Pulmonary-Lung Appt (good improvement), 2014/2015 - Happy (almost) New Year!

I try not to be one to put to much stock in 'wishing' or even really hoping (but I still kind of do regarding some things) that things like the shunts will stay working for the long-term. I think I realize my history and so instead try to be a realist and instead of 'hoping' for something to work for a 'long, long time' I simply appreciate when something (like the shunts) keeps working reasonably well day to day and appreciate each reasonably good day as it is. I guess I believe in not spoiling now wishing for something that may or may not happen but in all likelihood will happen (if that makes sense, not sure how to explain what I am thinking).
The reason I share that is today while driving to and then home from the Pulmonary Appt (about an hour drive each way) I kind of suspected the Xrays would show the fluid was even further improved and related to that I was thinking about this whole year, the various issues that have occured but also the various good improvements we've had (whether short or long lived) with various issues. Thinking about all of this just makes me think of the New Year and kind of wonder what it will bring but also renews my appreciation for those things we have been able to figure out or fix even if imperfectly and even if not a likely forever fix.
In any case today I saw my previous Lung dr at FMLH, she is who along with my Cardiologist 1st suspected the MPS or Lysosomal diagnosis 10yrs ago. She opted to repeat the Chest Xrays and 1 other looking at the lungs from a slightly different vantage point. Thankfully these showed the fluid is now "negligible" (Yay!) though initially she apparently had some concerns regarding "reduced breath sounds" on the R side. She thought perhaps the reduced breath sounds where due to this area having been entered so many previous times both w the 2 OHS and with the repeated TPL Shunt revisions so altogether, all of these surgeries creating scar tissue. I know Dr.Bragg has worked w the Peds Genercal Surgeon on the last 2 TPL Shunt revisions so Dr.Biller is probably right in her thinking.
She as am I was happy with the improved fluid as she prior to the Xrays raised the potential she may need to do a test where they insert a needle through the ribs to remove fluid and test it (something my PMD and the other Lung dr had also mentioned, a thoracentesis I think it may be called), definitely not high on my 'sounds like fun' list. Additionally this is a test that would be more complicated bc of the TPL Shunt and Dr.Biller commented along the lines 'I dont really want to get your Neurosurgeon mad at me'.
Thankfully it seems the fluid for now has fixed itself, finally (!!) and hopefully the residual cough which is pretty non-existant in the morning but worsens often significantly by the end of the day will sooner than later go away. Thankfully it's still the case where the cough is improved since prior to the VPS revision as prior to that the cough pretty much lasted all throughout the day. I definitely pray it will resolve altogether at some point though!
As far as any F/up she just said if I need her to call or let her know and she'll see me which like my PMD, Dr.Bragg and Cardiology if I call their Secretary's (sp?) or Nurse they open up Appts much quicker vs going through scheduling. In Dr.Biller's case we email some to (as do some of the Providers above) so I can often run questions by her/them without having to make an Appt or call their Nurses which is nice! B/c I hadn't seen her in atleast 5yrs., perhaps more, I wasn't sure if she would still see me but when I had initially emailed Dr.Biller she responded back saying she'd be happy to and would see what she could do as far as finding a spot "to sneak you in sooner".
I am always grateful when Providers remember me and are great about taking back on my care! My hope is the fluid that had been in the pleural space and which drains from the TPL SHunt will (other than when draining from the shunt) stay pretty minimal and not build up again! It's definitely weird (or atleast it seems like it to me!?!) it took so long for this effusion to go away (over 2mo). Any pleural effusions prior to this i'd never had symptoms with.

FMLH Infusion
I started at FMLH infusion last wk, the Nurses seem nice and 1 in particular who had me my 1st wk (last wk) at FMLH stopped by several times this wk to check in see how I felt after last wks infusion (felt pretty rough, the total volume was almost double what I normally get as the order when faxed over hadn't specified that I get the drug mixed in about a 1/3 the total volume most pts receive). Last wks Nurse took over for the Nurse I had today and finished the last 3 or so hrs of infusion and in general just seems to have an interest in helping sort our new rate/ramp up as she is who spent the majority of the time adjusting the rate Monday so we'd end up over 5hrs.
Thi Nurse last wk once we realized the drug was mixed in to much fluid and my cough was becoming increasingly worse as the infusion wore on slowed the total rate time down. This wk the drug was mixed in the right volume but my PMD had suggested I ask about having the drug (Aldurazyme, ERT) infused in over 5hrs run time vs my typical 4hrs to try and combat the cough (which had in general been getting worse for months during infusion) and to prevent what she and my Cardiologist feel is/was fluid overload and thus lung congestion secondary to the heart issues and a high sensitivity to fluid shifts.
Yesterday's infusion did go alot better, we monkeyed around w the infusion ramp up and final rate to get it run in just over 5hrs (I didnt end up getting home till a little after 8 Monday night after leaving at 9 that morning). My cough though for the first time in months didnt get worse with the infusion and was pretty much at it's baseline by the end of the day! I am awed and grateful the Nurses where so easy going about just switchng the infuse time from 4hrs to 5hrs without needing my PMD, Cardiologist or the prescribing dr. to write a new order!
Hand Surgery
As far as the Hand surgery next Thurs., it seems my Cardiologist wants to talk to my PMD about the INR mngmt pre/post. Since my PMD is out of the office until Monday the plan is to still stop the Coumadin this wknd but then I think he (Cardiology) wants to talk with Dr.Simpson regarding a plan if the INR where still to high when I test Weds prior to Thurs surgery. Hopefully INR will just be low enough and not be like this last shunt revision where it took forever to drop and was still 2.2 the day of surgery! Thankfully Dr.Simpson is great about talking to my other Providers when need be so I am not to worried about things getting sorted out.
2014 - 2015
Otherwise I am signing off this year (2014) with the simple wish that everyone be safe! I am admittedly wondering and curious what 2015 may bring. I pray for decent health and not to much changes (seems silly to wish for no changes as that seems unrealistic) + either for us to figure out something better for the shunts (headaches) OR at the very least the shunts stay stable working wise. It really would be fantastic if we could figure out a solution to no headaches but i'll also jsut con't to be grateful to have a Nrsgn that believes me and Providers in general that work fairly well together!
My wish and want is the same for the heart issues, to both feel better and as well for there to not be any big changes in this next year that require us to go in again and have to replace the next valve. Perhaps I might wish for things to stabilize or that we could figure out a better med regiment that had less overall side effects? I can wish, right!? =)
And last related to those 2 things I hope the fluid issue related to the TPL Shunt and the heart issues stays stable and the cough improves, ideally completely once and for all!
Blessings for 2015 to be a good and kind year to each of you reading this!
Erica

Merry Christmas (Our Savior is born!), New Infusion site, MPS Conf.

First let me say I hope each and every one of you had a blessed and joyous Christmas! Whether you believe in Christ or Heaven or not I know the comfort and reminder of Jesus birth each Christmas although it took place so long ago is a great comfort and solace that my sins are forgiven and a better place not of this earth awaits me!
This post has been a week or so in the writing both partially while in FL and then on and off since then - so my apologies!
Although like any family we have our here and there arguments or disagreements I am generally blessed with great siblings (the majority who come around) and blessed to have seen all but 2 of mine this Christmas days. I am continually reminded especially how thankful I am my Sister and I remain close. I am thankful my nephews Mom lets him come to our house and he still wants to and that my oldest brother is again part of our tradiions which then means seeing my niece!





Today (last Monday) I started ERT over at FMLH which went ok, I had my own room and was able to sit in a regular desk type chair though clearly the Nurses thought I was a little nutty for wanting to spend 5+ hrs in that kind of chair vs reclined in 1 of the infusion chairs (which I find really hard on my back and neck). =)
My Nurse and the others where nice and despite a few problems (when the order was faxed over my Genetics Team hadn't apparently noted the total volume so the Infusion Clinic Pharmacist went off of what the Aldurazyme insert says which is quite a bit more than my normal (250ml vs my normal 150ml fluid volume total). They opted to slow the infusion a bit but I was still able to get home by 5:30 after starting at 11:00.
Probably the thing that will most annoy me is they do vitals every 15mins, every time they ramp up the infusion rate and then every 30mins which is TOTAL excess when you've been on the drug for 9.5 yrs and even the MPS treating centers do it far less than that! At CHW we did vitals at the beginning, occassionally in the middle and at the end. I have asked my GC to re-write the Aldurazyme rx to be based on weight given the drug will no longer be paid for ahead of time and then shipped from a Specialty Pharmacy but instead the hosptial orders it and bills my insurance. Billing this way is totally so they can do a huge drug upcharge - what cost my Insur., approx $15,000 a wk or less will now no doubt cost double that unless the Insurance put in place a fixed price per vile contract which is also possible. If anyone ever wonders why our healthcare costs so much in America...
Otherwise the curious aside was the main Nurse I had kept commenting throughut the day (and I mean she literally kept questioning me about it) about the cough which was very mild when I got there around 10:30 and full out sounded like a mix between a lifelong smoker and a deep hacking/barking cough by the end that was and still is continous that night.
Because this worsening during ERT has been going on for months now although I hadn't really thought alot about it before the Nurse kept asking about it today but I emailed my Neurosurgeon (I didnt hear back which I don't always) to see if she had any thoughts. I also emailed and did get a partial response from my Cardiologist with more questions of his own. He first commented if the infusion was being given fast and alot of fluid he felt it could be causing "your heart pressure to raise leading to congestion of your lungs, and your cough". - I obviously have noticed the cough in general and been asked more times than I can count "are you ok", "do you need a cough drop", "do you need some water" or "are you sick"? All from well meaning people.
Because it's not hard to do and I try to keep the 3 or 4 Core Providers especially on the same page (and they so often work closely together) I had sent a message to my PMD regarding this infusion fluid seeming issues. She in-turn also has concerns it is to much fluid being given to fast - in most pts. she commented 150ml's isn't really alot of overall fluid, especially over 4 hrs but she feels my body is extremely sensitive to fluid shifts and asked if they (Genetics, Infusion clinic) could run it in over 5 hrs instead of over the current 4hr time period to break up how much fluid is being given each hour. Wouldn't be ideal to have to go longer though I do know others (for very different reasons, usually to maximize enzyme exposure) who go over 5hrs or longer.
She thought bringing this up to my Lung dr. may help as that dr is in the same Health System as my Genetics Team. I see Pulmonary next wk so have written this down, fwd'd my PMD's response to my Heart dr. and will talk to the Pharmacist and Nurses at ERT next wk. It seems from yesterdays infusion the Pharmacist seems pretty amiable (she is assigned solely to the Infusion clinic which is nice!) and the Nurse was willing to turn down and run yesterdays rate slower so perhaps they would be willing to do it on their own. Guess we'll see.
I really don't quite understand why so little fluid (from the infusion) when I do take rather large doses of meds to get fluid off is causing such increase in symptoms?!? Overal my headaches while imperfect are still better than pre-the VPS (shunt) revision as is the cough to some extent but that said the cough is still utterly terrible many days especially by the end of the day! I've come to the conclusion vs sounding like a smokers cough instead it sounds like a barking seal and altogether annoying! I can understand I guess why it would get worse as the day goes on given the TPL Shunt drains more when I am standing up and thus the longer I am up the more fluid that probably collects in the pleural space causing the irritation.
I just keep praying the cough will resolve and the fluid will start to absorb better?
The side pain over/by my R ribs and under/to side of R breast is pretty near a constant with some fluctuation, I often unconsciously find myself holding my side, almost as if to put pressure on the uncomfortable area without really realizing i've been doing it. Odd is the feeling for sure! I guess we'll see what the Lung dr says next week regarding all of this issues (cough, pleural effusion/fluid and ERT rate, etc.). I didn't hear anything further from my Cardiologist after answering his questions which is a little odd (that he'd ask those questions and not re-respond).
Included below is part of the recent Echo results, which as you can see are not to bad, the 2 changes are the L atrial (moderate) enlargement and the R atrial (mild) enlargement both of which are new from 3mo ago. These issues likely go along with the on-going cough and fluid balance issue between the TPL Shunt and Lung/heart and as well seeming even increase in cough/symptoms with ERT (infusion) though I don't get a large amount of fluid and is given over 4hrs.
We did deal w Severe L atrial enlargement prior to the 1st Aortic valve and subsequent 2nd re-do Aortic valve replacements but that had went away not to long after the 2nd surgery. The mitral valve atleast hasn't worsened since the Echo last Febr which showed it increased from a Mild to a Moderate leaking and narrowing.
Summary: 1. Hurler-Scheie syndrome. 2. S/p Kono procedure and aortic valve replacement with a 21 mm Flex ring St. Jude valve. 7/23/2013. 3. S/p subsequent re-replacement of mechanical aortic valve and closure of residual VSD on 9/3/2013. 4. No residual ventricular septal defect. 5. Trivial aortic valve stenosis. 6. Peak aortic valve gradient (A4C) = 14 mmHg; mean gradient = 9 mmHg. 7. Mild aortic valve insufficiency with no evidence of paravalvar leak. 8. Tethered and thickened mitral valve. 9. Moderate mitral valve stenosis -unchanged from previous study.10. Mitral valve mean gradient = 8 mmHg.11. Moderate mitral valve insufficiency.12. Moderately dilated left atrial cavity.13. Mildly dilated left ventricular cavity.

Otherwise I got back from the National MPS Society Conf. Sunday night which was at Disney World in FL (Contemporary Resort). The Foundation I work very part time for paid my expenses (flight, hotel, food) and was a lovely Conf. seeing both these 2 whom I work for (although they ended up having to leave early on Fri) and many other friends.
The Adult Conf. was defintiely heads and tails better than any of the 2 previous ones which was nice! I do think there's still alot more that can be done for Adults to bring more in to the Society especially at the regular Family Conf. which is not at all geared to those who are actually affected by the disorders but still overall it was good.
I toured Downtown Disney Thurs morning (I got in Weds early afternoon) and after Conference related stuff Thurs afternoon/evening, and most of Fri and then Sat. morning I went to Epcot with 3 friends (also adults 2 MPS I and a friend of ours w MPS II) and the 1's Dad. Very fun! :) Epcot like Magic Kingdom 5yrs ago was definitely not what I had pictured it as but we had a blast taking pictures and just seeing parts of the park. Overall we did probably less than a 1/4 of the total park but was fun and enough.
At the Conference as happens here at home I got quite a few compliments from friends and families who knew my story when I was first diagnoed and the often extreme struggles I had with many Providers getting answers. Now I often receive high praise especially for "how dedicated your Neurosurgeon is to your care and persistent" which I do hope Dr.Bragg knows that means alot to me! I am glad other families can see you don't always need an so-called "MPS Expert" to handle your or your Child's care to get great care! Maybe 1 or 2 of my non-core Providers are "experts" but non of my Core Providers are and yet I have the best Team of Providers i've had in years!
I'll try to get around to updating the slideshow with new pics sometime soon.
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I hope each of you reading this has a very blessed Christmas, I am hoping our families celebrations are calm and without much anxiety (on my nephews part) as well I am looking fwd to the Christmas Eve and Christmas Day services.
I'll update next week after the Lung dr appt.,
Thanks so much for stopping by,
Erica

3mo Echo, Cardiology Appt., Sunday School pics

Today's Cardiology Appt went ok, mitral valve remains stable at the high moderate leaking and stenosis but he'd prefer (and I am certainly ok with!) we con't to wait to intervene as long as possible. I think if I remember correctly he said the mitral valve remains stable from the increase in stenosis and leaking last Febr. (high moderate) and I think he said the atrium is enlarged and he felt that was due to blood returning (abnormally) backward from the mitral valve causing stress/pressure. He showed a diagram how the TPL Shunt fluid can be affected by the heart valve issues/enlargement (or maybe it was vice versa but I don't think so) and explained how he felt they all tied in to each other.
As far as the fluid in the lung space from the TPL Shunt he to feels as my PMD and Nrsgn do that it is multi-factorial. Unlike last appt though he seems to have changed his mind about the heart issues affecting the shunts and the shunts affecting the heart issues (was never rocket science to me given the hydrocephalus/pressure affects autonomic system and that controls things like heart rate, BP and breathing).
One thing I don't necessarily agree with him on though is he feels our cont'd issues with high heart rate are somewhat related to the shunt issues which I do not think is the case. I personally feel like regardless of when we get the shunts in atleast a reasonably good place my Heart rate remains high but then again it isnt as if we ever get complete headache resolution with the shunts. So I guess who knows?
He did make a comment regarding the shunt c' cardiac issues that 'I understand this better than I used to' (see above comments) which I am glad he admitted that and appreciated his saying as much. I've just always felt how couldnt a brain issue affect cardiac and vice versa? I also reaaallllllyyyyy appreciate that he, my PMD and Dr.Bragg work together (despite all 3 being at 3 different health systems) and try to go to each other coord. my care/questions vs when 1 may not know something trying to just wing it!
I am not sure who they see (but perhaps Dr.Bragg as I know his Nurse at one pt had asked if I thought she would see another pt of theirs and many of my Providers think very highly of her (given they know the extreme struggles I had prior to her to find a Nrsgn that would listen to me and believe me), but he commented with 2 other pts of his who also have shunts he's witnessed and is what changed his mind that when the 1 pt atleast had an EVD (external shunt) she had headache resolution and her heart rate was back within normal range. When she has had shunt issues but no EVD her heart rate to is high regardless of meds. Funny enough (perhaps only to me!) he like Dr.Bragg commented that it would be ideal for our heart issues if I could have an permanent EVD (I had commented how Dr.Bragg and I used to talk frequently about how much better I felt with EVDs compared to shunts) - I can't remember exactly what he said but something similar to what Dr.Bragg has said numerous times about how w EVDs the settings can be continously adjusted. I have a feeling they must work together with other pts and that she has (if they do) taught him a thing or two, which if that is the case I say AWESOME!
He still feels the many cardiac meds I take are needed regardless of what is causing teh high heart rate/strain on heart (multifactorial) but is partially it seems what influenced his change of opinion. I thought this was interesting given some months ago Dr.Bragg felt that there was a connection between the mitral valve having worsened and our on-going issues with headaches.
I am glad the cough isn't a big concern to my Cardiologist and while it's not the end of the world to me I DO have to say i'd gladly give it up. By the end of the day my chest and mid-back ache something fierce from coughing. This is saying something given the cough IS even improved from pre-this last VP Shunt surgery!
I don't particularly think my Cardiologist likes the fluid being there but I also don't think he was overly concerned although I also don't think it's occured to him prior to 2mo ago if there where periods where there was pleural effusions (fluid in lung space) I didnt have symptoms with it nor did I ever have this cough except after the 2nd OHS (which wasnt related to shunt). He was talking about Dr.Bragg (I can't remember exactly what but somehting to do with the heart stuff and the shunt stuff) and commented how he'd read he latest clinic note and I commented she seemed happy the fluid was reduced and that we both thought it was just another sign of the shunts complimenting each other. I've not read her note yet but he made the comment he wouldnt call what she wrote "happy" with the improvement but more "relieved" (I think that was his choice of words).
Either way we know it's an imperfect situation but better than it was and I HOPE the fluid will eventually (sooner than later preferably) resolve on it's own or perhaps just not build up as much so these secondary symptoms could improve? I realize given the TPL Shunt does drain to the lung space there will likely always be some fluid but that doesn't mean the related symptom are normal!

I've been thinking about some things since Weds' Neurosurgery and PMD Appt., perhaps more out of curiousity for the future but would raising the TPL Shunt a bit higher (I think the setting is around 50, lowest it can go is 30) and lowering the VPS setting a notch (though Dr.Bragg wasnt sure offhand what the setting was ie is it still set at it's lowest, pre-surgery '30' or is it at '40' the 2nd lowest setting.. Non-the-less the reason i've been thinking and wondering about this is the cough is very perstent as the day goes on and so while it's for sure improved since since the VP Shunt revision it is also still a problem. I don't know if we have room to adjust the VP Shunt down so we could maybe adjust the TPL Shunt up but this cough is fierce by the end of the day (pre-surgery it felt like an atomic bomb going off everytime i'd cough by the end of the day, now it just feels like a grenade (the force and thus discomfort I guess) by the end of the day as my mid-back and chest are ridiculously uncomfortable. The cough is just semi-consistent all throughout the day occuring more and more frequently as the day wears on. Pre-VPS surgery it seemed to just occur consistently at a much fiercer degree (again thats saying somthing given it now!)
My other thing i've been wondering about is will the fluid (CSF) eventually go away on it's own and thus the cough clear up? That may be a question more for my Pulmonologist (Lung dr.) when I see her the end of the month. Not urgent, more just curious questions for down the road and the fluid/cough question b/c well by the end of the day my chest is pretty darn uncomfortable.

My Pain Mngmt dr. semi-agreed (didnt disagree just felt my Primary dr could prescribe it) that a oral steroid burst may help the seeming sciatic nerve symptoms - Dr.Simpson in turn did just that and called in SoluMedrol dose pack which i'll start this wk.
Dr.Bratanow did talk about potential steroid injections and still does not feel this is a good way to go as her concern is scar tissue and the TPL Shunt (both potental for introducing infection and she is concerned the sciatic nerve symptoms are in some way related to the TPL Shunt irritating a nerve or related to both shunts though she isnt sure how this would be. She'd like to order a ultrasound to look at soft tissue in lumbar spine as she is also concerned there could be something similar causing this like occured with the nerves that where adhesed together prior to my 1st surgery with Dr.Bragg. I don't know, I just know for whatever reason these sciatic seeming symptoms where not as aweful when the VP Shunt wasnt working well and although the cough/lung fluid improved w the VPS revision the Sciatic nerve symptoms amped up big time. Literally I can be standing in place for as little as 1-2 mins and the symptoms begin and only get worse there out.
Last but not least - I am off to Florida to Disney (MPS) Conf. tomorrow, a little worried how tired i'll be but I should also have plenty of down time and I have all afternoon (I get in around 2 Weds) and night to do my own thing and most of Thurs as well. Instead of going to a Park Sat. afternoon i am most likely giving my Park ticket to a friend but not completely certain on that yet. I just think it would be to hard and uncomfortable to stand in long lines on back issues (which sucks a little actually!). Non-the-less should be a good Conf. =)

On a totally different note albeit i've had about a MILLION people ask me about the cough and if I was ok i've also had family and friends who know me well and who commented they thought this was the best i've looked in a long time s far as the headaches goes! =)
Attached is a couple pics from this past Sundays Sunday School Christmas party (I did a lesson the 1st 40mins of class).
Thanks for stopping by, will update on change in ERT (Infusion) clinic (=/) next update,
Erica
'SS Kids and their gift bags; an ornament and photo album (pics from this past semesters class activities)

'The meaning of the Candy Cane' craft - (very neat)


Our Christmas baking (part of it, I just decorate, lol)

Peds Neurosurgery (surgery) F/up, PMD (Hand) Pre-op Appts.

So what a day filled with ALOT of information, not earth shattering information but non-the-less alot of information to think about and store away or remember! In my PMD's case she writes down her thoughts/plan/suggestions at the end of Appts on the after visit summary and with my Peds Neurosurgeon I write myself notes as I walk out to my car.
I guess i'll just start at the beginning with the various things each Provider and I talked about!?
Dr.Bragg, who I might add is so sweet, while I of course am human and doubt her (as with anyone) at times she continually proves she cares and is always trying to think of better options.
I know or think in the back of her mind she still weights the potential for the cranial vault expansion but I think she must 1. sense my hesistation and 2. want to try to avoid it if we can or for as long as we can by considering other options?
She talked to her Codman Rep and although I am not sure if it is actually a Codman valve Dr.Bragg mentioned there is a new valve she may consider next time we have a valve occlusion issue (not if but when, I thought that was kind of funny) that she said is closer to the Strata programmable valves we first started with "and I know we didnt have very good luck with" but this valve has something different about it and she feels might be a good option to try. One downfall she mentioned was it has less setting options than my current Codman Hakin valve(s) which she seemed to have a bit of concern about but then at the same time we do really seem to only use the bottom 3-4 settings on my VP Shunt so who knows? I am open to trying it if/when we need to down the road if she feels it's worth a shot. Worst that happens I guess is we go back to what I have now. She commented in that conversation she cont's to ask her Codman Rep to keep an eye out for something similar to what we have now but better. =) I think it's sweet she seems to think about our issue a fair amount.
I thought that conversation was pretty funny given at the appt this afternoon my Primary dr was asking about and talking about the VPS surgery and the Pleural space fluid amongst other related things and she un-related to Dr.Bragg's comment said to me (along the lines): "I remember the 1st time I saw you for a shunt surgery pre-op, saying to you something like "well this should get you to feeling better again for a longtime and you kind of smiled, nodded and didnt say alot." She then commented along the lines "I now know your normal is to need on-going shunt revisions and for you it isn't unusual". LOL!? =)
Glad atleast she doesnt question my symptoms or sometimes lack of appearance of symptoms (given the time of day I most often see her) and that she goes with the flow and has been amazing at marshalling my other Providers regarding what information is needed for shunt surgeries (ie Coumadin and med adjustments per Cardiology, Stress dose Cortisol per Endocrine, etc). I like her and she's very good at what she does!
She's good at stepping in when she feels something needs to be done but not afraid to give the reigns to another of my Specialists if she feels they can or should handle the situation (whatever it may be). Most of all she's personable and she herself likes (especally) my Cardiologist and Neurosurgeon (her words today) which is good since she works with them some on my care.
With Dr.Bragg's Appt this morning we where talking about the Pleural Space fluid (hot topic these days) and she similar to what she had messaged me the other night commented along the lines "it's interesting how we seem to have another way to know when your VPS may not be working right and that the TPL Shunt causes such irritation" (meaning when it's the sole functioning shunt). I didn't think to ask and wish I would have but why the TPL Shunt would suddenly start causing such irritation to the pleural space and thus the cough and other on-going symptoms? Likewise I didnt think to ask if she thought the residual cough might eventually go away completely?
I think from what my Primary dr has said from the 2 of them (Dr.Bragg, Dr.Simpson) having talked there seems to be consensus that it is partially due to the cardiac issues but I wish I would have thought to ask for their thoughts on this more!
Regarding the INR issues with this past surgery we've formulated a new plan; instead of waiting to test the morning of surgery or just testing the level the day before but then cancelling any surgery if it where to high she suggested I test at her clinic-lab (or if already in Milw test there but make sure she gets the result) and if INR where to high she could relatively easily prescribe a dose of Vit. K to bring the level to range for a surgery the next day.
We'll try this in Jan. with the scheduled (out-pt) Hand surgery. I'm not sure if Dr.Bragg will follow a similar suite in any of her future potential surgeries when/if I am admitted the morning of surgery but I would imagine she may.
On my part it seems I also screwed up the Coumadin dosing for this last surgery, I had thought I was supposed to stop it 3 days prior to surgery (so that Monday for Thurs's surgery) but in fact I should have taken the last dose that Sunday and then skipped doses M-W for Thurs's surgery. I'm not sure the level would still have been in-range but it may have been. Atleast on my part good to know what I in fact should be doing for the future!
Because Sciatic nerve symptoms in my L leg left have been pretty bad again (standing for as little as a minute or 2 in one place is aweful and I am frequently waking up at night) she suggested I ask my Pain Mngmt dr about a possible oral high dose Steroid burst to see if this might help bring inflammation down. Several months ago we had been considering doing some kind of invasive injections in the area Dr.Bragg suspects (but isnt completely certain) may be the cause. Dr.Simpson thinks if we try a high dose steroid similar to what I take before surgeries/procedures or when sick (self injection) but with a different med it could potentially help the symptoms. This would be ideal to prevent anything invasive and thus potential for introduction for infection in areas around the TPL Shunt. My PM dr is a Anesthesiologist by training (amongst several other board certifications) so she'll have knowledge of this. Dr.Simpson was/is willing to prescribe it she just didnt want to "step on any toes". I can appreciate that.
Because there seems always to be many things going on she's watching the fluid around the pleural space (but like Dr.Bragg very happy it has reduced and like Dr.Bragg thinks it's interesting how the 2 shunts seem to play off each other ie when both work symptoms improved some. Pulmonary will con't to monitor that and we all hope nothing invasive is needed as far as pulling off fluid (to test or just to remove fluid)!
We pretty much covered every area from head to toe and talked about stomach spasms i've been getting again which is something in the past (atleast this particular type/symptom) i'd only gotten when I had some type of infection. I am not to worried about it as what's one more issue (right?) as long as I can get it to go away within a few hrs each time (is kind of gut wrenching, feels like your abdomen or stomach is being twisted in sporadic lasting knots that slowly subside each time). She asked that I keep her updated on this ie if it doesnt go away in a few weeks or gets worse; her concern is is there an infection somewhere albeit like w the pleural space cough/fluid I don't show symptoms of anything and CSF was clear from this last surgery (Dr.Bragg released her labs on her systems mychart). I think perhaps Dr.Simpson's bigger concern is with any infection/cold I rarely have fevers (probably due to abnormal autonomic issues) but even so I don't think she's to terribly concerned for now.

Dr.Bragg just said regarding f/up (after we hugged and celebrated my not seeing her over Christmas either right before, during or after for the 1st time in the 3 yrs i've been seeing her!) that she'd be around for the most part should I need anything. Hopefully I won't! It's sweet she cared enough to share what she thought her schedule might be.
Dr.Simpson pretty much said the same, keep her updated on various symptoms and if nothing else we'd talk the day before the Hand surgery.
A week from today I should be in FL at this time! And a week after that is Christmas which is what I am really most excited for; I love seeing my nephews and nieces faces!
Thanks for stopping by,
Don't forget to appreciate those people in your life whoever they may be who help in big and small ways!
God Bless,
Erica

CXR, TPL/VP Shunt (appreciating Provider openess and honesty)

I'm just going to say I love how alike my Peds Neurosurgeon and I think on some things, in this case how the 2 shunts function and aid each other - the TPL we've always considered to be the stronger, better functioning of the 2 shunts and seems this latest VP Shunt surgery proved this once again.
I wrote about the Pulmonary Appt last week and Chest Xray (CXR) results showing 50% improvement in the Pleural effusion (fluild build up in area around L lung which is also the space the TPL Shunt drains to). To coincide with this since the VP Shunt surgery and although the cough has cont'd to be pretty persistent throughout the day it is still at about a 50% improvement from prior to the VPS revision. (that is saying something given how many of my ERT Nurses have commented "that cough really doesnt sound good" and "if only you could get rid of that cough now" b/c it's there consistently throughout the day. Still the improvement I did get was nice - now instead of a constant (sounding like) smokers cough I just have that hack about 25% of the time and the rest of the coughing time is milder/not as deep.
I emailed the most reecnt CXR results (released on my PMD's MyChart system) to my Neurosurgeon and Cardiologist and my Nrsgn commented back (which is almost exactly how i've described the function of the 2 shunts to other Providers/my Nurses/family/friends):
"I suspect it improved because your other shunt is now working, putting less stress on your thoracic-pleural shunt. Kinda proves our theory that they work together but don't quite add up to two shunts. Just my initial thoughts."
I love that about Dr.Bragg, that she's so willing to share what her thoughts are and really when it comes to the shunts, either b/c i've learned from her or for whatever reason we think alike on areas. =)

I see her in f/up Weds morning (bright and early as it turns out) and then my PMD that afternoon (Hand pre-op) which perhaps less than ideal the time between the 2 appts BUT still better than making 2 trips to Madison on top of today's trip to Milw. for ERT and Thurs's trip also to Milw for an Appt!
There's been some question on my part about a ugag MPS test (test the level of glycosaminoglycans in the body in pts with MPS I) that was done and 2 drs in the MPS Community commenting the wrong test was ordered and results that we got wouldnt be accurate. My PMD asked that I bring the results from Genetics with me so pehraps she can help sort this out.
Next week in addition to the usual ERT (monday) is a Cardiology Appt on Tues (whch btw INR by some miracle was back in range today just 2.5 short weeks post the VPS surgery! Amazing given all the stuff given to bring INR down that Thurs when surgery was post-poned to Fri.) and then I leave for FL Weds morning for 5 days (Disney-MPS Conf.). =)
Will update after both appts if there's anything to update about.
Thanks for stopping by,
Erica
My Sunday School kids and I did the ceramic ornaments (stars for the star that lead the shepherds to Jesus in the Manger) as a craft though our lesson was unrelated.
The kids enjoyed it and something they'll have if they wish forever.
And last my tree this year - pretty simple :)

Pulmonary (Lung) update

I reached out via email to the 1st Pulmonologist (Lung dr) I saw 10 yrs ago last week, she is a dr. I started seeing shortly after I started seeing Dr.Earing (Cardiologist, he had sent me to her when lung issues where found on some of his testing) and i'd seen her for some years after that.
For a while b/c sleep issues/sleep studies where also being monitored I followed with her colleague and then for the past few yrs Dr.E just monitored with testing the Restrictive Lung issues as it wasnt really a big issue.
In any case because of the on-going pleural effusion and symptoms my PMD had wanted a Pulmonary consult again to make sure the fluid in the lung space wasn't caused by an infection (no real signs of this and right now the only real on-going symptoms are a persisten cough and the past day to today a little discomfort when I cough in my R-sided back and chest).
Earlier this week at my PMD's wish I saw a Lung dr at Dean, which is her hospital system and seemed to go fine. Per that Chest Xray compared to the CXR and CT Scan about a month ago the fluid was down by about 50%. I honestly suspect it varies day to day given somedays the cough is very mild and other days I rival a smokers deep hack. It is odd though given prior to a month-ish ago I had no symptoms from this and no cough and now can't seem to shake it. To quote my PMD in a MyChart message she sent after the Pulm. Appt and regarding potentially switching back to Dr.Biller at CHW/FMLH she commented "I am fine with this, thankfully no procedures are needed for this yet" - alot of confidence it will resolve on it's own for good, lol? I do trust this dr. just thought this was a funny comment.
Because that Pulmonologist wanted to keep tabs on the fluid and b/c my former Lung dr. was more than willing to see me again (she emailed back) I am going to go back to her and can see her the end of Dec.
She also could get me in the 17th of this month but I leave for FL for the Disney Conf. for 5 days then. Given Dr.Biller is at the same hospital as my Cardiologist it just seems like it would make sense to go back to her? My PMD and I guess if I understood her correctly my Neurosurgeon to feel it is likely a combination of the TPL Shunt fluid draining and cardiac issues causing the fluid to sit in the pleural space (where CSF drains to) causing the symptoms, so seems like it would make sense to me to have those 2 drs (Cardiologisy, Pulmonary) at the same place? My PMD was ok with this plan as she is good about reaching out to any of these drs when she needs to and when they need to they've reached out to her. Most send their notes to her as well.

Otherwise next week I don't yet know what's the schedule/situation but supposed to see Dr.Bragg for surgery f/up but per her Secretary she may have jury duty (I think thats what it was) and Anna would know today if she was going to or not (I forgot to call till late this afternoon). - So either i'll see Dr.Bragg or one of her NP's for f/up. I have appts on Monday (ERT, normal) and on Weds (Madison, West side) and Thurs (Milw) so am kind of hoping whatever the case is when I talk to Anna Monday we can perhaps make Weds work or I can shift something around to Tues or Fri with them and the other Appt.
Atleast I can say while not totally perfect I have been feeling pretty good, hopefully it lasts but at the same time I just enjoy each good day with minimal pressure headache that I get!
Thanks for stopping by,
Erica

VP Shunt surgery (revision, #32 or 3)..Discharged

Surgery which was delayed from Thurs to Fri due to to high of an INR was post-poned to Fri morning but finally occured! Because of the to high INR Dr.Bragg opted to admit to the floor (Neurosurgery) and 2 bags of the fresh frozen plasma + a small dose of Vit K. brought INR, which is the blood thinner level to 1.3 by Fri. morning. I needless was relieved and soooo glad to just be getting this over with!
Aldurazyme
(ordered by the floor Pharmacists and given while in-pt.)

Surgery itself was really short (apparently about 20-30mins) as Dr.Bragg ended up finding the problem right away at the proximal (top) part of the VP shunt (the part that goes in to the ventricle in the brain). I'm not entirely clear but I think she just replaced the valve as she said when she initially tapped the shunt pre-incision there was some flow, albeit sluggish.
Once they had the site open she said there was very little spontaneous flow and clearly a occlusion albeit she felt a partial occlusion. She also commentd to me when we talked that after they changed the valve the CSF flow was much more normal. She cont's to feel it is our "Drain, collapse, pressure builds, ventricles are forced open, drain, repeat" that causes these shunt occlusions. Given symptoms I can't really disagree.
Non-the-less I am just extremely grateful she was able to do whatever she did to be able to get me admitted over-night and do surgery Fri (vs having to wait till this wk or longer). I hope she really knows I appreciate her effort on my part AND not giving up when I know she wasn't entirely certain my symptoms where due to this shunt/shunt in general.
Talking earlier Tues night before I was discharged Dr.Bragg did make the comment along the lines of "In the future we know now the symptoms you had are likely a partial occlusion and not to let it escalate so much". I think she meant perhaps that while I often looked fairly normal as if feeling fine the appearance can be deceiving. I do get comments albeit well meaning from time to time on "are you still having headaches" I think b/c very few people see me at the worst points of the day.
I know people rarely see me when I feel badly as I try to schedule most appts in the afternoon or at the very least late morning and I rarely am around people at the times of day that are my worst.
I didnt really expect going in to get complete symptom/headache relief which I did not BUT I am grateful to be feeling somewhat better and while I do hope someday we can figure out something that would give full headache/symptom relief I also really know to appreciate the improvements I did get with this (revision) surgery.
I think really, atleast Dr.Bragg seems to believe me, to want to keep trying to help which snyone who has followed this over time knows is a fear of mine (Dr.Bragg at some point not helping. I am my own worst enemy when it comes to believing people and that they really arent going to give up!

Anesthesia
Apparently there may be some Anesthesia-airway changes as Dr.Bragg had mentioned something about Anesthesia and the Anesthesiologist (who'd had me atleast once before though admittedly I didn't remember her) mentioning that she felt my airway had narrowed further since the last time she intubated me.
As far as what Anesthesia uses each surgery they tend to follow similar approaches used in the past whether they use a Glidescope to better visualize and get the endotracheal tube in or fiberoptic intubation, etc. I am not entirely sure what the differences are.
I guess as far as this surgery they had a difficult time getting a secure airway though thanks to the glidescope instrument used other than being dry I didn't have a sore throat though which is always nice! I know my airway has already been a problem so hopefully this won't mean we can't still do the light sedation and airway bagging while they work to get the endo tube in. There are some instances where a Patients airway is so narrow that they have to literally be intubate while awake - could you imagine? I do think from what Dr.Bragg said it may not be to bad if any change but something that may vary surgery to surgery?
I do know from what the Nurses and Dad said Anesthesia/intubation took alot longer than the actual shunt revision due to issues apparently. Per Dr.Bragg she wasnt sure if airway was in fact changed or was the Anesthesiologist remembering my past surgery with her different than it had been.
The concern was apparently a tighter/stiffer jaw and reduced mouth opening as they tried to sedate/intubate.
Although they still did not shave alot of hair with this surgery and it is growing in already I must say this was by far one of the most noticeable 'cuts' i've gotten with a revision. I am not sure if that is b/c my hair is so much shorter in general but I am hopeful it will keep growing back at the pace it is over the past almost week!

On a side note although the TPL Shunt has been annoying no doubt due to the on-going cough (a month or so now) but I am VERY grateful at the same time it is draining so well as that shunt meant the different between feeling pretty bad over the last month but still able to function (modify when or how I did things) whereas I am very positive without this TPL Shunt I'd have not made it and would have ended up in the ER. It seems also surgery may have helped for whatever reason a small bit with the actual cough. I still have it, worse as the day goes on but no where near as bad as it had been. Odd!?
I do see the Pulmonary dr comng up and while symptoms arent perfect they do seem to have improved some with this revision, not sure why that would be but I won't complain!
The night before and after surgery they wanted 02 (oxygen/heart rate) monitoring which I must say is nothing short of annoying but curiously the O2 (oxygen) level would frequently dip in to the 80's no matter when we adjusted or changed the finger probe. The beeping eveytime heart rate or 02 went to high or low was nothing sort of annoying!
One of the nurses i've had before was asking about the cough pre-surgery (the night before) and made the comment factoring in the cardiac components along w the TPL Shunt that the symptoms I was having made sense "with those issues the fluid has no where to go". I guess I hadnt really thought of it like that but perhaps true.
Otherwise fairly uneventual surgery/stay, happy to be home and know of 1 or 2 things to square away (better plan) for future surgeries.
Thanks for stopping by,
Erica P.S. the # of shunt revisions all minus 3 has been in the past 3.5yrs, the prior 3 where since 2006 when I was first shunted - kind of crazy isn't it? And yet for anyone who wonders perhaps you can see why I am so grateful for Dr.Bragg for her persistence especially in times I know it wasn't all that clear it was the shunt
The best visitors to have - my Sister, Mom and Niece and nephew came Sat.

VP Shunt surgery delay, admitted for INR pre-surgery.

Today's surgery was cancelled due to to high of INR (how thin the blood is) which really sucked both since surgery had been rescheduled once before and bc I was already in pre-op. I knew whatever an INR draw showed yesterday though wouldn't represent today since my last dose of Coumadin was Monday so had figured it would be within a 'surgery safe' range atleast by this morning, 3 days off the med. Not so much, the level was 2.2 and "safe" is 1.5 or under it seems.
Thankfully once my Neurosurgeon came up to Pre-op she was able to sort out a alternate not quite bad plan - she was able to admit me to D6-4 (Neurosurgery floor) with the plan to give Plasma (FFP) and Vit K this afternoon in order to bring down the INR with surgery as an add-on case tomorrow (meaning we won't know a time and could be anytime during the day).
It definitely sucks to be here another day and for surgery to have been cancelled but grateful Dr.Bragg was able to be flexible and work out a plan to go ahead with surgery.. I just want it done!?!
Admittedly and this is quite stupid but this is the shortest I've ever had my hair during a shunt surgery so that part makes me a bitt nervous ie how noticeable is it going to be (the incision, small shaved area, like I said stupid) but non-the-less feeling better would still be better..

"I have a hard time sometimes explaining my Providers (and frankly how annoying it is sometimes) to family and sometimes friends and yet how in many aspects these same Providers have been great for my care and are very good on my Team. I know my Mom doesn't hold it against me but dang I seriously was not the nicest (lost my cool, said I wasn't talking about it anymore in trying to explain something when I could have just said something differently in a better tone) and admittedly we both where probably over tired and frustrated about surgery being cancelled again but still on my part not one of my awesome moments! There's reasons I am not a morning person I guess.
The reason I share this really is one of my Pastors from Church had emailed this morning regarding surgery and postponement and said something along the lines of "God most certainly given you a lot of patience. You may not agree with that, but trust me...coming from an outsider's perspective, you have been blessed with a LOT of patience. What an example for the rest of us to learn from!"
I thought that was ironic to read after feeling so frustrated this morning but at the same time I sent back a message saying I appreciated the kind words and vote of confidence! It's nice to know people believe in you I guess or think that you deal with things well?!?
The Plasma is being run now to bring INR down with the Vitamin K will be given in oral form, low dose (hopefully that in turn won't affect to much our ability to get the INR back in range once released from surgery/in-pt!) - Vit K is notorious per my Cardiology Team for having longer term affect on INR manageability.
It's ironic really that after 3 days off the Coumadin (blood thinner) my level would still have been so high (normal for a mechanical heart valve pt. but to high for surgery) given we (my Cardiology Team) and I typically seem to have a somewhat difficult time keeping the level within the acceptable range - more often than not in the past 6mo. it has been to low vs. to high.
In the future for surgeries I think I am going to question my Cardiology Team about testing INR the week before any planned surgery so we'll have a better idea where my level is (to low, just right or high.) Doing an INR would mean we could perhaps better tailor when I stop the Coumadin according to that Lab result vs. this time we hadn't done an INR since the 1st wk of Nov. and so had no real idea if my level would be 'good' and just planned the stopping according to AHA/ACA guidelines - my Cardiologist and PMD suggestion as in the past.
The last surgery I guess I had kind of forgot about it but I think we may have stopped the med 5 days prior to surgery (Neurosurgery suggestion) and the surgeries before that I was pre-admitted the day before (for other reasons I think scans or something) and so we had an idea if INR would be ok enough the next morning. Those surgeries we had to give Plasma the morning of surgery.
I will close on a somewhat positive note that although my cough (from pleural effusion) is cont'ing to hang on tight, worsening as the day goes on my INR when re-drawn some hours after this mornings had already dropped from 2.2 to 1.9. The Plasma should bring it safely down the rest for tmrw!?
I close this praying for a safe surgery tomorrow, thankful for my Neurosurgeon and thankful for my family and those friends who continually check in on these surgery days!
Thanks for stopping by, God Bless,
Erica

PFTs/breathing, Shunt surgery, PMR/botox

I'm not sure what made me realize or think of it but the Pleural Effusion symptoms that have been occurring for some weeks (with the cough that has been occurring for the past 2-ish wks) is the same symptoms that occurred for weeks after the 2nd OHS (open heart surgery). - I'm not sure what the deal is but the symptoms ebb and flow throughout the day, seemingly the worst at night. Definitely annoying and really wishing would improve or go away... Surgery is next Thurs and makes me a little nervous.
Apparently the consensus atleast between my PMD and Peds Neurosurgeon is this Pleural Effusion is caused both due to the TPL Shunt and partially heart related. Last year with these symptoms the TPL Shunt was temporarily re-routed to drain (along w the VP Shunt) to the peritoneal cavity so it would seem it was different causes each time?..
Overall I've not heard anything regarding the PFT results from Cardiology nor if they have a problem w the surgery going on, though they are aware it is next wk. (my PMD wants their blessing on the shunt surgery going ahead). I guess or would imagine they'll likely give myself/my PMD the ok before next Thurs or give us the PFT results but would indeed be a relief to know sooner than later. I don't imagine they have a problem w the surgery given the NP didn't seem to think there would be the other wk but there's also been somewhat mixed communication regarding the PFts and f/up. Non-the-less i'll just be happy when the surgery is over (hopefully next wk) and dealt with.
As far as the blood thinner (Coumadin) i'll take the last dose of that on Monday which gives 3 days prior to surgery though haven't heard if they'll require testing the day before of just test the morning of surgery (during the 2 hr pre-surgery time). Generally INR should be low enough within that 3 days range though last time (unsure if it was related to General Surgery working w my Nrsgn) they required the level to be within the "normal" range whereas other surgeries Dr.Bragg/Anesthesia has just done Plasma ahead of surgery if the level wasn't low enough.. Unsure this time.

The PFTs (breathing tests) where yesterday (Tues) with 6minute walk test and seemed to go relatively ok. I didn't ask the Tech what her opinion was but asked my Cardiology Team to to send the report to my PMD (outside medical center) and to let me know if they'll clear for the shunt surgery.. Per my PMD and her having apparently talked with Dr.Bragg last wk Dr.Bragg wants to go ahead with surgery if possible. I am sure hoping we can just get it done.

PMR F/up - Botox
Today (Thurs) was 3mo f/up for Botox (spine spasticity; botox injections calm the c-spine, thoracic and shoulder muscle signals from becoming overly reactive). Overall Dr.C did 15 injections at the various points trying a different strength to see if it would have the same benefit. Follow up once again in 3months. She commented that some of the muscles seemed more tense/tighter than normal, asking if I'd still been doing massage (Physical Therapy) which was a little surprising she could tell but I have not been to PT for a little over a month. It always surprises me how many of my Providers can really tell a significant difference between when I am and when I am not actively doing PT.

I'll update when/if I hear anything from Cardiology or my PMD or otherwise if anything new comes up regarding the VP Shunt surgery.
Thanks for stopping by,
Erica

PMD, Neurosurgery, Cardiology-Pleural Effusion

Yesterday I had the CT Scan with contrast which was fine, quick and not to much trouble other than the Tech having a bit of trouble getting the IV. Note to myself, apparently they know how to use Ports, so ask in the future ahead of time!
My PMD, Dr.Simpson messaged today with the results and to say she had spoken with my Neurosurgeon, Dr.Bragg regarding the results (see CT Scan results below and Dr.Simpson's comment right below this) - Below the CT Scan results are another message received later this afternoon from Dr.S regarding the Shunt surgery and her conversation with Dr.Bragg.
11/7/2014 12:33 PM CST
The ct scan report is below. Upon review of the prior ct scan and this one and discussion with Dr. Bragg. --the consensus is that the pleural effusion is likely multifactorial--partially possibly from the heart and partially from the shunt. Due to the area of " mixed Density area" you may benefit from having some of the fluid removed to ensure no infection contributing to the symptoms. When is the pulmonary appointment as they would be the ones to set that up? It should be done prior to the upcoming shunt surgery if possible since it could affect how the surgery was done/antibiotics, etc. PJS, MD
Ct scan showed: 1. Small to moderate sized right pleural effusion with associated consolidation at the right lung base. The effusion appears to demonstrate subtle mixed density posteriorly of questionable significance. This also may represent imaging artifact.
2. Catheter again seen extending from the spinal canal into the right pleural space. The tip of the catheter within the pleural space has changed since the previous study and now lies near the lateral interlobar fissure.
Regarding the Pulmonary consult I spoke w this clinic and then Dr.Simpson spoke directly with the Pulmonary On-call dr. and the soonest Appt is what I have in Dec. unless symptoms would worsen.
11/7/2014 Lets see what cardiology says and how your symptoms go. I know that Dr. Bragg really wants to keep the surgery at the same time for now. If cardiology is okay and you don't have any worsening symptoms I am probably going to be okay with proceeding with surgery. However, if symptoms get worse, you get a fever or feel more ill then we should probably wait until you can see pulmonary. PJS, MD

I emailed this information to my Cardiologist, his NP and Nurse and will f/up (likely call) them on Monday to see what their opinion is regarding surgery. It may be my Cardiologist wants to see what the PFT results are (those are Tues) so we'll see and i'll keep praying!
I don't particularly want to have surgery but I do want to feel better so I hope these symptoms either improve or at the very least stay stable.
Thanks for stopping by,
Erica

Pleurisy (Pleural Effusion), Hand Surgeon Appt.

Pulmonary/Cardiac
The CT Scan for the pleural effusion/R lung opacity is Thurs., which this is to to get a better view of the fluid area (Pleurisy, pleural effusion) w/PMDs concern if it is worsening cardiac heart failure or in fact just fluid from the TPL Shunt causing irriation causing the cough/other symptoms. After the Scan and PFTs, PMD asked that I make an Appt with the Lung dr. (Pulmonary) which i've been talking on MyChart w my former Pulmonary dr. at FMLH and will schedule w/her for further out and in the mean time see a Pulmonary dr. recommended at Dean (if that works out ok i'll cancel the one for Febr. with my former Lung dr.).
PFTs are scheduled for next Tues (ordered by Cardiology) and those + the CT scan will determine if my PMD will release me for the Shunt surgery on the 20th. She also wants Cardiology's blessing for this upcoming surgery based off whatever these test results are. I am dreading the shunt surgery for a few reasons but at the same time just want it to be over with already and so am praying fervently we can go ahead and my symptoms improve or do not impede surgery.
Overall the breathing/cough issue though it could be from the TPL Shunt (causing irritation) and could be Pulmonary related my Primary doc seems to think it's likely a cardiac issue possibly worsening heart failure secondary to the mitral valve which has been worsening.
If you can please say a prayer the CT Scan gives clearer answers to my Primary dr. and she in turn with my Cardiologist can release me for the Shunt surgery in 2 wks. I sure am praying, for as much as I don't want surgery I do want to feel better on the headache end.

To start off and then cap off a long Appt Tues. my car was overheating again which I must say given I was an hour and a 1/2 from home, on the far W side of Madison I was mildly freaking out!
Thankfully my brother who is VERY mechanically inclined was home and answered his phone + I happened to have car coolant in my trunk - I am not all that car savvy but do know how to check the oil, put in washer fluid, put in coolant (the basics) so with that emptied in I made it back to BD where my brother lives and he was able to do a couple things to hopefully fix the problem for now.
Definitely never dull, though I'd take dull for sure!?

Hand Surgeon Appt
I Saw the Hand Surgeon in Madison today (Tues) which it had been a little over a yr since i've had the R hand Carpal tunnel released again and Fat pad graft. The fat pad graft done by this Surgeon is due to 2 prior indepth CT releases and the prior Thenar Tendon transfer to protect nerves/hand use. All prior hand surgeries minus last yrs. R Hand where done elsewhere.
Given i've had the same symptoms in my L hand and the EMG/NCV nerve test done before the R hand surgery showed similar nerve entrapment issues w both hands we're opting to do this hand again now to.
This Surgeon plans to do the same in L hand as last yrs surgery for the R hand. Due to it being a more indepth surgery they splint/immobilize for 2wks, remove stitches and then last time they re-casted for I believe it was another 2wks w therapy I mostly did at home after. We've scheduled the Hand surgery for early Jan. which works best due to the Shunt surgery Nov 20th and we can figure out the Pulmonary/Cardiac issues in the mean time + the Holidays and an MPS Conf. at Disney right before Christmas.
Hpothenar Fat Pad Graft
The hypothenar fat pad flap is a technically simple procedure that allows the fat pad to be mobilized easily and placed across the palm as a barrier between the nerve and the radial leaf of the transverse carpal ligament, effectively preventing median nerve readherence ..

In God's hands, right?
Erica

Chest Xray results

Nothing to special to share, just posting what my PMD mycharted me earlier on Monday regarding the Chest Xray (CXR) results from Fri.
To: Erica Thiel
From: PJS, MD
Received:
11/3/2014 12:41 PM CST
Received:
11/3/2014 12:41 PM CST
So as we discussed the cxr final report is back and showed: New opacity in the right lung base compatible with infiltrate and effusion. Thus you have some fluid around the lung causing irritation. This can be due to heart failure symptoms getting worse versus infection. Albeit I think infection is less likely since you don't "feel sick with normal upper respiratory illness symptoms". No fevers or chills over the weekend? Please update the heart doc about the new effusion. I would also like you to see the lung specialist. Keep the Ct scan appointment though as that will help us evaluate the fluid area more.
I have placed the referral for the lung doc. The number to see the pulmonary specialist is 252-8000 to set up the appointment.
How are you doing? Any worse then Friday?
Dr.Simpson,

I've forwarded the results to my Cardiologist Team whom I spoke with today anyways for INR result (in-range at 2.08). I am not sure if they have any thought once they see this but the PFTs (breathing test) are next Tues and the Nurse will send the results for me to pass on to Dr.Simpson and to Dr.Bragg if needed.
As far as a Pulmonologist my Insur. Nurse Case Manager was given a name suggestion by a couple of her colleagues but I may also see my former Lung dr. at FMLH since she would still be somewhat familiar with my history. As far as the cause of the irritation/Pleurisy I'm not sure if it's from the TPL Shunt draining the CSF to this space and that CSF perhaps not being absorbed fast enough or if it is some other cause such as worsening CHF. I'm not sure anyone knows at this point.
The CT scan is on Thurs.
The only part worsened of symptoms is the cough is more persistent and frequent though still (thankfully) somewhat intermittent.
It's sort of crazy how it is always something!?
Erica

Halloween, eventful week

Trick or Treating was great; so thankful it is in the afternoon! That afternoon was so warm, sunny and NICE for once! No coats (hallelujah!)
I think it was awesome my Sis' let Zan goes as what he really loves!
Zander as a 'Monarch' - his true love and choice (so adorable)!

'Bells the Princess'

Earlier in the day, after Sunday School which was as usual well and lovely, (I love the kids I teach) sitting in Church I was seriously dealing with massive nausea - I always keep Zofran on me which I took more than one is supposed to but thankfully I was able to keep from throwing up! Otherwise the week was eventful with a family funeral (beautiful but sad as most funerals are - both a end and a beginning ie the end of a great man and the beginning of a great 'life' in Heaven.
Otherwise for the past few weeks I'd been dealing with SOB (shortness of breath-air hunger) episodes, R sided pain which eventually began to also occur under my R breast and in to my chest. This seemed to get a little worse vs better over a 2 week period and occurring quite frequently. Earlier this past week (Halloween week) after going back and forth with my Primary drs. office they (on-call dr., my PMD was out sick herself) advised I go to the ER due to the chest portion of the symptoms. Knowing on my end it wasn't likely cardiac as they suspected I instead asked the Nurse if I first could call and talk with my Cardiology Team which was fine.
Shortly after I called the ACHD Cardiology Nurses line the NP called me back and as I guessed my Cardiologist felt it was likely more Pulmonary related than Cardiac and did not think the ER was the best course to take. They want the PFT (breathing test) results though those aren't until the 11th due to my ERT Infusion being on the other day(s) they could have done.
Once the PFT's are done Cardiology is (I guess, per the NP) moving the Cardiology Appt. up another month (originally scheduled 3mo after last Echo, 2mo after I last saw them) to review test results and clear me before the VP Shunt surgery. I don't think it's that they think the surgery will be a problem, more they just want to cover bases given symptoms.
That all said in the mean time these symptoms are still occurring so I 'mycharted' with my PMD once she was back in the office and went in and saw her Fri. Her likely diagnosis is Pleurisy (inflammation of the lining of the chest wall) which is the same side as the TPL Shunt drains in to though we have no way to know is that the cause (if fluid sits in this space to long it can cause irritation) or is it a virus or who knows. In any case she (PMD) is ordering a CT Scan with constrast for this coming week as she to wants to cover all basis before the VP Shunt surgery and if symptoms don't improve i'll take a steroid pack (which I could have started yesterday but opted not to).
Otherwise below are some pics - thanks for stopping by,

Shunt revision rescheduled

It's hard to believe I am writing this and although I tend not to believe in bad luck perhaps the other week I jinxed myself when I wrote something to the effect of: "Dr.Bragg is out of the office studying for her final board testing but said she didn't have a problem doing the VP Shunt surgery while out".
Perhaps I jinxed it by doing yesterdays Pre-op Appt. (?) or perhaps I just feel like it's one of those things that oh so elusively out of reach (relief)?
I don't know but what I do know is that this sucks and honestly I'm probably going to worry between now and the new surgery date (Nov 20th) that Dr.Bragg will change her mind or doesn't believe me or something. Maybe it's an irrational fear but non-the-less when you don't really know it is for me a fear.

Perhaps that all sounds silly but when you've had the past experiences I have with Neurosurgeons I don't think you ever quite stop worrying when will this one stop believing and leave to? Silly I hope but non-the-less a worry of mine.
When I saw the missed call from my Nrsgns Secretary earlier I think my heart just momentarily stopped, I just had a funny feeling it wasn't going to be a good reason. I called the Secretary back and sure enough Dr.Bragg wanted/needed to reschedule surgery from the 30th (next wk) to later in Nov.
I had thought the reason surgery was delayed this 2 wks now was due to her taking her board testing sometime in this interval (but thinking about it now remember her saying something about it being in Nov.) and so I am not sure but think she was just doing very part time practice between when I saw her a few wks ago and now and then next week will be out for good? I don't really know.

After talking to her Secretary, Anna went back and talked to Dr.Bragg who was willing to con't on with the surgery next Thurs. but all f/up care would have been with her partner. Anyone whose been following this or my previous cb site for sometime knows my experiences with her partner have not been great ranging from flat out accusing me of being a "drug seeker" to comments along the line "I'm glad she wound up with you" (when rounding on a wknd day for her); believe me there's a reason I walked out of the one and only appt with this dr.
The only appt. I've to date ever walked out of AND I feel it is my gain to have Dr.Bragg as my Nrsgn - she is good at what she does and I think works well with my other Providers. She has believed me when I know there are many times my symptoms are less than classically presenting and though I never stop really worrying about her giving up I feel lucky to have gotten her on my Team due to her caring and persistence.
I know her partner is a good Neurosurgeon and several of my friends have kids who see him and they really like him which I am happy they have good working relationships. I would never say this dr. is a bad person just that we don't mesh as Patient and Provider and I feel heads and toes more comfortable with Dr.Bragg who has never discounted my symptoms or what I might feel nor accused me of making up symptoms. I think some drs are (my opinion) just better with kids and some are just more able to do both kids and adults.
It's also on my part not a male vs female dr. thing as several of my other Specialists are male including my Cardiologist and we get along well. In my Cardiologists case we are 2 headstong, stubborn people who have worked well despite our not always agreeing nature.
I write all of this here as with many things I write a bit apprehensively as I don't obviously know who (or even what Providers) may read this and I worry a bit it may offend someone and that definitely isn't my intent.
The reason I share it is b/c it is my experience and this all above plays in to how I feel today as I was deciding whether to do surgery still next wk or as much as it kills me go with surgery in a month. =/ I am guessing Dr.Bragg's Secretary probably thought 'holy cow' when I talked to her as I was in tears and upset (not angry upset, just upset) trying to sort out what to do.

Due to Dr.Bragg being there I've opted to reschedule surgery to the date later in Nov., I think it may be a long 4 wks between now and that surgery date but I am not very comfortable with post-op being under someone other than Dr.Bragg who is familiar with my care.
I bounced the thought off my Mom (who acutely remembers past experiences) and some friends and just decided since Dr.Bragg is so familiar with my underlying, potentially complicating issues i'll wait. Do I wish it was tomorrow or the as before scheduled next Thurs yet? Absolutely!
Pre-Op from yesterday should still carry over to next month so the only thing I can think of is INR testing which I imagine i'll have to get done the day before. Anna, the Secretary was going to place the surgery as the "1st case" so hopefully that will remain true and we manage INR the day before by outside testing.
To sign off I'll leave with this quote I came across on fb today:
"Hiding sick is very easy when your life is always being invaded by pain"
I wish more people could understand this; not all of us regardless of how we feel curl up on the couch as we have things we are responsible to and so you instead modify your life or plans.
Erica

Pre-op Appt, VP Shunt Surgery Thurs. 10/30

Today's pre-op Appt went well, nothing to special. The Appt was with my actual PMD(she's very part time but her nurses will add me on to her schedule) so went relatively quickly. We where able to use labs we'd just done several weeks ago so not much had to be done.
EKG was done at my Cardiology Appt last wk so that to did not have to be done. As far as the cardiac symptoms/breathing issues she just commented she'd have wanted a Cardiology consult had I not just seen them (she sent me) and so she was glad we didn't have to try to get that done in time.
Med wise unless Cardiology says otherwise (PMD was going to call them after the appt.) and unless Dr.Bragg/her office would say otherwise before next Thurs's surgery i'll stop Coumadin which is the blood thinner 3 days ahead of surgery. Med wise i'll take Lasix and all cardiac meds the morning of surgery (to prevent heart-fluid overload) though she wasn't certain about the baby aspirin. She feels it should be taken but is Cardiology's call.
In the past as far as knowing if the blood thinner was in-range for the day of surgery Dr.Bragg has done several different approaches from my being admitted to the floor the day before surgery to having me come early the day of surgery and doing labs several hours before at her hospital. The plan this time is admit the day of surgery and I assume just check INR in the 2 hr window between surgery check in and going back to the OR. I assume if they want something different they'll decide between now and then.
Weds. i'll be in Milw. and since it's just easier i'll have my Infusion nurse access my Port (vs leaving it accessed after Monday's infusion and then having to change the dressing when I shower each day leading up to Thurs.) so that is done and no need to convince Anesthesia or a Nurse to access it vs. starting a peripheral. My infusion Nurses real jobs are on the Access Team at Children's so it works out well; typically they will just add me to their schedule, i'll come in and takes about 15-20mins. Having the Port accessed before I come in saves a lot of frustration the day of surgery as Anesthesia usually is against accessing/using the Port and honestly even this way sometimes they only way they'll use it is if Dr.Bragg asks them (she has seen multiple attempts at peripheral IVs).
As far as ERT since surgery is on a Thurs., I may be out in time to get my normal infusion at CHW but per an agreement/arrangement with UW's D6-4 Neurosurgery Pharmacy I call and give them a heads up anytime i'll be having surgery so they can order the enzyme drug to have on hand. This way if I am in-pt I can won't miss. I've not yet called to let them know but the Aldurazyme then gets mixed by the Pharmacy and infused by the floor Nurse(s) using a slightly different ramp up rate than we use at CHW (for whatever reason it runs at a much faster pace if we use the ramp up rate I normally at at CHW so one of the Nurses had re-calculated their rate and works well).
As far as surgery I am just praying we figure this out, that any occlusion would be a simple find/fix and if need be Dr.Bragg works down the entire shunt system to rule out it isn't further down in the distal end. I trust her though and know she'll do whatever she feels is best.
I guess if there's anything else i'll update otherwise surgery is next Thurs. the 30th.
I've attached below a few copies of my Sun School kids at the end of class the past 2 wks. I typically take pics for various Church purposes and then make copies for the kids/their parents which the kids seem to really like having.
As difficult as mornings are these days (I feel quite out of sorts actually and in a zone sort of) I try to get up a bit earlier on Sundays so some of the symptoms are atleast partially worn off and honestly my kids are pretty easy going. I've also figured out how to teach without having to do a ton of actual talking the entire time.
In any case thanks for stopping by,
Erica
'The Passover'

'Moses leads the Israelites'

VP Shunt revision date, Cardiology Appt.

The VP Shunt revision date is set for Oct. 30th - pre-op Appt is set for next Tues.
While imperfect that the surgery date is further out and you might ask why (Dr.Bragg is out of town I believe for her board certification testing one of these wks.) it also isn't as bad as if I only had 1 shunt and that wasn't working optimally. Given I do have 2 shunts and the TPL Shunt (Thoracic to Pleural draining) is working well and we rarely have issues with it I do atleast have some CSF getting drained and headaches (and associated symptoms) while less than fantastic are also not as bad as they would be where I to have 1 shunt and it where malfunctioning. That said believe me I'd still take a drill and drain the fluid myself if I could at times..

Cardiology
I sort of had to laugh at yesterday's Cardiology Appt., I think Dr.E knew my PMD is who asked that I talk to his Nurse and make an Appt. with him but initially he was like (my paraphrasing) "I really don't think this is solely a cardiac symptoms as you have a lot of co-factors going on that inter-play and could be causing your new symptoms".
I kind of looked at him and just said 'You do realize that's why I talked to my Primary dr. first right?' LOL, I really like my Cardiologist but sometimes he acts more like a Father figure than he does a dr. with the 'know-it-all-ness' but then again he is a Dad to a bunch of girls so probably explains it. I know the 1st time I met him 10ish years ago I thought he was a little arrogant (but seemed knowledgeable) and i've come to figure out is just that he cares though he can initially come off a little brusque. If you don't know his approach well I could definitely see some pts. being offended; his personality is something of a joke (not a bad one) amongst his pts.
As far as what does he really think is going on? He commented from having read one of Dr.Bragg's more recent clinic notes (they are not at the same hospital but both practice out of the 2 Children's Hospitals in WI) that he does not think our cardiac issues would impact/influence the headaches and he does not think an issue with oxygen getting to the brain is the cause of the H/A's. His thinking if I remember right behind this was 1. The aortic valve is working well now that it is fixed and 2. the Mitral valve has less of an impact on this area (but definitely don't quote me on the 2nd part).
I obviously don't know and all I know is the aortic valve is functioning well post-the re-replacement last Fall and the Mitral valve is at the edge between 'we can wait' and 'we'll have to intervene' but he is hoping we can wait as long as possible.
As far as symptoms he had a few thoughts but really didn't want to adjust meds as he is concerned I am already on quite a few (5 different cardiac meds in several different classes to be exact) at relatively high doses and is concerned any dose adjustments would "upset the balance".
His 2nd thought is repeat the breathing tests as it could be related to the restrictive lung issues (same symptoms prior to the aortic valve replacement/re-replacement so seems less likely but anything is possible) and asked that I use the inhaler (Xopenex) I have more routinely (I have it but never use it now) and especially when going to be active.
3rd. Instead of repeating the Echo in March at 6mo's he is repeating it in Dec. (the day before I fly out to the Disney Conf. actually) and f/up with him after. If anything changes or new concerns between now and then let him/his Nurse know.
I personally think these symptoms are cardiac, basing them off of how I felt prior to the aortic valve replacement and how I've felt over the past few weeks. How I can sleep 8 hrs or I can sleep 12 hrs and I still feel pretty much exhausted. The weird sweating (not as bad thankfully) which I think is autonomic and impacted by the shunts, the breathing and intermittent but frequent heart rate changes on top of the intermittent blood pressure changes. That all said I also know I went like this for a period of a year or two before the aortic valve got to a 'severe' range so it is quite possible this mitral valve will stay the same it is to for some time to. This would be fine b/c I don't really want to go through another heart surgery and all that entails although at the same time I am amazed in the last few wks how little I noticed the seeming energy improvement after the 2 OHS last yr. until it just wasn't there anymore again these past few wks!?
I guess on a positive note since I had to be there the NP whom followed my care both in the hospital after both OHS and then for 6mo after during each cardiac f/up stopped up to say 'hi' today. Normally Dr.E's Nurse Jane does pre-appt vitals, med check, etc. but Nancy did so today, just to check-in which was nice. I might add both are extremely nice and easy to work with though. =) Nancy and i've talked on the phone a few times (INR results) since she hasn't really been closely tied to my care since last March but otherwise we'd not seen each other and so was a nice surprise. =)
Un-related to the actual issues I was there for but something Dr.E has brought up before, he brought up how easy it has been for them to work with my primary insur. to get approvals/authorizations. Cardiology like Dr.Bragg is an out-of-network Specialist exception and so my Insur. Nurse Case Man. when our Insur. had 1st switched to a HMO a couple yrs. ago got Prior Auths. (PA's) in place which cover certain care and tests in addition to the drs. each for 1 insur. benefit year at a time. In the case of Cardiology they apparently have other pts. who also have Dean Insur. and must have a fair bit of troubles getting PA's approved for out of network tests, drs., etc.
Dr.E has commented several times over the years maybe they should just have me doing the paperwork for them (lol) but really for me it's just been a matter of being persistent and involved, sometimes pushy to get things done though my C.M is a huge help to!
I personally think patients have to be informed of their benefits (what coverage they have) and almost more importantly request a Case Manager be assigned to their care as that 1 person is then your entire point person for ALL Insurance questions or issues. I know my Insur C.M works with my Genzyme Insur. (drug) Case Manager when it comes to questions on that end, and with the Neurosurgery fl. staff from time-to-time and has often been my go-between for the Medical Director at Dean when issues have come up.
Somewhat jokingly (and yet it's true) I commented that as much as I often hate asking questions I am not afraid to push for and ask things when I am not sure and that to is important in getting the care and coverage pts. need. He smiled, laughed and agreed about my not being afraid to ask questions either right away or later on. I think all my Providers know my hesitancy but also know I'd rather be informed than mis-informed or not understand and I'd rather as much as possible get things done on my own.

In any case no earth shattering news, and nothing exciting (which is ok).
I'll update again when there is anything,
Erica