Tuesday, February 24, 2015

Neurosurgery (shunt) f/up, Hand surgery f/up, etc.

This is one of those updates i've been thinking about for days but have not actually sat down to write. I have also been pondering what to write for a RfRD (running for rare diseases team) Patient Partner blog and coming up with a blank on that to.
This last Fri for the 1st time I chatted with my 2 new RfRD running partners on the phone (I have 3 this year, 1 returning, 2 new members) and thought i'd get a little inspiration there but haven't really? I have some thoughts for what to write this year (my 3rd year on the team) but no clear path on how to lay out what I have been thinking yet.. hopefully I will get there! I do really like my new runners though, they seem fun and we had a good chat, the time sort of just flew past which says a lot as I am not much for talking on the phone!
This last week I saw Dr.Bragg in f/up which went fine, really nothing to important out of that Appt other than we just watch and see and for now as imperfect as the headaches and resulting (pressure related) symptoms are it's somewhat better than it could be (could be worse) so she just said to f/up in a month or if needed call her sooner. I didnt think to make the appt on the way out and havent called to schedule yet but will get around to it at some pt. I think the worse is the pressure affects in-directly the sciatic nerve/symptoms down my leg (from reading online and having talked to a couple others w/former spine shunts it seems spine shunts and leg nerve irritation is not at all uncommon those less commonly known amongst our drs.. It is definitely a symptom that occurs related to the shunt(s) functioning less than well and not only occurs at night waking me up but intermittently as pressure builds during the day.
Breathing has been an issue the past 5 or so days (not so much SOB just intermittent feeling difficult to get normal air) though I honestly just don't know if this is related to fluid building up in the pleural space or something else or perhaps multi-factorial ie cardiac and shunt combined in nature.
This combined w the headaches and leg symptoms could drive a sane person batty! I swear really MPS is like a mind game not only trying to figure out issues but figuring out when something is really an issue to the pt of telling 1 of the Providers about and then IF it is WHO/Which one!

I was sitting at ERT writing some of this post yesterday (Mon.) and finishing the rest now, a day later. At ERT I generally have quite a bit of time even after having finished (what I can do there) my SS lesson plan, taking care of other phone calls, filling out any papers I have to and tyingup a few other loose ends related to other stuff. I figured since I had my netbook in my bag I might as well try to type out part of an update (at the time I was initially writing this i'd had about 2 more hrs of a 5.5-6hr infusion to go). =)
Today I saw the Hand Surgeon and he is happy about the hand surgery recovery. He commented the only concern he'd had w the surgery is when he did the hypothenar fat pad graft there wasnt much fat tissue to begin with for him to work with for the fatpad graft but he felt what he had he was able to get a good cover. As far as the splint I am able to not wear it other than he and the OT recommended with a few things to perhaps use it (but my discrection) and OT recommended at night as they are concerned this is already the 3rd CT release for each hand. I am not entirely sure wearing a splint would or would not prevent recurrence but don't know. I guess i'll just see if I would wake up w symptoms I can put it on but otherwise symptoms have for the most part been really good! Is always nice when something is good! =)
Typically with just a carpal tunnel surgery release a patient is in a splint for just 7-10days. Because of the hypothenar fatpad graft the healing process is longer though I was able to take it off at home when doing simple, light activities. I am glad to be done with another hand surgery!
I do have to share something funny that 2 of the OT's both of whom worked w me a bit over the last month (related to post-op care/splint care) said. I had just been taken to a table in the rm and they both, walking past said "Hey, Erica, we're fighting over who get to work with you today" =)
I have to sort of laugh about being in Milw. yesterday, and Madison tmrw + Weds as last wk it was Milw. 1 day and Madison 2 days. Last wk Dr.Bragg asked me at her Appt if I am ever able to coord. appts for the same days? Clearly it would be hard to do so when in 2 different cities but for instance like last wks 2 Appts in Madison and 2 separate places this wk I see the Hand Surgeon Tues at Dean West and PMR doc at UW in Middleton (botox injections in spine/neck) on Weds in addition to the usual ERT every Monday.
It would be great if it where a little easier to coord. appts though! Most rare disease pts know and understand this struggle though; just yesterday I was reading a funny article for 'Rare disease day' and 1 of the points was about never being able to coord. appts and doctors being in multiple different cities/medical centers.

On an unrelated note and i'm not entirely sure what has made me think of this (other than perhaps hearing, reading and thinking about the Ataluran drug so much in the past few wks which is the drug being studied for non-sense mutation (atleast one mutation makes no enzyme) MPS I pts and which is expected to finally hopefully go to trials this summer. This same drug is in Phase III trials for DMD which is a form of muscular dystrophy, a muscle wasting disease for pts who also have atleast 1 of their 2 mutations that makes non of the needed enzyme.
Anyways it occured to me that while DMD and MPS I are very different disorders it is almost striking how many of us MPS I pts where initially diagnosed with or thought to have/tested for some form of muscle wasting disease?! I know many other type I pts who where diagnosed in their teens who tested for or dx'd w some form of muscular dystrophy or muscle disorder and I myself in addition to drs later wondering about connective tissue disorders was tested for and the question batted around about a muscle disorder called Charcot Marie Tooth (CMT) primarily due to the significant nerve issues in my hands and the changes in joints + cardiac issues.
Just an interesting observation I guess. Last wk I had fwd'd an article related to MPS I and Ataluran to my Lung dr as I know her interest is Cystic Fibrosis + I know this drug is also in studies for that. She emailed back that she hadnt been aware the drug was a potential for MPS I (but thought it was exciting) and that her Center was in fact part of the on-going trials w CF pts and this drug.
Sort of funny how my PMD once she found out about the Elmiron shared she has experience w this with several of her pts and is willing to manage it if I do opt to start it. Elmiron is another drug I am potentially going to start for MPS I once I see the Genetics dr in Boston as he is willing to prescribe it and my PMD thinks it's a good idea to try.
I have known about both these drugs (Elmiron, Ataluran) for some time and yet had no real clue 2 of my own drs have experience w the meds separately. =) Small world and goes to show really how in many ways, many of MPS's issues counter-cross to other disorders.
Will update again sometime soon, if your the praying type please pray the headaches would stay stable OR even improve - that would be AWESOME! I know some around me have commented "you look good, you must be feeling good" which is nice to hear BUT I also feel guilty then and quite honestly most of the time I feel like I can't really be honest about what I am really feeling. =/
Thanks for stopping by,

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