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
Monday, January 30, 2012
Thursday, January 26, 2012
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
Tuesday, January 24, 2012
Revision # 14 / VP shunt
I'll try to make this coherent - may end up coming back in the next day or two to fix typos.
I wrote in yesterdays update about Dr.Bragg's plan for this surgery (how she was going to move the tip of the catheter from my frontal ventricle to the ventricle at the back of my head) but once she got in to surgery the plans changed. She decided to open up the frontal incision first (originally was going to do this last after getting the other shunt tip in place) and found that the shunt was not completely occluded and was working partially (once again) which she was surprised by given thurs shunt tap and inability to pull any fluid out of the ventricle. Her conclusion was that the shunt from looking at the trajectoy and placement of the tip of the shunt (a CT was done right before surgery this morning) was in less than an optimal position and thus when the ventricle drained the tip would essentially come out and cause issues with further draining. I cant remember exactly how she explained it but it had something to do with that. In order to place a new valve/reservoir and get the tip of a new cathether in to a better placement Dr.Bragg did say she had to drill away a bit more bone than other surgeries and the incision is somewhat bigger but if it helps i'll deal with it! At the beginning of surgery when she realized the shunt wasnt completely blocked as she initially suspected she paused the surgery for a bit (took about 3 or so hours total for a otherwise relatively simple placement.) and had her partner called (a world renowned expert on hydrocephalus) in who while he didnt scrub in stayed for the rest of the surgery and they collaborated on how to proceed and placement. She tried to get further pictures for me of the build up within the shunt catheter but these pictures did not turn out as clear as the prior picture she had taken and given me with the scope they use so she didnt keep them. I so appreciate that she keeps wanting to try and help me and that she is concerned about my best outcome (she/they where concerned with moving the shunt tip to the back ventricle if we could get this one to work well given ive already had 1 infection and risk of bleeding would have been greater in the other ventricle.) and that she consistently wants to make sure we are still helping, not harming but also get me feeling better despite all these on-going up and down trials. She's a good dr and I appreciate her honesty, down-to-earth-ness (my own word I know!) and concern about her pts. Even when I may feel bad she knows how to respond and help, not always a common trait in all doctors.In recovery I was upset because I hadnt known why they didnt do what she originally was planned and was scared she changed her mind and didnt want to do anything; she came by in the recovery room and explained it all to me, explained her concerns and that she wasnt giving up on me, we still had options if this didnt work for the long run and she wanted to minimize what we had to do when possible while still giving the best possible outcome. She then said to me if I wanted she could refer me to her colleague or another neurosurgeon if I was discouraged. My response despite my awe at her honesty and concern was simply that she IS one of my favorite providers and as long as she isnt giving up on me, im definitely not giving up on her and really think she is very good at what she does on many levels.
She also for now left the 2nd valve which sits at my collarbone in it's location as when they tested the distal part of the shunt it was draining. She is a little concerned that my skin isnt handling the valve tubing (and connector) but felt that we would be better off not messing with it and if needed that is a really simple procedure to move it back to it's correct location further down on the chest (for those who know where chest Ports are placed the 2nd valve originally sat in that location). Sh also said it's possible at some point we would have to add a 3rd valve to slow down CSF drainage and prevent the ventricle from collapsing (several other of her and her partners pts have a 2nd and a 4rd valve to). She was pretty confident today's surgery would work and felt like this might be our best outcome yet, I am crossing my fingers!
Anesthesia went ok, a difficult intubation but also not the worst ive had so that was good as it is alwats one of the biggest concerns. The head anesthesia doc wanted a copy of the echo from my Cardiologist before proceeding to surgery (I knew my cardiologist was ok with this surgery and had just saw him last week and emailed with him yesterday) so they managed to get that although the Echo reports are always very different from what my Cardiologist puts in his reports so I personally ddnt think it would be that useful anyways. After surgery I spent the rest of the afternoon with quite fast heart rates (120-130's but normal much slower except for the past couple weeks on and off) which also caused impressive light-headedness that was less than pleasant. Such joys.
I'll update when there's something to write - thanks for stopping by,
Erica
I wrote in yesterdays update about Dr.Bragg's plan for this surgery (how she was going to move the tip of the catheter from my frontal ventricle to the ventricle at the back of my head) but once she got in to surgery the plans changed. She decided to open up the frontal incision first (originally was going to do this last after getting the other shunt tip in place) and found that the shunt was not completely occluded and was working partially (once again) which she was surprised by given thurs shunt tap and inability to pull any fluid out of the ventricle. Her conclusion was that the shunt from looking at the trajectoy and placement of the tip of the shunt (a CT was done right before surgery this morning) was in less than an optimal position and thus when the ventricle drained the tip would essentially come out and cause issues with further draining. I cant remember exactly how she explained it but it had something to do with that. In order to place a new valve/reservoir and get the tip of a new cathether in to a better placement Dr.Bragg did say she had to drill away a bit more bone than other surgeries and the incision is somewhat bigger but if it helps i'll deal with it! At the beginning of surgery when she realized the shunt wasnt completely blocked as she initially suspected she paused the surgery for a bit (took about 3 or so hours total for a otherwise relatively simple placement.) and had her partner called (a world renowned expert on hydrocephalus) in who while he didnt scrub in stayed for the rest of the surgery and they collaborated on how to proceed and placement. She tried to get further pictures for me of the build up within the shunt catheter but these pictures did not turn out as clear as the prior picture she had taken and given me with the scope they use so she didnt keep them. I so appreciate that she keeps wanting to try and help me and that she is concerned about my best outcome (she/they where concerned with moving the shunt tip to the back ventricle if we could get this one to work well given ive already had 1 infection and risk of bleeding would have been greater in the other ventricle.) and that she consistently wants to make sure we are still helping, not harming but also get me feeling better despite all these on-going up and down trials. She's a good dr and I appreciate her honesty, down-to-earth-ness (my own word I know!) and concern about her pts. Even when I may feel bad she knows how to respond and help, not always a common trait in all doctors.In recovery I was upset because I hadnt known why they didnt do what she originally was planned and was scared she changed her mind and didnt want to do anything; she came by in the recovery room and explained it all to me, explained her concerns and that she wasnt giving up on me, we still had options if this didnt work for the long run and she wanted to minimize what we had to do when possible while still giving the best possible outcome. She then said to me if I wanted she could refer me to her colleague or another neurosurgeon if I was discouraged. My response despite my awe at her honesty and concern was simply that she IS one of my favorite providers and as long as she isnt giving up on me, im definitely not giving up on her and really think she is very good at what she does on many levels.
She also for now left the 2nd valve which sits at my collarbone in it's location as when they tested the distal part of the shunt it was draining. She is a little concerned that my skin isnt handling the valve tubing (and connector) but felt that we would be better off not messing with it and if needed that is a really simple procedure to move it back to it's correct location further down on the chest (for those who know where chest Ports are placed the 2nd valve originally sat in that location). Sh also said it's possible at some point we would have to add a 3rd valve to slow down CSF drainage and prevent the ventricle from collapsing (several other of her and her partners pts have a 2nd and a 4rd valve to). She was pretty confident today's surgery would work and felt like this might be our best outcome yet, I am crossing my fingers!
Anesthesia went ok, a difficult intubation but also not the worst ive had so that was good as it is alwats one of the biggest concerns. The head anesthesia doc wanted a copy of the echo from my Cardiologist before proceeding to surgery (I knew my cardiologist was ok with this surgery and had just saw him last week and emailed with him yesterday) so they managed to get that although the Echo reports are always very different from what my Cardiologist puts in his reports so I personally ddnt think it would be that useful anyways. After surgery I spent the rest of the afternoon with quite fast heart rates (120-130's but normal much slower except for the past couple weeks on and off) which also caused impressive light-headedness that was less than pleasant. Such joys.
I'll update when there's something to write - thanks for stopping by,
Erica
Monday, January 23, 2012
Surgery (revision) #11 / #14
This almost seems nuts to write but tomorrow will be revision #14 and #11 since last May 2011. I have to be at the hospital at 8:30 and surgery is scheduled at 10:30. Pre-op went fine on fri with my PCP's concerns being the cardiac issues and airway as well as making sure the cortisol replacement (cortisol is a stress hormone and has to be given in a large dose 1 hour prior to any procedures or surgeries to prevent the body from having extreme problems.) This is a med I take in a much smaller dose to replace the deficient cortisol every morning and afternoon. As far as cardiac issues my Cardiologist had just upped the lasix dose mid-last week which has helped improve breathing (reducing pressure load on the heart and taking off fluid) but he is holding off on increasing the diltiazem dose until I am recovered from this surgery - that increase would be for abnormal heart rhythms secondary to the atrial enlargement and valve issues.
Since I had ERT today the nurse just left my Port accessed and I will cover it before I shower and then change the dressing after so that skin doesnt break down as otherwise frequently occurs when any kind of dressing or material is on my skin for to long. (I have a pain patch I wear and ended up with essentially a first degree burn from it bc of such skin sensitivity and anytime my neurosurgeon uses dissolvable stitches she takes them out at the fup appt de to skin irritation.)
I think Dr.Bragg is only replacing and moving the proximal part of the shunt from the current location to a more suitable ventricle at the back of my head which likely means opening the current incision, the incision behind my ear and making the new one. If the 2nd valve that sits at my collarbone looks alright I think she plans to leave this in place though she had mentioned it is up higher than is normal.
I'll update when possible - stay tuned,
Erica
Since I had ERT today the nurse just left my Port accessed and I will cover it before I shower and then change the dressing after so that skin doesnt break down as otherwise frequently occurs when any kind of dressing or material is on my skin for to long. (I have a pain patch I wear and ended up with essentially a first degree burn from it bc of such skin sensitivity and anytime my neurosurgeon uses dissolvable stitches she takes them out at the fup appt de to skin irritation.)
I think Dr.Bragg is only replacing and moving the proximal part of the shunt from the current location to a more suitable ventricle at the back of my head which likely means opening the current incision, the incision behind my ear and making the new one. If the 2nd valve that sits at my collarbone looks alright I think she plans to leave this in place though she had mentioned it is up higher than is normal.
I'll update when possible - stay tuned,
Erica
Thursday, January 19, 2012
Neurosurgery and the ties that bind..
Today's neurosurgery appt turned out well or atleast better than I hoped except that I was right and shunt is basically failing again. I had been incredibly nervous (and I dont normally get nervous before appts) before this and before yesterdays cardiology appt because while I knew what I was feeling it's still hard because you dont always know if your provider is going to believe you. In all reality I need to have stronger faith in these 2 providers as they are 2 of the best on my team of specialists and both have good supporting teams. I literally cant explain why I felt the way I did but I was kind of depressed leading in to these appts; I guess because I want to feel better but in the first place I didnt want to be not feeling good to begin with. Ive had such weird symptoms lately between the nerve injury issues flaring up (bladder spasms which cause such gosh aweful and at times unrelenting cramps, a leg that is falling asleep randomly and often when I am standing as well as a period which is haywire -this month lasting basically a day and last month a couple days, I suppose due to endocrine-hormone dysruptiom from the on-going, intermittent shunt pressures/revisions. The cardiac stuff has been a little aweful at times with the rhythm issues literally just beating to its own very fast seeming chaoric tune althoug im not sure its chaotic but that it feels this way when there is so frequent skipped beats. I havent been sleeping well due to the breathing issues and the headaches which are separate but together create a really rotten time trying to fall asleep and then stay asleep and I sleep upright which helps this some but puts pressure on hips and low back and I apparently must move around often in my sleep as I often wake up with the headaches and feeling of trying to breathe past an elephant on my chest after tossing and turning in to a flat sleep.
At today''s appt I said to Dr. Bragg how I really jsut hadnt wanted to come in there with yet more bad news with the shunt and that I so wish I could come once or more importantly many times in a row and cheer that the shunt IS working right. (not that I was saying it was her fault as it is not). She and her nurse in their usual gracious style told me I ought to never second guess myself and although we've had a bit of a difficult time getting the shunt to stay working my symptoms have followed the same pattern each and every time and she had no doubt there was something wrong then as everytime ive said the shunt isnt working when she explored it, it hasnt been right. She did a shunt tap which showed the only CSF she was able to pull out was the fluid sitting directly in the valve/reservoir whereas she should have been able to pull some from within the ventricle to. This combined with when she depressed the reservoir it was slow to refill confirmed to her we should try saomething else. They will call tomorrow with a surgery date (she had asked if there was anything I wanted her to avoid scheduling around) as it was after normal clinic hours by the time I was done and apparently they are going to try to do it in the next week or 2. Apparently she has been brainstoming trying to think of ideas to make this work better in addition to moving the location of the proximal (beginning) end of the shunt from the ventricle above and to the R of my right eye to behind my head; a space that has less tissue and where the shunt tubing can be placed furher in to the ventricle. I had to give her and her nurse a little bit of a hard time when they are thinking of me days before im even coming in for an appt we clearly are spending to much time together; the funny part being several of my other providers will also commonly say they where thinking of me the morning before I saw them or several days before and some will send emails to see how im doing when they havent gotten any updates. Yikes, I am very glad to have caring providers but like iIsaid, clearly spend to much time together!
I have to call and schedule a pre-op physical tomorrow (#501 it seems like) although i'm kind of hoping since I did just have a pre-op in Nov for Dec's. surgery and I just saw cardiology yesterday that maybe my PCP wont make me come in; I can wish right!?
I'll update when there's something more to note,
Thanks for stopping by, leave a note if you like,
Erica
At today''s appt I said to Dr. Bragg how I really jsut hadnt wanted to come in there with yet more bad news with the shunt and that I so wish I could come once or more importantly many times in a row and cheer that the shunt IS working right. (not that I was saying it was her fault as it is not). She and her nurse in their usual gracious style told me I ought to never second guess myself and although we've had a bit of a difficult time getting the shunt to stay working my symptoms have followed the same pattern each and every time and she had no doubt there was something wrong then as everytime ive said the shunt isnt working when she explored it, it hasnt been right. She did a shunt tap which showed the only CSF she was able to pull out was the fluid sitting directly in the valve/reservoir whereas she should have been able to pull some from within the ventricle to. This combined with when she depressed the reservoir it was slow to refill confirmed to her we should try saomething else. They will call tomorrow with a surgery date (she had asked if there was anything I wanted her to avoid scheduling around) as it was after normal clinic hours by the time I was done and apparently they are going to try to do it in the next week or 2. Apparently she has been brainstoming trying to think of ideas to make this work better in addition to moving the location of the proximal (beginning) end of the shunt from the ventricle above and to the R of my right eye to behind my head; a space that has less tissue and where the shunt tubing can be placed furher in to the ventricle. I had to give her and her nurse a little bit of a hard time when they are thinking of me days before im even coming in for an appt we clearly are spending to much time together; the funny part being several of my other providers will also commonly say they where thinking of me the morning before I saw them or several days before and some will send emails to see how im doing when they havent gotten any updates. Yikes, I am very glad to have caring providers but like iIsaid, clearly spend to much time together!
I have to call and schedule a pre-op physical tomorrow (#501 it seems like) although i'm kind of hoping since I did just have a pre-op in Nov for Dec's. surgery and I just saw cardiology yesterday that maybe my PCP wont make me come in; I can wish right!?
I'll update when there's something more to note,
Thanks for stopping by, leave a note if you like,
Erica
Thursday, January 12, 2012
Cardiology and Urology changes
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! =/
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
Tuesday, January 3, 2012
May the New Year.. In the New Year..
It is always sobering reading specialists reports - I doubt they intend it but what they tell their patients and what they write in their reports is often different. My former GC once explained it as maybe drs have more time to think about what they are seeing and so unintentionally change or add to their opinions in these reports forgetting that they've told their patients something different. Once I heard that logic it didnt bother me much but it still doesnt make it any less sobering some of the time! One of the comments while my Cardiologist and I have talked about it, to see it in his most recent report was just one of those sobering moments. I wont worry about it endlessly but like I told my Cardiologist im human and that doesnt mean these things dont bother a patient at some point! Here are some of the comments from the report amd above are the cont'd and new findings:"Not an ifeal surgical candidate- continue to monitor and watch until symptoms continue to progress and no longer manageable or stenosis and related issues worsen. Maintained on current medications until then" - diltiazem long acting and lasix for cardio-pulmonary symptoms management. I had asked if we needed to change the cardiac meds I am on to slow this down not really thinking about the fact that it is the MPS storage causing these cont'd issues not otherwise 'normal' cardiac changes. Some of the changes are listed below - these are a combination of the MPS damage and further progression (ERT has not halted this area) and the congenital aortic valve issues.
Moderate Left ventricle hypertrophy
Severely dilated left atrium
Moderate aortic stenosis and Moderate Mitral Stenosis
Moderate Aortic insufficiency
Heart rate typically mid 90's to 105' range most of the time while blood pressure quite low at typically 90's/60's and often 80's/50's.
Abnormally skipping rythms but not more than a couple extra or skipped beats and mostly controlled yet. Increased heart rate is really the annoying one but even that is a 'more my normal' bc it has been years now so not as noticeable as it once was.
A theme of the past several year ends and new years; i'll never be the 'perfect' patient as I know full well I am too stubborn and want to be involved in my care (I dont see that as bad) and admittedly I can be kind of opinionated. I dont intentionally try to be but sometimes realize it or not I just am.. This year has definitely brought alot of new changes and some answers to things I long suspected but none of my drs or I could not really get answers to. The biggest issues have been the shunt (but im so grateful to be able to write this with definite noted improvement in my headaches other than the 1/2 a dozen times a day where the pinching-searing type sensation occurs and despite the vision stuff. ) and having found a neurosurgeon who really listens, really seems to care and goes out of her way to make me realize she isnt leaving. Related to the headaches and shunt of course have been the parade of surgeries but noteable during most of these including now is the periods short and longer where I did have much symptom relief including mid-back pain completely went away when LP shunt was tied off and subsequently taken out last month and constipation is so remarkably improved as is the headaches themselves. Low back pain has cont'd to be a pain literally whether related to not quite enough pressure/fluid being drained (Dr.Bragg's thought) or due to something else remains to be seen but on the other hand with the unexpected lumbar spine lamiectomy (bone removal) during the initial LP shnt revision in May and nerve bundle repair and the resulting side effects but continued healing of those said side effects has been while not lovely I can and do atleast appreciate that the symptoms ahove cont'd to improve! Id of course have rather not have had the complications in the first place but know and recognize in MPS much worse could have occured. Ive said it before but if that area needed to be decompressed im almost glad it was unexpected as then I had none of the pre-surgery spine stress to deal with and I know Dr.Bragg can manage these issues for me.
Other changes included to my Neurologist leaving my team and consolidating this care w Dr.Bragg managing all neurologic care and if needed she will consult the neurologist she works with on her complex shunt pts and whom I met previously prior to the VP shunt placement a couple months ago. And as well change in my Genetic Counseor of which has had the most impact in care and for which I now have a new GC but mostly dont have alot of contact with this group other than for infusions weekly and that is limited contact. Sometimes it does honestly feel like the more you do as a patient for your own care the less particular providers do in general be it to try and help find an answer or to a specialist, etc. That is the one thing I miss about my old GC she and I sure may have had our differences but she was ingenious at finding the good drs and getting things done. I have not one single idea how to remedy this.
I tihnk what I most hope for the new year is to continue to have opportunities like with Genzyme project (which we are modifying but cont to work on) and with what I do with APF. Both are so patient in my less than perfect ability (especially with APF) to be as involved as some leaders are in APFAN's outreach. I love what I do with this organization and pray in the new year I can cont to have the energy and time and strength to give and pray even that I may do more than I sometimes was able this year. I pray for good decision making in my care and the ability to cont to make decisions for myself regarding who I see and where. Related to medical care I especially pray I make good choices on who is and who is not involved in my care team and who if anyone of my providers I should make changes to.
I dont know what this year will bring but I do know I can decide how I react to whatever these things will be when they occur and I am going to try to cont to react in a cool=headed way that allows me to make good decisions, receive good care and not be a barrier to my own in-put. I dont knwo if that really makes sense but I guess really I just hope to keep being calm under fire and do a better job of caring less what others think and doing more to soeak up for myself or ask the quesitons I sometimes want to ask but dont.
I hope the New Year brings new blessings for your families and I pray it brings memories big and small!
God Bless,
Erica
Moderate Left ventricle hypertrophy
Severely dilated left atrium
Moderate aortic stenosis and Moderate Mitral Stenosis
Moderate Aortic insufficiency
Heart rate typically mid 90's to 105' range most of the time while blood pressure quite low at typically 90's/60's and often 80's/50's.
Abnormally skipping rythms but not more than a couple extra or skipped beats and mostly controlled yet. Increased heart rate is really the annoying one but even that is a 'more my normal' bc it has been years now so not as noticeable as it once was.
A theme of the past several year ends and new years; i'll never be the 'perfect' patient as I know full well I am too stubborn and want to be involved in my care (I dont see that as bad) and admittedly I can be kind of opinionated. I dont intentionally try to be but sometimes realize it or not I just am.. This year has definitely brought alot of new changes and some answers to things I long suspected but none of my drs or I could not really get answers to. The biggest issues have been the shunt (but im so grateful to be able to write this with definite noted improvement in my headaches other than the 1/2 a dozen times a day where the pinching-searing type sensation occurs and despite the vision stuff. ) and having found a neurosurgeon who really listens, really seems to care and goes out of her way to make me realize she isnt leaving. Related to the headaches and shunt of course have been the parade of surgeries but noteable during most of these including now is the periods short and longer where I did have much symptom relief including mid-back pain completely went away when LP shunt was tied off and subsequently taken out last month and constipation is so remarkably improved as is the headaches themselves. Low back pain has cont'd to be a pain literally whether related to not quite enough pressure/fluid being drained (Dr.Bragg's thought) or due to something else remains to be seen but on the other hand with the unexpected lumbar spine lamiectomy (bone removal) during the initial LP shnt revision in May and nerve bundle repair and the resulting side effects but continued healing of those said side effects has been while not lovely I can and do atleast appreciate that the symptoms ahove cont'd to improve! Id of course have rather not have had the complications in the first place but know and recognize in MPS much worse could have occured. Ive said it before but if that area needed to be decompressed im almost glad it was unexpected as then I had none of the pre-surgery spine stress to deal with and I know Dr.Bragg can manage these issues for me.
Other changes included to my Neurologist leaving my team and consolidating this care w Dr.Bragg managing all neurologic care and if needed she will consult the neurologist she works with on her complex shunt pts and whom I met previously prior to the VP shunt placement a couple months ago. And as well change in my Genetic Counseor of which has had the most impact in care and for which I now have a new GC but mostly dont have alot of contact with this group other than for infusions weekly and that is limited contact. Sometimes it does honestly feel like the more you do as a patient for your own care the less particular providers do in general be it to try and help find an answer or to a specialist, etc. That is the one thing I miss about my old GC she and I sure may have had our differences but she was ingenious at finding the good drs and getting things done. I have not one single idea how to remedy this.
I tihnk what I most hope for the new year is to continue to have opportunities like with Genzyme project (which we are modifying but cont to work on) and with what I do with APF. Both are so patient in my less than perfect ability (especially with APF) to be as involved as some leaders are in APFAN's outreach. I love what I do with this organization and pray in the new year I can cont to have the energy and time and strength to give and pray even that I may do more than I sometimes was able this year. I pray for good decision making in my care and the ability to cont to make decisions for myself regarding who I see and where. Related to medical care I especially pray I make good choices on who is and who is not involved in my care team and who if anyone of my providers I should make changes to.
I dont know what this year will bring but I do know I can decide how I react to whatever these things will be when they occur and I am going to try to cont to react in a cool=headed way that allows me to make good decisions, receive good care and not be a barrier to my own in-put. I dont knwo if that really makes sense but I guess really I just hope to keep being calm under fire and do a better job of caring less what others think and doing more to soeak up for myself or ask the quesitons I sometimes want to ask but dont.
I hope the New Year brings new blessings for your families and I pray it brings memories big and small!
God Bless,
Erica
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