Wednesday, March 23, 2016

Closer to a decision.. Now we figure it out

It hadn't occurred to me till earlier today and this is most definitely TMI (so not something I'm entirely comfortable sharing but I know others experience the same).
Really ever since I've had a LP Shunt I've had bowel movement issues, which required some less than ordinary thinking to sort out/at least be able to go (again like I said, TMI).
This issue had completely resolved when I started ERT (I used to get terrible stomach aches and intermittent (again TMI ) diarrhea for yrs that often occurred after I ate certain (many?)  things.
Well anyways after the MVR in Jan. it was like this just resolved again, was nice actually.  I didn't think to much about why it was but was definitely nice especially given other things where /are taking their good natured time! Ironically this was the same time period the headaches where getting worse again but didn't occur to me why it might (possibly) be.
Today/tonight  after Dr.Bragg getting a lot more CSF off the VP Shunt (amazing how I felt/ feel!!!) I kept admittedly wondering on and off today as I have for some wks (since the OHS really) is the LP Shunt working as well as it needs to be?

I know when she tapped that shunt some wks ago she got very, very minimal fluid off but she also thought it was probably bc the VP Shunt was draining more. I didn't think to much of it, thought perhaps she was right. Who knows she may very well be!? The reason I guess I wonder shunt is bc how bad nights are (literally waking up between 1 or so every morning and often awake for hrs w the pressure like feeling), the intermittent short term vision blurring (she thought this could be 'occular migraines'), there to maybe she's right though I  wonder if not the shunt bc it always occurs at times in the day where pressure is higher and then literally improves as I can feel the shunts kick in. These also never occur at times of day where I don't have the pressure like symptom(s). And this bowel thing normalizing so and that only ever occurs seriously every time  we've  either not had an LP Shunt in place or it turned  out LP wasn't working.
I don't know. The very intermittent vision  is very similar to the "migraines" I got as a kid right up to when i was 1st shunted but these are shorter lived (bc I assume pressure drains eventually).
Is a little hard to explain this one. I only ever get this when not enough pressure draining though and never get them at periods like Nov-Jan where it was really, really good. 
I don't think she's given up by any means, she wouldn't help in the ways she still does  but I do a little bit miss her in the beginning where she tried to rule things out or perhaps thought outside the box more. I am not sure exactly how to say what I'm trying bc like I said I don't think she's  given up, she still asks questions about my overall issues, she clearly cares just is more tentative perhaps? 
Heck it's not even that I want any surgery, I DON'T  I just want to feel better!!! To feel as good as I did for instance pressure/symptom wise going in to the last OHS. Yes it wasn't 100% perfect but we where oh so much closer and that was liveable.

So many around me would say to me they hoped that would last as they could see clear as day the difference. I can remember my Sister asking me if I thought that was the surgery (when we put the 2nd shunt back) that would finally work long term? I am more than sure I said I hoped but wouldn't hold my breathe as it doesn't matter who did/does the shunts, it doesn't matter we just have troubles keeping them working consistently really well (like full effect) long term.

I did finally ask Dr.Bragg some more questions today. There's many more to ask but I just wanted to ask a few today.
  Honestly  we'd been talking about her leaving and options for the past month or more now but I just wasn't entirely ready to ask to much or ask her opinion to much (wasn't ready to make a decision maybe, needed time to think about it all, definitely don't mean the above like I didn't /don't trust her opinion. I just in general need time to think about things before I talk to others or especially my Providers no matter who they are. ).
I am leaning towards 1 doc more than the other and wanted to verify if he had experience with the robotic /stealth machine Dr.Bragg uses as that was a big deciding factor.  She says he does so that would be good atleast.
I don't mind he is brand new, sure it means he has little to no real world experience but new docs generally tend to  think outside the box more, tend to be more familiar  (at least a little) with rare diseases like mine and (at least again generally speaking, I am hoping it's yhe case with this doc ) aren't as afraid to ask others opinions or thoughts. This is what I really, really appreciated about Dr.Bragg when I 1st began seeing her. She'd been in practice I think for like 2 yrs but still the new ones aren't yet jaded and get to know you as a Patient and Human generally before they do get so. =) Then if they do get jaded they know us as a Patient and human so it's like anything they feel isn't taken out in us or they trust us? Not sure but still I am OK w young docs.

Most of my Team where brand new or very new when they joined my Team and all currently on my Team are great! Of course as many know I've picked and chosen the Team I have now over many yrs and they are  at numerous different health systems (Children's in Milw., FMLH, Milw, Dean in Madison, UW and UW AFCH in Madison + My Genetics Dr in Boston.

I  cant say I am ready to start over, training a new doc nor do i want to start over with a doc. Can't change it though, right?
Just have to pray this doc is as good as Dr.Bragg is and that she'll let the new doc or myself contact her if needed and that the new doc is as caring or kind as she is?

We talked about lots of things today, not just about the docs switch (which is ok, that's a little depressing topic ) so I got some questions answered but she also said (and I agree) we have time to keep sorting this all out.
1 of my concerns which I didn't think to ask her is if we can set up a mtng with this doc, either before she leaves over the phone or at the very least can she fill him in on me and even though she'll already be moved to AZ by then (=/) the 1st day I see the new doc can she talk to him ahead of time  and he and I Conf. with her?
Many pts have what's called the Complex Care Team at UW which they can have help them transition to the new doc but I really have no one else there so am definitely going to be planning this as much as possible.
Trying to make sure we plan and have plans in place  so the new guy is familiar  with my complex history and if possible Dr.Bragg, myself and him can talk before he takes over my care. Can't hurt to ask and I think Dr.Bragg wants to help make this as successful as possible.

The NP from Cardiology (ACHD Clinic)  emailed me last wk both to let me know something was set up and  she wanted to let me know she'd  sent in the Cardiac Rehab referral.
More than that I'm her email she wanted to let me know this was her last wk in the ACHD Clinic as she accepted a position in-pt with the Peds Cardiology kids (also at CHW).
Her thoughtful and kind words re the impact I've apparently had on her, her learning about complex care and dealing with it and how this would help her in her new position really meant/means alot.
I'll definitely miss her being on the ACHD Team as she truly cared and was a great advocate + like many of my Providers really listened.  I felt comfortable telling her symptoms I sometimes feel a little intimidated telling my Cardiologist  (lol, I think bc I am stubborn, he is stubborn,  we're both opinionated and sometimes even despite these own traits myself he intimidates me)! Doesn't mean he's not a great doc, he is and 1 I really like.
Anyways that aside I see Dr.Earing (Cardiology) and Dr.Kovach, (Heart Rhythm doc) next wk.
The new beta blocker helped slow heart rate some but side effects where to significant so I stopped it. I need to be able to function and that med just makes me feel snowed . I'd guess Dr.E will have something to say about this but when is significant side effects out do any benefits given? I am hopeful we can try something else or figure out how to improve this as slower Raye clearly did help some.

Dr.Bragg asked me today what Cardiology was/is up to,  what are they trying to sort (accomplish maybe?) as it is a little confusing I'm sure to anyone just watching from the periphery even if they know history.
As I explained to her (but probably now very well) the bigger issue w overly fast heart rate even though mine only ranges 100 - 150s is the harder a heart beats (the faster the rate) means the less time there is between each heart beat for properly oxygenated blood to get from the heart ro the lungs  (or maybe its lungs to heart) thus good oxygen to heart and body which can affect many things if I understand it all right.
Before this MVR the pressures between the heart and lungs was significantly increased and the 1wk Echo post this 3rd OHS as I've written about before showed moderate stenosis.
Getting heart rate down, getting rate slower (between each beat) can apparently help the valve function and rid of the post surgery moderate stenosis (the goal).

Anyways, thanks for stopping  by,
Will try to update after next wks Cardiology Appt.
Am opting to see Dr.Bragg again the following wk,  the day before I fly to Boston vs next wk.

Happy April (almost)! May it bring warm weather, good answers and figuring some things out? Warm weather for Marathon wkd wouldn't be bad either =)


Tuesday, March 15, 2016

Few Appts, Cardiology F/up. Trying to figure things out

**I wrote this post earlier this past wk, just haven't hit send. -f

Today's (Tues) Cardiology Appt was ok, a bit frustrating for sure, not due to any one thing or issue but just because it's so very rarely clear cut with my MPS and the associated, secondary issues. Frustrating because it seriously feels like about the time we sort one issue and get it improved something else happens and sets that 1st issue back (the shunts having finally been working better, not perfect but so much better after Nov.) and then we did this 3rd OHS/MVR in mid-Jan. which we needed to do no doubt but the shunts (headaches/pressure) just haven't been the same since and I often feel like crap (the headaches/pressure follow a very predictable pattern) which honestly is just frustrating b/c I just want it to be good again. It was really awesome when we had the 2nd shunt back in place and I'd gotten new glasses (stronger script with bifocals) and those 2 things together meant I was finally able to read with much less issue. I slept better from a pressure/headache stand point and appetite was just better. Then bam, we did the OHS (Heart surgery) which in and of itself went really well, recovery was so smooth minus a few very small bumps (very small easily fixed things) and I got out after just a wk. which was AMAZING!
The headaches though where back  after surgery and have only seemed to slowly progress since then getting more aggravating. I don't know what to even say about them other than shunts are so good when they work well but (atleast in my case) they are so damn finicky and frustrating; I hate having to (what feels like) constantly ask my Team for help and I hate being reliant on my Providers to try and feel better.
As far as what did we decide at the appt? Nothing earth shattering, they'll  do a 2nd post 3rd OHS Echo around 3-4mo I believe  Nancy (NP) said (the 1st was a wk after surgery, this showed the Moderate stenosis in the new valve. A TEE which is a Echo done internally (in a sense) via a probe down the throat or on means like that, not entirely sure how it works when intubation yet) but that Trans Echo looked perfect. Very good. The 1 wk Echo post surgery showed the moderate stenosis but my
Cardiologist felt/feels this would/will improve if we got HR down (that's been solidly frustrating as it doesn't seem to budge much despite all these heart meds) but hopefully he's right and the valve is just fine. 
He doesn't seem worried so thus if could get freaking HR down the other symptoms I experience intermittently  (but semi frequently depending on activity) will hopefully improve to..
I think my Cardiologist said it himself he isn't absolutely certain how best to help this. He brought up the HR could be affected by pain an thus why it's been harder to control but we both agreed that seems less likely as my chronic pain is very well managed. We talked about it could be secondary/related to the shunts not working well enough but there to we've had high heart rates when the shunts have been very good (Nov-Jan for instance, with the EVDs, and other occasions) so that seems unlikely cause. He mentioned a few other things that could be interplaying but didn't seem to think those where causes either. He hopes with time it will settle down but we don't know as I've dealt w this high heart rates for some yrs now, atleast since after the 2 OHS for AVR/Aortic root. It's also increased in rate since the 3rd Heart surgery.  Like most freaking things  who apparently knows?
As far as how to try and get on top of it he seemed to admit some uncertainty but suggested we try a beta blocker again (oh joy) again in addition to the numerous other meds we have im place given this overly fast HR could be related to the MVR and perhaps (but we really have no way to know) will improve in time. He seems to feel that is less likely but also seems to be hanging on to a bit of hope for that. 
BB wise after much hesitation I agreed to give 1 another try and will take it at night, along w most of my other heart meds (some I take in the morning or as in a few some are 2x's per day). If it hasn't done anything in a 6-8wk period I am not staying on it though. I firmly believe if a med is causing worse side effects than it is helping then what benefits have been gained?
Otherwise he mentioned possibly starting/adding Amiodarone which is a more potent (I guess) arrhythmia med. With this he said it actually has less side effects than BB's in the way of fatigue but in other ways one has to be more careful I guess (sun, exposure and a few other things I can't remember are heightened I guess) but that he is more reluctant to start so is waiting atleast till after we meet with Dr.Kovach and look at big picture. And to see if the addition of the BB helps. I hadn't thought to ask but will talk to him and Dr.Kovsch, my Heart Rhythm dr. to see if the Ivabradine dose can be increased. I can't remember fully but thought Dr.Kovach may have said the very highest doses he's  used/seen used is 10mg, 2x a day which I am currently on 7.5mgs 2x per day. Isbworth asking as has so far had no side effects and prior to this OHS in Jan was at least helping some.
I'd  be more than ok not starting the new BB med, I'd be ok if we didn't have any of these heart meds really! 
At some pt I am going to say screw it all and stop em all. I don't mind the Ivabradine as I do think that is helping some, HR is still way to high (they'd apparently prefer 60-80 if I remember right) and perhaps the Digoxin is helping heart  muscle, I'm not sure. The 2 meds to get fluid off help but the doses are frustrating and frustrating it's like there no end in sight.
 I honestly had hoped after the MVR we'd be able to stop 1 or 2 meds vs adding 2 more meds! Frustrating of all as I come back to time and again is how  good the 1st wk was 
even if we where dealing with little things (UTI for instance) and being so tired but in general overall symptoms I just felt better. Simple and straight fwd once would be so nice. I can remember saying to multiple people in that 1st wk, especially once I was home (even if I was tired, it was a tired from how little sleep you get in a hospital at 1st) that I literally felt 'high on happy' bc I felt so good for once,  I literally think I'd forgotten what that good felt like!

I'll f/up with Dr.E and with my Heart Rhythm dr. in 1mo, in which i'll see Dr.Kovach 1st, we'll talk everything over and then i'll meet with both Dr.Kovach and Dr.Earing after. We'll try to formulate a better plan I guess. If lucky this med will work, I wont have side effects and god willing (please????) i'll get off some of these freaking meds at some pt.
Dr.Earing brought up how he doesn't see signs of Heart Failure, which is a good thing but I forgot to ask with all the ridiculous meds I take and the 2 to get fluid off wouldn't those kind of put HF under control? I don't know and will try to remember to ask next mo. I wonder that I guess bc of how I feel (some points better than others).

On a different note, they are going to try and get Cardiac Rehab started, their concern is the Rehab place will likely flip out over my heart rate I guess given I am taking meds and it's still high. 
Nancy, my Cardiology NP is going to fax over the most recent EKG and make the CR place aware this HR is basically my "normal" vs in most people I guess it would be more concerning. We shall see, right? **On that note I am opting to try the local bandaid station (community hospital ) Cardiac Rehab and that is set for the end of the mo/1st wk of April. It's supposed to be 3x a wk but I let the place know what ERT  I'd only be able to do 2x/wk (did the same after last OHS abeit was a different center) and that some wk I'll likely not make it even 2x a wk due to other things.

I keep telling myself, reminding myself and hoping I/my Team can get me back to the way I felt that 1st wk of the OHS. As I mentioned above  I literally in that 1st wk or 2 said to numerous people I felt so good, I felt "high on happy" because I hadn't felt that good from a heart stand pt in such a very long time.
 I pray we can get back to that 1st few wks and we  can get my back to feeling much better again!!!!
*The same really for the  headaches/pressure, I pray I/my Team can get back to how good I felt (wasn't perfect but was so so close to perfect, as close we'd had in a long while) with the 2 shunts. This OHS was needed, yet  it is hard how good the results where at 1st and  how different it has been since that 1st week to 1.5wks. Equally hard how good the shunts finally where, not perfect but sooooo much better then the surgery  and just like 3 steps backward again. I feel like this never ends! 
I WORRY with Dr.Bragg leaving (moving) end of May are we going to be able to figure something out 1st?
She said to me a few was ago re this (my paraphrasing) 'We'll  figure it out, we always do', I pray she is right and we can get the shunts back to a better place before she moves. I am worried. =/

I guess my sentiments about the frustrating heart symptoms  i the same I feel about the headaches/shunts with how good the headaches finally where before the Heart surgery then how backwards they have went since  with no clear solution why. FRUSTRATING!!!!! 
During shunt taps Dr.Bragg gets some fluid and does give some relief but it always comes back. To boot I kind of doubt whoever I pick to replace Dr.Bragg out of the 2 names she's  given /suggested will be willing to do shunt taps like she deserves to give relief.
Neurosurgery Appt / F/up -

I am pretty much in as much a loss now as a few wks ago which way to go re  choosing a new Nrsgn (Former Resident now Attending or Current Peds Nrsgn Fellow in Canada coming in Aug.).
I overall I just worry whoever I pick are they going to be like Dr.Bragg as far as really listen, not assume anything (for instance unless both shunts are completely failed I rarely look "bad") and work with me  as she has to keep trying to get to better even when better seems continually  short lived? Seems to continually slip away?
Some of upsides to Current Attending on Adult side
1. I looked back at my surgery records and he was in on 3 surgeries it looks like w Dr.Bragg.
2. Several Nurses from Neurosurgery flr, a Friend (whose daughter is also complex Hydro, sees Dr.Bragg's Partner)  all think he has a personality a lot like Dr.Bragg. All seem to think he has a good bedside manner which would be another plus. Good bedside manner usually means they'll take Patients view in to mind.
 This all would be a good thing if true (like I've said before I kind of remember this Dr just not well enough to really remember what he was like). 
I want a Dr who realizes, I know I may not always be right BUT I do know my body and I DO know pressure symptoms so I DO know when it's somehow related to shunts even if shunts aren't directly failing or more seems to usually be the case shunts  (in my opinion ) either intermittent occlusion or perhaps partially faiing. I really don't know on this last part just my feeling as its so clearly pressure and shunt related.

- I do worry a bit w the former Resident, Hydro isn't his apparent interest or Specialty so keeping up on research? Not that much ever seems to happen but being aware my situation is as far from normal as you can about get.. Would Dr.Bragg let him contact her if needed in the future and would he (and she after awhile?) be willing?
4. I really don't want to need any of my Providers help or to have to go to them but whoever I pick has to be willing to listen, to work as Team and to work with my other Providers.
5. As important to me, can I keep Dr.Bragg's Team - this may sound odd (I don't know) but I've had several Providers who where decent but their Staff sucked (when even other of my Providers said as much, you know it's not great then).
Being perfectly honest I really hope I don't have to teach a whole new Team (Nurses, NP, Secretary) that i'm not tying to be impossible but my schedule can be impossible to coordinate (various Providers schedules and in different Cities re Appts/ERT, life).
- Her Team knowing what goes in to coord.  surgeries, Adurazyme in-pt, etc, I really don't want to give that up, I like all of them. 
The Team now is golden at this so I guess I am going to ask Dr.Bragg can we meet together  w the former Resident and would there be any way we could talk to the Fellow in Canada who is coming (can't hurt to ask)?

 I know she's  said she thought  the Canada guy was nice but 1. Sometimes Dr's are different to each other then they are to Patients. ..Just about any complex Patient will say this. 
Would it help if we could talk to the coming Fellow to? 
As far as him it really, really comes down to wanting to keep her Team and I don't know how that could/or would work w the  now Attending whose worked w Dr.Bragg..

Needless if it's not already evident I am worried about all this, worried about things going back to how they where before I began seeing Dr.Bragg almost 5yrs ago.

I guess other reasons I am wanting to do as much as I can before Dr.Bragg moves is to try to figure this all out as much as possible with whisper i pick for a new dr. to try and make sure it  will really work..
As far as my trying to sort this as much as possible  with Dr.Bragg includes I trust her and I know how bad thins went when I'd been given her Partners name and been referred there prior to Dr.Bragg being at UW. ---
2. A whole bunch of my (former) Dr's had talked to her current Partner way back when and I even emailed w him prior to the Appt and yet that Appt was near a disaster from my stand pt. The 1 and only over I've ever walked out on. I don't ever want that to occur again.

3 Some have suggested I try the Former Resident-Current Attending and if it didn't seem to mesh I could always switch to the other new guy when he comes.     I've thought about this, the only worry is Dr.Bragg  will already be in AZ by then and I feel like I need her help to go over w my history/her impressions w/any new doc? I don't know how to do this 1, guess I need to talk to her some more about this possibility..

Not sure yet! I guess I really do need to start writing down questions to ask Dr.Bragg and/or to ask her and the other docs.

Some of the things I will miss most once she is really gone:
If I was in/going to the ER I could let Dr Bragg know and she'd  coord it all. I hope this will be the case w whoever the new doc becomes ( in new Teams case, whoever I pick let their Team know ).

Above all I hope whoever I/we chose will realize I don't want to need them but since I do  can we make a Team and work together well. Can they just respect I don't want them but do or will need them so can we figure this out together and can they be persistent (not give up)  as Dr.Bragg has always been... THIS is the part that most scares me  actually! 

After  my Appt in Madison Weds w/Dr.Bragg, I felt good from a Headache standpt (I slept pretty good Weds night to, yay!) I stopped at Hobby Lobby and HyVee since I was feeling good.

 At Hobby Lobby I found a few new crafts for Sun School which is always fun, lol.  I think it's quite possible I enjoy finding different craft ideas to much =) - I needed a little cheering up though in general (no real reason). :) Afterwards due to it being Lent I went to the late afternoon Service at Church which Lent is my favorite time of the Church Season no matter (reminder there's better to come thanks to Christ giving  his life for our/my sins; his selfess act) and ultimate Resurrection - a new beginning I guess really. 

Anyways it is nice (atleast for me) to be able to be in Church during that midwk Service these past few wks and be reminded as hard as things have felt lately, not feeling the best there is a so much better place one day.

The reminder to me during Lent of what was given for me despite my many sins is a reminder there is a  place where pain just won't be, where I'll feel better, free  of feeling these heart, shunt and any other issues one day. It's a reminder I am grateful for personally! I know it's not right for everyone but I am  grateful for my faith.

I truly believe everyone should have their own rights, beliefs and practices but I am grateful for what I have as it helps.

 If you believe in prayer please say one we can figure this shunt and heart stuff out if it be God's  will. 

Thanks for stopping by,


Friday, March 11, 2016

Peds Neurosurgery Appt, have names... Headaches uncertain

Talking to Dr.Bragg this morning (Thurs) at the Appt which went OK (admittedly I was praying there would be some simple answer to these pressure  symptoms though don't know what that would have been) but as usnoftenbthe case no such case. As Dr.Bragg said and I am grateful to her for  (my indirectly quoting) 'We'll  keep trying, we'll  figure it out, we always do' - I pray we do. It's so classic pressure symptoms and yet when she tapped both shunts she was able to get a little fluid off each. Not a lot but that is it seems likely bc  they are draining.

I honestly don't know, the relief was temporary and symptoms back by last night but sometimes even that 1/2 a day is like a reset on other symptoms. I woke up around 3 last night (this morning I guess) and couldn't fall back asleep dye to symptoms which sucked, a lot and vision when trying  to read this (even using the stover bifocal part) and with the screen enlarged is out of focus (blurry I guess) so that sucks. I reallllyyy  like reading so frustrating when this occurs! It's actually like the symptoms are slowly coming back, it was a sudden onset of all of it,  which I think has often been the case (slower onset vs sudden).

Dr.Bragg and I where talking about possible replacements for her (she told me about the Dr coming from Canada who will come in Aug. or as well about a former Resident now on the Adult side she thinks may be a good fit).
The former Resident is at least familiar w my care, issues and presentation so she thought that might be a good fit as he's been in on  some of my surgeries.
She also talked about the new Dr coming from Canada (I am asking around with Canadian friends to see if any know him from their own kids and if so what they think. 

It turns out though the new guy from Canada is a Fellow just finishing which I am not opposed to, I tend to think the young docs, newly out of training or fairly new (as was Dr.Bragg's case and the case with many of my current Providers, my Cardiologist for instance was only a few wks out of his Adult Cong. ♡ Fellowship at Mayo and at CHW maybe a month when I 1st started seeing him. My PCP wasn't in practice to long either when I 1st started seeing her and sane w a few other of my Providers. In all honesty about 1/2 my Providers are very young and 1/2 are by no means older but had a little more experience and then a couple  that where in practice linger when I 1st started seeing them.

I think a mix isgood and as pts especially very rare disease pts we have to be willing to train new Dr's bc the 'experts' certainly don't seem to be Doug it.  Even if our Dr's (or my drs) only sees me and 1 o2 other pts w MPS in their lifetime or practice at least they've seen it.

So an upside to the former Resident is he knows my case and I'd imagine knows the Stealth guided machine Dr.Bragg and her Partner use but then means new overall Team (Nurses, Secretary, etc). - 

I did ask Dr.Bragg since yesterday after thinking about this all some and a Mom (her daughter doesn't have MPS or a shunt but she does know rare disease and she's a former Researcher so she knows this world and needing to find good drs) suggested I ask Dr.Bragg if she thought I might be able to see the now attending/former Resident but  could i still keep Dr.Bragg's Team (NPs, Nurse, Secretary) given they are so familiar w the many facets of surgeries (other Providers involved (Cardiology, Endocrine, PCP), how to order Aldurazyme, INR mngmt, etc.) and Dr.Bragg seems to think it might be an option. That now Attending/former Resident is on the Adult side and Dr.Bragg's Team is in the Peds side so I'm not sure hospital would work but worth trying to find out, right if it might? Never hurts to ask and check? 

I guess she'll  talk to all of them and her and I will talk when I see her next and we'll keep figuring it out? I am glad we have time and yet wish we could slow time!                                       I do think she'll help if needed after she moves but really I still wish she:where going to be here! If only the hospitals and ruracracy (sp?) weren't so backwards.

Dr.Bragg's NPs, Nurses and Secretary are phenomenal about working w my PCP, Endocrine and Cardiology Teams + Genzyme and Insur. Case Manager regarding surgery needs, knowing how to order  Aldurazyme when in-pt and many other little things so I am hopeful maybe this could work.. I admittedly only remember the resident a little (but that's part for my norm, I am not great at remembering people after i've not seen or talked to them for a while. 

The former Resident/now Attending has great reviews by friends of me whose kids also have complex needs and thus also frequent hospital or surgery stays and interaction. That's a good thing :) 1 compared his personality to being a lot like Dr.Bragg's which also wouldn't be a bad thing, she's  not a 'let people walk all over her ' kind if person but she's  fair, cares, thinks outside the box (absolutely what I look for in all of my Providers) and she's persistent/believes me vs when it's not clear cut she doesn't just assume I'm making it up (I like her but I'd really actually rather be somewhere other than in any of my drs offices asking for help).

Sometimes a Dr's Team means as much as the Dr. does to make things go smoothly..Do I want to start that all over? I honestly don't know. So is it more of a potential upside to go w a new Dr but her Team still be there and familiar w my care? (If it doesn't work out keeping her same Team but the former Resident as my Surgeon),   I honestly don't know yet..

Dr.Bragg also said she would have privileges at the Adult Hospital in AZ and a few other of her pts are considering following her. I wouldn't move to AZ but do have a friend whose son has MPS I to who they go to Phoenix Children's and has said if I ever wanted to come see Dr.Bragg I could stay with her.
I know I couldn't see Dr.Bragg as my sole Neurosurgeon, it just wouldn't make sense but perhaps once a yr she could overall manage? I don't know if that makes sense either but perhaps a thought. 

She suggested something along that line (seeing her out there), I guess many this to figure out and in time it will sort out.

On a cute, unrelated note Dr.Bragg has gotten to know my nephew (Zander) some through my being in-pt and Z's love of coming to visit. Dr.Bragg made the comment she would miss Zander,  I think that little boy has a great fan in her =)

On a different note we where talking about her Partner I think more in the setting of if I went with the new Nrsgn her partner managing my care in the mean time (believe me I'd try my hardest not to need to), I am sure he's good at what he does and I know he's helped her plenty in my surgeries but I also know his and my personalities dont mesh well and hard to get past the many things he's said to me over the yrs about my shunts/not having an awareness of my MPS (as the cause for the Hydrocephalus) and that MPS then even causes Hydrocephalus for some.
Like I said I know he's a good Nrsgn just (and she's  probably right though I don't get that very 1st encounter before she was there) that my issues just make him feel uncertain or weary...
I dont always get when people are being sarcastic (and will be the 1st to say as much) and maybe that's just what his attempts where over the past almost 5yrs i've  been seeing her? Attempts to be sarcastically funny about how complex my care is and needing Dr.Bragg's help? Who knows, I guess I just like People and Providers especially who say things fairly black  and white and (especially if we don't really know each other) I wouldn't say things that if that was the attempt that could be misconstrued... 
On a different note Dr.Bragg tapped both shunts which she got some fluid off each, not a lot but some fluid so those seem to be working I guess. That is good but so frustrating (I know my body) the symptoms are classic shunt/pressure occurring worse at night/morning improving greatly to virtually nothing as the day goes on.
She did use the reprogrammer to double check both shunts where at the correct settings (each set at '30', lowest, India best draining the most amount of fluid)  and those I think where OK but I also am not certain, actually. I don't know with the Codman reprogrammer if it can tell them what the settings are currently at, prior  to being reset? Not sure but so far the symptoms came back some hours after we took off some fluid.
She did offer to tap the shunt(s) (I assume just the 1) and take off fluid again next wk. Not that I really want to have to have her help again, I really like Dr.Bragg, I really like all my Providers but I'd prefer to not need their help! 

It honestly seems unlikely as I've never had a correlation before but taking off fluid today helped some, but I can't help but wonder 1. regardless if that's all we could get off is/was it not enough (but it really was all we could get off both shunts) or 2. and this is what seems less likely as it's never been the case before (even when we've had better control of HR such as w the Ivabradine, HF/HR med) and only had 1 shunt in place for those months and other times we had 2 shunts in place for instance headaches where still on/off is HR affecting symptoms? 

I honestly don't think the 2 are related as when we do get HR down, such as when I was being paced by the external Pacer after the OHS in Jan. I had the return of headaches then. This said  could /does overly fast HR affect the shunts/headaches?  I don't think so given it never seemed to in past but I don't know?
 I see my Cardiology Team next wk and am going to ask many questions about why Heart Rate has been so difficult to get under control (ranges 100s to 140s mostly since this last OHS, was more high 80s to 100s prior) and even a new med (Ace Inhibitor) since tjid last OHS in addition to the others I was on pre-OHS, re-added) and could that affect headaches and if so what else can we do? Even if it doesn't affect the headaches (my Cardiologist didn't really seem to think so in the past if I remember right) what are options we have to get HR down?

I am tired of meds that aren't seeming to work being perfectly honest. I also am a tad worried about these headaches and not getting them back under better control before Dr.Bragg leaves end of May! 
If I'm really honest, honestly it confounds me how the shunts are sooooo good when we 1st put them in then something occurs,  say the OHS or an occlusion on other occasions and is never as good again till complete (total) shunt replacement is for some reason. I'm not advocating that I just often wonder is there a reason with MPS that complete shunt replacement seems to give better relief than partial (say just a valve) replacement does?
I guess it really doesn't matter right now as we're not headed to any revision but just something I've often wondered when dealing with a shunt seeming issue and I know a question many other MPS-Shunt Parents (whose kids have shunts) wonder to.

I need to remember to ask Dr.Bragg next wk, if we should do MRIs before she leaves.
I actually strongly dislike them (laying flat and still) but know MRIs are typically done/recommended  every 1-2 yrs w MPS I pts and suspect my Genetics Dr in Boston will suggest them. I see him in April so not sure.

Unrelated but related  I have been having hip pain at night which in the past has been low back related, related to shunt. That lokebso many tings though, who knows, is never clear and not as bad as it has been other times. More just curious and a trying to sleep/be comfortable on top of the headaches

Anyways I guess in general related to trying to make a plan re Dr.Bragg moving  I don't know, I want to slow down time bc I don't want Dr.Bragg to leave but other things I am looking fwd to in Spring.
I know Dr.Bragg knows my issues/presentation  (peculiarities perhaps?) and how to help and being perfectly honest no matter which of the 2 Dr's I'd choose will either think outside of the box like she does? Have I ever said I don't really like change (atleast in Providers who are good and get my issues )? - To hard!

Anyways, thanks for stopping by, will try to update after next wks Cardiology Appt.

 Next wk is a little weird as I infuse later in the wk (scheduling per the clinic) and see Cardiology Tues, Dr.Bragg on a non-clinic day Weds.
Thanks for stopping by,


Ps the 2nd picture is part of a comment from 1 of my closest Providers, she is intimately involved in my care and been involved in many of the  lead up to Shunt revisions, making surgeries w Dr.Bragg happen and coord. care happen.
I don't think and yet I know Dr.Bragg's leaving UW has left many of my Providers uncertain in how to replace her and quite aware how well she's  made my care flow. It's neat been neat knowing how much Dr.Bragg has impacted not just me but how many comments I've gotten from other Providers (both prior to knowing she was leaving and since) that they've really appreciated her communicating with them and her as a whole not giving up, helping them when needed and her belief I'm me. AZ is lucky to get her and I know I'm not the only one both Patient and from,Providers if mine who thinks that and is sad to see her leave come May!

I don't often share things my Providers write to me but this was worth sharing bc she's  made an impact not just to me.

Sunday, March 6, 2016

How to replace the seeming irreplaceable? Worry, Uncertainty, Loss

5yrs, it will have been 5yrs I've been seeing Dr.Bragg when she leaves her UW practice  and moves to AZ. :/
5yrs I didn't always trust her enough but 5yrs she never gave up and never stopped trying to help.
5yrs she kept learning about my disorder (MPS I) and 5yrs she worked with my Team in an effort I've never seen in another Neurosurgeon. 5yrs she believed me, became like family and became a Dr I truly respected, trusted and knew wouldn't give up (my trust may have wavered at 1st, yet (and especially ) she never gave up or lost trust in me and stopped trying to help me and I always respected her for this and  continually gave her high favorable ratings to my other Providers. I don't know if others ever did but many asked me if I though she would see other Patients  of theirs.
She was exactly what I needed for my Hydrocephalus and I can't tell you how hard this has been thinking of her moving, of her not being at UW when I have shunt or other neurological issues and as my Mom, who also really liked Dr.Bragg, said (in as many words) 'I can't imagine how much her not being there must scare you'  as my Parents know what it was like before Dr.Bragg came and my struggling to get someone to believe me and take on my shunt issues).- My Mom is right, I try to not think of it but as I've tried to fall asleep many nights I've fallen asleep in tears,  out of worry (whose going to take on and believe me and not give up like she has; is it going to go back to how it was before).
I know worry is wasted energy yet how do I not worry about an issue that isn't exactly my most stable?
I think sometimes about if say my Cardiologist ever left (unlike Dr.Bragg, in Dr.E's case seems less likely) there are 3 or 4 other ACHD Dr's in his clinic I get along with well and who he's  taught some about my MPS).

I HATE WORRYING And yet I am, alot. :/
Last wk, while in San Diego I was so tired and wasn't feeling good so between being busy w the few sessions I did attend (I didn't go to any of the mornings ones though several looked really good and there where others I just was flat worn out/not feeling great so I skipped) but what I did attend was a decent distraction topped with being around the friends who where also there (we often hung out in 1 of our 3 hotel rooms or the 1 night I had dinner with a former Pharma-BioTech friend and that to was very low key.
These where good, needed distractions.
I got back in to Madison Fri night in the snowstorm which was a peach to drive home in (normal 45mins approx drive took 2hrs) and Sat, my Bday ironically I slept, cleaned a little and slept through the rest of the day. Not exactly exciting  but much needed  and helped some.
The headaches and some cardiac symptoms have been kicking my arsh - sort of ironic given how many people said to me how good I looked in pics or they couldn't do what I was doing being at the Conf. so soon after the OHS. (which is a nice compliment even if I didn't feel great much of the time, I am the person that if I'm not puking keeps going, trying to not let MPS stop me to much).
If I never did anything when I didnt feel good seriously I'd probably do something once in a blue moon? I do wonder sometimes if that stops my getting  answers when having issues sooner but I just can't sit and feel bad about feeling bad.
I felt good for approx. 2- 2.5mo after we put the 2nd (VPL) Shunt back in,  up till we did the 3rd OHS and that I honestly think had to do w the surgery  and fluid affecting the shunt but who knows really. 

While wasn't perfect I sure accomplished more many days even while dealing w significant heart issues. Was nice and I pray so hard we can figure that out again before Dr.Bragg leaves. :/
Now? Now I feel like a sack of potatoes or something between the return of some of the heart symptoms (probably due to ridiculous fast heart rate despite the stupid boat load of heart meds I am on) and the headaches, oh the freaking headaches. They may come and go, worse at night but they sure suck! :/

Talking to my Mom Sun, we where talking about Dr.Bragg and her leaving come the end of May, my stressing about who to see (Dr.Bragg's said she'll help and won't let it go back to the way it was at UW prior to her coming BUT I worry, I worry a lot and have spend so many nights since finding out admittedly crying myself to sleep thinking about what will happen when she's gone ). I hate having to rely on someone so much. :/
I don't often let people see my emotions and often when people think they know what I feel they don't but lately my emotions are on my sleeve I imagine.
I am worried, really, really worried about her leaving and will there really be someone who gets what Dr.Bragg did that my hydrocephalus and MPS (which caused the Hydro) isn't a  'normal' presentation' or issues?
Worried and stressed about this is putting it midly and I wonder will it ever be like it was (currently is till the end of May) with Dr.Bragg where she actually cares, gets my issues and doesn't give up?
She's not your typical Neurosurgeon and I worry about this that I won't find another someone like her..
As Mom and I where talking about this the other day, my Mom asking questions about different Hospitals and Neurosurgeons (like where my former, 1st Nrsgn was) and 1 at Dr.Bragg's hospital but who is Adult trained not Pediatric who we liked but are uncertain could that Dr take my care or manage it for awhile till I potentially found either the Dr that's apparently coming late summer or just someone different? I don't know, have to talk to Dr.Bragg.
I don't think it would be good to see her Partner as he clearly doesn't like me and I don't in turn trust him so I honestly don't know.

I'll for sure talk to Dr.Bragg more when I see her in clinic and pray she can help me feel better before she leaves so we have time to sort a longer term replacement. I wish she wasn't leaving though!!! :/ Why do the good ones always end up leaving and my basically perfect team  now has to be put back together? 

In talking to a few of my Providers (primary dr, Genzyme Case Manager, Insur. Case Man. and Endocrine dr) have all said to keep them up to date and as my PCP put it "it is disappointing to hear, she's been a great Provider for your care and will be a huge loss, hard to lose and hard to replace her." - They all essentially said the same and none are certain how to move fwd, we all hope Dr.Bragg can help sort a good plan, with by some chance someone good to replace her. I pray she will stay in touch, she doesn't have to but to have her help and thus support maybe that would make it a little easier. All of these Providers above minus my PCP where there before Dr.Bragg so they know exactly how hard it was before she came to my Team.

I see her in clinic on Thurs so will try to update after and am praying she has some ideas. As she said "we always figure it out" so I hope this time to.

So nervous about this change and scared and uncertain and wish it was a bad dream - she's so good I don't want to lose her and I know for a fact I'm not the only one who thinks this and worries some.
I know God has a plan, I have  prayed repeatedly for God's grace as I continually have doubted him.

I apologize to sound whiny or whatever I sound, just scared I guess.