short term therapeutic relief) to get by till we have surgery to put back in the VP Shunt. She tapped the LP Shunt reservoir which is not painful and pulled off the amount of fluid she is able. It isn't a lot but it does help, almost like a 'parting of the clouds' temporarily.
I didn't feel terrible while seeing her but it's still a noticeable difference on my part after I get up from the table - truly like my head clears and best of all, weirdest probably of all, despite there being some nerve irritation in my low back as fluid is being pulled off (nothing that would make me respond in a way to make the pulling discomfort noticeable to anyone else I don't think) the residual back discomfort that I get (much improved nerve irritation since we went back to a Codman Hakim valve but not as good w this mini Codman version of the valve as it was w the full sized valve which perhaps that sounds weird but for whatever reason it's true).
In any case the residual discomfort that is there, worst with sitting was completely gone on my drive home and has cont'd to be good tonight! The headache relief wont last as long as the back relief but it's as if it all combined gives my body a mini break, a break i'll gladly take given the shunt taps themselves minus that nerve-pulling sensation don't hurt.
Surgery itself was switched to the 12th instead of the 10th, her Secretary called today to ask about switching the day, and explain why they needed to. Originally it was going to have to be the following wk, the 18th (or 1 of those days) as I didn't want to do the 1st wk of Nov. given Dr.Bragg will be back for a few days and then out for a few days again. This 2 day change does mean having to re-arrange a few things but being perfectly honest I feel like this is what I do constantly so what's a few more calls, right? I'll survive, I just can't wait for the shunt to be back in! 
Given Dr.Bragg offered to do a few shunt taps during that time and if I cone up w any questions about the VPS replacement I can ask those to at the shunt tap appts I am OK it being slightly delayed. I'd rather do surgery when there's the best chance Dr.Bragg will be arundel the whole time! 
I am always surprised Dr.Bragg is so kind (but most of my Dr's are, maybe it's as simple as if we as pts appreciate our Providers they treat us well?) I don't know but I am always so grateful to her for her being willing to do little things to help me! She said to me if I needed anything she could help with/had any questions for her while she is out I should let her know and she'd  get back to me. She is going to a Conference part of her initial time out but just said she'd see her msgs.
 No Provider has to make themselves accessible to a Patient so I just have to say I think she is really wonderful. I know I've said it before but I feel blessed to have her on my Team. 
I sometimes think about say 5 or 6yrs ago at this time I was still looking for help and answers and now? Now she just never gives up. I don't know how I got so lucky but God is good!

On a totally different note, I wrote this earlier in the wk, a friend had written something related to her daughters surgery and I was overly tired which is when I tend to reflect on the shittier side of all we (MPS pts., rare disease pts., chronic dx pts.) deal with. -
Honestly I don't know what made me think about this, perhaps reading a friend's post about her daughter, w MPS I facing more surgery and the uncertainty at times of what decisions to make. Thinking some lately about the heart stuff, the shunt stuff but especially about the heart/lung stuff and although I don't let it stop me how it does sort of hang over every other aspect of my medical care...
I kept thinking about that night, it's shitty we have to sign these consent forms pointing out the risks of our surgeries. Crappy we have to sit in our drs. offices and hear the risks and how high risk we are for surgeries but how these surgeries are needed for better quality of life. We consent to these surgeries and procedures, yet we know they may not always work.
We spend so much time on the phone scheduling, coordinating, dealing with insurance and many people, who have no clue assume our lives are so easy b/c they have no idea the amount of time all of these things take on top of the amount of time we spend driving to, sitting in and talking to/working with our Providers.

How despite this all many of us still try to be involved in things that have nothing to do with our care (or in my case a combination of advocacy,  my work w Gene Spotlight and outside interests in-related to MPS). How sometimes we're  so exhausted it's literally a fight to pull ourselves out of bed, in to the shower and through the task/activity/mtng at hand b/c we just want to sit down in the chair or crawl under the covers and sleep and yet we (I atleast) feel guilty if I don't go to whatever it is I have going on so I go.
How we often deal with nausea and headaches and pain while we go about the 'normal' things we do. How cracking open a med bottle, opening up a thing or 2 or more of nausea med to prevent from throwing up while trying to focus on the Church service or Sunday School class or whatever your doing with family or friends that day and yet most have no clue (and sometimes that's ok).
How many people assume your abusing that handicap parking spot "because you look so normal", "do you know that's a handicap spot " and all you want to say is 'no shit asshole' but you don't,  instead educating (lecturing sometimes) the person on not assuming, on what MPS is, etc.
We know chances are if we do opt for a surgery it may or may not work and if it does work, initially we may come out feeling worse or more discomfort on 1 hand (although if goes as planned we may also experience relief on the original problem) until our bodies heal.
Some surgeries are easier than others, the ones that seem like they should be the hardest (OHS?) albeit difficult at 1st tend, at least for me ultimately to have a quicker recovery than others.
How you feel guilty when you don't feel like your recovery is going as quick as those around you seem to think it should? Still on that pain machine? It's not my Providers who I feel guilt from about this but oh do I feel guilt sometimes when in-pt, as if I am supposed to heal according to someone else's well set plan.
I don't think people always get, while some surgeries are pretty straight fwd and small, the surgery itself wreaks havoc often on an entire MPS pts body due to positioning and other factors.. I don't think family or friends or Providers mean to make pts. feel guilty but sometimes unintentionally it happens. Believe me, as pts. we wish we could heal faster to and feel more normal faster to!

When it comes to surgeries we hope by signing these consent forms, sitting through 1 more pre-op physical, 1 more "these are the risks, this is what we're going to do" that we make the right treatment choice. We hope (pray!) we get the right Anesthesia 'Team' (if we're lucky as difficult airway Pts. it's someone who has had our case before) and who takes the time to work w the whole "Team" to make the best decisions for surgeries or procedures that bring us through safely w the least amount of issues or hopefully no issues.
We hope the entire "Team" works together, truly as a Team for our care to make surgery/procedure or even just care the best it can be.
As medically complex, complicated Pts. we just want Providers that care and when really lucky albeit no pt. (including myself!) really ever wants to need a Provider or Providers time so often they become as familiar w you as some of your own friends are, if or when this DOES occur you want the type of Providers that truly do care enough to care about you as more than just a diagnosis they have to treat. They know you as a person with outside interests and likes and want to help you succeed in life...
You want to be a person first, a diagnosis 2nd.  
In reading some more about the Ivabradine (Corlanor)  I was curious about some things to do with it. I learned a little bit, some things I knew, some things I knew where my own Cardiology Teams wishes for the drug and other info I read was just interesting.
Ideal Heart rate is between 50-60bpm. On average, recording HR via an app on my phone daily the #'s have been low 80s to low 100s w an occasional mid-70-80s heart rate.
So far, at least thankfully no side effects on this new 7.5mgs dose adjustment. Thankfully also the med when I picked up the new script came as a 7.5mg pill so while I'll split my old pills to use them up in the future will be nice to not need to use the pull splitter on these!
The only others med I have to split some days is Coumadin (I have multiple different strengths of the med) but those are easily split by hand even w limited hand/thumb strength!
From reading the Ivabradine (Corlanor) website they list 7.5mgs as the maximum suggested dose though apparently my Team has seen 10mgs used in some HR resistant pts.
Lastly, last wk Dr.Kovach had said I guess to let him know if there where any issues or no changes in 2wks (would be next wk) and the website also suggests any dose adjustments be done at 2wk periods.
I love the 'super hero' shape >

Sunday School Class

Just b/c he's so cute =)

Kinked catheter from prior to 2nd LP Shunt revision 9/24 -

Thursday, October 8, 2015

VP Shunt (surgery) scheduled; Heart Rhythm Appt (dose adjustment)

Surgery with Dr.Bragg to re-replace the VP Shunt is scheduled for Nov. 10th, while it stinks that it is a month from now (I knew this from talking to her the other day) I also would rather do it then vs. say do it early next wk and then she only be there a day or two of the stay, anything go wrong and she not be there. I can wait. I am very, VERY much looking fwd to feeling better, hopefully (on headache front) being able to sleep better but i'll wait.
She is out something like the 16th-28 (she told me this) and then the 1st few days of Nov. so once back i'll have a few other things better sorted anyways. After talking to her Secretary today I had 1/2 a dozen other phone calls to make (I was at ERT so gives me something to do during that 5-6hrs I guess to pass the time). I was able to get the CT Scan scheduled (she uses this for computer assisted planning/placement of the Ventricular part of the VP Shunt), the pre-op is scheduled (closer to the end of the month.
I asked Dr.Bragg if I could see her next wk on 1 of her clinic days to potentially have her tap the shunt and remove CSF. She was amenable to this (love her!!) and so this is scheduled for Thurs. Given it doesn't hurt when she does this BUT does help for headaches for a very short period (is only a few hrs but for whatever reason really does seem to reset of sorts some of the other associated symptoms for a bit longer). I am grateful she's willing to do this when she clearly wouldn't have to. Depending I may do the same the end of the month when she is back for a few days before she is back out again for a few days leading up to shunt placement surgery.

Dr.Kovach, Heart Rhythm dr.
I seen Heart Rhythm dr. today., At the last appt they talked about upping the Ivabradine dose to better control heart rate which in turn the hope is would better help oxygenation between the restrictive lung issues and heart and perhaps symptoms.
Dr.Kovach essentially said today b/c heart rate was "still relatively high" he'd up the dose to 7.5mgs 2x's a day (from 5mgs, 2x a day) and do a Holter (Zio patch) monitor in 2wks. If this doesn't bring HR down he said he'd likely try 10mgs per day as long as I con't to tolerate the med ok (so far no side effects which is nothing short of amazing with heart meds) and if that still didn't bring HR down enough he'd consider "adding something back". This option would be less than ideal as I already take this Ivabradine, high dose Digoxin (for heart muscle), Spironolactone and Lasix (both for the heart failure, fluid control) + of course the Coumadin. That is already a lot of heart meds in my opinion! Hopefully this new med adjustment will help bring HR's down more consistently. =)
If symptoms aren't helped by this adjustment or they opted to go up to 10mgs 2's/day it's likely our last hope before they'll do the mitral valve replacement (open heart surgery). Dr.Kovach made the comment if we get to the highest dose of Ivabradine and still not ideal situation after he mentioned potentially adding another different heart med back he said "or do we just bite the bullet and fix your mitral valve (replace it) and is that the only way we get your heart rate under control". (something to that affect, I wrote it down but perhaps not exactly word for word).
He mentioned seeing after the VP Shunt if heart rate is down and then the shunt replacement brings it down even more we'd have to adjust the med dose to a lower dose again. This of course would be a good thing, though I am skeptical this will occur as heart rate has been an issue for the past 2+ yrs since the 2 OHS despite our having had 2 shunts in place all of that time leading up to May. Although it was never perfect the 2 shunts was better than it is now as far as controlling intracranial pressure but seemed to ever have little affect on heart rate.
As far as f/up, HR/Med dose adjustment (if further needed) Dr.Kovach is opting to do f/up the wk after the VP Shunt surgery as he is on service (on call I guess?) for his Dept. the week before my surgery which would be a month from now/starting this med. He is doing the Zio patch as I mentioned above 2wks from now and said he would review that and based off those results I guess would perhaps make med adjustment before surgery. I am not totally sure on that part.
Either way my Cardiologist, who I see Dec. 1st for a 3mo f/up and 6mo Echo, (from reading his notes, things he's said to me if I understand correctly) seems to believe even if we do OHS it will help some symptoms but not all of the symptoms given (I guess?) high heart rate and lung issues. I was re-reading some of the clinic notes prior to today and have to admit I kind of glossed over some of the info previously about heart rate causing or affecting the increased pulmonary pressures and the thoughts this was cause of some of the symptoms.
One other note, after initially having problems again with the Port yesterday, which thankfully worked itself out vs our having to do TPA again (clot buster) I was able to get INR drawn after ERT. My Cardiologists Nurse called today and albeit surprisingly (but a good thing!) INR is in-range already at 2.29. Jane said to me something like: "your levels never go up this fast, usually it takes your body a good couple wks" so we'll recheck in 2wks to make sure it isn't to high.
Perhaps the best part of today was having lunch with my former ERT, Infusion Nurses. Both where my Nurses up till last yr. when the CHW Genetics clinic closed and both still do my INR/Port access now at CHW so I do see them but still very nice to catch up a little. =) All 3 of us just met in the CHW West Café Lobby for about an hour today prior to my heading up to the Heart Clinic. Erica

Monday, October 5, 2015

Surgery F/up with Dr.Bragg (scheduling for VP Shunt), Next up Heart Rhythm Appt.

Hi All, What a week =) Not a particularly bad week, I mean sure the headaches have definitely been there as has the on/off breathing issues and sleeping that makes me really, realllyyy hate (dread?) sleeping but those things aside (MPS aside really) things have been ok.. My car decided it wanted to do it's own thing last wk as I wrote about I think in my last post and it turns out we figured out what the problem was. Thankfully my brother was able to make one of the parts that had broke/was partially broken and the other part was relatively cheap + he was able to replace. This apparently explains why my car seemed to be overheating without actually seeming to over heat (antifreeze leaking).
Needless I was in Milw. late last wk again and it seemed to be doing the same thing so I took it over to my brothers again Fri (Beaver Dam) and he then figured out the problem and overnight fixed it (I took his car home, which is the exact make of my former car - a little unnerving). I am DEFINITELY glad to have my little car back! Beyond grateful to my brother to.

Dr.Bragg, scheduling VP Shunt placement, CT Scan
Today's Appt with Dr.Bragg went well I think, she has a new Nurse (not sure if a 2nd one or if this one replaces her former Nurse Lisa, who was very nice and always very helpful if needed). In any case Dr.Bragg brought that Nurse (Laura) in and introduced her. It didn't even register at 1st that Dr.Bragg meant she was a new clinic Nurse. I can be rather dense picking up information given sometimes. =)
In any case Dr.Bragg showed me the former Xray from last wk taken of the LP Shunt and where it was kinked (apparently the Radiology people read it as normal at 1st, Dr.Bragg made the comment "it's a good thing we check our own films" and then commented to her new Nurse how the shunt was kinked and we'd had to go back to surgery as an urgent case 2days after the 1st surgery. I should have taken a pic while there but did get a copy of the Xray film (CD) so will post a pic of that in 1 of the next updates perhaps. =) It was like a circle with the tubing really - I guess I could see how it could have been overlooked perhaps by the Xray/Radiology folks.
As far as putting the VP Shunt back in Dr.Bragg is out for the better 1/2 of Oct and part of the 1st part of Nov. so i'll talk to her Secretary and schedule surgery for early/mid-Nov. She asked if my Cardiology Team (Dr.E or Dr.Kovach, Cardiologist and Heart Rhythm drs.) where making any changes to the cardiac meds I am on which I kind of blanked but I do see Dr.Kovach this Thurs. and I know there was talk of upping the Ivabradine (Corlanor) dose from 5mgs 2x's/day to 10mgs/2x day). Given surgery is essentially will be a month or more from now this gives time if they do adjust that med dose for it to kick in and any issues should there be any (not that there should be any, is a pretty side effect free med amazingly) to sort out and my Team to see how it does before any surgery.
Before surgery she'd like what is called a 'Stealth CT' which basically means she uses a scan which is able to be uploaded in to a computer system and this helps guide her/her Team in positioning and placement of the VP Shunt. It also means the surgery can be done with a minimally invasive (much smaller) incision vs how many VP Shunts are placed under. The scan itself is really quick and as far as surgery doesn't affect me one way or another other than more accurate catheter placement due to my teeny tiny vents (ventricles) and brain/skull that are pretty non-compliant.
Given we are doing a Stealth CT Scan instead of a quick brain MRI I won't have to see either Dr.Bragg or 1 of her NPs after to have the LP Shunt re-programmed. I like all of her Team of course but I am not complaining about 1 less thing to coord. =) Maybe I can coord. the scan the same day I do the pre-op. or some other appt., I really need to get a Audiology and Neuro-Opthalm. Appts caught up so could always do either of those as well (not a huge deal to me, my Genetics dr. and Primary Dr. remind me each time I see them, to do these per MPS protocol). Will see. There's always grocery shopping to, my favorite reason to go to Madison. =)
I'll have to do another pre-op with my Primary dr. which is almost humerous; I actually think 95% of seeing Dr.Simpson is just for pre-op Appts as most of my other issues are managed by someone else so I rarely otherwise see her. Even her MA teases me good naturedly when I come in about this fact. =) Funny but not funny, right?!? My Primary does do a great job of pulling information together from my Neuro-Endocrine dr. and my Cardiology Team and coord. all of this (making sure all info is in place) so often times it is useful as she makes the appt run extremely smoothly so generally isn't to torturous. =) I have a few questions for her/or my Cardiology Team so this lapse in time to surgery gives me time to sort those things out. Isn't anything to important just a couple questions I have for either related to the heart stuff and surgeries.
Dr.Bragg made the comment when we where talking about timing for this surgery and about waiting to do it when she would be most around (vs say next wk and then risk being in-pt or having issues and she not be in town) about being in-pt for the holidays again (I was close to being there on Thanksgiving last yr but we avoided it, and avoided Dec. althogether though the 2yrs before I was there during and right up till Christmas). She apologized for this but I think we should still be doing this surgery early enough I should almost certainly be out by the time Thanksgiving roles around and Christmas well I am making it my mission to make it a 2nd yr with no Christmas in-pt! =)
As many (but not all I would imagine) know Monday's are typically the day I get my weekly Enzyme Replacement Infusion in Milw., this drug/infusion of course runs over quite a few hrs. (5ish) so isn't generally possible to coord. other appts the same day and especially appts in other cities. Generally I do INR checks, next door at CHW (Cardiology Teams hospital) before I go over to ERT but that's about all. 
I have to say though and it always amazes me bc Mondays are my normal infusion day on days like today where I see Dr.Bragg and thus move my infusion to a different day she pretty much always asks if I've gotten my infusion already or was I able to reschedule it.
I know I've been seeing Dr.Bragg for just over 4 yr but still it amazes me she always remembers I get this ERT and is always concerned that I not miss it. She genuinely cares which is so sweet. Sure she's my Provider and has done a lot of surgeries for me but still it's not like she'd have to remember this detail. :) 
Random pics, Sunday School, etc.
I shared a few pics of various things below including 1 of some of my SS kiddos that where at Sun School today - in total I had 2 kids missing but was nice to be back today (only my 2nd week out of the 4wks SS has been going).
If anything to update i'll post a new entry after Thurs's EP-Heart Rhythm Appt. and will update info here in next entry what the dates are for pre-op, CT/Stealth Scan and Surgery date.
I don't think i'll see Dr.Bragg between any of this and the actual surgery date but if I did come up with any questions I know I can get ahold of her Team; the only ques. I didn't think to ask her today was if we could use the regular size Codman Hakim valve vs the smaller one when we do re-replace the VP Shunt next month and that I can ask her beforehand.
Thanks for stopping by,