Diamox short term trial; Lumbar drain info

Dr.Bragg and her NP both stopped by separately; Sue is writing the discharge orders. Dr.Bragg thought trying the acetazolamide (diamox) would be a good idea and even if we cant do it long term (checking with cardiologist and will fwd any response to her) it may give us another idea how well a 2nd drain would work. She mentioned that she would be looking at my scans again and talking to Interventional Radiology to make sure placing a lumbar external drain could be done in xray (I believe she would do it there awake) so that she could have a clear picture/image when she is placing the drain and be safely above the laminectomy from last May and know we are away from the areas that are abnormal. She thought a few weeks rest. I think 2 weeks on the diamox would give us more than enough info if it would help and how it would help my body/CSF; I am grateful she will let me try this in the mean time (and Cardiologist already responded back that a short term trial was ok) and maybe will do even more to bring down the last CSF pressure that is driving my vision batty and making sleep impossible. Literally waking up in the morning is like trying to pull myself from what feels like a dazed coma. Here at the hosptial I slept upright; literally almost leaning forward and last night finally slept for about 4 or 5 hours. Today the headache is there but not as bad as before this revision and for that matter it never is really like a headache it's the pressure or as if your wearing a head band or something compressing the skull from the inside out. Not nice!

We (I) know pretty well that this latest revision did the job in restoring my CSF pressure back to where it was when we placed the programmable medtronic-strata valve in Febr. (low setting) and a few weeks will do good for my body (I think we will likely do a 2 week trial on the diamox before I go back and see her but uncertain). In the mean time it allows other things to get done and also can gather info which everyone knows is what makes me tick. :)

I will continue updating throughout this all and about the other upcoming tests, appts, etc.
Thanks,

Erica

External Lumbar drain, LP shunt addition and Cardiac Perfusion testing

After a miserable night of no sleep (maybe an hour and sitting up the whole time) and the nurse asking multiple times if I was ok because of the way I was falling asleep/did sleep Dr.Bragg came in this morning and I think the first thing she said was "your still feeling pretty crappy yet, huh?" Meaning she wondered yesterday if this shunt catheter move would help as much as we needed as she thought more CSF needed to be drained. The catheter being moved is a good thing as it still will be less likely to occlude but she must have thought that we would likely end up doing some form of additional drainage to as she came in and said she had been talking to her partner about why he did the LP shunt in one of his pts that also had a VP shunt and that patients symptoms she said  had very similar mechanics and issues to mine including small the small non-expanding ventricles. That patient has done really well with the 2 shunts and her partner apparently told her he would recommend trying the 2nd drain in me. Her partner as ive said previously is well respected in the hydro community and has alot of experience with shunts so although I am apprehensive about him I respect that he is willing to help my neurosurgeon plan the best course for me. Both feel our best bet is likely the 2nd drain so lumbar and brain pressures are equalized/level and if there is any issue with communication between the spine and the brain this will help mitigate this.  She wants to give it a couple wks for my body to re-coup from this surgery and initially she thought we would place a lumbar ELD via radiology using Xray guidance so as to go higher than the previous laminoplasty and also ensure proper placement. I think if im not mistaken she said we would be able to do this awake and then would monitor for a few days. If this showed improvement then we would place the lumbar peritoneal shunt in general surgery under anesthesia and at the higher lumbar level. Im unsure what valve setting she would use or if she would do a programmable with this to but she said she still has tricks up her sleeve and we have options. If needed and miserable she said we can also condider moving this ELDS (external lumbar drainage system) to sooner rather than wait the couple weeks. I think we will likely decide final when I see her in normal clinic fup which is typically 1 week after discharge. I could have gotten out of here tonight but have some questions for Dr.Bragg and although i do look fine I just wanted to ask her specifically about short term diamox in the interim. I would rather be home but know they have things here to help that I dont have and i'll get out tomorrow.




The CHW-ACHD (heart center) clinic coordinator called this afternoon and scheduled the Nuclear Med Perfusion Stress test - she  scheduled this test which will take 3 over hours over at FMLH for April 10th. I'll then follow up with my Cardiologist a month after this. This test will tell us if the heart muscle is getting enough adequate oxygenated blood and if so we can postpone valve replacements fairly safely as well chest pain not as likely to be serious (it also identified coronary artery disease which is known to happen in MPS although not well documented in data). If it came back abnormal with areas of the heart not getting enough oxygenated blood then we would know we have to consider surgery sooner. Please pray it is negative and we can continue to manage symptoms with medications for a while! I will start the new beta blocker (bisoprolol) likely on mon to give my body essentially a week post-op recovery.

Wish me better sleep tonight and the next few nights - it hasnt helped that the much higher  post-surgery(every 12 hrs) stress cortisol replacement dose is given at around 7:30 in the morning and 7:30 at night. I am down right exhausted tonight and vision getting blurrier and fuzzier despite wearing glasses so I am hoping to be able to sleep even if it is upright.

Will update soon,

Erica

#13 or something, surgery-revision, other things.

This I believe looking back through old entries was #13 Since last May and #16 overall for shunt revisions. Yikes! (although still nothing compared to some with shunt issues)/ My Neurosurgeon did tell my Dad as well as me in addition to her ideas below she has other tricks up her sleeve if needed. I am grateful as always to have her on my team.



I'll elaborate on these but these are the short updates I posted to fb earlier today:'
"Surgery took about an hour - will update a little while but was up on the floor by 10:30. Anesthesiologist today was from the cardiac anesthesia tema; very good and interesting to talk to. (Explaining how they would pre-emt any cardiac issues in surgery due to underlying issues). Will update findings, plan, etc a bit later. Tired and admittedly a lil crabby from being so tired."

~"Will update blog later but in pre-op Nrsgn explained how she was still thinking about my case over night but had decided on re-locating the tip of the ventricular catheter from the lateral (front) ventricle to the 3rd ventricle (same incision) using the endoscope as this is a stiffer area she believes is less likely to cause intermittent occlusions from intermittent collapsing. If this too doesnt ...work (re-occludes, obstructs again) she believes placing the LP shunt again in addition to the VP shunt would be our best option to allow more drainage. She would place this catheter in the lumbar spine at a different level than the previous LP shunt due to the spinal nerve root surgery last May and knowing the previous area is abnormal. The previous LP shunt was initially just tied off when the VP was placed for this very reason but bc of the CSF infection in Nov we had to remove both shunts and didnt replace it. We had frequent issues w/CSF fluid pockets and the lumbar tip came out of the sub arachnoid area at the end so we do hope not to have to place this again. If we did she said she can use a Y connector possibly to connect both shunt ends in the peritoneum to avoid a 2nd entry area there. The catheter itself she told me Dad wasnt as clogged this time (possible indicator of needing firther CSF drainage though as this is since having it on the lowest programmable setting (drains more)) but she did clean it out/move it."

Dr.Bragg this morning made the comment she was a little nervous that the shunt catheter wouldnt show any blockage (the reservoir was slow to refll and same symptom progression as other times) and although I didnt talk to her directly post-op (well maybe I did and honestly dont remember it!)  but she told my Dad that the catheter wasnt as occluded with cell debris as it has been in the past but I believe she still changed that catheter and she also moved the actual tip of the catheter (by extending it's length) to the 3rd ventricle whereas before it was in the frontal (lateral I believe is the name?) ventricle and this area should provide a stiffer background for the shunt to drain and the hope is help the ventricle from collapsing and occluding as often. She said this is more likely to become affected by the Choroid Plexus in this area but that balanced against the former position she felt this may help us  better.  I hadnt thought alot about her comment regarding being concerned the catheter wouldnt show any occlusion till after surgery as I honestly wondered the same thing she did and post-op I wondered was it bc she then she would know with more certainty that additional CSF drainage would probably be the step needed to stop the 'left over' symptoms? (mostly vision, some issue with laying flat although was 75% improved last time) That results in the  occlusions as at this lower drainage would it be helping push the cell debris through more easily regardless of the sticky-MPS storage substance (more force?) and yet not quite enough drainage to completely resolve the issue? I dont know and have written it down as 1 of 3 things to ask her.
She still feels if this occludes/symptoms dont completely resolve again that we likely need to drain more CSF and feels adding the LP shunt back as a lumbar drain (and possibly being able to connect it via the same peritoneal/abdomen incision via a 'Y' connector) to the existing VP distal catheter may be our next step if we have the same issues again despite this change.  We hope to not have to do this but if this shunt fails as have the others despite our newest change in location then she thinks adding further drainage will help increase the brain compliance (more fluid drained, more space I think) which can help symptoms. I am already at the lowest setting on my VP shunt and dont know what setting a possible LP shunt would be but it is in the background as a 'next step' if we require that.
 I do think far more of my drs understand the shunt issues now vs even a couple months ago (based off talking to and explaining in depth Dr.Bragg's thinking to my PCP and to my Cardiologist and their responses)  and that was nice or reassuring as ive struggled a bit lately with knowing was I making the right decisions as underlying I knew I wasnt making a hasty, un-informed decision but you still sometimes quesiton yourself.

I have to say today was by far the most the underlying cardiac issues have ever been a factor in any surgery and even Dr.Bragg who was in my pre-op room when Anesthesia was talking to me about their induction and surgery plan to mitigate cardiac symptoms asked afterwards if issues with the valves had gotten worse very recently bc of how concerned the Anesthesiologist was. I just said that it has been a almost gradual but steady up-tick over the past 2 years and although more symptoms I still sit at the moderate range (severals degrees short of severe with the Aortic stenosis according to the anesthesiol) with most of the stenosis and leaking valve issues and then the L sided filling pressures are very elevated + L atrium is severely enlarged. I added that my Cardiologist gave his blessing and is changing around some of my meds post-this surgery + we are doing the cardiac perfusion stress (dye) study to make sure all of the heart is getting properly oxygenated blood and if abnormal then we have to more seriously look at moving to double valve replacements but if normal we can post-pone for awhile as other things are sorted and because of the surgery risk for that and big change it would mean with daily coumadin. Dr.Bragg sits on a ethics committee with the Anesthesiologist I had today (he was from the cardiac service vs the normal neurosurgery anesthesiol. I usually am assigned) (since it is a brain surgery typically each anesthesia doc is 'sub' specialized in an area).. I appreciated  all the info he gave me which was great and appreciated as was his humor and his desire to learn about my issues +I learned alot from him. He seemed to want to do the best plan he could to preempt any surgical-cardiac issues. He laid out the complete induction and surgery plan and it was a different approach to some extent than others have used but was as I said interesting his reasoning. (which all did make very good sense). I think Dr.Bragg learned further about the cardiac issues and found it interesting as she stayed in the room and listened to the whole conversation which was about 10-15mins. 
As for why we are hesitant to want to put in a second drain right away (besides it just being yet another foreign device in my body) with the previous LP shunt both w/my former neurosurgeon and with Dr.Bragg (is fairly common in LP shunts) we had many issues with pseudomeningoceles (CSF fluid filled pockets) around the lumbar drain of varying sizes and degrees and also had a problem with the reservoir becoming flipped (causing CSF fluid build up of it's own)  and in the end the lumbar catheter tip had backed it's way somehow out of the sub-arachnoid (I believe that was the fluid filled space) and was not working. We also of course had the issue with requiring the laminectomy and nerve root repair (multiple levels of nerve roots compressed in to a ball she had to take apart and repair)  last May + the complications this caused. That all said she said if we did do this she would not place the lumbar catheter back at the same level and would move it higher or lower to stay out of the area we know is very abnormal. That nerve root issues was also more likely bc of the MPS storage and repeated lumbar punctures both before I had teh original LP shunt put in and the lumbar punctures since until I started seeing Dr.Bragg and she placed a reservoir into the shunt set-up.

In post-op one of the Anesthesiologist ive had for 2 previous surgeries (last month and in August (he checked!) :) stopped by to say 'hi' and see how I was doing + to wish me luck with this revision. He was a fairly quite guy so I did think that was pretty nice on his part since I wasnt in the main post-op recovery holding area and instead was with a sole nurse in a separate room. (isolation)

Earlier tonight I got an email from a 'new' (to me and to ERT infusions) MPS I Adult who found me via a search engine/website and reading this blog; it is actually always kind of exciting that this blog really helps others with both MPS I and with hydro (ive gotten seveal emails from people with that who found me/this site through search engine searching).  This person is in their early 40's and similar in build to me and says she also has similar joint characteristics/posture as well. Pretty neat! We plan to talk on the phone later this week when I am home. She does not have hydro secondary to the MPS it appears.

I have been getting updates from the main Genzyme Patient Marketing person ive worked mainly on the 'Adult MPS I' Survey and the deadline was extended to next monday + I was asked to comtact adults I know and remind individuals to consider filling out the informaitom (which goes directly to Genzyme to be quantified). We have gotten a fair response so far but want to get as much date and individual response as possible. :)

I was Asked to participate in a radio interview for a program called 'Winning Life through pain' in June for my role at APF regarding my experiences with chronic pain/chronic disease and the strides ive made in over-coming/getting on top of this pain. (pain management, physical therapy, multi-modale medication approach and daily stretching).

Im not sure if i'll get out tomottow but do need to ask Dr.Bragg about the lower spine and leg/feet symptoms as today even has been a little difficult - I range from sitting upright, to sitting on the side of the bed as I am now to laying on my side to try and prevent worse symptoms. I took a nap and was definitely not very comfortable as I have a low back ache/discomfort now since and not sure how well i'll sleep tongiht despite getting average 7 hours of sleep in the past 48 hrs (if even that much) and not alot in the past 2 weeks either.  We will see - it desnt help that because of the surgery 1 hour before and 12 hours post-op (which was about 7:30-8 tonight) I got a very large stress dose of solucrted which is a steroid and can cause inability to sleep. Yay, lol.

More soon,

Erica

Echo and Cardiology

What a day for sure, wow! My Cardiologists nurse who helped me arrange the Echo and Appt for today came in to the actual appt ahead of my Cardiologist and said to me "You must be exhausted after a day that started that early and ends this long" and she was so right! Thankfully I had only intermittent episodes of the headaches and other periods where I had headaches (pressure) but wasnt as bad as it can get so I wasnt as exhausted as I otherwise would have been. I got to the Cardiology clinic at 8:30 and the Echo took about 45-50mins total start to finish. Afterwards I hop, skipped and jumped (ok maybe just walked) :) across to the Curative building where the Genetics clinic is and where my weekly infusion is. After getting accessed this went till about 2:20 and then back to the Cardiology clinic for the Appt with my Cardiologist. (this way I could leave the Port accessed overnight for tomorrow's surgery vs having to de-access it to get the Echo pictures).

So the Echo looked about the same so far the heart muscle itself isnt showing signs of hypertrophy (weakening. thickening) but my Cardiologist knows the L sided filling pressures are very abnormally high and thus creating severe L atrial enlargement. This could be the cause of the chest pain and shortness of breath and skipped beats/arrythmias but the aortic stenosis and very abnormal aortic and mitral valves as a whole with leaking and stenosis also factor in. He is ordering a cardiac perfusion test which they inject dye in and watch how this goes through the heart and muscle of the heart as the person exercises and this will tell him if the symptoms are from not enough blood and oxygen is getting to any part of the heart. The test will also tell us if there is diffuse coronary narrowing which he said is very different than CAD in a non-MPS person and in the literature has rarely been reported to cause symptoms but he still wants to make sure it isnt the cause/addition to our issues. What I appreciate is that he factored in my joint and lung issues and left it up to me to do the test or not; he felt strongly it would guide us if we could safely push off doing the valve replacements but he also didnt want to cause a severe up-tick in joint pain for 3 or 4 days after at the expense of ruling out or in a cardiac issue. I just said that while I didnt exactly want to do the test I knew it would alos give us the best chance of knowing a different look at the cardiac disease progression and would tell us/give us a better picture of if valve replacement is needed sooner than we want or on the flip side if we can push it off as we want for as long as possible.

He is changing the medication I take and although i'll continue to take Lasix 40mgs (this was upped back in Jan) he is switching the diltiazem long acting which is a calcium channel blocker to bisoprolol a beta blocker for which we will start at a low dose. This will be done after this surgery tomorrow. He gave his blessing so to speak for the shunt revision surgery saying "you've had alot of surgeries in the past years and handled all of these reasonably well; while we dont know when your heart will act up and cause a problem with a surgery I think you will do pretty well." I appreciated the candid and upfront opinion and also how to him it isnt just 'cardiac issues' and instead is a whole person with many underlying issues and finding the best solutions for quality of life. He talked about the symptoms I am having and how until we do a valve replacements he probably isnt going to be able to get me feeling as good as he would like but also doesnt want to do anything to make matters worse.

I just appreciate that he is one of the docs I see who is very down to earth, very knowledgeable about MPS and looks at it from a 'whole person perspective' vs 'Im a cardiologist and this is all that matters to me." :)

I have to be at the hosptial at 5:30 tomorrow with surgery scheduled at 7:30. It will be as much a surprise to others as to me what we will end up doing with the shunt. :)

Erica

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.  
~  .
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

Pre-op, Cardiology and being an Imposter..

I cant help but feel like an imposter to this disorder- yes ive had dozens of surgeries, dozens more procedures, and definitely do feel everyday like I expect someone with a chronic disorder probably feels. Instead I feel like an imposter because I generally look healthy, I generally dont look alot like I have MPS (whatever that look is supposed to be?) and I generally try to lead a fairly normal (albeit fairly un-normal) life. I just feel like this, well to be honest Im not sure why I feel like this? But I still do sometimes... I think it is why I really appreciate people who dont assume how I feel just based off how I look and instead really know me and try to not assume anything.

Today's pre-op appt  went well ; she made the comment afterwards something to the effect of how I am actually a really healthy patient with alot of underlying chronic illness. I have to agree; sure there is alot of cardiac stuff, CNS/shunt/spine issues. autonomic-endocrine and joint, etc issues but as far as taking care of myself I am very independent and at a good weight and know what to do to prevent getting sick alot. (supplements like copper/zinc and fish oil). I think it's always kind of nice to hear when your doctors think your doing a good job managing what is essentially a really stressful job (phone calls to insur., paperwork, appt scheduling, appt rescheduling, more rescheduling and trying to make things work together (have already cancelled and rescheduled 3 appts since mon afternoon and scheduled 2 others). Making sure you have the meds refilled on time, making sure prior auths for those said meds are filled out by the prescribing dr and then calling back that said prescribing drs when no PAs are filled out a month later. Having to get to appts when you dont feel great. communicate and be your sole advocate and voice and to some drs convince them (thankfully very few as I only keep the good ones on my team now a days) that 'hey, just bc I look fine doesnt mean I dont feel like sh^t.' In addition to that all (and more) keeping the responsibilies you agree to be it projects for APF or the blog for WI-ACHD or in some cases to Genzyme. People cant possibly understand what  a life is like with MPS bc its not just living with the symptoms for some of us adults it is also managing everythng else that goes along with a progressive, chronic, multi-systemic disorder. Managing insur and in my case multiple insurance is in itself time consuming enough. So, yes, it's nice to hear a compliment when a provider thinks you are doing a good job. :)

She always asks how am I coping with it all and I said what I usually say 'what choice do we have?' Sink or swim essentially and I chose to swim and fight and by mostly taking it 'one day at a time' when it comes to the medical stuff I can deal with it and it just is what it is. I talked about people asking me when im going to stop having shunt revisions and why do we keep doing it + I understand most people know very little about hydro but that it does get frustrating sometimes when people try to second guess what they dont even know. . (I was purely venting) She just mentioned that while others may have thoughts only Dr.B and I know what is right and we will know if it ever got to a point where we werent making a difference. Right now we  (Dr.Bragg) talk often about the shunt issues and what our 'next plan' is and both Dr.B and I agree that we are still making progress and we do get complete symptom resolution its just a matter of the non-compliant venttricles and thus the brain tissue/cell debries gets clogged in the shunt catheter when the ventricles collapse. Laurie (NP) said if one thing comes out of all this pre-op things it has helped her learn alot about the complexities of shunts and especially underlying MPS and shunt issues as well as given us a chance to get background history in med record caught up/ It also has been a pathway for some providers communicating a little when it comes to sharing records with each other, checking with each other on questions before these surgeriies and aware of all thats going on. It was a good general visit in which many things incidentally came up and I got some advice on various symptoms + management which is good and nice.  One of many things we talked about and for which  she was a little concerned  was  the leg pain/numbness and feet going numb even with walking and thought it might be low back - this is also what my PT thinks and so I do really need to ask Dr.Bragg about it.  The pre-op was with the same NP who has done all the previous ones and who is most familiar with my care and she was just sending a note to my Endocrine dr + as well calling Dr.Bragg's nurse with a question.


 Although when the shunt is  not intermittently obstructed I feel reasonably "ok"/ Like today I was up for a few hours before I went and picked up my nephew and by that time I was feeling fairly good + Z and I had fun making lunch and drawing till he got picked up for  his second class. By the appt this afternoon I was exhausted and had a headache but was still not horrible so I dont think anyone would have even been able to tell. On the other hand when the shunt is not working well (mid-afternoons, nights and mornings are the worst) I am incredibly nauseated, having these aweful stomach-bladder spasms again, and head feels like a ton of bricks + vision very fuzzy/foggy.  I dont want another surgery but  am looking fwd to it being over and to feeling better again.  I dont think Dr.Bragg is certain what she wants to do yet and it may be a 'get in and look at' the shunt before she makes a final decision. I sent her an article about something shunt related the other day and she responded back yesterday that she would try to answer my ques including about a second shunt/final plan which is nice that she cares enough to take that time but I also am ok if she is just planning out what to do between now and next tues's surgery.
One of these times we will get the shunt working well for long term.   Like ive also said before I get that people who know very little about shunts question why all these revisions but when something has like a  60% failure rate in general in the first month and I know many, many other pts both with and without MPS who've experienced the same problems I dont question what we are doing. I said to my NP that people wouldnt let their kid suffer or in a less than perfect analogy you wouldnt leave  broken plumbing, right? Well I think of the shunt as broken plumbing except I have a good "plumber" so to speak.

I think I may have mentioned last week here that I got an email from the Ortho at UofMn - Dr.Walker who gave me the names of 2 Ortho-colleagues he knows who work at AFCH-UW (my neurosurgeons hospital). I fwd'd the email to Dr.Bragg's nurse and she responded that they like both drs and if I have any problems getting scheduled to let her know as they would help me get in/get an appt with either of the 2 docs. Both of these apparently handle very complex cases so I will be curious how it turns out!

I talked to the Cardiology secretary friday and she wasnt sure what my Cardiologist wanted scheduled (he had only said in his fup email that he should see me in clinic) and so was going to talk to him and get back to me.
** Jane sent me an emal, (she is Dr.E's nurse) and they have an Echo scheduled for thurs afternoon at 4 and appt at 5. I still am conflicted if these symptoms are really cardiac but guess Cardiol. must have some idea. I as I talked about alot recently always hate going in to appts when unsure about something and it does make me a little nervous! He's a really good doc that I do like, do trust and is easy to talk to it's purely just my not liking uncertainty and asking for help. I dont think this will hold up the shunt revision. I'll try to write a post about that appt/results thurs night or fri.

In the mean time going to quit and will be back here soon,
Thanks for stopping by,

Erica

Shunt failure aka intermittent obstruction and occlusion.

So I flipped around the original appt from today to next thurs with the appt w/neurosurgery that I had changed to next thurs and made that today. The other appt is a hand surgeon appt more or less because of carpal tunnel re-occurence (not new though has been since last spring I just put it off) and since it has been well over a year that ive had the thenar tendon transfer in my right hand I guess i'll get details on my L hand at the appt next week. Im not sure if i'll have it done in my L hand though -not because it didnt help alot in my other hand but because the recovery with no use of that hand is so long (3 months hard cast) and I dont have tons of strenght in my R hand plus even if L hand bothers me atleast I can still open containers. We'll see.

So anyways I saw Dr.Bragg today and she in her usual good style tested the shunt reservoir refill time right away and noted this is very slow to refill so no need to even do the shunt tap as she knew it would just show very little CSF able to be drawn out due to partial catheter obstruction again.
Not that I didnt know the answer but I guess out of hating these shunts issues, disliking taking her time (she really is so fantastic though and she + her nurse really are so easy to talk to.)  and just wanting the shunt to be good for a whole lot longer of a time I asked her do alot of their  shunt pts really have this happen so often and she talked about a few other of their patients experiences and how the small non-elastic ventricles create the suction instead of continous drain. (today's analogy was like sucking jello through a straw; you may get some but it will leave some jello (brain material) behind in the straw (shunt) and this in time causes the straw (ventricles) to become clogged.) She also said she really believes even though we got a much better symptom result with this latest valve (the programmable)  and having it set at the  lowest setting she does think I still need to drain more CSF and talked about oneof her partners patients who has both a VP shunt as well as a LP shunt to maximize drainage and symptom resolution long term. I dont think she has fully decided on what she will do this next revision and didnt seem like she wanted to add a 2nd drain so I do think per her style she will be looking at different options right up till surgery which is fine.  One other option she talked some about was moving the tip of the shunt catheter further in to the 3rd ventricle and I think the foramen of monroe but honestly cant remember if it was that or the foramen of magnum to get the tip to a space that is more 'fixed' and less elastic and less likely for tissue to occlude because less likely for ventricle to collapse and occlude and eventually obstruct.

Her nurse is calling on mon with the surgery date and in the mean time I will schedule pre-op since the last surgery a month ago (this weekend :=/ ) we slid under the 30day pre-op rule w/just 2 or 3 days before it would have expired. If you have a pre-op on file from one surgery and have another surgery within that following 30 days the paperwork and pre-op results still count so this time we have to get it done again as this will be 3rd surgery since mid-Jan (and many moons more since last May) but the last pre-op was mid-Jan. bc of the surgery in Febr being w/in a month. I think my PCP knows the drill well by now. I'll see if I can do that mon after ERT since my  PCP is next door to CHW/infusion.  I asked Dr.Bragg if we could not do the revision next week since although I want to feel better I want to keep up a promise I made to my sister for my 5 1/2 yr old nephew  a couple weeks ago Sara, my sister had  asked if I might be able to watch Zander on tues-weds-thurs and fri next week in between his 4K, early childhood and ABA classes. I cant watch him thurs but agreed for tues-weds and fri and really he is so busy both in 4K (which i'll drop him off at and pick him up from) from 7:45-11 and then ABA usually 11:30-1 and tues early childhood he goes to at noon. I'll have to get him up, help him pick out clothes, make breakfast and lunch but otherwise not to bad.  I drop him by his Dad on Weds and fri at 2 so I can get to the others things I have going on. For that kiddo id bend over backwards, stand on my head and do cartwheels (non of which I can do!) to make sure he's happy as he means the world to me. I can live with my symptoms for that extra week if it means being there for him. Lisa, Dr.Bragg's nurse said to me on the way out that not that many patients who need brain surgery put someone else before their own needs/how they feel and she's so right and I told her Z is as close to having my own kid as im ever going to get so sometimes we do nutty things to ourselves for someone who means everything and the moon! :)

For those unfamiliar with shunts who may wonder why so many shunt malfunctions and revisions I thought id post a  tidbit of info on the statistical average for shunt failures in the first month alone which is 50-60% and 45-50% the first few months and not a whole lot better after that. Research is on-going in how to improve this and my neurosurgeon + her colleagues are continually researching and looking at different ways to get long term results in their hydrocephalus patients. I am glad she thinks outside the box and too doesnt just judge me how I 'might' be feeling based off how I look. (ie some people assume if you look good you cant possibly feel bad). Very few people as ive talked about before are great at just looking at me and figuring out how im feeling.

Dr.Bragg had asked about pain medication if there is anything ive used in the past (she has asked this before in an attempt to help) that really would help these headaches especially since I asked to wait a extra week on the surgery and she is just that kind of provider that really wants to help her pts in any way.   I am on a 1 every 7 days pain patch, a neuromodulator (for nerve discomfort/pain) and a immediate release pain med as needed and the only thing I could really think about on the way home after we had talked about it some was a steroid but even that im not sure can be used as I take twice daily hydrocortisone which is a form of long term steroid for cortisol (bodies stress hormone). I may ask her nurse about that when she calls mon but the things that really make the H/A's worse I avoid like laying flat, laying back against pillows (versus fully propped up) and during the day the headaches ebb and flow.   A couple things ive thought of to ask Dr.Bragg and which I emailed her from our conversation and some subsequent reading I was doing on pubmed and google yesterday was 1. since she commented about do we need to drain more CSF can this theory be tested by placing a lumbar EVD (external drain usually placed in the ventricle) in addition to having the VP shunt open and running? The other was can a brain and spine MRI dye study be done to see if dye gets fully through spine and brain with no obstruction from compression or  potential obstruction(s).

As she was leaving Dr.Bragg commented jokingly that it's a good thing she likes me and my family alot since we spend so much time together and I replied back to her and her nurse that I also am lucky to have in her particular position on my team since she gets it! And I totally mean it, I cant imagine going through all of this with someone who just wasnt as good natured as she is and I also am grateful as ive said before that she has an interest in hydro!

The only other thing really going on is the cardiac stuff and my cardiologist was going to review my chart and try to come up with a 'next step' - im not sure what he'll decide if anything and will fup on that in the next few days. Its just another symptom so no rush on my part and I still dont really know that it is cardiac related. I think it is but not sure. ** I got an email from him today and he wants me to come in to the clinic =/ so ive talked to his secretary and they are figuring that out; im unsure if it will be next week or when.

I guess i'll try to update soon but otherwise be well and enjoy this weather wherever you are reading this from! Here in WI it has been absolutely gorgeous for March and no snow to be seen anywhere around!!

Erica

Life, it's a many Zebra'd thing...

  Welll if there where a theme for this week it would be I keep coming back to how after all these years after diagnosis and the years before that of so many drs I still hate asking for help. I still dislike bringing up symptoms to my providers and I still have a really hard time calling providers to ask what they think I should do when I dont know what to do or know what to think.  I posted s/t about this on fb the other night and got some insightful replies from friends (MPS) who themselves deal with alot of providers for their own kids. I dont really know if im ever going to be really comfortable asking my providers questions or calling up and not getting jitters when I am asking for advice. I know (think?) in my last post I commented that I have gotten more comfortable asking questions, and I do think I have but I literally get shaky feeling (I always joke if I got nerves about my actual health issues like I do about having to ask or admit when things arent great id be in a insane asylum) bc I will put off making calls for hours or days or as long as possible before I will call say a providers secretary or nurse and ask them something. Not intentionally to hurt someone but I often wont admit when im not feeling good and while there are some people who are fantastically perceptive (a couple of my providers just have this way of knowing as does my nrsgns nurse but otherwise I likely wont tell how im feeling bc either I dont want to talk about it or as ive mentioned before with family and friends I just want them to be family and friends and to be an escape.
 I just dont like asking people for help and not bc I dont think the other person wont have good advice or wont know how to help or because they havent reassured me many times that I should call or ask questions but bc I dont want to be a burden to people whether it be family, friends or providers. There are so many big things that happen within MPS that I especially struggle with the little things that im not sure if I should bring up or if I should see someone about or if its say a different neurologic issue but the shunt is so on-going do I still bring up the other thing(s)? I know the answer is yes but I still struggle to do it and often wont.

I know I talk alot about the health issues and not alot about the rest of my life but really that is what this blog was intended for, for me to chronicle my experiences and feelings and the ups and downs and almost to have a record to come back to when I cant remember something. (I cant tell you how many times ive come back and looked to see when a particular shunt surgery was or when the Port was put in or when I did one thing or another. It also is intended to help other people understand MPS from a patients perspective and help families if they wish especially with hydro in MPS families as this is a disorder that so little is written about in our disorder and is so misunderstood by many. I thankfully found a good neurosurgical team and so while my experiences have been a winding rollercoaster I still think it is information that can help other families. I also really want this to be for other MPS adults to realize that we are our own voice and advocate (cliche I know) and these issues like hydro DO and CAN occur in our much less common, much more rare attenuated-MPS I older adults classic signs not withstanding. I do hope this helps others and maybe even some doctor out there but really it is for me, for my sanity and a way for me to put a voice to what I am experiencing.
I received an email from Dr. Paige Kaplan's (CHOP) Genetic Counselor yesterday and they are nearing finishing the MPS I skin biopsy studies using ataluran the test drug. They are now asking for urine testing to do GAG testing for a 1 week 1st morning void to use as a pre-human study data. Sara mentioned that for the actual skin biopsies the results have been mixed with the drug working well in some skin samples/mutations and not well in others but they dont know yet whose it worked in. As for the actual study they are thinking they may open it up (the drug-human trial) first to those of us who participated in the actual skin biopsy testing/study phase and this is being planned. (planning for the actual study) No dates are being released yet but Dr.Kaplan in an earlier email had said she suspected by the end of the year so im not sure if she will change that date to earlier. IRB still has to be written and approved plus arranged. This drug if it worked would be used in conjunction with the ERT infusions and not as a replacement - the drug is thought that it may get in to the brain, heart and joints.

As for actual issues going on - I havent been sleeping much - in the last week I havent fallen asleep since I think thurs before 3 in the morning and since sun have been waking up with the same dang symptoms from shunt. I called my neurosurgeons nurse today and asked her if I could move my appt up from 2 weeks from thurs to next thurs and she asked me why we where not making it this thurs? IIve already rescheduled an appt I have this thurs several times due to shunt issue and feel bad doing it again so just going to deal with it till next week since only nights and mornings are bad so far. I also told my sister a few weeks ago id watch my nephew tues, weds and fri next week form 6:30-2 weds and fri and 6:30-12 tues (and maybe thurs) and drop him off at and pick up from his 4K class and really as bad as I feel in the morning (really incredibly bad but i'll just get up a couple hrs before I have to be over there and then Z doesnt wake up right away when im there either). I dont want to not watch Zander bc no matter how I feel id bend over backwards for that little boy to make sure he's happy and I know it makes his day super happy when im there. (the other day I got the sweetest wake up call from him and he was inviting me over to his house as my sister-in-law and niece where also already there.) 

Otherwise my Cardiologist is super great and as much as I hate asking him or any provider for help he is trying to figure out the best way to help manage a new symptom (a few months but hadnt said anything till now bc I thought maybe it would go away or was nothing and maybe it still is). With him he'll communicate easily via email and then either respond with his decisions either to run a test, repeat a test, come in for a fup or email with an idea or he/his nurse will call. He's been on my team since the beginning of dx and along with one of his patients is co-founding the WI-ACHD group for his adult congenital heart patients to bring patients with ACHD together which has been a fun new project. 

So I know ive mentioned a few times here briefly about tentative dates for going to UofMn for the neuropsych-MPS I longitudinal study (on-going, yearly) and the date keeps changing mostly bc of my shunt surgeries. So I was talking to the Neuropsychologist there Dr. Elsa Shapiro about some changes they are making to their protocol for us MPS I programmable-shuntee pts in which they have a new neurosurgeon. I hopped on the UofMn website and lo and behold the new neurosurgeon is none other than the  neurosureon I was referred to/my Intratehcal study dr worked with while I was in the IT study 3 1/2 yrs ago and when Dr.Dickson was suspecting I had beginning shunt issues. This nrsgn came from OSHU in Portland, OR and I had spoken to him over the phone and via email several times + he untimately referred me to my current neurosurgeons colleague (this was about 1 1/2 yrs before my current nrsgn would come to the hospital)  and that lovely experience. I really had the sense talking to this neurosurgeon then that he knew alot about lysosomal diseases and was/could think outside the box. While I wont switch from my neurosurgeon as she and her team have been amazing it will be interesting to meet this other nrsgn at UofMn! (they are changing the IRB so the new doc can re-program their programmable shunt patients after the fMRIs are done so that they then dont have to send us to UCSF to get the imaging on top of going to see them in Mn. I beieve at UCSF it was a Dr. Peter Sun that had been re-programming the VP shunts of their MPS I pts after fMRIs where done at his hospital.
On top of the new neurosurgeon they are also actively recruiting a new Neuro-genetics dr (this was the speciality Dr.Charnas had before he went to Shire btw) - this would be fantastic as really neurology wise (neurologic-non surgical) my care has never quite been the same since Charnas left and he was good at helping me figure out when to get MRIs, diagnosed my hydro, managed the spinal cord compression in between visits with Dr.Schwender (Ortho-spine) and overall just helped bring my care together really well - he and my former PCP worked so well/communicated so well together and ive never had that since. He is also who thought I should do the IT study when I did and helped facilitate that initialy back then. He has been gone from UofMn since Nov 08' literally the last time I was him was on my decompression surgery for neck  - election day  08' and the other days that week and since have ran in to him once or twice at meetings but not actually spoken to him as we where going different ways. I know some of my providers from then commented over time that they missed having his input - most providers since then have changed for various reasons but care still hasnt been the same. Thankfully my neurosurgeon was more than willing to manage both the neurosurgical and non-neuro-surgical aspects of the MPS and that has made a difference + she has a colleague who appears to really understand our MPS differences whether he had saw MPS before I dont actually know. (I met him the day after I had been admitted by ambulance and was quite well out of it). I still think though even having someone at Mn who could help advise us maybe on things like autonomic (who knows they may not even have that knowledge but can hope) and just maybe give insight on some things maybe we could be missing. (what I dont know and I would talk to my neurosurgeon before just adding someone else to the team. I respect her and trust her judgement and try not to step on feet.  Keep your fingers crossed Mn gets this Neuro-genetics dr they are recruiting! I tihnk more needs to be understood in both the attenuated and severe MPS I neurologic population and its a shame more isnt studied on it so more drs can learn! I was finally lucky enough to get my nrsgn who understands the differences in hydro in lyso diseases/MPS and want more families to not have to go through the years I did before they find someone too!

If your wondering why the title of this entry, I just saw that somewhere and liked it and is true. Sometimes with care I wish providers would not think a zebra and instead think 'hey this might be a horse!' (issue ie more common) or the opposite 'hey this really might be another one of these fantastic zebra issues!' Not that there is really a right answer  bc with rare diseases there is no easy answer and no right way to manage a patient I dont think. Just as patients I suppose sometimes we wish things could be done a little differently or a little more smoothly. :)
All for now -

Erica

On turning 29

So this is another one of those updates that has been happening over the past few days so I will try to make it make sense! :) Yesterday I turned 29 and I figure my 28th year was filled with enough surgeries from start to finish to cover this year and maybe a few more years too??? With the hip injections, Port placement, (for making ERT easier) the initial shunt revision and lumbar spine laminectomy/nerve repair (end of May) and  11+ shunt surgeries since  + a handful of additional small shunt procedures since. (ICP monitor placement, shunt taps, etc). That was nutty! I know this will sound quite odd but ive always liked odd years better age wise not that there is anything special really but in this case I can hope this year may be a little quieter and maybe, just maybe the shunt will stay working for a long while. As my Mom said the other day when we went someplace together, even a year would be a welcome break but we'd certainly take many years of a working shunt!
Ive had quite painful sciatic nerve type pain for the past 6 or so weeks which makes sitting for any period of time really uncomfortable - my favorite place to sit and do stuff here on my laptop is on my bed (vs my desk which is usually covered in papers and documents, records, etc) I love just sitting leaning back against pillows (upright) legs straight out in front of me or curled up but definitely doing less of that these days. Ive been back in PT regardless for the past 2 weeks so am hoping this will help some in addition to the stretching ive already always done everyday. We will see - im sure it will (whatever it really is!) will heal and go away as it always seems to.

I have been feeling really good  for the past 3 days - a little tired on and off sun and today (this was written on mon)  but likely because I got up early with Zander on sun and had him from early Sat afternoon, overnight all through sunday when we had a birthday party with my siblings and a few relatives at my parents house.  I then fell asleep around midnight Sun night and was back up to head to ERT early mon.. Really the only symptom that remains is vision though which is not perfect but so happy to not have all the other symptoms, I hope it lasts!! Most of my siblings came to my parens house as did Zander and I sun and a few other family members for my twin brothers and my b-day (which is actually today) so it was definitely a busy day/busy weekend!

My neurosurgeons nurse sent me a message earlier today that she talked to the infectious disease docs and gave them my normal UA result (from the UTI and klebsiella infection) and apparently ID docs told her that at UW the policy is in pts who've been diagnosed with 'extremely drug resistant organisms' the isolation measures will stay on my file indefinitely! Wow! This means for every little thing from clinic appts, to any tests, to surgeries, to in-patient meal delivery, etc., etc EVERY person who comes in contact with me has to due full on measures from gown, mask and gloves! This after we did 2 different courses of antibiotics recommended by ID and the UA came back negative 2 weeks or so later! And the funnier part is where I get ERT the nurse wear a mask and gloves to access my Port but otherwise no one has ever done these measures and same with all other appts at CHW and FMLH since!  Sometimes healthcare actually is quite backward to me and makes me laugh purely out of it's craziness.. Definitely odd but also not anyone's fault, the UTIs have been an on-going issue every since the spinal nerve/bladder injury last May and signals just dont appear to communicate right yet. Both my neurosurgeon and the Urologist feel it would take about a year which will be end of May/June for the nerves to really heal.

                                        
           I found this picture  of the same programmer (magnet) device used to re-program my current VP shunt valve.

This 2nd picture is a copy of what my 1st programmable valve (teh 2nd valve is a fixed pressure) looks like and another view of the re-programmer device.

I read a really great analogy on hydrocephalus today - I used to be able to describe the problem ok but sometimes explaining it to other MPS families or friends who had previously never heard of this issues and in the case of some of the MPS Families where now facing this secondary diagnosis describing it in easy to understand terms was sometimes a little difficult for me. Ive gotten some really great (and funny) analogies of which ive shared some here from my neurosurgeon but today also read another great one about how the brain is like a kitchen sink either it's always running (kind of like the broken toilet that always runs from a few weeks ago) or it is a sink that should always be on but the sink holes become clogged or partially obstructed and  fluid isnt getting drained and builds up. The treatment is of course just like fixing a broken sink is to  create a new sink or create a new plumbing system to divert the fluid (CSF) elsewhere.

Picture of how the VP shunt is placed/threaded.

Ive been busy this past week on and off adding a few resources here although there are a couple articles I need to re-locate that where really interesting to me that I will add to the new resource guide on the left of this entry (further down the page.) I also have been adding to and changing different things about the WI-ACHD (adult congenital heart disease) blog and finding it quite interesting + informative working with the other 3 people involved in this. I love new projects in addition to old on-going ones! :).

More soon - take care,

Erica

Neurosurgery 2 week post-surgery fup

I saw Dr.Bragg (neurosurgery) today and we will just take a wait and see with the shunt meaning see if it stays functioning, see if the vision stuff improves, see if the morning/night symptoms improve (shunt when upright is able to fight gravity and the opposite is true when laying down even though I do try to go to bed sleeping up-right) and see if this low pressure is able to keep the shunt from re-occluding again. Symptom wise I really do feel pretty well minus occassional symptoms which is incredibly, incredibly nice!! We are at 2 weeks post-op today so half-way to our approx month point where we seem to always re-occlude. So far I am optimistic!

I asked her opinion of the Intrathecal (IT) study to use it as a means to possibly keep the shunt from re-occluding and she didnt think it would make a difference. She said she and her partne see this occluding issue very commonly in their patients who have small ventricles but who also have shunts as they are able to use their endocscopes to peer down the shunt tract and visualize what is blocking the shunt. She said that what she has found in my shunts appears the same as the brain material they see in her other patients with similar small ventricles who are shunted and so she doesnt think it would make a big benefit. I trust her opinion. I updated 3 of the MPS docs who have given me their opinion that they felt IT could be of benefit and one of these drs replied back pretty quickly that he agreed that the cause was definitely from cell shedding clogging the shunt but he added that even if there was MPS (GAG) storage it wouldnt be visible in the shunt as the storage appears clear in the CSF fluid and that the build-up or clogging material would then also appear like any other material in non-MPS hydro pts with occluded shunts. He wondered why the shunts keep occluding (my neurosurgeon feels this has something to do with the small ventricles) and he thought that the IT could still help. I guess at this point I am going to go with my neurosurgeon opinion, it wont hurt to gather these opinions and if we cont to have shunt occlusions then I will re-consider the IT study. I am hoping we dont though cont to have the occluded shunts! Like my Mom said even a year break would be nice! :)

It seems incredibly hard to believe it has been 2 weeks tomorrow since this last surgery and yet I am glad we are this far out and it isnt last week or the week before as I feel better now - this week! I hope it continues!

Because of the isolation measures Dr.Bragg and her team + the floor nurses at UW have had to take for the past month since my 2nd to last surgery about a month ago (5 or maybe 6 weeks ago) in which I had gotten a UTI post surgery and was an infectious antibiotic resistant bug (klebsiella - XCR) I did print out and take the results of a recent UA result to her this appt. This UA was for this most recent surgery 2 weeks ago for which I did not need to have a repeat pre-op physical (because we where doing another shunt revision 3 or 4 days shy of the 1 mo mark that the previous pre-op physical had been done) and so the result was supposed to have been faxed to her but apparently never was so the isolation measures where never removed. Even she said that she wouldnt do the isolation measures if she absolutely didnt have to by hospital policy and so now she and her nurse are going to talk to Infectious disease docs and see if they can get the isolation measures/precautions removed from my file. Hopefully! It is almost comical watching them gown up, wear masks and gloves and then peak their heads in joking around about the time it takes as they are getting it all on. Atleast they are all good natured and we've had a few good laughs about it if nothing else. :)

I had a conference call with Genzyme PR Director earlier this week and we have the survey for Adult-MPS I pts finished minus a few minor changes I recommended and we agreed upon. It looks like we will use a program called survey monkey to complete this which should be good. I cant include the survey here but once it is finished I will upload the final results though that will be some time. I am very happy we are this far and that Genzyme agreed to and was interested in focusing on figuring out what are some of the needs of adults with MPS I and better figuring out how to help this community!

I am supposed to go to UofMn next week for NP testing but havent heard the arrangement yet so maybe it has been put off which would be ok with me. I do haveERT mon,  PT  tues and another appt scheduled thurs  in Milw so wont mind if I dont have to reschedule these.

I have been incredibly busy in between other things working on the ACHD website ( www.wi-achd.blogspot.com) and making changes and upgrades to various things with that. It has been both good and distracting, although really I dont much need another distraction. All that aside I am glad to be working on the project and I think once we get more done this will turn out to be a great group! I also met with  my regional manager for APF after my neurosurgery appt yesterday and we went over some projects APF is working on as well as some ideas for here in WI.

I received a certified mail letter today from a apartment that I have been wanting to get in to for the past couple years - it is back in Waupun which is where I lived for a short period after my sister got divorced. Mileage wise it would be a bit further from both Milw and Madison but time wise it would remain about the same as it is all highway time vs now I go through several small towns/cities to get to Milw. and rural roads to get to the highway to madison. My one caveat/concern about moving is that I have taken care of some stray/feral cats outside my apt building for the past 3 years that ive lived here - the ones that ive been able I used a live trap my Dad got me and took to the local human society which adopts out the friendly ones and gives the feral ones to local farms. I honestly dont know if I can just leave these cats as I do care about them and I cant just see abandoning them as someone clearly did to the original ones. I know alot of people probably think no big deal and wow what a thing to let get in the way of your moving but to me these are innocent animals that deserve a reasonably good life. As it is now I have a wooden shelter my brother re-built for me (the 1st was stolen last summer) and straw a friend gets for me and I change their water in the winter 2x's a day so they get something to drink + feed them the same dry food I feed my own indoor cats. In other words I care about these animals just as I do about my own inside cats. I really dont know what to do right now.

Ive attached the latest email from Dr.Kakkis regarding his opinion of the shunt and IT: again just interesting and potentially helpful to other families.

(Erica,
The difference in the MPS patient is that you will likely shed more cells over time due to the storage in the meningeal cells, making more debris than usual.  Second, you cannot see MPS physically.  It is clear in water  so she always sees just the cell debris.  Any goeyness would be hard to see as a film on the filters adding to the plugging but no doubt the cells are the thing. 


The question is why do your shunt get plugged so much? 


My thought is that IT still could help. 


Emil)