Revisions, Cardiology, Thoughts...

This week has been down right exhausting as ive been watching Zander my 5 1/2 year old nephew 3 days (getting him up in the morning, taking him to school, picking him up, making lunch, lots of playing, etc). I had a bunch of other things also going on this week and on top of intermittently feeling pretty bad and not sleeping well by fri night I came home and fell asleep by 8 (typically dont fall asleep till atleast midnight and often later when shunt is working and when its not working all bets are off for any significant amount of sleep) and although I was supposed to meet my Mom and sister at 9:30 I didnt set my alarm right and woke up finally at 20 after 9 on sat.. Sat. I was still tired and sore (joints) and pretty worn out so hoping to get sleep tonight to. Zander wanted to spend the night and I felt bad bc I do like it when he stays. I guess in all reality the body knows when it can only handle so much and so when you finally reach a point you are so exhausted it just catches up. I think the same holds true with how my body sends pain signals as ive been back in PT for about 5 or 6 weeks now and it seems like when one area is worked out and mostly taken care of other areas can crop up; almost like the bodies sub-conscious way of not giving more than it can hande. Defintiely atleast feels that way the past week with low back, hips and knees as I feel much like a little old lady on top of the head stuff!   ~~~~  ~~~~

I cant even explain what this shunt revision feels like; 1. because I dont know the exact plan for what my neurosurgeon is going to do (she is so gracious when it comes to email and I have sent her a few articles I read about shunt valves  with 1 shunt valve having 8 settings vs the curent 5 my medtronic-strata valve has (more options I think for draining further CSF (although I wasnt sure from the article if the extra settings where in fact for actually turning the shunt to drain less) and she emailed me back to say she was going to look over the article and information + get back to me. I in turn emailed her back to say she didnt need to re-respond as we could talk before surgery tues or I can call her nurse monday to see if Lisa  knows what ideas Dr.Bragg may have more closely settled on. I had kind of hoped we would do a brain-dye study that can show if there are specific areas of the brain that are causing obstruction or communication issues and same with spine but am not sure if either Dr.Bragg didnt think it would be helpful (it was originally an idea she had brought up before the last revision) as well as wondered about the ICP/EVD so we could try a few things but I know that Dr.Bragg doesnt make hasty decisions and that she will make the best plan she feels can help and I am ok with that. My in-control side that likes lots of information is defintiely a little nervous for this revision becuase of not knowing exactly what we are doing ahead of time but over all I am ok.  I do plan to ask her about the low back/leg/foot pain and numbness as both my PCP and Physical Therapist expressed concern it could be spine related especially given my spine history and frankly the symptoms are down right annoying. I am hoping she might have some thoughts; I do also see the new Orthopedic dr (see below) in mid-april who apparently Dr.Bragg has worked with on some of her other pts and who does have alot of spine experience.  
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I think what has been hard about this revision is ive really had alot of people both MPS families and non-MPS friends weigh in on their thoughts often im very sure in a well meaning intention but when you as a person already are muddling through your own feelings about something and doing your very best to make the very best, most educated decisions (with the opinions of countless MPS drs, my neurosurgeon and I having talked quite a bit and with reading articles to learn more about intermittent obstructions/occlusions in shunts) I did and do feel like ive made the best decision I could for my situation. Ive tried to explain to friends who've asked if maybe I wasnt essentially trying to chase a golden goose (so to speak) and get a headache resolution that maybe we are never going to achieve that in fact we have gotten complete symptom resolution and these shunt revisions have nothing or atleast very little to do with that and everything to do with actual, physical occlusions in the shunts that have been occuring very often (as evidenced by the revision surgeries and amount ive talked about the crazy shunt here). and preventing worsening in symptoms re-occurences secondary to these shunt issues, like vision (blurry, fuzzy), endocrine hormones worsening, autonomic symptoms almost completely resolve with each revision, sleep and how severely it is affected when the shunt isnt working, the headaches themselves, nausea/vomiting, etc. Ive really tried to share some of the infromation ive learnd about these issues with MPS families especially those whose own kids have had shunt issues and in some cases havent been closely looked at bc the nrsgn doesnt really believe the family/individual. Ive pointed out that this isnt just me, that shunt issues occur in something like 60% of all people w/shunts in the first month after implantation or revision as well as share some of the stories my neurosurgeon has shared with me. I really want to help turn the tide of better shunt understanding in the MPS community (ie how intermittent obstructions can make a shunt seem like it isnt the culprit due to normal ICPs on shunt taps, etc) and how when one dr wont listen sometimes we just have to keep looking till someone has seen what we are experiencing and listens and wants to help. Im comfortable with the decisoins ive made and my core (relavant to these revisions/neurosurgery) providers are very aware of and have signed off on/given permission for these surgeries all with us all knowing it can have a toll on my body. Its how we manage the post-surgeries that matters really.


In a 'whats going on' note I called AFCH on tues while Zander was in class and talked to the Ortho nurse and she in turn  scheduled an appt  with Dr.Noonan who was 1 of 2 names I was given by Dr.Walker at UofMn. This appt is in mid-april and I have copies of most of the records i'll need and just need to get copies of the MRI/Xray images from FMLH. I kind of wonder if him and my neurosurgeon wont end up co-managing the spine issues as from what ive read this dr also does alot of spine surgeries in complex cases and that would be fine.  --- I also had to reschedule my Hand appt and see this doc in 2 weeks but have been having problems with my pinky finger on right hand - it wont straighten and like when typing now is really actually painful; in the past ive only had a short term problem with 2 fingers getting 'stuck' and this was remedied fairly easily by some kind of steroid injection in 2 places in each finger and no problems since. I am seeing my hand surgeon for my other thenar muscle loss (had the tendon transfer 15mo ago in  hand) as well as re-turned CTS symptoms but will mention this finger issue. I am amazed at how much a simple pinky affects typing!
My PCP sent me a mychart message with a few notes post-the surgery physical and one of the urine test results came back positive for increased protein which this is the 2nd time this has occured so she mentioned in her note that they (she and my PCP) where investigating this and planning to order either a 24 hour collection or a urine spot test and would get back to me. Im unsure what this means but very well may be nothing as it was only a trace amount of protein each time - they are going to re-do the urine test in a month.

Thursdays Cardiology appt was  rescheduled due  to an emergency to  monday so my Cardiologists nurse and  I worked it out I will do the Echo at 8:30 before I go over to ERT (infusion) that way I can still leave the Port accessed after infusion is over (for tues) and then after infusion is over at 3-3:15 i'll go back over to the Cardiology clinic for the actual appt with my Cardiologist. I still dont think it will actually hold surgery up.

My primary insurance Dean Health Plan is switching to using their own facility as a distributor of the aldurazyme in May vs going through a middle company (increased cost, more hassle and more chance of screw ups occuring/mis-communication). This also may help with being able to do aldurazyme at UW when in-patient there if needed since Dean is in Madison and should prevent some of the many hassles we've had with making sure there was drug always on-hand at CHW for infusion!

I'll try to update a quick note monday night after I get home from ERT and the Cardiology appt. We are doing the Echo at 8:30 then i'll go over the ERT and when done will go back to the Cardiology clinic in CHW around 3-3:15 for actual appt with my Cardiologist. The nurse and I did this so that I could leave the Port accessed from mon's ERT infusion overnight until tues's surgery. My neurosurgeon is fantastic about our using the Port for surgery and makes the anesthesiologists when they want to add a second line put it in after I am asleep as she has seen how hard it is to get a periperhal line access.  (I have the things to cover it for showeing and also when needed to flush it with heparin although shouldnt have to this time.)

Take care - thanks for stopping by; feel free to either sign in as a follower or send a comment. (I know some of you have been having problems leaving comments, thanks for the heads up).

Erica