Wednesday, December 31, 2014

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!

Friday, December 26, 2014

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.
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,

Tuesday, December 16, 2014

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,
'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)

Wednesday, December 10, 2014

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,

Tuesday, December 9, 2014

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,
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 :)

Friday, December 5, 2014

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,