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)