Wednesday, April 8, 2015

Dr.Bragg, Neurosurgery Appt, VP Shunt tap, ERT Infusion. Next wk to Boston

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Global Genes Project article
Article I wrote for Global Genes - anyone who gets their blog emails/updates will have received this already. Although I am in the process of having my article for the Running for Rare diseases Team edited that to will be added at the bottom of this site when it is done and i'll share here (in a new post).
http://globalgenes.org/raredaily/35-years-with-mps-1/

I did see Dr.Bragg on Monday asked asked her about the spine symptoms, why they seem to get so much worse when the VP Shunt is working imperfectly and she said she feels it is due to the TPL Shunt likely picks up more slack, ie fluid (CSF) when the VPS isn't working optimally. This in turn likely causes the TPL Shunt catheter to expand which likely then rubs against spine nerves and the chest wall. The chest wall symptoms are more fluid related I think as it is not at all like last yr where the catheter truly was rubbing against my chest space BUT it would still make sense that as this shunt does more, more fluid accumulates in the pleural space and between that and the cardiac (heart) issues it is likely to much for my body to handle and the fluid irritates the chest wall space.
The spine nerve symptoms like I mentioned above is likely just bc the catheter expands in size and bc spinal canal is already small it likely is sitting close to 1 side and irriating these nerves that then manifest in my low back/mostly leg in a referred pain/discomfort sense.
Dr.Bragg did tap the VP Shunt again and although she wasnt able to remove much CSF (2ml which isnt alot considering there have been times we've been able to remove as much as 20ml's I believe) BUT it does seem to be just enough that it gives the TPL Shunt a slight break (I don't understand it but won't complain!) as the leg symptoms where much improved for yesterday AFTER having been a horrible morning. Literally in the waiting rm and in the exam rm I was sitting with my legs up on the seat I was sitting on, straight out in front of me, leaning back just slightly as that for whatever reason seems to ease the strong pain just a little. It was AWEFUL yesterday. Standing for just checking in at the reception area which takes about 2mins my leg hurt already. It tends to hurt while I drive but no where near as bad as when I am standing.
Anyways so Dr.Bragg couldnt get alot of CSF off, she did say the shunt tap flow looked a bit better than last wk but she still feels we may end up having to do a shunt valve revision. For now given she knows I really want to go to Boston for the Running Team/Genzyme and Boston Marathon wknd activities she is ok doing periodic shunt taps and we'll do one again next Weds, the day before I fly to Boston.
Whiile the symptom relief won't last the whole time or even close to the whole time it will as Dr.Bragg put it "give you a little bit of time where you feel better" (she put it something like that, not those exact words) and even though the headaches will very likely be back by Thurs morning I should be able to sleep good Weds night, I don't fly out till mid-Thurs morning and I have a direct flight on the way there. I am not sure how next weeks tap will go but with this one she could get intermittent spurts of CSF and she made the comment "that's probbly how you feel" (again something like that) - which is true! The headaches are intermittent! That is the one benefit of it being just an partial (likely) shunt obstruction vs when I have complete shunt malfunction as although I don't feel great I atleast have 1 and basically a 1/2 shunts working and it's better than when just 1 shunt is working!
We'll decide on surgery again after next week shunt tap or con't to periodically do these taps and watch the valve ie see if it gets worse as far as does the reservoir fill even slower or does CSF become even more sluggish when she is trying to pull fluid off for shunt taps? Only time will tell. I think she said yesterday she felt we'd likely end up doing a revision; she kind of asked me if I thought we should do it, to which I said it was her call, I can tell her my symptoms but truly she knows whats best and I trust her decision making regarding all this. She's done enough revisions on me she'll know when is the right or best time! =)
] As long as the TPL Shunt is working and the VP Shunt is working some we have time to figure out these shunts and any potential revision. It is when either shunt completely stops working that we have a very acute situation that requires surgery within a day or so (as soon as INR/blood thinner level) can be safely brought down.
Ironically it was 4 yrs ago this past weekend not only that I got the Port-a-Cath placed, which was a few weeks before I first saw Dr.Bragg's Adult Neurosurgeon colleague (Dr.Brooks) and not all that long before I started seeing her then after either!
I hadn't realized it but 1yr to the day after I got my Port placed is the day Dr.Bragg and I decided to place the Lumbar EVD (external shunt) to see if this would perhaps give us an idea if a 2nd shunt would help our on-going issues. Obviously we did decide on the EVD and then 2nd shunt placement and although it's been perfectly imperfect having the 2nd shunt has made our shunt revisions in my opinion go from being very, very frequent to generally every few months. I realize to many that may not seem great BUT that 2nd shunt has been a blessing and if there where a way to drain more fluid in another way i'd jump up and be like 'hell yah' as we know I do best and feel best the more fluid I drain!


Cardiology, INR / Event (EKG) Monitor)
I talked to Cardiology the other day after INR (blood thinner level) which was a little low though this may have been due to I think I forgot a dose of the Coumadin last wk., I am not sure though. I take this med alone right before I go to bed whereas all the other meds I take in groups eithr in the morning or evening other than 2 afternoon doses of 3 meds. Anyways so my INR was not in-range at 1.71.
I also talked to them about the heart rate monitor given it has been such a pain both with the patched causing pretty bad breakdown of my skin (after just 8 hrs I have sores and spots of bleeding despite using the sensitive skin patches). Once I transmit these last recordings though thankfully they said I can just send back the Event monitor; Yay!
I see them the end of April to go over treatment decisions ie the new med, this event monitor recordings, symptoms, etc. and we'll make decisons ie up the Cardizen or something else. I do have a built in heart rate monitor on my phone so when I would do the event monitor recordings OR the days I couldnt wear the event monitor but had symptoms I would atleast record heart rate on this app and then it stores it and gives a timeline of each hr + date/time when each was. I sent that to them as well. Not sure it will be helpful but can't hurt I guess.
Boston / Boston Children's Hospital
Next week when in Boston as i've written before I am seeing Dr.Bodamer a Geneticist at Boston Children's Hospital, he just moved there a couple months ago so is in the process of getting his MA State license but wanted to accomodate my being in town already so made it work to see me. Since he may not have 'patient priviliges yet' his Nurse and Secretary emailed me yesterday asking me to send Med Records (I keep copies of all records but jeepers wait till the last minute!) and to say that he would be seeing me along w a colleague of his who is also got alot of MPS experience apparently. After a realllly, reallllllllyyyy long day yesterday (hour drive to Madison to see Dr.Bragg, shunt tap then 1.5hr drive to Milw for labs at CHW + then next door to FMLH for 5-5.5hr ERT Inufsion and then an hour drive home. I got home about 7:30 and was pulling out and scanning in medical records till atleast 10:30! Glad it's done though!!!
I get in to Boston Thurs afternoon and am planning to have dinner with my Genzyme Insurance Case Manager who will likely meet me at my hotel. Friday after my Appt. I am meeting my good friend who also was my runner the past 2 yrs for lunch which I am also looking fwd to! If I have time after the Appt and lunch i'll probably need a nap at the hotel before the friends I am stayin with pick me up around 5.
Sat morning the family I am staying with who is also on the running team-patient partner and I will go to the run that the RfRD Team is doing (21 miles I think) to cheer them all on and after i'm not really sure. I AM sure the wknd will be exhausting!! Sat night the RfRD Team hosts a dinner of sorts at Genzyme Center (the main location for Genzyme, it IS gorgeous!) and after if runners/patient partners are free to do as they wish.
Sunday I am unsure and then Monday unlike in years past there is no pre-run breakfast so all families and anyone cheering runners on will congregate again at Mile 14 in Wellesley for the race. I have to fly out at 4:45 so will have to leave mid-afternoon but plan to take the train or something like Uber out to the airport. My flight home is anything but great w a layover (flying there is a direct flight) and I get back late. I rescheduled my ERT Infusion for that Weds of that week so as not to miss altogether. A friend of mine is teaching my Sun School class the 19th.
I sm praying I feel good and excited albeit a little nervous for the whole week (Thurs/Fri) and weekend to come!
Stay tuned, Thanks for stopping by,
Erica
Happy Easter from 2 very cute little and not so little munchkins!

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