Post-op 1 week

Sorry ive not updated since late last week - I did get discharged on thurs w/quite the discharge process once again - it's gotten where my Dad just works the entire day because we know it rarely goes fast but this one made even the last one in Dec seem somewhat smoother! The big issue wasnt my neurosurgeon signing the papers or her NP stopping by in the afternoon (to fill out a script and we ended up chatting some in the hall)  or even Pharmacy that all went well but because my morning nurse got done early and someone else took over but never came by to de-access my Port. I finally got a nurse to come by (98% of the time I get the same nurses now but was a different one ive not met who took over) and anyways she didnt know how to take out a Port needle (flush with saline, make sure there's a blood return, and then flush with 5cc 100u heparin to prevent any clots from forming till I used the port today (ERT infusion).  So once I walked them through this all then they had to order up heparin which took another while and needless was a long day! My neurosurgeon originally came by around 11:00 and we didnt get out till around 7 - I slept from almost the moment we got in the car all the way back to my parents and then fell back asleep about 11 thurs night till 10 the next morning once I got back to my apt (I leave my car at my parents before surgeries and then drive home from there).

Headaches remain about 50/50 with when they are there affecting vision and nausea and just not great but when the pressure is not there I really do feel great - very odd. I fup on thurs. though no stitches as the only incision is dissolveable.  Ironically already a few weeks ago I had been moving around other things on my schedule so I could have atleast thurs morning free (this was before surgery was scheduled/knew surgery was going to be done again) so I could go to my nephews 3 mo autism-ABA (routine) eval - I didnt go to the last one due to conflict in schedule so was looking fwd to this - Sara and I usually get lunch on the way back and is a nice way to hear whats going on with Z progress wise though I am at his house enough when the head therapist is there. One of these times i'll be able to have an open schedule to go with again. :)

**Add in note: The increased lasix dose seems to actually be helping quite a bit with breathing (to reduce load pressures on the heart and lungs secondary to the severe atrial enlargement and aortic/mitral valve issues. - Last night I ended up taking some lasix late in the evening (I take a dose before ERT and also after ERT but for the past 2 weeks have been on a doubled dose of lasix as well) (about 8) which of course meant going to the bathroom till early morning (secnd night of being up till 2-2:30), but this significantly helps the breathing issues. My  Cardiologist is holding off on increasing the cardizem (diltiazem long acting) dose until ive recovered from this shunt surgery - I think he just wants to make sure the increased heart rate and rhythms arent related to the shunt and give my body time to heal before adding in something else as well. I have a sleep study the end of Febr. (could have done it thurs but quite a bit going on this week already). and so suspect perhaps he may wait until this study is done or atleast for a few weeks if we do increase this med dose.

Thats about it for now - will try to update again soon,

Erica

Post-op 1/2 revision 14

I say this only because it is true today; MPS truly sux and right now I am disliking it akit; most of the time I can deal with it fine but after this day id swear my body is on a one man mission to make me miserable.  This morning I had symptoms like a UTI but also wasnt to sure since I do have bladder issues and go from having retention issues to having to pee quite often alot despite meds I take. A culture came back negative and then shortly after the new upped lasix dose was given (6th day at this dose) and I peed like a siv so it didnt matter what I felt for the next 6 hours. Tonight the symptoms came back but with a vengeance with urinary frequency and pain with peeing as well as lasting afterwards. A new culture showed some signs of a infection (possibly from the cath post-op in recovery as I didnt have a foley in during the 3ish hour surgery and couldnt pee in a container so the nurse had to cath to drain 700+ of urine out of bladder while waking up. Im not sure but would suspect that's where any infejction has come from. The docs are doing a more indepth UTI lab and ordered Cipro and ive taken the top dose of both the vesicare and the levsin now which help bladder spasms if it is that. I am sooo tired I am going to feel drunk soon from lack of sleep as last night I slept only about 2 hours or 3 tops.  Lightheadedness was on and off today but not as bad as yesterday and heart rate was so-so but still noticeable. Headaches are at a 50/50 range ie 50% of the time I can feel pressure building up (CSF) and 50% of the time I feel pretty good as the fluid (CSF) is draining. I literally almost can feel it in certain parts of the cathether. Strange.

My neurosurgeon and her NP stopped by earlier weds night and the NP asked how I deal with this all and still stay so positive? I didnt really know how to answer that other than to say that I cant hardly get upset at her or my neurosurgeon, the nurses or anyone else just because my body seems hell bent on hating me quite often; it definitely isnt their fault and I just appreciate their helping and caring. She made the comment I could be the poster child for living with chronic pain which was a nice comment and especially since I do advocacy work with the American Pain Found-Action Network and it is a passion of mine. But in all reality even when I have bad days and I get frustrated at some providers (not this team) in all reality I know I cant hardly (most of the time) take out my bad luck on other people, shoot even like yesterday when I come out of surgery and am crabbier (not that I was to the nurses or my dr) I feel guilty because I dont ever want to be that high maintenance pt and hate the thought of that. So I guess how do I stay positive? I dont have that answer other than ive found my passion and it's separate from my disorder and so when I do feel even slightly better I really try to help others and I enjoy all of the experiences I get to have w/APFAN and I also still get to do alot with MPS with Genzyme, etc.. I also am the lucky-ducky aunt of 6 amazing nieces and nephews and get to spend alot of time especially with my 5 year old nephew who reminds me constantly (he has high functioning autism so has his share of complexities in his life) that even when things are frustrating we still dont have to let it define our lives. I dont know if any of this makes sense, given im pretty darn tired but just my thoughts. I think in all reality I just want to try and be a nice person and a decent patient so I can get along with my providers and they like me back and I think it can help foster better communication, trust and care.

My parents came up to visit tonight (they live about an hour from the hospital) and since we are big in to scrabble these days my Mom brought the new one we had bought a couple weeks ago. My regional manageer/director of grassroots advocacy also came up to visit and she ended up playing a game of scrabble as well and we caught up on ome of the latest goings on with APF and APFAN as there really has been a boat load of changes over the past few months. The company was really nice although as usual since I know most of the nurses here they are nice to talk to as well.

A friend of mine who also happens to be quite young (early 20's she is amazing) lost her little girl Olivia to her MPS battle this morning, Olivia was less than 3 years old and a couple months post-BMT. This is a sweet family and I feel so badly - I never want any child to lose their battle but Olivia and Brittney off all people is hard. My prayers are with her and her family. ^^Godspeed^^ Angel ^^Olivia^^ may you fly as freely and sweetly in heaven as your body did not allow here on earth.

I may get out tomorrow- will update when I know something,
Thanks for stopping by,

Erica