Monday, April 28, 2014

Neurosurgery adjustments, Cardiology, Boston Marathon wknd

Sometimes I put off these updates where it seems like it might end up being long, which even though I kind of like writing it's a matter of putting thoughts together from all the things that have occurred since a last post!

Although I didn't expect to have to I ended up seeing Cardiology today (Dr.Earing) after I called to give them the usual heads up that my Infusion Nurse was running the pre-ERT INR result across the path from ERT (Genetics Clinic, Curative) to CHW's lab.
Jane, Dr.Earing's Nurse answered their phone and I gave her a run down of some of the symptoms that have been occurring over the past week and few days (the shunt or pleural space seeming irritated/uncomfortable when breathing, worse when laying down. The feeling like I am breathing under-water sensations, (also intermittent) which worse at night and HR being very high despite the BB med + weight being up quite a bit over the past few days (I don't weight myself but Michele my ERT Nurse also commented on the big jump today pre-ERT).
Jane in turn talked to Dr.Earing who asked if I was able to come over after ERT which I was and so I saw them.
Per Dr.Earing Magnesium and BNP levels where fine (magnesium can be affected I think by the high Lasix dose and BNP is one sign of heart failure they look at). B/c these where normal and b/c he was hesistant to increase the Lasix (gets fluid off) dose any further (quite a high dose already 2x a day) and I've not tolerated a higher than 80mgs dose of Nadolol (beta blocker) + this med hasn't done a lot to get heart rate down as it is. He also did not want to adjust the Spironolactone and didn't really want to switch to a Calcium Channel Blocker (CCB) due to the affect these have on blood pressure and mine already being very low. Instead he opted to add a low dose of Digoxin which is a med that helps a "stiff" heart to pump better I think was how he explained it.
This is a med he had talked about last time and feels it is the best bet with the least likely of addt'l side effects.
He made the comment that he wanted to do a Chest Xray but like anytime he sees me he wanted to be cautious in any decisions he made and make only baby steps compared to how he might treat most of his pts regarding what interventions he might add or try. He added that b/c of the MPS he always feels like he is learning from me, "you make me think everytime" and regarding the Chest Xray he said "I won't likely know how to interpret it" (given the TPL Shunt). He said if he needed he would access the Care-WI network for UW's Med Records and compare this CXR to ones done recently post-surgery by Dr.Bragg.
I am just grateful he is usually willing to admit if he doesn't know and he isn't overly aggressive about treating new symptoms but at the same time belives me and want to try to improve quality of life.. I think really those are 2 of the best things you can ask for from a provider!?
He did say he was surprised the Heart rate hasn't come down yet given we are this far out from the 2 open heart surgeries (OHS's) and that in most patients Sinus tachycardia-post Heart surgery improves within a few months. His big concern is why the Mitral valve worsened so quickly after the 2 OHS's when it was mild after both and from what he has read it isn't standard even in MPS pts for it to suddenly just progress after another valve is replaced. He did comment as he has in the past that the fast heart rates likely has some do to with the mitral valve gradients increasing.
He added something along the lines "You con't to do things your own way" (he was joking, regarding my body, the MPS makes it's own rules).
I keep the normal, as scheduled f/up in a month to see him/Nancy and actually forgot to ask about what the Coumadin/INR level is and when do we need to re-test that (done at ERT so is easy enough).
Regarding if I had any issues with the Digoxin or problems between now and next months f/up he commented that given I am at ERT (same hospital) every Monday to just keep them in the loop if there are any issues and he was good with arranging his schedule to just see me after ERT (hopefully won't need to) =)

I think I wrote an update the other week after seeing Dr.Bragg about how we where going to post-pone making any adjustments to either shunt last week and she had said if I wanted I could see her on Tues once I got back instead of waiting till Thurs. I initially had thought I would do this but it turns out I got very little sleep Sun night in to Mon and got home very late Mon night last wk with very little sleep again in to Tues morning.
I just was to tired to want to drive to Madison. Hopefully whatever we try tomorrow will help. The uncomfortable-ness (is that a word!?) around the TPL Shunt and especially when I lay down has cont'd so I am hoping maybe Dr.Bragg will have some thought on that to. <--- We turned the VP Shunt down by 2 settings to 50 last Thurs I believe while the TPL Shunt is at 40. This helped some, not entirely but still some is better than nothing!

Thurs, April 24 -
I saw Dr.Bragg today (almost a wk ago) and initially we where going to adjust both shunts by one setting but then giving the odd and often strong discomfort I feel along the TPL Shunt we opted instead to leave that at '40' (30 is the lowest) and adjust the VP Shunt from '70' down to '50'. She said if I cont'd to have symptoms I should call and schedule to have the shunt adjusted again although it could be w either of her NP's to (whom are also good). As stated above there is a small change in headaches though I still feel so tired. Really I often feel like I've slept about 4 hrs when I've been going to bed earlier and though also having to get up earlier than usual I still managed 9-11 hrs the past few days. It could be a combination of all the various symptoms going on though, who knows. I don't know if I should have the VP Shunt turned down another notch or not. I thought maybe I'd wait till mid-to end of this current wk to see.

(This was written a few days ago, before today's Cardiology Appt and before knowing they would want to see me).
Probably not related but have been feeling symptom of short of breath (the feeling like you are trying to breathe underwater or have held your breath to long and can't get enough air now, not the 'I just ran really hard' SOB feeling. Seriously who knows who symptom like this belongs to though?
Honestly I often don't know which specialist a symptom 'should' belong to but this started worse yesterday especially when I went to bed but tonight occurring even while I am sitting completely upright in the chair. Hoping it will just improve and if not I guess I can ask my PCP tomorrow who she thinks I talk to or what does she think in general.
Monday i'll re-check INR for Cardiology so while I seriously hope for pete sake this is no longer occurring then if it where I can mention to them when Nancy, the NP calls with the result and any med adjustment (if needed). AGGHHH sometimes!!!!

(Boston) Marathon Weekend

I left for Boston on a Fri (Good Fri!) and though the flights where a bear (I also didn't have a direct route so took longer) I was happy to be in Boston once I got there; my friends the Siedman's whom I was staying with again picked me up from the airport.
Sat I was able to sleep in (thankfully) some and met up with Jessi in Copley Square after Jenn dropped me off at the train station which I took in to town. From there we took some pics near the finish line (the BAA 5k was just finishing and was a ton busier than it had been last yr so harder to get as close) after which we went and met up w some of the Genzyme-RFRD's Team members who where also getting manicures with us. Jess and I and one or two others who got their nails done opted for MPS purple and one nail done in Genzyme green w/a purple MPS ribbon decal on that one finger! AWESOME!

From manicures Jessi, Colleen (another runner) and myself headed out to Allston, one of Genzyme's manufacturing plants (the very first time I was to Allston back in 07' Aldurazyme was still bottled there but it is no longer) where we helped finish setting up for Sat. nights GRT-Patient partner/Family dinner (very fun!).

The dinner was a good time, at the table Jessi and I sat at there where about 6 other people including off hand 2 other runners, the GRT Coach and us + another family. SO FUN!

As far as actual race day Jessi finished in under 4 hrs (3 hrs 45mins I believe) and did AWESOME! Although I missed being home for Easter I was also sooo happy to be there to root for Jessi and so happy to see her smile when she crossed mile marker 14 (our Genzyme/Running for Rare Diseases Team headquarters), we quickly got to hug and say a very quick few words and then she was off again! I had a semi-early flight (there weren't great options for that Monday night when I booked) so didn't get to congratulate Jessi after but we've exchanged emails, texts and other messages since! I can't wait till next year again!! THANK YOU Jessi and the entire GRT Runners! I posted some pics throughout this blog entry and added some to the slideshow at the L of the blog updates.

Jessi is mailing the poster/picture that Genzyme/RFRD had at the Banquet of Jessi and then myself and Ben (IMO if you look closely at the pic means 'In memory of') which was I'd guesstimate about 2x the size of an 8/10 frame! It is sooo cool!!!! Thankfully the Siedman's will also be able to get of the poster/print made as I think according to Stuart and Jenn it seemed to mean a lot to them that Jessi also ran for Ben's memory. This whole weekend w the Siedman's and with Jessi + the Team was simply amazing!
As a really cool side bonus Jenn took me to the Church where Ben's funeral services where held for their Easter Service Easter morning and the service was simply put BEAUTIFUL! The sermon ('The 1 lost sheep (how the Shepherd left his 99 to look for the 1) and 'it is good') was one of the best I've heard in a very long time, this is saying something as I've felt the Pastor's at my Church are very good! The Choir and instruments where beautiful and the Church was gorgeous + the day was fitting! I pretty much slept all Sun afternoon but then Jenn made a dinner which was VERY good and some friends of all of ours came in that night from NY (their sons also have MPS III). Such a great wknd!
I am so very thankful to the Siedman's, to Jessi and Genzyme + the Genzyme Running Team and to Vanderpool's + all the wonderful memories from this wknd! I was happy to be home late Monday night but at the same time even though it meant missing Easter here I am so grateful for all the memories! Paying what I did for my plane ticket was even worth it! =)
As an added bonus My Mom made a Easter-ish dinner again yesterday so those of us kids who wanted would come over and be together!) =) Zander, my nephew spent Sat night and after Sun School and Church he and I went over to my parents where several of my siblings and there families also came. All was good!

Thanks for patience in getting this update written,
God Bless, take care,

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