Old news (sort of), New news, Catching up..

Dr.Simpson my PCP is wanting to schedule an appt due to muscle spasms in stomach (likely shunt related given previous history of these prior to our having moved the Thoracic shunt to the pleural space last winter and pre-the heart surgeries (having had to temporarily re-locate this shunt to drain in the abdomen where the 1st (VP) shunt lies) and due to muscle spasms in other areas. I've been having muscle-type spasms in L side (fingers, calves/leg, foot and oddly last in mid-side/upper stomach). These all tend to be short lived but repeatedly occur other than the abdomen/stomach spasms which go on for hours several times a wk. I think PCP perhaps wants to get a better idea of what it all is, i'll humor her I guess. I had just sent her an email-mychart asking if she had any thought on how I might be able to prevent it when she suggested seeing her given the longevity of some of the issues and how the shunt related spasms have been an on/off issue. Since I am still semi-fairly new to her (just over a year now) while she has learned a lot about my history and MPS she also still tries to stay very involved in what various specialists are recommending, adding potential recommendations and looking in to options. ------------------------------------------------------------------------------------------------------------------------- I emailed a bit w Dr.Bragg (Neurosurgeon) again last week, I had asked her in my initial email if she thought when we where able to move the thoracic shunt back is there ever a way to tunnel the valve of the shunt so it sits more on the side (more muscle/tissue/fat) so it isn't as noticeable (moves) when I might bend down/twist or sit back. There I s some concern there could be permanent scarring from the multiple heart surgeries, done in short order and thus the many chest-pleural tubes so will be a problem we'll have to sort out first. If there is no permanent scarring from the 2 heart surgeries and repeated chest tubes- 3 the 1st time, 4 the second time we'll move on. She did email back to say she was willing to consider other options for where the valve is secured/tied down (right now sits basically over the rib area/spine area between my spine and side and VERY noticeable when I move as it also moves. It isn't about a cosmetic issue but about the discomfort the valve places! Not an awesome feeling, not painful just not awesome feeling! I will see her sometime in mid-Nov (in 2 wks which worked best w my schedule) as this is coming up on week 7 or 8 post-the 2nd heart surgery and Cardiology has said we have to wait till closer to week 12 plus she would like to wait to around that timeframe to (3 month mark or so) in order that any scarring or issues from the chest tubes may be healed. I'll likely call and schedule next week for later in Nov. and we'll figure out a plan and schedule from there. <--- Obviously I was writing this in multiple parts! ------------------------------------------------------------------------------------------------------------------------ On a different note I was writing most of this from the National MPS Conf in San Antonio (is held in a different city each yr) and spent some time talking to a Genzyme-Registry person. I asked her if she 1. knew of any other way the registry could be updated w our info from say surgeries, bigger issues if our Genetics drs/team arent doing it and 2. would pts ever be able to access this info in the future? - Apparently Genzyme has roled out a newer format to their other Lysosomal-enzyme treated Registries and will be doing the same in late 2014/early 2015 for MPS I. In the newer formatted Registries any of our specialists will be able to get a passcode and be able to upload their info related to us (say a surgery or outcome) to keep the Registry better updated on all MPS I pts as right now they have very poor compliance w just Genetics drs having access.. I really think my Cardiologist and Endocrine dr would update their info as they are very interested in MPS I info and would then get info in return, in general. I tend to think Dr.Bragg might also from time to time be willing to update shunt/surgery info or have one of her staff and for that matter my PCP likely would to. I think it will be a good thing for access for all of the MPS I community! Those of us MPS I pts who have drs participating will then also be able to get a summary of our submitted info (can't remember this part for sure) compared to other MPS I pts or perhaps it will be just a summary of what was submitted on us. No-less it's a start and I think will be good for information for the MPS I community as more articles can then be written and shared... -------------------------------------------------------------------------------------------------------------------------- Otherwise things have been somewhat quiet; I was initially sitting at the San Antonio airport writing this waiting to fly home but am now at my Apt finishing. The Conf was fun - not much info if any for adults but nice to see friends. It is good the info is there for families w younger kids and hopefully someday there will be topics/speakers for adults to. Was interesting to see the ones you think are friends but w all the fb drama avoided me(and on the other hand how many made comments about what was going on supporting me/saying they know I would never do what was said); honestly im not sure I mind bc people who know me really know me! I shared 2 joined but separate rooms w Allison (Gene Spotlight) whom I work for, we each had our own bathrooms, each had double beds, our own mini-fridges, etc. We generally just left the door between our rooms open other than when we where changing or showering and had a great time catching up, talking. Either talking back and forth while we where each in our own rooms or sitting on each others beds chatting and going to the various sessions together. Thurs night before the MPS I-attenuated syndrome breakout session we met up w a friend of hers who lives in Tx and had driven down and went down to the San Antonio riverwalk to walk around and eat which was fun and nice. I'll try to attach a few pics, I didn't take a ton but took a few - i've attached a couple. :) ------------------------------------------------------------------------------------------------------------------------ I have a few appts (Cardiology for instance) coming up again in the next few weeks (have just mostly had routine things like Pain Mngmt fup and Cardiac Rehab in the past 2 weeks) and con't Cardiac Rehab and so if nothing else will update after those. We've cont'd to do labs for the Coumadin (blood thinner) weekly right now and are slowly tweaking the dose back up, Cardiology suspects it will semi-even out soon. I have labs again before ERT on mon. but right now the dose is 6mgs 5 days a week and 4mgs 2 days a week (M,F). ---------------------------------
Thanks for stopping by,
Erica