Cardiology and Urology changes

Let me preface this by saying this entry has been written partly last week and now tonight as well.

I had talked to my Cardiologist last week about some symptoms ive been having (which have been controlled by meds for several years fairly well up to this point) and he wanted me to come in this week and get a cehst xray (CXR) and repeat Echo done before seeing him. This was earlier today and while the tests are the same as in Nov he is adjusting the  meds I am on by doubling the lasix dose (water pill) to be taken either 1 in the morning and 1 in the afternoon or both in the morning if tolerated and easier. This will be to reduce the cardiac workload secondary to the valve stenosis and leaking (insuff/regurgitation) + enlargement and thw idea is to reduce how hard the heart is having to pump due to the 2 valves and enlarged atrium. If this goes ok and breathing symptoms improve in 3-4 days we'll keep this dose and  if symptoms do not improve Cardiol. suspects the symptoms  may be as much lung related which is a different issue. If on monday the lasix is going ok but I am still having the rhythm issues we will increase the diltiazem by 1/2 to 180mgs - this is a longer acting med which helps control heart rhythm irregularieties. Today in additin to the Echo and CXR he ran several labs including one known as a BNP which measures if a person is in heart failure (high = bad, low= good) and will again repeat labs on monday (I can likely have them done at infusion and drawn from the port-a-cath to check how my body is handling the increased lasix dose and make sure several markers arent being messed with adversely.  In addition he is having my sleep med pulmonologist do a repeat sleep study (has been a few years as the one supposed to be done last spring ended up getting pushed off due to the shunt) to make sure there isnt obstruction going on as the breathing is the worst at night although these breathing issues also  often do occur secondary to atrial enlargement in people wth valve issues.  We talked about valve replacement more indepth again which has been a recurring theme the past few appts and his opinion was that it's a hard call when to do this as we'll have to do both valves at once which is harder on the body, a longer surgery and more demanding + both would have to be mechanical due to my young age and  the risk of MPS deposits occuring on tissue valves and re-occurence of the same issues. This of course would mean coumadin amongst other possible things and frequent blood tests to check levels and increased risk with other surgeries when on this blood thinner. We'll push off any replacements as long as we can and hopefully that will be a while as I told him and truly mean it; this is one surgery that scares the bejeezus out of me. I cant even begin to imagine that surgery and nor do I want to! =/

I saw Urology  last weds for the bladder spasms (secondary to spine nerve injury) and waking up many times a night to go to the bathroom/retention issues. This Urologist  is very sub-specialized in spinal nerve injuries and  was a little surprised by my up-tick in symptoms but said this type of spinal nerve injury is like the changing seasons; starts out cloudy, warms up a little, rains some, storms again (nerves repairng/recovering and Dec's surgery could of course have been a  potenital irritant although my neurosurgeon did not have to go back in to the nerve root areas where she decompressed in May but swelling I imagine still occured when she removed the LP shunt. He said that it takes about a years time in this ttype of injury to expect more normalcy though may not be rhe same as before  last May's lumbar laminectomy and shunt revision surgery. He is  leeping the vesicare and is a  adding levisin a sublingual med at  nightime that lasts for several hours and is for people who have problems getting to sleep due to having to get up frequently to go to the bathroom. Urodynamic testing will be repeated in June.  Oy vey - all this and only 28, it's supposed to be a old persons problem! :)

Neurosurgery Appt Fup was rescheduled for this thurs due to a snow storm - I ventured over to my sisters to play with my 5 yr old nephew and see my niece and the roads werent to bad on the way there but definitely much worse on the way back! I hate to say this and have been avoiding saying it but I think the shunt is on its way to failing again unless this is just a intermittent roller coaster effect related to something else. Needless if it is the shunt I am going to be incredibly disapointed as it is tiring feeling good, not feeling good, and on and on and on.. not that this is anyones fault just that it doesnt make it any easier to try and get back to planning out the normal things id otherwise be doing such as with APF. Im tired of shunts and headaches and nausea and feeling like someone is sitting on top of or inside my head.  Id like to pound on the wall but what good would that do besides cause damage i'd have to pay for later!?  I see neurosurgery tomorrow  so I just pray she has ideas and maybe these headaches will go away; its always possible.

The Adult-MPS I survey is expected to be completed next week (we are taking a different approach than originally planned with the genzyme/myself/select MPS I Adults) and will be sent to me per the Patient Advocacy Director for MPS I at Genzyme; i'll review it and we will then send it although we want to reach adults beyond just the few who are members of the Nat'l MPS Society and those also who may not be on ERT. (there are some).

Unrealted but just need to write because it's weighing on my mind and bothering me tonight, I really stink at not caring what other people think - I guess because im always trying to share what I know/experience to help other families and yet because I 'dont fit the mold' I guess of what people think MPS is supposed to be I do feel a little like people judge befre they really know me. I wish I was the type of person who didnt give two craps if others liked me or not - I mean dont get me wrong I have my 'close' friends in the MPS community - I just dont really see keeping in touch with 3 or 4 dozen diffeent people when I do have a life outside of this disorder and I am close with my family which is what counts to me.  The 1/2 a dozen MPS people I am close with dont take offense that I dont really talk on the phone or email that much and we catch up here and there. I think what im trying to say is I dont get the junior high attitude of some people, if they dont like you do they really need to talk behind your back or act so juvenile and spread crap when they really have no idea who you are?

Anyways I guess this is all, thanks for stopping by,

Erica