Wednesday, June 26, 2013

Nothing we can do for now..

Didn't take long, no real surprise I guess but my stomach has stayed pretty swollen (think early preggo look) and seems to ebb and flow as far as how swollen it is throughout the day. At first I didn't realize that odd pressure or nerve like symptoms along the shunt catheter line (from mid-back to stomach) was more than likely related directly  to the swelling and secondary incision discomfort/pressure seeming in/around stomach and it seems related to CSF drainage/not absorbing properly? This I guess wouldn't surprise me as it is the reason we took the 2nd shunt out of the peritoneal area and placed it in to the newly chosen pleural space to drain back in Dec. Wasn't perfect but was a ton better than having both shunts draining to the stomach where I forever seemed to be having stomach spasms and issues. I know it's only temporary but sometimes when your in the moment of something and dealing w the symptoms a "temporary" change isn't all that easy like it likely would be if we where having no underlying adverse absorption symptoms and had changed the shunt only because of the infection or some other reason back in Dec and now had to change it back but worked fine. I am hopeful the heart surgery will go well and soon enough we can put the TP shunt back to it's former draining location. Dr.Bragg has talked about and again this morning that we will be able to do this and she thinks will just require 2 incisions (1 for the pleural space tunneling of catheter) and the other to disconnect the current shunt set up from the 'Y' connector as it can't just be pulled through. I definitely don't want summer over but can't wait till we are able to do this and maybe put an end to this seeming never-ending shunt saga! :)

I wrote this email to a friend earlier as she had asked how things where doing and if I thought i'd get out soon. I had fwd'd her something related to heart valves and minimally invasive valve replacement (interesting stuff!) which hopefully someday will apply to her case as well as mine and any other MPS individuals to avoid the 'open heart' portion of heart surgery! Here is the email and pretty perfectly sums up my frustration today and past few days:

I think we are sort of in a crossroads; we had to move the Thoracic pleural shunt back to the peritoneum (for the heart surgery as you've probably read) and had no choice about that but also where replacing the valve w what we think will be a great benefit as the particular Codman valve has worked really well in my VP shunt. The TPL shunt now being back in the peritoneum (TP shunt, lol) is causing grief of it's own with distended stomach and discomfort along the shunt tract I think bc the CSF fluid isn't absorbing properly (the reason why we had moved one of the shunts to the pleural space in the first place) and so is really uncomfortable and headaches worse than when I came in last Fri. Eventually after the heart valve surgery is healed we will be able to move this shunt back out of the peritoneum to the pleural space but for now I honestly am struggling w the thought that I could be going in to this heart valve surgery feeling really aweful headache and symptom wise and not to mention it could  be months until we can resolve the shunt issue and move it again. I truly feel like we get sooo close to being in so much better of a place w the shunts (we where closer before having to move it and think that the new valve would have been the tipping point had we been able to leave it to drain where it was) but then it's like a mirage, something always happens to stop actually getting to the 'finish' line'. Super disappointed and frustrated. I don't need to vent to you though, you get it.

We turned down the Thoracic Peritoneal shunt by 2 more settings today (tues) so it is now at it's lowest setting (30) and does seem to have made a bit of difference but no-where near enough. I just think the big difference is CSF not likely absorbing as well as it could.  I know it isn't my eyes directly but everytime I would try to read the past few days I had worse headaches and back to the really blurry/double vision. Definitely is odd to explain but was like where I would be  staring at the screen or trying to read and I would I get almost mind-numbingly tired and headaches. Since there is next to nothing we can do here I think I will be going home today; Dr.Bragg mentioned it and I agreed bc while i'd give just about anything to feel better and get to the "finish line" once and for all I don't see a point in being here if they don't know what to do w me right now. Soo very frustrating when you know what the answer is but can't do anything about it immediately!!!

Will update when there is something more, more than likely from home.
Thanks for stopping by,


Saturday, June 22, 2013

(#27) Assuming what I think/feel, TPL Shunt revision update (finally)!

Attached below this is a pic of Dr.Bragg and I from this morning; she is sweet to take a moment (while here talking) to do this. I will be sure and make her a copy.

So this whole basic blog is more or less a vent; bc I am tired of people thinking my venting means either A. All I think about is medical stuff/I have no life outside of MPS or B. That my working for the rare disease foundation/family is somehow a bad thing bc I am doing yet more around medical stuff. To point B I say, really? If those who say or think these things really knew me they would know I have always wanted to work in the healthcare field as a Nurse or something related since I was a little girl! This job is a perfect blend of being able to make some extra income while working part time and still be able to deal with all the of the often very never-ending medical issues (having to manage all the paperwork, insurance issues, appt scheduling/trying to coordinate and the family I work for who runs this foundation completely gets MPS on a personal level so they are sooo flexible!

Does it really matter what  a patient does for a job as long as they are happy and for that matter the job doesn't stress them out or affect their ability to function? NO! It sure shouldn't! This is what I enjoy so to be perfectly honest it completely baffles me that people who say this stuff probably barely know  me  but apparently think they do and think they have the right to judge my happiness bc I happen to have a rare disorder that does consume a lot of time but then I also chose to work within this same disorder for multiple reasons including it is what I enjoy and it does make me happy (being able to help others, being able to see/review what is coming down the pipeline for possible treatments for MPS, often communicating with researchers, etc.
Earlier today I am not sure where she got the information of who even told her these things but an OT eval was ordered (which was fine) and she was asking questions though I do think it is curious why drs don't tell their pts what they are thinking or their thoughts behind orders/evals? One of the several things that made me raise an eyebrow was her comment how "my parents are my only or major support system"? I asked what made her say that to which she didn't really have a response other than info that was apparently given to her. Anyways, I explained to her that my parents are who come with me to surgeries and are the main visitors I get but that I have  a large and varied support network at home between my church, my fellow few MPS Adults and parents of MPS kiddos as well as I do have provider's who check in with me on a regular basis (Pain Mngmt especially) and perhaps a important detail to me is that I am  not one of those people who likes to be surrounded by people all or even most of the time! Instead I prefer either to spend time w my nephews and nieces or at my Apt reading, writing or  doing whatever I like in the peace AND quiet! I pointed out that I write about my MPS experience but as I've said all along I had wished often when I was first diagnosed w the MPS that there was some other Adult w MPS writing about their journey/path so I could have followed in order to give me an idea on what I might expect or what I might do differently to make my care back then be more smooth. My blogging now and my main use of facebook is to share what I am experiencing so I can hopefully help someone else in the same or similar shoes I was 9-10 years ago! Judging by the many emails, messages and pm's I get I think I am able to do just this in helping others both with MPS and many times people w Hydro who are also thankful for an honest, open perspective.  I just don't think that I have to share EVERY aspect of my non-medical, personal life and that much of my personal (and boring to most I would think) life is in a way or as much as it can be separate from the MPS/medical stuff! It drives  me crazy that people "think" they know what I am feeling or thinking or that they assume they know how I feel about procedures/surgeries or issues. If I wanted people to know how I feel about something I would share (and often do, atleast partially) but some things are just mine and not everyone's business.


Friday TPL Shunt revision/Change
Sorry for the delay in updating here regarding Friday's (very!) early but seeming successful Thoracic Pleural Shunt to Thoracic Peritoneal Shunt revision/change. I am actually glad surgery was the first case and although I felt like I was (again, seems the theme of the week) in a daze all week it was good to be done early. I tried napping several times but everytime I would do so i'd end up with nausea, headaches (all intermittent) so taking a nap was never a success. I fell asleep about 10:30 tonight but the IV pump began beeping about 11:30; of course! I am sleeping fully upright so am hopeful I won't end up with  a super sore upper/mid-back in the morning. Dr.Bragg had said in pre-op this morning the Reps (or herself?) had the new Codman Hakim valve set at 70 so we had plenty of wiggle room to go up or she felt most likely go down in order to drain more fluid.
Surgery itself went well; haven't had a chance to talk to Dr.Bragg (another surgery; told Dad she would stop by this afternoon) but as planned the TPL shunt valve was changed from the Strata valve to the Codman Hakim (same as VP shunt) and distal end now re-routed via a 'Y' (?) connector back to the abdomen. A-line placement went ok, anesthesia went ok and PCA (pain) line connected.
I asked Dr.Bragg if she thought down the road it would be an potential option to re-replace/convert the current (changed for the cardiac valve surgery) TP shunt back to it's former TPL placement (thoracic pleural) as I had good results with this + stomach spasms subsided to virtually complete improvement (85% improved i'd say) after Dec's change. She said that this was a definite option and one she was not opposed to considering down the road if need be. (so thankful for her open mind and that she doesn't discredit my questions or concerns!!) The blood thinner (coumadin) will make future surgeries more tricky but not impossible so I am grateful she will con't to help down the road and after the CV surgery!
Dr.Bragg's Nurse and I where working on the Aldurazyme order earlier this week for Monday so I believe they have the correct dosing/mixing instructions from CHW (slight change from last in-pt) and I think the plan (which could have changed I imagine) was to order today (?) once I was on the official hospital census.
My Stomach/abdomen and side are somewhat sore (worse w movement) and thoracic (mid-back incision) area is much like a knife digging in/being twisted + do have  a on/off headache (we have room to turn down the setting, could partially seem worse due to being so tired) but other than being tired I am glad all went well and glad as usual to have provider's like Dr.Bragg who care/listen and are open to my thoughts.

I'll update again when I know anything; I had emailed Dr.Mitchell the Cardiothoracic Surgeon earlier re a CV surgery and the programmable shunts questions + he responded. I do have to get  alittle info from Dr.Bragg as he wasn't to familiar with the programmable shunt valves.
Thanks for stopping by,


Thursday, June 20, 2013

TPL Shunt revision 5:30/7:30 (Friday), Hand Surgery, Cardiothoracic Surgery/Cardiology Appt updates

This week I feel incredibly weary, tired, and although I probably seem a little down to some around me I actually am not, just very tired which brings out my venting side.

The shunt surgery is scheduled for 5:30 arrival and 7:30 OR time. The other Nurse that works with Dr.Bragg called today saying that Anesthesia was asking them for the specific Echo report and details so she was going to contact my Cardiologist's Secretary though they must have gotten this sorted out. Usually it is 1 of 3 or 4 Anesthesiologists who have my case so I imagine they just want to know if there was any changes though even Catie said "we have the Cardiology note which lists all the Echo details and history and aren't sure why that isn't enough".

The Cardiothoracic Surgeon Appt went ok this morning; I honestly was so tired I felt like I was in a daze (to many things on my mind and a long day of other appts/stuff after that appt so my I felt like I was trying to process the info and look interested while also wishing I was elsewhere! I think my brain has been going at a hundred and 50 decibals this week, running through everything I still wanted or needed to get done before tomorrow. I even said to the Surgeon that I just was tired so I honestly didn't have a lot of questions to which he said if I thought of any after today to just email or call and talk to him and ask. The basics are we definitely agreed on a mechanical valve (what my Cardiologist recommended to him and what other MPS drs have recommended to me). We'll for sure replace the Aortic valve; the mitral valve should be ok for now but they'll look at it while in replacing the aortic valve. He ordered an Echo for the day of pre-op as there is some specific measurement related to the valve size he is looking for and not listed on previous Echo's, they will also do a trans-esophageal (down the throat) Echo once asleep before they start the valve replacement to get a different, more direct view. Oddly he didn't mention the pre-op Echo but I received a mychart message so called his Secretary to verify if this was needed as I then had to reschedule something else the morning it is set for. The actual surgery they expect if no complications to take about 6-8 hours (including anesthesia) and he thought the hardest part may be equaling out Coumadin/INR levels post-op but also said this is usually easier (less med needed generally) in aortic valves than with mitral valves. He also mentioned they may be able to wean off anesthesia within a few hours of surgery but wouldn't do so till stable in the CICU. In-patient could  be as little as a week; I know my Cardiologist felt the post-op would be the hardest so I have to admit I hope it is that short but knowing w the shunt surgeries how long some in-patient, post-op stays have been and the heart being affected by so many more of the underlying MPS issues (restrictive lung dx, autonomic, cortisol-steroid and shunts) it will be  more of a surprise if it really is,  I guess we will find out soon enough. No matter I just hope it goes well and everyone (specialist wise) plays nice together as they have been; that the shunts are no issues during and that the Port (which Dr.Mitchell thought they would be able to work around) would be ok. He also said that a lot of times due to hypertrophy of the heart in severe cases of stenosis like my case with such chronic, low blood pressure/heartrate that post-surgery patients often will say their heart feels like it is going at warp speed but also have a ton more energy. It freaks just about everyone out but I am used to the low BP/heart rate by now (86/50 today) though will gladly take more energy post-op; maybe that will make the healing easier!?

I emailed back and forth with Dr.Bragg's Nurse and she is verifying the Aldurazyme dosing and fluid (amount) mix and although they where going to order prior to my surgery Friday they now plan secondary to hospital policy/patient census requirements order the Aldurazyme once I am admitted and in-house. We should still be able to get the med for Monday or if nothing else even Tuesday will do. I am happy regardless that Lisa is helpful and she + Sue (NP) are helping to coordinate getting this (extremely expensive) drug there so I won't miss while in-patient!

I met with my Insurance-Nurse Case Manager today which was nice; the other day we had agreed we should keep in touch once she leaves her CM position at DHP in a few weeks (going to be a Nurse discharge planner at one of the other bigger hospitals in Madison) but today she wrote down her cell # and told me I should definitely call her once in awhile, keep her up-to-date. We took a picture together and she had moved around last Fall (actually about 30 mins from where I live, a 1 hour+ commute to Madison) and mentioned if I was ever headed to the Dells to let her know she'd love to meet either of my nephews and thought they would really like her Farm. Neat! =) I will definitely miss having her on my team; like I said in the last post, we didn't always agree but she moved mountains to get things figured out for my care and especially for those providers of mine who where out-of-network, in-network exceptions! I am hopefully, cautiously optimistic that whoever she has take over my case will be as helpful/caring/useful as she was, I will definitely miss having her!
                                                       Peggy (CM) and I

The Hand Appt today was amusing; I still haven't talked to the actual Surgeon and after the PA looked at the incision she had the MA (Yes, the Medical Assistant!) remove the stitches which I could have smacked her; I truly try to give people a chance but in my opinion an MA has no business removing stitches (I have no problem if an PA, NP or Nurse does) and to boot she didn't once wash her hands OR wear exam gloves during! EEWW!! The OT and PA recommended 2 more weeks in the splint though I can take it off to shower (AWESOME!!!) as long as I am not doing anything with to direct of contact for the 1st week on that incision and then slowly transition out of the splint. Under the splint is a sleeve which should also help w incision sensitivity and they recommended wearing this if need be after the splint is done for a bit to help though I do have a scar-incision type dressing that just lays directly on the incision site to help scar tissue and can use small amounts of Vit. E oil/Cocoa Butter. The PA recommended following up (finally) with the Surgeon in 6 wks which would fall the week after the Cardiac Valve surgery so I mentioned this and she was ok with doing it the week before the CV surgery. So fast fwd to making the appt w the Secretary and I mention needing to make the fup surgery before the 7/24 surgery and she (Secretary) seemed like she was upset I couldn't do the only 2 dates the Surgeon was available the end of July? I just wanted to ask; what am I supposed to do reschedule the CV surgery for a Hand fup? Grr!! Anyways they couldn't apparently make anything work w Dr.Bliss so the fup is back w the PA? Odd, just very odd set-up! Like I said I have no problem who fups are with but I would have liked to talked to the Surgeon and find out his opinion of the surgery outcome atleast once!?

Say a prayer tomorrow (Friday) surgery goes well and no compications!
Anyone who reads this who happens to come in contact with me I apologize ahead of time if I am crabby; i'll try not to be but with little sleep and already exhausted...
Thanks for stopping by,

Tuesday, June 18, 2013

Insurance Nurse Case Manager, TPL Shunt surgery vent

I called my Insurance-Nurse Case Manager with a question about the on-going process Dean has been working onin  trying to set-up an in-house Pharmacy for managing and shipping Aldurazyme since our benefit year renews July 1st and so thus does the specialty Pharmacy contract. Dean is cont'ing to sort this out and is able to provide the drug through their own means in-house is just a matter of essentially how will they bill and reimburse themselves (I don't quite get it) but my CM said it basically comes down to if they (Dean) by and sell the drug themselves they have more control and while it will still be incredibly costly for them at least they would be keeping the cost in-house in a sense. (I was a little lost on this too!) - I did find out sadly that my CM (Peggy) is leaving to go to a different job, she had started at DHP a few months before my parent's employers switched from WEA Health Insur to DHP so we've really been on this past 3 yrs journey w Dean together. Her last day is in several weeks and she did say she'd we should exchange emails so hopefully we will do that from time to time, I think she just would like to keep up-to-date on what is going on medical wise with my life because she has been there through so much of it in the recent past. I am ok with that. =) We didn't always agree on every decision but she just about made mountains move and especially in the past year when our plan changed from a Point of Service (choice in providers) to an HMO (usually a small network of providers the insurer contracts with whom you can see) as she got exceptions in order for me to stay with many of the specialized specialists I see all of whom where outside of Dean's HMO network. She made the Medical Directors aware of my situation and diagnosis and why we needed the special exceptions to see the providers I do out of network and I never had a problem getting those approvals then or now. I am hoping that the CM she plans to ask to take over my care will care as much as Peggy did and will be invested in dealing with providers on my behalf when we need something I am unable to do on my own. She would call my specialists if necessary to get whatever paperwork complete or work with my PCP and I when necessary and just all around was very good at what she did; like I said we didn't always agree but she still cared!
I have been up to this point other than writing  a little about the shunt surgery here otherwise trying to just put it out of mind (out of sight, out of mind?) but really feel far from enthused about this; which I know your thinking "well how can you be enthused about any surgery?", right? The thing is, you really can't but you can look at the positives at least ie what positive impact will it have and get by with that. This time I am really not digging any of it, (as I think i've written previously) having to put the TPL shunt back in to the peritoneal space by the VP shunt sucks (how's that for sounding childish!) actually and especially so when we finally figured out places that worked for both shunts to drain separately and with the 1 Codman-VP valve I've been doing better so I really think w 2 Codman valves (1 in each shunt vs 1 Codman and the 1 current Strata valve) I would do really well. Sometimes I curse the never ending crap with this disorder; why can't we just have one resolution and that be it for a while? I don't mean to sound resentful or whiny which I know I do it just is hard sometimes to understand. I am still hopeful atleast that maybe there is some, small chance Dr.Bragg would put the thoracic shunt back in to the pleural space sometime down the road after the open heart surgery is stabilized and healed/sorted out.  I am also praying Friday's surgery will be a morning surgery as afternoon surgeries rank right up there as completely aweful (no food for so long of a period being the worst though admittedly sometimes I will chew on hard candy bc I feel incredibly crummy when I don't eat anything and then get very crabby/short tempered). Several of the D6-4 (neurosurgery) nurses are on fb and so I asked the night shift ones if they work Fri and only 1 of the 3 I really like (there are others I get along w well to) is not on vacation for the next week; go figure! I do hope both have good vacations though as although I wish they where working I also know they completely deserve the time away. I definitely wish we had been doing this surgery on a Tues or Weds vs a Friday though. =/ In fact I wish we didn't have to do it AT ALL!

I know I just updated yesterday so i'll stop whining and update after Wed's and Thurs's Hand appt (removing stitches, see if the splint can be taken off for good) and the Cardiology/Cardiothoracic Surgeon Appts (thurs).
Thanks for stopping by again,


Sunday, June 16, 2013


This has been a pretty chaotic past week although this next week promises to be just as chaotic just maybe slightly less so. Lots of appts (yuck, I really, really, REAALLLLYYYY  miss all or most all of my providers being in Milw., was SOOO much easier!) I feel like ALLL I am doing the past few weeks is drive, Appts, drive, appts, drive, appts, drive ,appts, drive. =/ Last week I threw teaching VBS in to that mix Tues-through Friday mornings with appts every afternoon either in Milw or in Madison and then Friday afternoon I drove to the Chicago area to stay with some friends. Last week was ERT (Milw) on Monday, a bunch of running around on Tues afternoon and getting a haircut in Columbus. Weds was a initial post-surgery Hand Appt at Dean-East (Madison), Thurs was Pain Mngmt Appt (Milw) and then VBS each of those mornings till 11:30-12 plus headed straight to Chicago Fri afternoon.

This week I should atleast be able to (hopefully) get a little more sleep. Monday I  have ERT in Milw again (we are changing the infusion rate, slowing it down), Tues is Dentist, also in Milw., Weds is back to the Hand Clinic at Dean-East in Madison (get the stitches out, hopefully get rid of the splint for good) and Thurs is the Appt w Cardiothoracic Surgeon (Dr.Mitchell) + my Cardiologist (Dr.Earing) to go over the Valve replacement surgery in July, followed by an Appt w my ERT-infusion nurse in IR (also at CHW) to get the Port re-accessed for Thurs's surgery. Then back to the Dentist Thurs afternoon. Friday is the TPL shunt revision surgery (replace the valve type and move the distal end of the shunt). Which on that note I did send Dr.Bragg an email tonight though it's possible I won't know till Fri just asking if she thinks down the road, post-Valve replacement surgery would we be able to place the TPL shunt back in to the Pleural space (I dread having both in the stomach and no-matter if the spasms recur (I pray they don't) having both shunts in the stomach is just not a comfortable thing maybe bc of the body structure of MPS pts?

Although I didn't think the Chicago MPS meeting was very informative (when will these meetings ever have something also geared towards individuals affected by the disorders?) I did enjoy going and especially spending time with friends and loved spending Friday/Sat w Jamie and her family! I actually didn't take many pics so don't really have any to post but the drive no-matter if gas prices are just about killing me (feels like it) was soo worth it! I also didn't think driving in Chicago was as bad as I thought it would be, I will definitely go back! The toll booths definitely kill me, like on an "really, is that really necessary?" level! I can't imagine families like my friend Jamie who have to drive back and forth to Chicago multiple times a week in IL and have to pay those tolls AND gas prices AND hospital parking costs! INSANE! I don't think to many hospitals actually think about what it costs families to begin w to bring their kids or themselves to the hospital not to mention adding in all those other costs!?!?!?! NUTS is what it IS! Although I am sorry for Jamie for the driving she will have to do I am super happy to say they are moving to the border of IL and WI soon!!!

I vented about this on fb but one thing I just kept thinking about on my drive home from Chicago/Zoo yesterday (3 1/2 hrs) was far to many drs won't give new drs a chance and don't seem to realize the only way we are going to get more drs who understand what MPS is and are themselves 'experts' in it is by referring pts to those drs who for instance have good bedside manner but little or no experience w MPS. We need to start slowly training these drs who don't have much or any experience to recognize the significant issues that occur w our disorders but which to many drs INCLUDING to many "expert" drs fail to either pick up on or don't believe are an issue and getting more and more drs who can see pts and help pts vs having 1 maybe 2 drs in a geographic area who are so-called "experts". (In my opinion there are NO experts). I think I am proof alone that giving new drs who are interested and want to learn but who have only a little knowledge or experience of MPS a chance   can get pts a lot further than sticking w the same old 'tired' group of old drs who won't change their mindset and won't think outside the box. My drs have done well w my care and NON of them are experts but they have been interested, curious and have wanted to learn!

Changing topics ever-so slightly; I do have a pretty great group of  'Core' Providers, they seem to care, seem interested and have each in their own ways learned a lot about my issues, the MPS and especially their particular areas they each treat... That said, what I've written below, which is basically me venting has NOTHING to do with them.. I am quite thankful for them!
 I have several providers who can't seem to fathom that 1. No, I don't actually want to see all these drs and have all these surgeries, it sucks actually to be perfectly honest. 2. It's like it is a mission to make it quite known they can't fathom how any patient can have so many health issues and thus providers and thus of course appts!? (umm, hello?! Have they ever saw other MPS pts? Read anything about it? These are not unique (to me) issues!) Why do some providers find it so necessary to question decisions we as patients make? Not related to their issues either but decisions that really have nothing to do with their area and for which they often have no business commenting? As if we the patient(s)  really already don't loathe the disorder and process enough and then providers who just "have" to give "THEIR" opinion have to question why we see so many provider's and "why" there are so many issues? Ummm, one suggestion?! A Search engine and type in 'MPS', grr!?!?

How can some providers just get it and others although they can be helpful they are clearly, completely clueless about the actual disorder? I am having issues  a provider and just frustrated so this is where this vent comes from. =/ I know it is sort of random, I  just was feeling really irritated and frustrated when I wrote it earlier last week.

On a very side note all of my out-of-network, in-network specialist exceptions (Neurosurgery, Cardiology, Cardiothoracic Surgeon, Neuro-Endocrine, Neuro-Opthalmology and a few others) where re-approved for another 1 year Insurance specialized in-network exemptions. Good news! =)

I'll try to update later in the week, maybe after Thurs's appt w the Cardiothoracic Surgeon and Cardiologist.
Thanks for stopping by,


Friday, June 7, 2013

Hand Surgery, Botox injections, VBS.

The Hand Surgery went  well Weds and not to painful. I am basically one-handed other than a little bit of hand use but was still able to drive myself to Madison to the botox and labs today. Thankfully the complete arm-regional block was pretty well worn off by this morning as that was interesting and proved difficult in trying to get dressed last night! At the hospital today a random doc in the Elevator asked if I tripped and hurt my arm and then  I also stopped to grab a bite and a lady asked what I did or was it carpal tunnel and did I do a lot of typing.. (lol) I explained a little of the storage issue and this being the 3rd surgery so I knew what to expect and personally I think it hasn't been as painful because to some extent there is a lot of permanent damage to the actual nerve.  I rarely go in to a lot of detail to people as most are only semi-interested and personally although I am happy to educate new people on a new (to them) disorder as chances are they've never heard of it but I also don't want to bore people and really I don't think everyone I don't know needs to know everything about me!  She asked was the surgery painful as  she had heard  I guess that this is one of the more painful surgeries. I kind of laughed saying this was the 3rd time and a piece of cake compared to some surgeries I've had, which is definitely true this time. Funny the things people in random public places will ask (about what the Port is, do I know I have some line under my skin or what is that tubing under my skin (shunt), etc? I sometime want to ask; "No I didn't know I had a line,  what line, OOh my gosh are you kidding I have no idea what it is!?!? LOL!
 We used a regional nerve block for the surgery which worked fine and I was able to be semi-propped up as they prepped for surgery and during by leaning back on pillows in the OR (the Anesthesiologist was great about that as well as no issues w giving the Solu-cortef! (Hallelujah!) While they used the standard Oxygen and did an EKG prior to going in to the OR due to the heart issues which where also fairly standard and normal. The regional block was placed while still in the "holding" area and done with ultrasound so also was not painful.  As far as the bandage that is about 3/4 length up the arm/to elbow and first OT appt is this coming Weds. I guess the bandage will be switched on Weds but stays on until the following week (2 wks from surgery) when the stitches come out and then a splint has to be worn for another 2 weeks after that. This apparently means I  will go in to the shunt surgery/revision with the surgery (R) hand still splinted; I had misread a mychart message about this a few wks ago and not that it's a huge deal just different than what I thought till now. I assume the length of this splinting is because of the hypothenar fat pad graft, done after the carpal tunnel re-release and synovectomy (to help protect the newly re-exposed nerve as so much damage has been done from such on-going severe CT recurrences multiple (3) times now and so many hand surgeries, including the Opponensplasty (ie the thenar tendon transfer 2+ yrs ago). I imagine this must take more time to heal than just plain "simpler" carpal tunnel surgery. Not entirely sure, since I can still drive I can deal with it. Showering is a DEFINITE pain though!

The botox today which I was also trying to get done before the 2 up-coming surgeries was done by a new dr doing one time injections for my Neurologist who has been out for a few months; he commented how un-affected I looked once he figured out I had MPS. Once he started the botox w EMG guidance in the nerves he was almost speechless as he said and was plainly obvious the muscles are extraordinarily over-active (spasticity) and  especially he said for how mild I look as far as how affected. He was quizzing me about my form being particularly rare (1:500,000) so I hope he now realized not everyone who has a lot of the affects has the more standards "looks"! The nerves due to muscle spasticity literally sounded just like a very loud, stormy thunderstorm (the crackle, thunder-ish loud crescendo's) in the 30 some injections he did across neck, thoracic spine (down to bra line) and across shoulder's. I will say he wasn't to bad at the injections, no numbing spray and only one of the 30-some injections made me suck in my breath and jump! I think I wrote in the last post that I may be switching in-time for the next set of Botox (every 3 mo) to a PMR dr my Neurosurgeon and her nurse recommended. Ive talked to the office and just couldn't make my schedule work with that drs in the next 1 1/2 months so they will keep me n their computer system and I will call and schedule a new-patient appt after the Heart surgery, hopefully in August if all goes well with the heart surgery. My Neurosurgeon and her Nurse really, highly recommended this PMR dr.

We start VBS next week; someone else is teaching Monday for me and then I have Tues-Fri with class running from 9-11:30,  2 1/2 hours though Friday we have a picnic after for the kids and really the other days it amounts to only about 30-mins that us teachers have for our actual lessons. The other 2hrs area short  opening service each day, short recess, craft/snack time and then music followed by closing back up in church. We alternate between the school and church throughout the morning and I will have an assistant + about 11 kids in addition to whoever isn't registered and comes the day of (mon.) and signs up.  It looks like I will only have 2 of my SS kids and so far about 8-9 other kids based off the roster from our planning meeting tonight. I think it should be ok, and I am looking fwd to seeing "our kids" again both those in my class (going in to 3rd-4th grade ) and our other kids who are now in the 5th-6th grade class for VBS and then for SS this Fall.

Earlier this week I had 2 upper, back teeth pulled which other than having to end up icing the one side that night (they had to cut it out and place stitches) the healing has been very fine. Otherwise I have a lot =./ of cavities to get filled, most small with one bigger. YUCK! That all has to be done before 7/24, the surgery date and in-between the shunt surgery, in-patient stay plus a lot of other stuff. Yippee.

Unless something changes I am planning to head to a friends who lives outside of Chicago next weekend and go to a MPS meeting at the Zoo plus then drive back either Sat night or early Sunday morning. Our kids sing in the late (10:30) service for VBS that sunday.
Will update again when there's anything new, all including labs is set for Dr.Bragg's shunt surgery and I meet w the Cardiac Surgeon and my Cardiologist the day before (20th) at CHW.
Thanks for stopping by,


Monday, June 3, 2013

Pre-op Appt, Provider's working together re: Shunt and Heart surgery.

I am really amazed though equally happy how my core providers (Cardiology, Neurosurgery, PCP, Neuro-Endocrine) have seemed to band together with this upcoming triad of surgeries (Hand, TPL shunt, Open Heart-valve replacement)  and come together in talking to each other via phone as well as exchanging clinic notes with each other. I am totally impressed! I wish it always worked this seemlessly and without much effort on my end but no-less I will take it right now!
Today, Dr. Bragg (neurosurgeon') nurse, Lisa called and asked/verified that my Endocrine dr was just sending them her clinic note and was not in fact the one doing the pre-operative physical notes/approval as she (Lisa) had received and was reading a "very detailed and long" report from Dr.Maas (they usually just get Dr.Maas's pre/post-surgical recommendations for the stress-dose cortisol dosing) and Lisa just commented that she would keep an eye out for my PCP's pre-op paperwork but appreciated the report from Dr.Maas. My Genetics Counselor stopped by ERT (infusion) today and commented she had received my Cardiologist's most recent note and had a few questions.
Additionally I have heard from Endocrine (Dr.Maas) since the appt with her last week asking to be kept up-to-date on the specific dates of all the surgeries so she can make sure her notes/recommendations get to each of the appropriate provider's before each of the surgeries. Cardiology and Dr.Bragg (Neurosurgery) spoke 2 weeks ago within a day or two after I saw Dr.Earing (Cardiology) and are both on the same page as far as what needs to happen with the TPL shunt (moved out of the pleural space and back to the abdomen by the VP shunt distal end) and the time-line between Dr.Bragg's shunt surgery and when she will release me for the Cardiac-valve surgery. She will release me a month (4 weeks) after the shunt surgery and  usually she sees pts a week after surgery or a week after being released from the hospital and then a month after the 1 week fup.
I probably wrote in one of the other posts the actual OHS-valve surgery is just over a month after the shunt valve surgery (June 21 and July 24) with the pre-op Appt at FMLH (cardiothoracic surgery dept) is 1 1/2 weeks in to July (10th).
I think the last provider that needs to be involved is my Pain Management dr., to come up with a post-op in-patient and out-patient pain plan as it will have to factor in what I currently take and whatever they opt to use there, usually in-addition. She is aware of the surgery though and knows Dr.Earing, my Cardiologist so shouldn't be to hard and as well I see her atleast once and likely twice (once montly appts due to the way controlled substance medications are prescribed/monitored) before that OHS surgery. She has worked with Dr.Bragg on the shunt surgeries so that isn't to big of a deal.

Friday I had the pre-op Appt with my PCP for this Wed's Hand surgery as well as for Dr.Bragg's shunt surgery in several weeks. I have to do labs sometime this week (likely when at Dean for the botox) but otherwise the appt was fine. Her only recommendations where 1. they not do general anesthesia (per my Cardiologist) which we knew and which she confirmed with my Surgeon. 2. Limit the amount of fluids during surgery (also per Cardiology) and 3. She suggested taking the Lasix, bisoprolol and Spironolactone the morning of surgery (in the past we have skipped those doses and the bisoprolol is usually a night medication). She felt doing these ahead of the surgery would help pre-empt the heart failure symptoms I had with the last in-patient surgery and that I would feel better overall from a breathing stand-point since they do have to give some fluids during the surgery, regardless if it is just a regional nerve block. I will just make sure and take the meds several hours before I leave for surgery regardless what time it is so I am not peeing every 15mins on the way there and in pre-op!

Will update after the surgery, otherwise the only other update really is that the Pastor heading up VBS (also ran Sunday School) just called and all of us teachers are meeting Thurs night for the pre-VBS meeting to go over next weeks lesson's and acitivities. I am nervous about that and especially since someone else is teaching for me Monday but I also think it should be kind of fun. :)
Thanks for stopping by,