Pre-Op Physical, Hand and Neuro/shunt, Thanksgiving

Tues Nov 20th?
Today, I saw my PCP for the pre-op physical for Dr.Bragg's shunt surger(ies) and for the Hand surgery and kind of had to laugh; I knew from the start (and was ok with) that my new PCP knew pretty much nothing about MPS before she had met me but she did some research/homework before our first mtng and atleast knew what the big issues where going to be. (her doing that research alone proved she was willing to learn.). Today she commented that this should be the last time I needed to see her for a pre-op appt for the shunt stuff for a long while and we shouldnt ever have to do a hand surgery pre-op again given this surgery (3rd time in 10yrs for R hand) was going to be so extensive. I am unsure if she (probably) just thought I was kidding as I laughed and said something along the lines of "I am sure we'll be doing this again in a few yrs for the hand stuff" the reason being for my saying that of course is carpal tunnel in us MPS pts isnt the same as CTS in non-MPS pts/general population as for us it is caused by GAG accumulation and inflammation and doesnt matter how extensive of a surgery surgeon do it will recur and the surgery is more to preserve nerve function and relieve symptoms. My concern all along with this Hand surgeon has been I dont think he really gets the MPS factor and I think he thinks (it seems like) he can make it go away for good this time by doing the additional things and protecting the nerve with grafted tissue post-release. I am a lot hesitant and more than a little reluctant how this will go. =/ ?

Weds Nov 21?
I saw Pain Mngmt for fup on Thurs and she of any of my providers knows me very well and we where talking about the carpal tunnel and associated surgery in Dec and I made the comment I thought maybe I should get a second opinion from my former Hand surgeon (switched bc of Insur but he did the 2nd releases on each hand and at same time the tendon transfer on my R hand.) She made the comment in all these years she has known me now (5 or 6) and through all the surgeries she has seen me through (more than 2 dozen in that time) she had never once heard me express concern about if the surgery/surgeon was doing the right thing and she strongly felt I should get a second opinion if even it just means the 2nd surgeon (probably my former) where to say they agreed (or not) with the plan currently set to be done mid-Dec. which is more extensive...   I gave her copies of the c-spine MRI and she made the comment my headaches could be caused by the severe narrowing at multiple levels below my previous fusion  since the headaches (different than the shunt headaches) are much worse anytime I bend my neck..  I did talk to my Ortho-spine surgeon in Mn's nurse and sent up my MRIs and Flex/Extension Xrays from the past year and recent as well as surgery notes to them and should hear back in the next week or two what they think.

Family pics from Thanksgiving ... Thanks, Jenn! (sis-in-law)

 

Nov 26th - Happy Birthday Lis! (my niece)

 

My Insur Case Manager called late this afternoon and was a nice call; while we dont always agree on everything we talked about the Hand surgery ( I had emailed her about what my PM dr and I where talking about) and our thanksgivings and I generally feel a bit more settled when I can just hear other people's opinin of what I am thinking about/wondering about and in some cases worrying about. I have gotten a little better at asking for advice although I still if I dont feel comfortable with what someone else might think will stick to my feelings and sort through things on my ownmore often than not,  I still dont quite kmow what to feel about this hand surgery but the surgeon himself seems to come recommended albeit my concern as ive stated before I think is what does (or more likely does he not) know about MPS and how that affects CTS and the other procedures he is doing and although I actually in reality dont mind if someone else does the follow up it does still make me wonder howi nvested is a surgeon in his pt if he isnt interested enough in doing his own fup t at some point?

 Monday is the surgery with Dr.Bragg to externalize a portion of the 1 shunt and let it drain outside the body for a few days to see if under-drainage is part of our problem and then if so/if this works well we will go back in and re-internalize the shhunt in to the pleura (chest) I guess.  I will be happy when it is over. I dont actually know how many days we will drain over but wouldnt think more than a couple days and then re-internalize it but should call and ask about that I guess. I really want to get the night and mornings with these shunts figured out as I think we are so close but I am just not enthused about this (not that I am about any surgery but this one especially I think) but I guess it's the price yah pay and I do think we are doing the right thing as the 2nd shunt has helped but just not like w the external drains and that is what we want to understand or figure out what is different.

 

Since I have ERT on Fri and surgery is next Monday one of my infusion nurses who is also on the access/Interventional Radiology team at CHW agreed to re-access my Port on Fri so I will just have it in for a few days on not a entire week but also wont have to have Anesthesia deal with accessing it. Much better to have nurses do it ahead of time then have Anesthesia try to convine me they can just do a peripheral IV or wait for access team at UW.

 

My Sunday school kids sing the following sunday (9th i believe) and would like to be there. :) I have class this sun (The birth of John foretold) and then the 23rd which is a Xmas Party for each of our classrooms so should be a fun time with the kids and probably figure out a activity and a craft to do during the hour that day.

 

I actually am atleast 3/4's done with Xmas shopping and the hardest 2 (My Parents!) are done - I just have part of my sisters, my nephew Quinn, part of my niece Alissa's (b-day and Xmas) and part of my other nephew Jordan's + cat toys left! I am waiting for 2 things I ordered on ebay for my nephew Zander 1 of which my parents and I split the cost on which is nice so then being he is my Godchild I could get him something else to. :) Ive had alot of fun w Xma shopping this year and ebay has definitely helped to!

 

I want to add that although I didnt write anything for Thanksgiving (this has been a week-long post in progress) I am very grateful for my family, for my nephews and nieces and especially my sister for letting me have Zan whenever I want basically and as well to the family I work for (I love this job) and those people in the past year who have helped me in various ways. I really appreciate all that people do and as well for those who believe in me and want me to work for or help them. :)

 

Thanks for stopping by,

 

Erica                                                                                                                              

Ridiculous

Neuro-Endocrine Appt was ok, she is continuing to keep fups for now at 3month intervals due to the shunt issues (we go back and forth between 3 and 6 mo fups and testing) and the lack of periods as she wants to follow that and see if it resolves. I assume it is just from the stress on the body of up and down pressure and the effect this has on my Endocrine system on almost all levels, who knows. She ordered the usual array of cortisol, thyroid, female hormone and blood chemistry testing.

I went to check an appt date in Jan as this week ive literally spent no less than 6 hrs and probably more scheduling and rescheduling numerous appts for Dec due to the shunt surger(ies) and in-pt starting Dec 3rd. Appts rescheduled have varied from Rheumatology, hand surgery and those 2 post-op fups, Urology and testing to go with that, Neurology-botox and Cardiology of which I am waiting to hear back from a couple yet (from monday, ugh). Anyways so Tues I originally had a pre-op scheduled for the Nov 29th hand surgery but we where going to use this date for Dr.Bragg's surgery anyways AND the date would have still been within the 30day requirement for the Dec 20th Hand surgery, Lo and behold some person WITHOUT asking me if I still needed that appt EVEN THOUGH I TOLD THE HAND DEPT I WAS RESCHEDULING DUE TO ANOTHER SURGERY cancelled and rescheduled the pre-op to later in Dec. I called scheduling for my PCP to try and figure out what to do but PCPs schedule is now booked and the scheduler had me leave a msg for her nurse about what happened as maybe they can make some exception for an new pre-op date. When I talked to Anna, Dr.Bragg's secretary mon she had asked me to try and get this done soon and was happy that it was set for next week meaning they''d get the paperwork a few wks ahead of time. I am so fit to be tied with this Hand dept and their ridiculous scheduling and the fact that they just schedule appts w/out verifying if it works for the pt (I literally would not know about appts and changes if I didnt check mychart for other reasons) and the fact that the dr doesnt do ANY of his post-op fup (ALL done by his staff) at any pt after surgery and just the fact that this drs ofc and Dean seem to think pts can drop anything in their lives to be at the Hand centers whim. Afraid not and incredibly pissed as this hasnt been just once w the scheduling crap but multiple times and ive only been seeing them since Oct. I very much dislike this Hand center and prefer my former surgeon.


ARGHHHHH /(Just to vent a little more),
Stay tuned its never dull, =/?

Erica

Scheduling to externalize...

I know I never posted after getting released from the hospital last weekend (sat) but will update now..

I saw Dr,Bragg today and we agreed to proceed w the surgery to externalize a portion of the VP shunt which she will use the same incision we made last week from what she said and I think if I understood correctly will tie off temporarily the distal end of the VP shunt in case this external draining wouldnt work in which case if it didnt work she said she would elect to place the catheter tip back in to the stomach area and although I dont quite get this part (we covered alot of info and I quickly forget my train of thought or dont think of questions commonly till after we've parted,) but she said in that case she would be able to tie the 2 catheters togeheter and pull it through vs having to re-thread the catheter w their tuneller. If the externalization does work we would place the catheter in to the pleural space it seems which is what surrounds the lungs I believe and a pretty easy procedure (I think).  I am not poitive on this but I think we would only be moving the one shunt drainage tip and not both of them although thinking about this now I wish I would have remembered to ask that and will ask her nurse somtime befoe the surgery as I think Lisa would either know or ask Dr.Bragg for me or I could email Dr.Bragg and ask her but likely easier to just as Lisa.

We are trying to work out dates now for this all to occur as she is out of the ofc the week of Thanksgiving (2 wks from now) but also was trying to think of a way she could get this done before then or atleast she externalize and drain the shunt and maybe her partner could do the 2nd surgery to re-install it either in pleural space or if it didnt make a difference back in the stomach. I am not as keen on having her partner do the 2nd procedure just bc I trust and know Dr.Bragg and she knows me so it seems in all likelihood we will probably end up doing this all the week after Thanksgiving. I dislike the idea of feeling this bad the majority of the day for another 2 1/2+ wks but would rather wait to have Dr.Bragg be there. This of course means I would have to re-schedule the hand surgery which is Thurs the 29th which is also not great news as those symptoms are pretty bad but the headaches and resulting issues are worse. Have I said I hate MPS lately?

Next week I do have appts w my Endocrine dr (3mo) and w my Pain Mngmt dr as well as mtng a friend of mine after infusion on mon so probably in all likelihood it would be better to have the shunt surgeries the later part of the month. It would be nice to not need any of the surgeries at all. =/

It seems from what Dr.Bragg was saying today she may be planning for a week altogether for the surgery, draining, and re-install/revision surgery as she was also figuring out even if we did do the surgery next week I would be in-pt over the weekend but could her partner get me out in time for the holiday. I dont want to be in-pt for Thanksgiving. They say timing is everything well I seem to pick the best times of all for just about everything. =/

On a different note my sunday school kids sing in both church services 2 sundays from now and although my co-teacher is the one directing our class and the other classes (I have no music ability what-so-ever organ or directing wise!) id still like to be there so hopefully that will work out too. These are sweet kids and so far has worked out ive not missed any of my sundays for teaching (2 on, 2 off) and hope to keep it that way. :)

I will have to reschedule the Rheumatology Appt in 1 1/2 wks as I have a pre-op (again, such joy) that same day which was originally for the Hand surgery as we couldnt get this last neurosurgery note to cross over (a wk outside the 1 mo timeframe) but it seems it will likely be for the shunt anyways again. I see my Pain Mngmt dr next week and will see what she has to say about seeing Rheumatology anyways since it appears it wont be w the dr she wanted anyways. Guess I am bringing my new PCP in head and feet first as far as how nuts the MPS stuff really is and can be.


One last note my GC and I are setting up an appt w the Geneticist (not his biggest fan) to map out the higher Aldurazyme dose and our 6mo-1yr plan for what we'd like for it to help and how will we measure any potential benefits if it would help. I am unsure when that will be but hoping to start the higher dose soon.

Stay tuned, its never dull although on the medical front I wish it would be!? I am looking fwd to seeing this friend of mine on Monday, although she doesnt have MPS she gets it to an extent and has been dealing w her own chronic issues and right now I could use to vent to someone/talk to someone who understands how frustrating this all does get to be somedays. She is my age and another friend I met when she was doing her school shadowing w one of my providers.

Thanks for stopping by,

Erica

Not knowing, complex decisions, Care

Dr.Bragg was in a really long surgery (well in to the night) so I didn't see/talk to her at all today but her NP stopped by this afternoon and we talked for quite a bit about this all similar really to the conversation I had w her other NP yesterday about giving each option a trial period which was something Dr.Bragg talked about on the phone on Mon when she called me (ie if this smaller surgery to remove the 2nd valve didn't work as we hope she'd like to give it a week or two before we did anything else.) and making sure we don't do to many things at once and if something does help not know what it was that helped. Its a good plan to have and I appreciate Dr.Bragg being tentative just need to remind myself one day at a time is better and even though I desperately want to feel better I need to be patient.

I was a little confused talking to Sue about getting out of here as on one hand she said if Dr.Bragg where to decide to do something early next week (seems un-likely) it would be better for me to stay in-patient but then she also said she wasn't sure what Dr.Bragg would want ie my getting out/care and it was probably be better to talk to her when she came around (Sat morning I think) and make sure she thinks the ultrasound was ok (we never heard anything about it from Radiology) and see what her thoughts are ie cont to wait and see or something else.

Admittedly as I've talked about in the past the hardest thing I have always struggled with regarding the medical stuff is simply not knowing a plan or even more so not having a (if even just) tentative timeline for when things might occur. I am the type of person who does well w plans, information and knowledge of when things might happen or what we/my draw are thinking and might consider trying. I think part of that is knowing that the provider(s) care (which I know Dr.Bragg's team does) and even more so being able to arm yourself ahead of time, I suppose to give yourself a distraction and feel like I am doing something (I don't know this one) for my care. Again about the best I can figure to explain my quirks...

I am hoping to be able to talk to Dr.Bragg for a few mind in the morning and see if her thoughts follow along what she was thinking Thursday and if she will let me go home as well regardless if we're waiting and watching (my first guess what she'll chose), trying one of the things previously talked about or?? I always feel more settled when I know a little of her ideas no matter the situation. Thankfully she is patient and seems to like to share her thoughts or possible ideas.

I in all this as usual just desperately want these headaches to GO AWAY! For good! It was a semi-rough day emotionally on top of the physical headache symptoms that very ebb and flow. I try to not focus on the un-knowns to much but today and even yesterday was failing semi-miserably at not having control and not knowing + even the worry am I going to spend the rest of ny life feeling this crummy so much of the time..(obviousmy none of us could answer that anyways as it is ultimately in God's hands). This afternoon my sis happened to call and I vented some to her as well some good friends in the MPS community left sweet messages in text and fb messages trying to cheer me up as they know the roller coaster ups and downs and how hard it is in not having simple answers or easy fixes/solutions.

I am grateful for those friends many whom are parents of MPS kiddos not actual Adults w MPS and for family who cares even though they don't always really get the actual frustration. (There is simply know way one can understand unless you've been in endless decision making, coordinating and day to day-ness of a chronic, complex and very time consuming issues like MPS care.) But my family is still there and tries and for them I am grateful, very grateful!

Thanks for stopping by,

Erica

Revision #16, 2nd Valve removal, Wait and Watch; test(s), Ideas.

Today has been kind of  long day but hopefully tomrrow i'll feel even some better. Pretty much we wait and see if the CSF stabilizes now between the 2 shunts. I was talking to them about (as has  been a past fear too) that I do worry about this being on-going for the rest of my life and not feeling good so much of the time w small parts of the day I feel better. Thankfully Emily, Sue (NPs), Lisa (Nurse) and Dr.Bragg are so easy to talk to  albeit I dislike really talkin to anyone about how I feel. Atleast they care though.

Dr.Bragg's NP told me one of the things they have done over the past few days is talk to her partner Neurosurgeon and he feels as has been a discussion point in the past that this is related to a compliance issue and will take lots of
 "tinkering" to find the right, best set-up for my body and CSF pressures/flow. We know the 2 shunts compliment each other and have helped keep each shunt from malfunctioning or atleast so often its just a matter of why is it no where near as well as the external drains? Who knows. =/ They did

They are going to do an ultrasound (vs a CT scan which I would have to drink contrast and since I havent been able to keep down alot of fluid (on IV fluids yet) and have no appetite (have eaten very little food) so the ultrasound is the simple means to get imaging although will take a bit longer tomorrow than a CT. This will give them a way to non-invasively look at where the distal ends of the 2 shunts are draining in to and make sure there is no CSF that should be absorbing in to the peritoneum that is just sitting or collecting causing issues. If there is no visible fluid collections it doesnt neccessarily mean there is no absorption issue this would just be the 'easy' way a not well absorbing CSF/shunt would present as. There are apparently multiple ways mal-absorption issues could occur w shunts and drainage problems. I cant quite remember that part of the conversation to well. I think Dr.Bragg said there was a chance they would go back in and explore the stomach but I honestly dont remember this part of the conversation well and am sure we will talk more after the ultrasound.  I am unsure when I will get discharged but suppose it depends on being able to keep down enough fluid and food. I am hoping to get out.

If we did decide to do an externalization of the shunt(s) we would likely take the VP shunt and disconnect it similar to w this valve removal surgery and externalize the shunt at the R upper chest area an then drain from there. I am unsure if we would do an open (free flow vs controlled)  drain (from the shunt) or if we would try different regulated pressures to see what amount of fluid worked best.  If Dr.Bragg did end up moving the distal end of the shunt she said she wouldnt put it in the heart due to the significant cardiac issues and instead would likely drain it in to the chest/pleural area I think she mentioned when we talked earlier today. Initially she had considered maybe the atrial part of the heart could be a drainage point but my Cardiologist was against that due to all the cardiac issues  and already having a Port (access device) in place.  She was more than great about that not being an option to consider.

I should know a little more tomorrow.
Thanks for stopping by,

Erica