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

4th week Post-Op Cardiology Fup Appt

Today's (Thurs) Cardiology Appt went well, the Labs show Coumadin level is trending down which Dr.E and Nancy (NP) actually consider a good thing as they said this typically means the pts appetite is improving and overall body health is improving. I'll repeat these (INR) and other labs next week at Cardiac Rehab but dose was increased back to 4 mgs and likely in time will con't to increase some. Otherwise the Chest Xray shows the fluid around Heart and lungs is much improved (I kind of suspected as much as the cough is very improved over the past couple days) so we'll keep the Lasix dosing at 60gs 2x's a day and Aldactone at 25mgs 2x's a day and Bisoprolol daily at least until the next fup. The reasoning or thought behind this was that since I have improved over the past week w these meds Dr.Earing didn't want to upset the apple cart and cause a set back or fluid retention again. I follow up in a month so maybe then we'll be able to lower or take away some of the heart meds, at that point it would be about 2 months post the 2nd open heart surgery and 3 months post the 1st OHS.

Cardiac Rehab is going fine and actually not very hard, I didn't really know what to expect with it but really it is just their having you hooked up to EKG/heart monitoring the entire time you are working out and they monitor heart rate/blood pressure and heart signals. Really it is mostly stretching to begin with then working out atleast right now on the treadmill at increasing pace as tolerated each week and then switching to a bike (for instance this last Weds a recumbent bike) and following that some more stretching and several short laps around the gym to cool down. I haven't broken a sweat yet. I'll typically do this 2 days a week but for instance this week and last because of scheduling on my part was only able to make it on Weds' and the same in a few weeks due to ERT on Monday's and often other appts on Thurs's.

I guess the only other new thing is I am going back to my Apt tomorrow (sunday) which will be the 1st time I have slept there in over 2 months (I have gone back and forth just haven't stayed there) so I think that will be nice! Today we had a ACHD (adult congenital heart) Picnic in Mequon which was nice and basically entails a group of my Cardiologists clinic pts who also have Congenital heart issues of one cause or another meeting up to chat, eat and just be around other people who get it although lots of the conversation centers around our lives in general. Next week I am having dinner w a different patient of his who had a heart transplant and who I actually met through my Pain Mngmt dr. She and I have talked several times since meeting at my PM drs office (by chance) and being introduced and she is close to my age so should be nice. I like food. =)

I don't think there is a lot going on otherwise medical wise, I emailed with Dr.Bragg last week and we are going to wait till atleast mid-Nov if possible to begin to talk about moving the 2nd shunt back to it's former location. She (Dr.Bragg) is a bit concerned there could be permanent scarring in the pleural space from the 2 heart surgeries and subsequent chest tubes used during both. I am not sure how we know if there is permanent scarring but pray this wouldn't prevent us from moving the shunt back as I cant fathom both shunts draining in to the stomach (peritoneum) permanently. =/

The only other thing is due to some b.s drama w a group of catty women the family I work for arranged for us to have a connected hotel room at the Conf and as well helped me arrange my flight so I can fly back on Sat instead of on Sunday so I can go w my nephew and niece trick or treating on Sunday!
Will update soon,

Erica