Sunday, May 25, 2014

Neurosurgery Appt, 5/21

I actually wrote the rest of this the other day and then forgot to post it so will just add a bit to say that our adjusting the shunt helped some; enough for now which is AWESOME!! This weekend has been pretty good, tiring but good enough which I will take! Yesterday I went garage saling on and off with my Sister, nephew and a friend of hers - the garage sales kind of stunk but was still fun and the weather has been GORGEOUS finally!
Today was the 1st Flea market in Cedarburg (about an hour from where I live) which is huge, I typically end up sitting down multiple times throughout as it is incredibly tiring on your body but still fun. I didn't buy much but being outside is good enough. =)
Afterwards we ate in Hartford and came to a Greenhouse my Mom, Sister and I go to (is a craft type store to) which means more flowers! I haven't taken pics yet of my potted flowers and need to get more dirt but love what I got today - many varieties of flowers and colors!
This coming up wk is a cookout at my Sister's, ERT Tues, Cardiology Thurs., and PT Fri + may meet a friend after.

I saw Dr.Bragg yesterday (Thurs) due to the increasing headaches yet again, we talked about various thing including the newest cardiac med (digoxin) and she commented about how the heart (heart rate) issues likely play a small part in the headaches as does what she believes are changes in ICP (intracranial pressure) needs even in shorter periods of time. I had commented how it is sometimes frustrating that we get improvement w/a shunt adjustment for a few wks and then is like it starts all over again.

We opted to adjust the VP Shunt setting down to '40' (I think sometimes I am losing my mind, lol, I could have swore that is where both shunts where set at when we adjusted the VP Shunt some wks ago but I don't always keep track either).
We both agree it isn't that the shunts aren't working (is completely different to tell since I have 2 shunts, so if 1 isn't working very well the other can pick up some slack) but it's possible 1 of the shunts isn't working very well and if this setting adjustment doesn't make much or any difference she said to come back in and she'll likely tap the VP Shunt to see how it is flowing.
It seems like she must think about my driving in to see her as she'll often comment along the lines of my having to make another trip when we're having issues but I am just grateful she cont's to try and cont's to try to think outside the box; that she still seems to take the whole situation in to account..

On a completely separate but I guess related note, I just realized today, it has been 3 years since I started seeing Dr.Bragg, 3 yrs I think since our first shunt revision (that had turned out to be more complicated than originally thought) at that time I still had a LP Shunt (lower back, ran around where valve was implanted on the side and ran in to the abdomen); an hour or two surgery ended up being 6+hrs as it was more complicated due to what she had to do w the spine nerves, shunt, etc. Non-the-less 3 yrs!?!

It has been 2 yrs since she placed the 2nd Shunt (the VP Shunt was added in Oct the yr I started seeing her, the LP Shunt had been tied off at that time and in Dec of that year it was removed when everything was first taken out due to a CNS infection. The 2nd shunt was added in May 2012.
Almost 30 (if not that many) shunt surgeries and procedures later, 2 OHS's and several other smaller surgeries un-related to her and she's stuck by my care which is AMAZING!
I know I doubt that Providers will want/chose to stay on my Team and chose to still care and yet deep down I know with her and with my Cardiologist they are in it for the long-hall and I as their patient need to be more trusting even in the times of un-certainty (remind me of this next time I am writing here about being nervous/scared/uncertain)..

I am eternally grateful she took on my care and that she never gave up, that said I really would love it (see above, soo psyched, hope it lasts!!) if this adjustment would make a bigger difference and we wouldn't have to do anything!?!?!
Thanks for stopping by,
Happy Memorial Day, remember the reason for this weekend!

Monday, May 19, 2014

Hard to explain; a little depressed, MPS really stinks.

I can't really explain why but I have felt a little depressed the past few days (unusual for me, usually even in the worst of moments I am able to find something good) I guess maybe because physically I don't feel great and even with the new med a few wks ago and adjustment to this dose last week (cardiac med, digoxin) I still feel short of breath often (not a typical I've just walked a long distance SOB but a feeling like your trying to get enough or better air) and is occurring often. No matter how much I sleep I still feel tired and feel worn out (in a tired way) which kind of stinks as some of these nights I've slept close to 10-12hrs and so shouldn't be tired.
Headaches which I am very positive are un-related to the breathing thing and which where really good for a few wks have been an issue again which is a little depressing to. I've written many times about my apprehension at asking providers for help/telling providers when issues are recurring yet again and I am sure that is likely part of why I feel a little down.
I don't know, I guess maybe i'll just never get used to asking other people but especially providers for help and in a case like this where it is 2 different things it is really getting to me.

I think the cardiac stuff is depressing b/c not only did we just do the 2 OHS (open heart surgeries) less than a year ago (July and Sept) but then in Febr. the 2nd valve that has been an issue (mild issue w stenosis and leaking) for years showed it has worsened (moderate narrowing/leaking) and symptoms where mildly occurring again around that time, worsening still more since. then.
Hopefully meds will slow down or stop the valve from getting worse for many years but at the same time the way these symptoms are is frustrating and not at all awesome considering it was less than a year ago we had just got rid of the very same thing by treating (replacing) the aortic valve.
I am hopeful the new med (digoxin) will eventually work better to slow down heart rate which Dr.Earing had said he was a little surprised it was still in the range it was as many months out from the OHS's as we where. My hope is the beta blocker, digoxin and 2 meds (diuretics) will help to get fluid off, will help slow heart rate down so the heart has more time to rest in between each heart beat and thus not as much effort being forced on the mitral valve-the valve we are trying to avoid having to replace for as long as possible.

Side effects of the Coumadin are already a-plenty so I don't look forward to the day we'll have to use an even higher dose of this to get INR in to an even higher range. On one hand I really, really want to avoid replacing the mitral valve (the higher Coumadin dose, higher side effects as well as I am sure a 3rd OHS would be it's own issues) and on the other hand I just want these stupid breathing issues to go away, preferably without surgery would be great. Thankfully at this point we aren't yet looking at another OHS and Dr.E will do his best to avoid it as long as we can.
I can only control so much of this right now so just do what I can (meds wise, INR mngnmt) to avoid more surgery and am choosing to not worry now but that said it doesn't mean the symptoms don't bother me and doesn't mean I wish I didn't feel as crappy/do wish I felt better! (INR has been out of range since the last surgery the end of March though was close to target range 1 or 2 times. I re-test again next wk before ERT).
I also f/up with Cardiology from the Digoxin first being added a month ago next Thurs (28th I think). Dr.Earing per Nancy, the NP did opt to increase this dose last Monday to double as heart rate was still in the high 90's as he is still aiming for under 70 (briefly mentioned above).

Headaches are just depressing, it literally feels like a never ending cycle, we get in a good place but it never seems to last long and no matter that I know it's really something with the shunts I still feel questionably crazy b/c the nights are on/off bad and mornings-for the first few I feel as if I am in a daze with a clamp or pressure or something over my entire head. This all slowly (crazy I know) melts away generally in a few hrs., somedays quicker than others and almost always gone by the time I am around anyone.

I didn't know her super well and had only met her once (in our rare disease, MPS community some times you are closer to the people you never meet or only meet once then to the people you meet often though) but this morning a friend of mine, who was just a few years younger than me and who also had MPS I (in her mid-20's, post-transplant for MPS I-H) passed away unexpectedly from an otherwise not that out of the ordinary surgery.
Stacy was someone who had also been through OHS so although we had different types of valves (she tissue, myself mechanical) we would talk about this and about other things related to our MPS. What i'll especially think of and remember her for is she also adored cats and we would share pictures with each other (those photo posts) of different cat memes/posters we each saw on fb.

Sometimes you literally, almost can forget that MPS is a life threatening, all encompassing disorder and then you wake up one day or log on to fb one day and see a friend, so close in age to your own un-expectedly passed away and it is like a cold, slap in the face.
I am glad to have known Stacy the little that I did and for her family, well they will be in my prayers as I can't imagine the pain her Mom (who is especially sweet) and her family are feeling tonight. Godspeed Stacy, while the earthly loss is great, your heavenly gain is beyond anything, any of us can imagine!

There are other things bothering me but some of these are more to do with people and things I just need to think about and figure out. I am really grateful to those who are there through my good days, my crabby days and to whom the MPS/medical stuff isn't who I am but they also realize my life isn't like the average, typical persons and still are there. Thank you if you are reading this!

I am sure I will buck up and cheer up one of these days, for now thanks for stopping by and thanks for following.


Friday, May 9, 2014

Various Appts, not much else.

This week has brought a few more appts (imagine that, right!?) with normal Pain Mngmt f/up (required by federal or state prescribing laws for opioid med prescriptions) and 3 mo f/up with Physical Med Rehab dr (Caldera) for botox injections which are done in the neck, across shoulders and upper spine + some of thoracic (mid-spine) region to calm muscle spasticity. PT as well but that is close to home, the same city as my parents live and not to far from where I live.

Pain Mngmt wasn't really anything new, if anything we talked more about what's going on with other aspects of my care (how the shunts have been, cardiac issues especially) and she asked about what I've been working on/if I am working on anything big for GS or other things I am involved in. (Sun School ends in 2 wks, 3 of us Teachers have been meeting to put together and finish our final group project activity as well as the craft (making kites for all the kids, 'soaring with God') for all grade level kids.) Anyways she is involved with one of the Pain Academies and always talks about different things she/they are working on around Pain prescribing/awareness and bringing attention to Chronic pain issues (specifically in recent years how pain mngmt has been vilified).
Someone she is working with is apparently interested in working with patients at some point down the road and so she I guess wanted to give a heads up. I obviously have plenty to do either way but if need be would help where needed if I could to help as lack of awareness around what medications can do for chronic pain pts is a huge issue.

Dr.Caldera, my PMR doc is pregnant which I knew last time, (her 4th kid) so will be out come July for 3 months (i'll see one of her partners, a dr actually who worked w/my Neurosurgeon after my 2nd shunt was placed for the next set of injections) but talked about a group she has been wanting to start for some time and is hoping to get off the ground in Fall once she is back. This would be focused for her Adult pts who have Congenital disorders so this includes disorders like MPS (something your born with), CP, SB, and other congenital disorders that affect the muscles and which pts age out of Pediatric services.
Some pts like myself do still have some Pediatric specialists who also treat adult pts and some Specialists who are duelly trained in Adult and Pediatric specialties (so joint Internal Med. and Pediatric Residencies or like Neurosurgery where they do a fellowship after training is complete in Peds Neurosurg. But other pts due probably to the type of specialists they see do not access to Pediatric Specialists once they age out (I guess).
In any case the idea she had was patients would be able to exchange information, specialists, resources and just generally stories + help each other which seems like a good idea. =)

Headaches have been somewhat ok, last weeks adjustment helped a little more, and really a little more is better than nothing more. Do I wish it was as good as some of the times or for instance like w the external drains? ABSOLUTELY! But I guess this is better than nothing? I'll take what I can get at the moment and only hope something improves or at the very least nights and mornings get better.. I can hope!
I am supposed to schedule a f/up with Dr.Bragg so will do that in a couple wks if I con't to feel at minimum this 'ok'. Ugh, I am tired of asking her for help, tired of the stupid shunts and tired, tired, tired of the headaches and thus related symptoms so I just hope things will stay the way they are and I don't have to call/see her sooner!?!? - It's not that I don't like her (I do) just that I don't know that she knows how to help right now and I feel at a loss b/c I know these headaches suck and I know it's the exact same symptoms at a different degree than other times (always been variable at different times) and I honestly just feel whiny, not that she makes me feel this way but seriously how do you just keep asking for help, over and over and over again for the same issue?

Last but not least (sorry if these updates are kind of boring, I am not much for regaling people with stories, I basically only blog for the purpose of updating about the goings on w/the medical stuff) the new cardiac med, Digoxin atleast doesn't seem to have side effects and though it hasn't slowed heart rate down (the real goal) it seems from what I've read it can take a few wks for it really to kick in fully?
I have to re-test INR again Monday while at ERT and give Cardiology a heads up when my Infusion Nurse takes the lab over so when the NP/Nurse calls I guess will let them know if HR is still as high as it seems to be now. It is definitely annoying (feels a little like a constant butterfly fluttering in your chest!?! SOB is a slight bit better but that to, it's not like it's really noticeable to anyone else b/c it's not as if it is the 'SOB with activity' but instead is just the random and worse at night SOB that makes it hard to get enough air or deep enough breath I guess-hard to explain that one. Same as was before the AVR.

On an un-related to med. stuff note, for Sun School and Mother's Day this wknd I bought some wooden bird houses and bird seed + am having the girls paint and/or decorate these houses + write a little note to their Mom's and we'll attach to the bird house (there's a rope at the top) a little saying 'Spreading the seeds of God's word'.
Hopefully the girls like the idea, seems a little different than the more average/more often flowers or having them make a card and has an applicable Christian theme... Our story also happens to be 'The workers in the Vineyard', so while not directly related it does go along with one of Jesus other parables. I really try to find crafts that have wider, real life application as a reminder that God's hand is in life all around us. I always hope this is effective, who knows?
Thanks for stopping by,

Thursday, May 1, 2014

It is what it is... but what is it? Who knows..

I guess this is a bit of a continuation from Monday's Cardiology (and overall) update.
I really wasn't sure if I should have the VP Shunt adjusted and hemmed and hawed about it earlier in the week even though I did think it could help. In the end I did opt to make an Appt with one of Dr.Bragg's NP's today and have that shunt adjusted by a small amount.

Meanwhile last night I had posted on my fb about how uncomfortable the area around the seeming TPL Shunt draining end had been and especially how much worse it was last night (but I also was fairly certain it would improve as it has done this 'get worse, get better, get worse' over a few wks now with last night by far being the worst symptoms wise though). Anyways, apparently Dr.Bragg saw this post as she was walking through the waiting room while I was waiting earlier today and stopped briefly to say she saw the post, would mention the symptoms to the NP's and she wondered was it cardiac related (I don't know)? I just said to her I was sorry it always seems like one thing or another and I hate that I seem to need to ask for their advice or help so often. As she usually is she was gracious but non-the-less I never feel great when new issues crop up and feel quite frustrated by these symptoms. =/
Sue, the NP turned the VP Shunt down by 1 setting (both shunts are at '40' now and opted to do a repeat CXR since we weren't sure what came of the Cardiology one (see further down). As seems to most often be the case, b/c why would my body ever want to give a clear cut answer (!!!!) the CXR looked fine, ie no CSF collection in the lung space, the shunt tubing is not out of place and overall seemed about the same as previous, after surgery per Sue and Radiology's report.
Although I hate being emotional I did break down in tears b/c although I don't really want there to be something big wrong I also just wish there was an answer as this clearly isn't getting better and I am frustrated at my body, especially lately. Couldn't I just have some time where there was just nothing going on and the MPS stayed at bay, ie things where calm?!? =/
Sue I think felt pretty bad and I do think she believed me, which atleast helps. I just think (and cant blame them) they don't know what to do. Dr.Bragg didn't want to adjust the TPL Shunt to drain less (Sue thought maybe this would help the discomfort) as she didn't want to do 2 different things at once, which I do really understand. Sue also asked if I thought I should be admitted so they could try adjusting the shunt over a period of days (I guess) which I didn't want to do as I feel like sure we could try that but overall otherwise I would just be sitting there and I can feel bad at home as easily as I can feel bad in-pt.. Here I can atleast do things when I feel somewhat better + hopefully the minor shunt adjustment will make a small adjustment in the headaches if not in the breathing/pleural area? We agreed if symptoms don't improve by next week i'll call and let them know, and if need be come back again (next wk is really busy with other appts and meetings) which doesn't sound great either as I've already been there 3 wks in a row.
I guess I also just hope it improves between now and then by some slim chance as I truly just don't want to keep having to ask Dr.Bragg for help?

Cardiology INR
Cardiology (Nancy, NP) called with the INR results as even though these where done on Monday morning and I saw them Monday afternoon after ERT I forgot to ask about the result and Dr.Earing vaguely discussed the results as a part of the overall other few labs he had requested separately. I hadn't realized Dr.E was including the INR result when he went over the other lab results and to didn't know when to re-test (I hadn't thought to ask till later as there is often a lot to remember as is already!)
In any case the INR was slightly low at 1.9, whereas my range is 2.0-3.0 but per Nancy who called today with the results and next testing request said that Dr.Earing was ok keeping the Coumadin dose the same and re-test in 2 wks (from last mon). I am glad atleast the dose wasn't tweaked!
She also commented how she had been updated about the cardiac symptoms and the seeming Neurosurg. issues and to please keep them updated either via email or via their ACHD Nurse line + also give a heads up when I retest in 1 1/2 wks.
They don't feel the discomfort with breathing is cardiac related as both Dr.Earing and Nancy both separately stated to the affect of "it would be unusual this far out from OHS for a patient to have lung issues/fluid collection related to heart issues" + the Chest Xray's both their's and now the one done at Dr.Bragg's where clear for fluid build up.
Non-the-less they would like to be kept updated to what becomes of these symptoms.

To put it mildly I feel incredibly frustrated today which seems to be the story of my life related to the MPS these days and just wish once lately there could be a clear cut, relatively straight fwd answer?!? I do kind of wish Cardiology and Neurosurgery would talk to each other though I also get it's not as if I am either of their only pt and both are extremely busy.
I just wonder though if it wouldn't help for one to exchange thoughts with the other? I don't really know..
Stay tuned, Thanks for stopping by,