Saturday, March 28, 2015

Neurosurgery Appt, shunt tap; VP Shunt.

It has actually been a relatively short few days between the last post but I did see Dr.Bragg and that Appt to went fine and thankfully we have a tentative plan. I am so grateful she never gives up.
Dr.Bragg opted to tap the VP Shunt and although she was able to remove a small amount of CSF it took quite a long time, a very slow process whereas usually it is pretty spontaneous and takes a very short amount of time to receive however much fluid is wanted. She commented in the process a small amount of blood came out while she was trying to drain CSF, I don't think that is significant but she felt the shunt is likely occluding again and probably need a revision. I'll see her in a wk but she jokingly said "maybe we should put you on the 6month plan" (for shunt revisions). LOL, though she's not really wrong as it seems to be about true. For now the valve (reservoir where she taps to test/remove) CSF does refill so that is alteast good.
When I see her next week she'll re-tap the VP Shunt and we'll decide from there about shunt revision and if so details. While in Dr.Bragg's office we where talking about if I had anything coming up which I don't other than the Boston trip which I was thinking at the time was not till the end of April. Instead when I went back and looked at April I am supposed to fly out to Boston the 17th so the wk after Easter I believe that is? In any case Dr.Bragg had asked me to msg her later Thurs to let her know if the shunt tap relieved the headache and secondary symptoms (it did, so glorious for that short time!) so I let her know about the dates.
Thankfully I do have the 2nd shunt so that gives us time as long as the VP Shunt is working atleast a small amount.
Dr.Bragg thought we could likely do periodic shunt taps to relieve the headache symptoms once she is back next Mon. so I am hoping she may be able to tap the VP Shunt and remove some CSF indeed periodically including maybe that Weds before I fly out Thurs morning? I don't know if it's the best idea I go to Boston but I realy, really try to just live life as normal as possible in-between the medical stuff? I know I could rest as needed and it is with families and friends that get this rare disease stuff in Boston and I am seeing a dr at BCH that Fri so although unlikely if anything did come up I would know medical personnel in Boston beyond just the Genzyme folks... I would imagine maybe we could do any revision then one of those next days after I got back? I fly out that Thurs, 17th and get back late the 20th.
I truly don't want to have to do another revision but at the same time I wish we where doing it tomorrow... Hopefully things just go ok over the next wks. I am looking fwd to it being Holy Week and Easter!
Although headache symptoms where back by Fri, today (Sat) the symptoms actually once I got past this morning weren't to bad and the cardiac (heart) symptoms where pretty good - I was out and about with my Mom, Sister and nephew (Zan is by me for the night and i'll take him back to his Mom's after Sunday School and Church tmrw) and although really tiring i've also learned to just sit out some things (wait in the van) and I try to do enough to have fun but also not wear myself out so completely it isn't even fun after a little while. I am hopeful this next few wks we can do the shunt taps and make things reasonably ok for a day or two at a time which although the symptoms come back they honestly atleast the leg and secondary symptoms (minus nausea) seem to take a few days to build back up to be so bad again. Almost like the nerves get a small break, a sigh of relief in a sense from pressure? I don't know just what it seems like.
Zan loves to take my phone and take pics of relay towers, power poles, power lines, cell towers, etc when we are out and about and so days like today sometimes it's just fun listening to him and watching to see what he deems "that's awesome"! =)
Tomorrow is the last Sunday School for a couple weeks, Palm Sunday (hard to believe it is a week away from Easter!) so after our lesson I have ceramic crosses for my kids to paint/decorate and a small gift for each of the kids. Zan typically enjoys doing these crafts with the kids so although it's a mess to clean up after it is usually fun =)
Will update again sometime soon,
Thanks for stopping by,

Tuesday, March 24, 2015

Cardiology Appt, med adjustments

Today's Cardiology Appt went ok, as is often the case with this I think it went better than I thought maybe it would, by that I mean I am always a little nervous he'll think I am over thinking symptoms given I know I rarely have said symptoms (is intermittent like most things I experience) when in clinic and likely look fine. Thankfully he is good and seems to always look at the big picture ie pts probably wouldnt chose to be here if they genuinely didn't feel good.

I'd written down a general brief overview as best I could describe what i've been feeling and Dr.E plus his NP, Nancy asked their usual litany of questions to get an idea of what might be going on. He admitted he isn't sure and feels it probably is multifactorial ie probably both heart and shunt related due to the 1 shunt draining to the pleural space and the affect the cardiac issues have on lung issues (honestly I can't reexplain what he said about this other than when there are both heart and lung issues and a shunt that drains to the lung space it makes it harder for fluid to absorb properly or maybe it was having this fluid draining there is somehow affected by the already over-worked heart function? Not sure! Always makes sense when he explains it but not entirely one of those things I can re-explain.

In reality he has no real way to know what of these issues is causing the symptoms but does feel it is atleast influenced by the heart. Could be due to arrythmias or due to the mitral valve or again a combination of issues.
Some of the things he felt would give the best impact on symptoms are things he was also not comfortable doing, for instance the diuretics (Lasix and Spirononactone, meds to get fluid off) he feels are already at the max dose he is comfortable with and because my blood pressure is so low but heart rate is overly fast he is concerned what option for meds we have to use/go up on/try.
Initially he was leaning towards doubling the Beta Blocker dose as this can help heart function while slowing heart rate but I questioned when are we treating one symptom only to make another issue (side effects) worse and in turn not improving Quality of life? We went back and forth on this for a bit with his strongly leaning towards an increase dose and my being against it. After a bit he thought some more (I appreciate that he's not afraid to stop and re-think ideas and admit he needs a min!) and asked what about adding a Calcium Channel blocker but keeping the Nadolol along w the other meds I take (4 other cardiac meds)? While not thrilled with the idea I wasn't opposed to it and said as much to him and to Nancy that although I don't think anyone ideally wants to add even more medications to their list of what they take if it meant not increasing the Nadolol i'd try it. Fast fwd another couple mins and we settled instead (yay!) on simply starting the Calcium Channel Blocker (Diltiazem CD) and stopping the Nadolol altogether as he was to concerned blood pressure would sink to low given it is so low already. I don't think this is his ideal med arrangement but I also don't think there is an ideal arrangement at this pt. He did leave the Digoxin, Spiro, Lasix and Coumadin med doses alone as well as the baby aspirin.
The plan is i'll stop the Nadolol tmrw and start the Cardizem on Thurs and we'll titrate the new med up to get to a decent place and ideally slow heart rate while giving time for the heart to increase filling pressures in-between heart beats (I think thats what it is described as). My Pharmacy had to order this partcular med so in order to avoid any lapse in one heart med and the new one Cardiology instructed to keep taking the Nadolol until I receive the new one and then start the Cardizem the next day to avoid any undue stress on the heart.
I follow up again with Cardiology in a month and obviously do INR in-between there so will talk to the Nurses/NP in-between but we'll re-eval everything at the next Appt.
For now I have a 'event' monitor which I am supposed to use anytime I have symptoms and then the kicker is you have to transmit the readings via a landline. I don't have a landline and don't know many who do! I am hoping I can either trasmit at my Church maybe or at my Dad's office when I get to town but the recorder only does 6 things at a time. Fun, fun times!
Should these meds not improve things enough Dr.E circled back over and over again to a mitral valve replacement "i'm not sure it would help all of your symptoms but we might have to do it, your valve isn't the worst i've seen but it also isn't pretty".
He seems genuinely concerned this would be really difficult and an unknown outcome as far as getting through surgery (lol the bluntness) BUT if that is where we end up I have confidence i'll do just fine and we'll put in place the measures needed to make surgery a success. I believe she is still at CHW so hopefully the same Anesthestiologist who is familiar w my airway could do the surgery and ABOVE ALL hopefully we just won't need to worry about it for a while! I know a mitral valve replacement would make other surgeries a tad more tricky given blood thinner dosing is different/slightly more complex but there to not going to worry about it till we have to.
For now will just pray this med helps and be grateful I have a team that is insightful and tries to think outside the box.
I see Dr.Bragg Thurs and hoping she can help figure these headache issues out. That is an Appt I think I am reallllyyyy nervous about. Why? I don't know, maybe bc I find it difficult asking for her help continually. In any case it is in God's hands and he knows the plans he has for me.
Thanks for stopping by,

Wednesday, March 18, 2015

All these symptoms (head, heart); Life cont's on

Everythng seems to be in place for the Appt in late April with Dr.Bodamer at BCH, this means i'll fly in to Boston Thurs afternoon stay overnight neat the hospital at a hotel I reserved and see Dr.Bodamer Fri morning. He is the dr willing to prescribe Elmiron, the anti-inflammatory type drug (it is a drug originally approve for Interstitial Cystitis). I have plans at the moment to meet up with my Case Manager from Genzyme (company that makes the enzyme replacement drug) for dinner and Fri going to stay with friends who are also on the RfRD Team and Marathon wknd activities begin Sat morning.
In all honesty I think i'd probably give my left foot to feel even a little better before that trip! I wish we could adjust the shunt valve(s) or figure out what is going on and not to mention figure out why the TPL Shunt is causing such (at times) extreme irritation to the sciatic nerve and other times the pain/discomfort is less but still not that great. It really makes little sense!? The headaches and thus leg discomfort seemingly due to nerve irritation continues at it's lovely own drumbeat. I am frustrated by it but at the same time what can I exactly do about it on my own!? NOTHING! I hate to keep asking Dr.Bragg for help though am going to have to again as I can't take this. Even simple things like standing to brush my teeth or today standing intermittently in Church was down right aweful!
Honestly I think I wish we could just get back to whatever caused pre-Jan to be better! I can't help but wonder if neither shunt is working as well as it should be, I mean I know based off my own symptom progression and previous dealings (many times over) that the VP Shunt is working just not optimally is my feeling. I feel like when this gets to that pt then it puts more pressure on the TPL Shunt which is super irritating (literally) to. Sometimes I could just yank that damn shunt out of my mid-back! I honestly wish it just was positioned differently or something. Something needs to change =/
Non-the-less I don't know, I guess it's just day by day with these crappy symptoms and keep trying to do the other things as much as possible that do make me not hate these shunts quite so much! =) I absolutely know the shunts help and make life better but they sure work to their own beat and as they please vs the times we've had really pretty good! I guess in other words it's just have to chose to smile despite often feeling really kind of crap?

I think I may have written about it in my last post but in talking to my Cardiologist Nurse after the last INR draw a few wks ago regarding the (breathing) symptoms I have been experiencing we agreed if I was ok with it I could update them this next INR draw (today) to update them on how I felt and if we should move up the Cardiology Appt.
On a good note albeit aggravating bc it means I stay on the near double dose of Coumadin my INR today was 2.03, 2 wks ago it was 2.01. This as I said is good but I had really hoped i'd be able to drop the dose back down! I am unsure why it is suddenly taking such a higher dose of Coumadin to be even at the bottom range of my INR range but wonder if dropping the fish oil dose I take to just 1 capsule a day is the reason. Doesn't really make sense to me, but maybe.
Anyways so I talked to the Cardiology Nurse today and she is talking to my Cardiologist to see if he wants the Echo and 6MWT scheduled sooner to. I am guessing the appt would be sometime in the few weeks as the symptoms are bothersome when occuring and occur intermittently (but semi-often) throughout the day BUT also I don't think is an urgent issue. Just another irritating issue that likely won't have a clear answer and honestly if I had to guess would bet it has something to do w the shunts not working optimally and headaches/others symptoms building up so frequently.
My PMD wondered if the lasix (fluid reducing med) dose needs to be adjusted. I already what I consider a crazy dose so am hoping we could figure something else out or switch a med vs increasing that dose. Even my PMD commented in her same note regarding these issues she realizes how sensitive i've been to adjustments in this med. I don't think there is ever a simple answer! A nice easier answer would be we could just adjust something over the phone and not have to go in! I know I am deaming (bc Cardiology feels the meds are "really at max doses already" so isn't as easy as just adjusting any 1 dose.
For several months reducing the amount of fluid I drank and our reducing the ERT infusion rate helped symptoms overall although that was more the residual cough and pleural effusion fluid and that improvement was a combination of the Nov. VPS revision and this changes in fluid. This symptoms just seem different, like symptoms i've had in years past. Hard to say BUT not exactly what I want to be doing, not only dealing w headaches and having to repeatedly ask Dr.Bragg for help but now damned cardiac (seeming) symptoms to! GRRR!!!! <--- pardon my whining/PnM'ing btw.
The best I can describe the symptoms is a winded feeling, hard to catch my breathe BUT not the same as say i've just run a race.. Also an 'Air Hunger' feeling, almost like you feel when trying to breathe underwater. This is by far the more frequent of the symptoms and the worst! Is thankfully intermittent though and honestly even when it's occuring i'm not sure it'd be obvious to anyone around me?
Believe me when Jane, Dr.E's Nurse was asking questions I am sure I sounded like a flat out moron trying to think of the best way to describe what I have been intermittently feeling! You'd think living w/this everyday a person could just explain it but so is not my case!

Will update others things in a future post, watching Zan tomorrow after school. While I rarely have loads of energy I very much enjoy being with him! Bella, my niece, Z's sister was asking me the other day to go for a walk with her which I didnt (thankfully my Parents where at my Sister's to and Dad went w Bells and Zan!) but is fun to just do relatively low energy things w the kids and often Zan (or Bell) is just happy to have my phone and take pics which are fun to watch! )
Will update soon,
Thanks for stopping by,

Thursday, March 5, 2015

To another yr of possibility.. If your going to have an Appt on your Birthday, Neurosurgery..

It is another year of possibility... another birthday.. Happy Birthday to me (and to my brother Aaron) =)
This morning like most mornings started off rather blah and bland (my words for not feeling great) and transpired to a better afternoon. Although I hadn't thought of it when I made the Appt I had an appt with Dr.Bragg today so made my way there early this afternoon. Thankfully that timing atleast works out so I am feeling more human, I don't feel like a grouch and having a conversation doesnt feel like it is picking needles out of a haystack (my description for how difficult it is sometimes to come up with the thoughts to make conversation with someone when not feeling good)..
Because she's Dr.Bragg and well bc that's who she is she realized it was my bday and as she opened the exam door she and her 1 NP started signing 'Happy Birthday' (totally embarassing BUT really sweet of them!) =) She had this sweet picture they had printed out (she clearly knows me to well what with the cat and all) and which she and much of her Team had signed. Additionaly bc she's silly but in a good way she had this skull (LOVE IT) and these kitty ribbons - I might see if I can hot glue one of those ribbons to one of my headbands. It's good to be a little bit like a kid sometimes! I happen to know 1 niece of mine who will love these to. =) 

So anyways the reason I was seeing her was really due to not feeling well, clearly even if I like Dr.Bragg I wouldnt otherwise chose to just want to see her in clinic! It's a nice enough place BUT i'm not crazy!! Alas b/c the leg symptoms have continued to progress despite my 'imperfectly perfect' shunts (they work but never well enough and over time seem to work less and less well due to the ever repeating drain/collapse/pressure builds/drain/collapse/pressure builds over and over process) she opted to tap the VP Shunt and if nothing else said she would take some fluid off to see if this gave relief. The tap went ok, initially she had made some comment it felt a little different than a few wks ago but I didn't ask what she meant by that. I know I ocassionaly will depress the reservoir to try and get CSF seeming to flow which for whatever reason helps then shortly after helps the L leg symptoms and the reservoir doesnt always seem very 'squishy' these days (doesnt seem like it refills very quickly all the time maybe?) but I am not entirely sure if thats normal and something I completely didnt think to ask her. Ironically this morning in my stupor I was trying to think of what it was I had wanted to ask her about and couldnt and now I realize thinking about it this clearly is what it was (why would depressing the reservoir to seeming get CSF moving seemingly help the leg symptoms?) Aother question for another day I guess.
I think she said she took off between 2 and 3cc of CSF (I am not enirely sure how significant an amount that is) but asked me to let her know if it helped both the headaches and the leg symptoms. I could tell a difference (which is often the case) by the time I got out to my car; it literally is like a 'head clearing', as if a bowling ball like pressure is being removed from your head AND my leg didnt fall asleep/hurt AT ALL during the drive home!! I know it's only temporary BUT this still is an Awesome, Awesome bday gift that I am REALLY grateful for!! God is soo good and my Neurosurgeon is wonderful for trying little things to even help temporarily. She asked that I update her on if the shunt tap/removing CSF helped which is easy enough, I am glad she cares =)
I am admittedly curious to see if this removing a bit of fluid will help breathing tonight and sleep amongst the other things. My Cardiologist is apparently concerned about the new/recurring symptoms but told his NP he felt I would know if I needed to come in (likely med adjustment then) or if it was multi-factorial which is what I also suspect ie shunts affect cardiac stuff, cardiac stuff affects shunts, etc. I have no doubt when the shunts are being a little/lot less than optimal heart rate is much more forceful and fast.
I know there is no easy answer for her but I also know many drs wouldnt try anything at all so I am always grateful when she is willing to try something... She talked about she'd seen her Shunt Rep the other day and they where talking about a newer Strata-like valve but it has 8 settings vs Strata's only have I think if I am remembering from my Strata days only 3 or 4 settings.
Dr.Bragg said she didnt feel this would be a great choice for me as it has so few settings which at the time talking about it I didnt think to much but I am curious now are those settings higher draining or are some of the settings able to drain low as does my current Codman valves? Whenever I talk to her next I want to ask her about this as well as about a valve we talked about a year or so ago that albeit notoriouly difficult to reprogram apparently has more setting options...
Their re-programmer for the valves I have in both shunts is non-functioning at the moment so I am hoping at the very least once that is fixed we can potentially adjust the settings on either of my valves to try and help..

On a slightly unrelated note, for sometime now i've been trying to restrict how much fluid I drink, some days doing better than others but defintiely noticing a difference the day after I have drank more or less. This is cardiac in nature but slightly aggravating as you never realize just how much you miss or want something till your trying to not drink alot of it! INR, per Cardiology this week was just barely in-range at 2.1 AFTER 2wks of near double the usual Coumadin (blood thinner med) dose I take! I about fell over when Nancy,the NP called and told me that. Honestly i'm glad it's in-range but jeesh at that dose I figured we'd be safely higher so I could reduce the amount I take every day again!

On a reallllyyyy un-related note Dr.Bragg and I where talking about her daughter and my nephew and some other things and got on to the subject of where my Parents live. Tursn out she (Bragg) is giving a talk in B.D towards the end of the month! Good that this town is FINALLY branching out and getting over their seeming insulated self and trying to better educate their Providers on various Neurologic and other issues that occur in pts! Althugh I saw a Pediatric Cardiologist in Madison growing up I often wonder had I seen someoone more specialized earlier on for some of the other issues sooner (as a Teen I started being referred to other Specialists) would an MPS dx have come sooner? I wonder this bc I look at others in the MPS I community and many don't look that different than I do nor have most had anywhere near as many issues as I have had.
Insurance Case Manager
My Insurance Nurse Case Manager called the other day, just to check in and was asking about the shunts, headaches and symptoms and how things in general are doing. I mentioned how i'd despite really not wanting to do it made an Appt to see Dr.Bragg for later this wk (today) and how I know I 'look' fine but I sure don't feel fine alot of the day and that I wished we'd find a better answer with the shunts ie a better valve or setting or perhaps the TPL Shunt causing irritation to my spine nerves just makes the overall headaches that much more aggravating to me. I don't know but I said while I in no way blame Dr.Bragg and AM grateful to her and I am certain it must be hard to her b/c I rarely present as 'looking bad' even if I don't feel great (other than times such as in Jan when the 1 shunt was completely not working).
She (Nurse C.M) said something along the line how well I seem to do keeping a positive attitude and not getting mad despite on-going, intermittent issues and that most pts she knows would not have the grace/patience. All I could think is I hope I really do come across as she seems to think? I don't think I am impatient by any means but I sure hate feeling like a nuisance (not that Dr.Bragg really makes me feel this way). ... It IS just HARD having to continually ask someone for help!
Perhaps that conversation and today's Appt are God's way of continually reminding me I should just trust more and doubt less? As i've always said, I really do trust Dr.Bragg and most of my Providers but I have a really hard time sometimes not having answers and not doubting they won't give up!

My wish to myself is this next year of being 32 is blessed and good and I grow in whatever way God deems fit, that my health may be well or that we may if it is God's will figure out these headaches at some pt and the cardiac issues perhaps stay stable.
Thanks so much for stopping by,
'Happy Birthday' (from Dr.Bragg and her Team)