Wednesday, May 29, 2013

Shunt surgery date, Valve replacement(s) surgery date set as well.

I talked to Dr.Bragg's Secretary today and as well afterward talked to the Cardiothoracic Surgeon's Secretary and here are the pile of dates we have set. I totally had thought the heart valve surgery would be in early Fall but she (Laurie)  asked me to ask Anna/Dr.Bragg this morning when I first talked to her (the Cardio-Surgeon's Secretary) to see how long after the shunt surgery Dr.Bragg would release back to Cardiology. It turns out Dr.Bragg will release me a month after her surgery (was my guess, obviously had to many surgeries with her!) and the heart-valve surgery is scheduled for Weds, July 24th at 7:30 with pre-op Xrays, EKG, etc the day before (23rd). I also meet with the Surgeon and Dr.Earing June 20th to go over the plan for this surgery.

Dr.Bragg's shunt surgery to move the TPL shunt from the pleural space and to place the Codman, programmable valve is then set for Friday, June 21st. They give the time of surgery the day before when the nurses/anesthesia call. Below are all the up-coming surgery dates as well as pre-op appts. While Dr.Mitchell and Dr.Earing are at CHW and see pts in clinic there + surgery itself is at FMLH (much like Dr.Bragg sees her pts at AFCH but does Adult pt surgeries in the connected Adult hospital). I've had many other surgeries at FMLH in the past so it should be ok, anesthesia typically has gone far better at UW but I also know Dr.Earing will be on this and with my Neuro-Endocrine dr at FMLH she can guide the use of the steroids/cortisol replacement pre/post surgery which will also be good.

Surgery date(s)
May 31, Pre-Op Appt, PCP, Dean
June 5th, Hand Surgery - Dean Medical Center
6/20, Meet with Cardiothoracic Surgeon (Dr.Mitchell), and Cardiologist - CHW
6/21, Dr.Bragg shunt revision to move TPL shunt distal end to abdomen, replace valve
7/23, Pre-Op Appt, CHW (Xray/Imaging, EKG, Anesthesia, etc)
7/24, OHS-valve replacement(s) surgery, 7:30-FMLH (Dr.Mitchell, Dr.Earing)

In an email this morning my Cardiologist mentioned they are trying to plan a ACHD-patient education lecture series and thought I might be interested in helping once recovered from the valve replacement-open heart surgery + suggested I should consider getting involved. I think it sounds kind of neat! I am typically always interested in programs to help other patients or providers better understand whichever issues it is we're working on, in this case Adult Congenital Heart issues.

The thoracic valve is being as aggravating as ever today, and especially bad tonight; I was talking to my Pain Mngmt dr via texting and she asked me to please call Dr.Bragg and let her know + suggested I call the on-call staff tonight but I will wait until tomorrow likely. YUCK! The worst part is definitely how irritated my neck/shoulders and mid-back are, right now secondary to the valve area discomfort. I am leaning back against pillows but still terribly uncomfortable and un-pleasant. I HATE THIS!

On a side note I am driving down to stay by a friend (MPS family) who lives in a suburb outside of Chicago the wknd of Father's Day,in June,  there is an MPS meeting we are planning to go to together. Her son has  a shunt and dx'd with MPS II + a cutie, always reminds me a lot of Zan and similar in age as well.

I'll try to update if there is anything new, the Secretary for Cardio-Surgeon's ofc had left a VM late this afternoon that I missed asking me to call her back so I will do that in the morning before or after a dental appt I have in the morning.
Thanks for stopping by,


Tuesday, May 28, 2013

Heart Care, the Specialists involved; Insurance, Hand surgery plan..

These are the days I would trade and be somebody else; as my Infusion Nurse said and I often say as well after she commented "I don't know how you do it", she then commented and I agree "One day at a time, right!?" Between stomach spasms (very odd, haven't had those since before the shunt infection was treated last Dec.) and the shunts replaced/moved. I think the TPL shunt valve sitting in my thoracic spine has shifted or something as every time I move or sit back I can now feel the valve for the past 5 or so days and is especially annoying. Probably my 2 biggest dislikes in life are the thought of anything to do w the ribs (and the valve seems to be rubbing against these) as well the other is anything to do w the nose. No-less this has been especially uncomfortable and especially sitting back is super sensitive; more often than not the past few days I have sat semi-sideways which in turn puts excess pressure on the upper spine/neck I guess bc I don't have full-normal movement in my neck.
At the Pain Mngmt Appt the other day, something she usually does per her exam but my PM dr wanted to look at my back to make sure all newer and previous (older) shunt/spine incisions looked "ok". She commented that there cont's to be some swelling/fluid at the former lumbar shunt (last opened up, shunt removed in Dec.) and was concerned about that; I've noticed the CSF pocket but isn't big enough to cause a problem, if anything it could be bigger! =) The valve is higher up in the thoracic area of the spine + definitely is loose/moves around or something. I think Dr. Bragg had put a pin or something in to hold it in place when she changed the valve back in winter and told me to be careful how I moved/lifted (or it was something along those lines she had advised) as she said it could come loose. I wonder now if it has? Not painful but is odd and kind of un-comfortable and can definitely feel the valve seeming to move + not a comfortable feeling at all! Definitely hope it improves as I believe Dr. Bragg is out next week and although I don't know what date they are looking at for the shunt revision yet, (another fun matter bc then I am still waiting to give other people answers on things I've been asked if I would do)  I am guessing, hopefully the shunt revision will be mid-June that way I can do the things I have planned and not have to move to much around? Hopefully I would know an actual date tomorrow (Weds)? Dr.Bragg had said she would be out for a minor procedure/surgery I believe next week so I realllyyy need this valve to settle down and not be an issue not to  mention the Hand surgery is next week and VBS the following. Grr to MPS and especially right now!

I think I posted this last week but the Cardiothoracic Surgeon's Secretary at CHW called last Thurs wanting to schedule those appts (not sure if she also was scheduling surgery or that will come at the initial mtng), I know Dr.Earing wants to get this done soon, after Dr.Bragg releases me on her end from the shunt surgery recovery.  The hospital that Dr.Earing (Cardiologist) and the  Cardiothoracic Surgeon are at is a Children's Hospital with a pretty large team of Specialists (3 Board Certified/trained ACHD drs) and the largest ACHD patient program in WI. The Children's Hospital is ranked  #3 Nationally and is ranked #7 for Congenital Heart care/surgery in the Nation. I don't know much about the Surgeon or for that matter yet how long they expect the valve replacement(s) surgery to last or even what they tentatively think in-patient post-op length might be. I've heard as short as a week from a couple Adults and as long as 3 months, all where MPS I Adults. Seems pretty variable, I guess?... the shorter end would be nice and for all to go smoothly would be great! I was initially for a long time freaakkkkeeeddd out about this surgery but have come to the conclusion from talking to other Adults both with and without MPS that the surgery itself and recovery may not be to different than a spine surgery and the first few weeks the hardest. A few patients where even back to driving after a few weeks, I pray that is me!   Dr.Earing as I've written thinks  that post-op is going to be the most difficult (because of the restrictive lung issues, hydrocephalus/shunts, Endocrine-steroid issues and all the other potentially complicating factors that will likely affect this surgery) but maybe i'll sail through relatively easily? . Who knows, but I can hope!
Surgery will be scheduled so Dr. Earing  (my Cardiologist) can be in the OR w the Surgeon (Dr. Mitchell) and so that Dr. Earing (per his wishes) can follow my care alongside the Surgeon "to make sure everything is being done and monitored appropriately due to your underlying issues". I have to call the Secretary for Dr.Mitchell back as soon as I know when Dr.Bragg's surgery is planned for so they can schedule appts w Dr.Mitchell/Dr.Earing and plan the actual surgery time.

My primary Insurance is an HMO but Dr. Earing (Cardiologist) is an out-of-network specialist w in-network approval (as is Dr.Bragg, Neurosurgery (UW), Dr. Mass in Endocrine (FMLH) and a couple other of the specialists.) The reason primarily these drs where approved as 'out-of-network, in-network' exceptions is Dean's Specialists reviewed my records and told my Insurance-Nurse Case Manager my care and issues where to complex and needed to con't to be followed by the sub-specialists when we switched from the Point-of-Service plan to the HMO plan and they did not feel comfortable seeing/following me. Good for me atleast! :)

On a completely side note this is the 3rd time a different provider has asked me about referring a patient of theirs to Dr.Bragg, previously my Pain Mngmt dr planned to/is referring a shunt patient of hers, and my Cardiologist's Nurse just last week also asked me about sending a patient of theirs with complicated shunt issues to her. Today it was my Neuro-Endocrine dr talking about  a patient in a similar situation as I was prior to finding Dr.Bragg, where the original Neurosurgeon put in the shunt, revised it a time or two and then refused to see the patient any longer. Since my Endocrine dr works w the Neurosurgeon's at FMLH she often sees the patients that have secondary endocrine issues as a result of shunt/hydrocephalus (or in this case Pseudotumor Cerebri) and was a huge advocate on my behalf that I was having shunt issues when my former Neurosurgeon and some of my other drs didn't really believe it (due mostly to ventricles). All 3 providers  comment how impressed they are with Dr.Bragg's persistence, willingness to learn more about MPS and willingness to keep helping in addition her willingness to have taken on my case when essentially she was then dealing with "someone else's work". All have raved about her  although thinking about it off-hand I think only 2 of the 3 have spoken with her one or more times and Dr.Maas I think might have? I can't remember back in the beginning especially..  I think they are just impressed that she took on my case, has stuck with me and is so willing to "think outside the box" and "talk to other provider's if she has questions". I  know how good she is and has been with my care and her willingness to answer my never-ending (i'm sure) questions in addition to her patience with an pretty complicated situation! I am glad others of my provider's recognize how good of a provider she is. In all actuality I am pretty impressed with how many of my "core" drs now share patients with each other; Pain Mngmt shares multiple of my Cardiologists patients due to referrals from him and likewise from her to him after I had such good luck with each provider and they cont'd to see this through the many up's and down's. I think also she (Pain Mngmt)  saw his diligence in following my care and piecing out that the issues aren't just cardiac or lung but he looks at the whole picture (which is something all 4 of these core drs are great about, looking at the big picture in the context of their specialty/issue) when it comes to the MPS dx which not to many drs take in to account  a patients entire health picture vs just focusing on what they are trained in.  My Endocrine dr shares multiple pts with my Cardiologist and with Dr.Bratanow (Pain Mngmt). Now they are starting to refer patients to Madison to Dr.Bragg,  I think bc I've had such good luck with her. I think all 4 are great providers so I am glad other patients get to have them and benefit from their experience/caring/knowledge!

Otherwise the Pre-Op Appt is this Friday with my Primary (Internal Med) dr and the plan remains to do the Hand surgery with just a regional nerve block. The actual surgery will consist of a 3rd and more in-depth (hard to believe they can get anymore detailed than last time) carpal tunnel release, tenosynovectomy (cleaning out the tendons in the hand I guess) and the hyopthenar fat pad transfer. Initial follow up is a week after surgery where the bandages are changed and then the post-op fup is 2 weeks post-surgery. I am not entirely sure actually how long the dressing/splint stay on with this surgery? Never actually thought to ask.
I also have to call and schedule botox injections, I haven't done these since late last fall and my regular dr who did it then is out I think until August yet but my appeal to see a PMR dr that Dr.Bragg and her Nurse recommended cont's to be denied. My PCP called and spoke to the Med Director at Dean but their denial was based on "an in-network provider can do these" which is true but not the whole picture. I can always use my secondary insurance to see this other provider or I can also appeal w Dr.Bragg/her nurse's help (Lisa has offered to help if I want her to in the past with this) to Dean as a patient. I am not entirely sure what I am going to do yet but I did give my Dad the records to have faxed by the Secretary at his work to this other (out of network) PMR dr.  I can and probably will appeal the denial but in the mean time think I will have the botox done by one of Dr.Mangin's (my current dr, a Neurologist doing the botox) partners as the neck/shoulder and back muscles are crazy annoyed despite on-going PT.

Thurs I also see the dentist again for a cleaning and I guess molds as 2 back teeth that where cracked w separate anesthesia intubations  are going to be pulled by the Dental School's Oral Surgery Dept.. Yippee.

Never actually ends, all this "fun, fun, fun"!
Will update when I know a shunt revision surgery date and appt dates w the Cardiothoracic Surgeon.
Thanks for stopping by,


Friday, May 24, 2013

#27 Shunt revision, OHS-valve replacement schedule, VBS.

Dr. Bragg emailed regarding  having  talked to Dr. Earing and having talked to the Codman Rep. The Rep said they have a specific setup from what I understand for the TPL shunt but Dr. Bragg wants to stick w what we have and place the same (Hakim) programmable valve we have in the VP shunt into the  TPL shunt + then move the distal (end) of the shunt from the Pleural space back to the stomach (this part completely sucks). I don't completely understand what she meant about now wanting to change out the whole system since quite a bit of it, it would seem will be changed w moving it back to the stomach/abdomen and putting in a new valve but at the same time she knows what she is doing and that's pretty much what matters!
The reason for moving the distal (end) of the catheter back to the stomach is Dr. Earing feels and Dr. Bragg said she agrees with + worries when they place bilateral chest tubes these will mess w the shunt set-up. She was going to talk to her Secretary about what date works best for her schedule for this surgery and wanted to do the TPL valve change (from Medtronic-Strata to Codman-Hakim) and distal catheter swap in the same surgery. I talked to her Secretary and asked if based off both our schedules we could look at the 3rd week of June as she is out the 1st week and she thought then I could finally get the Hand surgery done,  that next week Pastor Hirsch had asked if I would consider teaching VBS to which I finally told him I would after waiting all week to try and get an idea when this shunt surgery was going to be. .

I also got a call this morning (fri) from the Heart Surgeons Secretary just letting me know my Cardiologist had spoken w Dr. Mitchell and  wanted to give me her direct # plus asked me to call her next week to schedule once I know when the shunt surgery is. They want to schedule a appt to meet w/Dr. Mitchell and Dr. Earing as well as go ahead and schedule the  valve surgery as soon as I find out when the surgery w Dr. Bragg will be (to revise the valve on the TPL shunt and move it back to the abdomen). Dr.Earing wants to be in the OR apparently during the actual surgery and wanted it done during a time when he is on-service so he could also monitor my care.  This to will take a bit of coordination on their parts but on the flip side of that bc summer is their busy time maybe the actual valve replacement surgery won't take place till Fall?
She made the comment that they would make sure and coordinate this heart surgery for a period after Dr. Bragg gives the ok/clear to proceed post-shunt revision.  I am looking fwd to being able to breathe better all of the time and probably sleep better after this but like I've said before this surgery is not high on my list of things I really, really want to do! Not to mention having to be back relying on other people for however long is crappy!

Next Tues is an Endocrine Appt at FMLH  and after I have ERT at CHW which is next door (usually Monday's but bc of the holiday) + then on Friday is the pre-op Appt for the Hand surgery. That is supposed to take only about an hour to 1 1/2 hrs and hopefully not any longer since we are only doing a regional nerve block and I will be fully awake. The good of that bad (having to be awake) is although it doesn't actually un-nerve me to be awake I still would rather be in lala land but the Surgeon had said I could be sitting semi-upright during.

Since I don't when the shunt surgery is going to be and is what I was basically waiting on to tell Pastor y/n if I would teach VBS, I just went ahead and emailed him yesterday and said I would teach 4 of the 5 mornings (2 1/2 hrs). I'll  have to move around appts I have to afternoon (I think just a hand surgery f/up w/OT which should be flexible since they have multiple locations and OT's.) and anything else was already in the afternoon.  I am unsure if there will be 2 of us teachers or how many kids we have (we have a meeting before it starts to get our class materials)  but I know at least several of my Sunday school kids will be there which is nice, they where a great group. :)

That is about it, will update next week if or when I know something,
Thanks for stopping by,


Tuesday, May 21, 2013

Cardiology Appt, Valve replacements being recommended.

It wasn't even a week ago Dr.Bragg asked me  (again) when I thought Dr.Earing was going to green-light going ahead and doing the valve replacements (she is concerned I think bc although her surgeries will be more complicated from a blood thinner stand-pt post-valve replacement now the anesthesia part is so much more difficult and really time intensive.) I think to she is curious if that will have any impact on the headache symptoms?  Regardless today Dr.Earing walked in and said he thought it was time and no medication adjustments or additions we did where going to make a big enough difference and he felt we needed to do this. I guess on a thankful note he must also have figured out there really isn't anyone in the MPS community w much experience in Cardiac-valve surgery and so he was recommending a Cardio-Thoracic surgeon at his hospital (a Mike Mitchell) who he feels has experience w complex heart surgeries/complex underlying, complicating issues and is who he plans to refer me to. He did say that he had a few concerns the biggest being the TPL shunt will have to be moved as they use the same area during open heart surgery for tubes/wires/monitors (unsure what exactly) and so 1. he had already tried callling Dr.Bragg when I went down for a chest Xray after the appt (his nurse came down to Radiology to ask me something) and 2. He was a little concerned about the shunts and if Dr.Bragg when he talks to her felt like she would need to be present during surgery or immediately after he would reluctantly refer me to the Cardio-thoracic surgery dept at UW so I was where she would be available. He would really like it to be at CHW though and if Dr.Bragg doesn't feel she needs to be there during surgery/doesn't feel there will be an issue w the shunts then he would like the surgery sheduled during one of his on-service calls so he is around and can make sure everything is being watched/monitored and taken care of (he's pretty particular but it's also what makes me really appreciate and like him as my Cardiologist + trust his judgement). I am wondering since we apparently do have to move the TPL shunt for the valve surgery if once I am healed from the open heart surgery and if I had the same stomach symptoms recur as before we moved the TPL shunt from the peritoneal area would we be able to replace the catheter back in-to the pleural space down the road? (I don't know, it is also possible as I think I may have wrote about last week that the stomach symptoms where more from the CNS infections and less from the actual shunts/CSF absorption). Then again, who knows maybe I would do well with both shunts in the stomach area again. Can't help but wonder. :)

He wasn't sure if the Port (for IV access) would be ok during surgery but just said it depended and bc they would have to place so many wires/monitors and lines during that it might have to be removed but would more or less likely be an un-known until they where doing surgery.
He is keeping the meds I am on the same (lasix 40mgs, 2x's per day, Bisoprolol (a beta blocker) 1x per day and Aldactone 1x per day till surgery. He also green-lighted the Hand surgery as long as we do it with only a local nerve block and no general anesthesia. He did add that if we had to do any absolutely necessary surgeries (shunt for instance) then he wasn't opposed to General Anesthesia, just that careful monitoring and fluid mngmt be done which they have been doing since Jan at UW anyway w the Arterial line and careful fluids, etc. His big concern is any surgeries causing congestive heart failure due to fluid overload.

He made the comment this time which kind of surprised me (given past comments) that he didn't think surgery would be the really hard part he thinks the immediate post-op recovery will be the very difficult part. I guess because of the restrictive lung issues, shunts, blood thinner initiation, etc. Fun.. (not really) I am sure it will all be a barrel of monkey's and a load of fun. (sarcasm)

A surgery date will really depend on the shunt stuff so a little variable, he will call me after he talks to Dr.Bragg and I am unsure (I didn't think to ask) if there are any other tests that will have to be done, in the past he had mentioned repeating coronary artery block testing (a pretty simple test w imaging) but he may not do this either, really not to sure. In the mean time I have the quick-brain MRI for her tomorrow because she was thinking about changing out the TPL shunt to a different valve (see last post, no information since last Weds but maybe, hopefully know something tomorrow when the NP resets both shunt valves, am hoping Dr.Bragg might be around I could talk to or the NP knows something. Either way one of my Pastor's at church had asked earlier this week if i'd be interested in teaching VBS the 2nd week of June to which I said I couldn't do monday (bc of ERT) but maybe could move around other things I have already scheduled the other days and would let him know. He was willing to find someone else to tag-team and teach for me on monday if I can do it. I said i'd let him know by Weds afternoon if I could so am hoping to know something from Dr.Bragg tomorrow, I would actually really like to teach this as I would have some of the same kids from Sunday School and is just a week-long 2 1/2 hrs each day. I think the only other appts I have right now that week are post-op Hand fups which can be rescheduled to afternoons I think. I asked Dr.Earing when he thought (a guesstimate) the heart surgery might happen ie fall, winter, etc and he said it really depended how I felt but he was leaning towards sooner. I already commited to teaching SS next fall although w the same teacher from last year and hoping to be able to at most only be out a month or two and maybe if we need to can have a substitute teacher if my co-teacher isn't able to do it the weeks I may be out. Not a big fan of not being able to commit and especially because of health stuff!

I'll update when I know something more,
Thanks for stopping by,


Wednesday, May 15, 2013

May 15th, Neurosurgery Appt, up-coming.

Today, May 15th, is National MPS Awareness Day...
Fittingly I guess for the Awareness Day I spent most of this morning and part of this afternoon in Provider offices; first in Madison seeing Dr.Bragg and then this afternoon at PT. I am dead-tired!

Even though I trust her very much and know in reality she will be there to help me I still get nervous everytime I see Dr.Bragg, I get butterflies calling her Nurse or Secretary though both recognize my voice (yikes!) when I call and both are  nice/good at what they do. Why, do I get nervous then? Because I HATE asking people for help, hate that there is on-going things I need their help with and I wish I could work out problems myself! It doesn't matter that i've done this over and over and over with dozens of drs in the past 8-10 years i never get less nervous calling.  As much as I have to ask other people for help, I sure hate it no matter if it's providers, my parents, ANYBODY!

Anyways so Dr.Bragg and I talked about the future after the heart valves are replaced and what impact did she think this would have on my shunts or future revisions? I was nervous about this. I do realize people still have some surgeries while on blood thinners but I also talk to many-a MPS Adult who have had drs tell them they wouldn't operate solely because of their being on Coumadin/blood thinners. Thankfully Dr.Bragg just said that when we are faced with this issue we will formulate a plan and most likely would be (if the Coumadin couldn't be stopped ahead of time) admitting me the day before surgery, starting IV heparin and running this throughout the surgery then post-surgery she/they would start me back on the Oral med once INR levels where stabilized usually taking she said a few days. I get the feeling she is a little concerned they are cont'ing to post-pone the surgery, though i've gotten that from other MPS Adults to who keep saying "you want to make sure you do it before there is permanent damage." I also had an friend/fellow MPS Adult email me tonight saying she definitely thought the decompression surgery was worse than the valve replacement surgery so that as odd as it sounds was good to hear! Since i've had numerous spine surgeries then maybe the open heart surgery won't be as bad as I think!? Dr.Bragg did also say that since we typically end up taking a few days to let pain settle down and get back to the normal pain med regimen it shouldn't add to much addt'l time to any future revisions.

Her suggestion she said she had been thinking about and needed to confirm with the Codman Rep that we could do now for the headaches was take out the Strata (5 setting) valve in my TPL (thoracic-pleural) shunt and if she is given the "it's ok" put in one of the Codman valves either the 'Hakim' which I currently have in my VP shunt as well or there is a semi-newer (Certas) valve made by Codman that is resistant to being changed by MRI (something we do frequently for the shunts and  for back imaging) but also has many more options for settings (18 or 20, not sure which) like the Hakim valve. In the case of the Certas I would then I think be able to have MRIs done at other hospitals (UofMn study for instance) without 1. having that hospitals Neurosurgeon (in the case of UofMn) to reset the valves or 2. be tied to only having MRIs at her hospital, say if another provider wanted one done for a different reason.
She mentioned putting an anti-siphon device in the VP shunt but felt like this would essentially be the same as our having previously had 2 valves on the one shunt and wouldn't help symptoms.
And one other thing she talked about was putting the TPL shunt back to draining in the stomach but she said bc we never really did confirm if it was an absorption issue that caused the stomachaches (I actually think those where directly tied to the shunt infections as I only got those symptoms when I also was either being treated for the CNS infection or in the case of last summer/fall a un-treated, not yet found CNS infection. The stomach symptoms cleared up w both infections as soon as we treated the infection, took out the infected shunts and replaced with clean, non-infected shunt hardware again. I don't think she is leaning towards moving the TPL distal end back to the stomach regardless, more or less I think was just musing outloud (a trait I fully appreciate, I like knowing what her thoughts are, even the ones we don't use.)
As far as when we wll do the shunt revision surgery I am not entirely sure but I think due to Dr.Bragg being out the 1st whole week of June and her thought being if I wanted i could finally, still go ahead adn get the Hand surgery done on the R side (if Cardiology approves) and would also be able to keep the other appts like Cardiology, etc next week and what I have also the following week. I am unsure when I will find out when they want to exactly schedule the surgery for but at the latest I imagine at next Weds's shunt reset appt they should have more info if not before.

I had originally changed a Pain Mngmt fup appt from tomorrow to last week and then ended up re-moving it back to this week (thurs) but got a call from Dr.B's Secretary today that she needed to move it to next week. I am just glad she atleast called this time! Unfortunately though this means an appt nearly every day next week with; ERT (infusion) on monday, in Milw (CHW), Cardiology (1 month) fup on Tues also at CHW, Quick-brain MRI and Appt to have both shunts reset right after (10:45/11:30 in Madison (AFCH/UW) and Pain Mngmt also in Milw now on Thurs. YUCK!  I am looking for a new laptop (typing on my netbook) so may still go to Milw tomorrow to Best Buy or other computer type stores but sort of depends on how much energy I have.

On a completely different note we have our last Sunday School class this Sunday till Fall (my co-teacher and I have both agreed to teach again next year, 3rd/4th grades) but that doesn't start until our 1st mtng in July and class again till (I believe) August. Very hard to believe the year is over, it was incredibly eye-opening to me though both in that i'd never want to be an actual teacher (our kids where great, some we will have again next year which is really nice) and 2. I definitely felt like it got easier as the year went on and I developed more of a rhythm or set-up to our Lessons and activities the weeks I taught (every other). Of course knowing the valve replacement is coming down the pike I am not entirely sure how that will impact SS, it may be that Joan teaches those wks I am gone, much the same as this year with the numerous and long shunt in-pt stays or it may be that I try to get a sub from the pool we have to teach while I am out. Joan and I have talked about it some and to some extent will just depend on when Dr.Earing (Cardiologist) thinks is best to do the surgery. He has both been leaning towards doing it sooner (I think i've written about this in past entries) or it may be that we re-evaluate in 3-6months depending how the symptoms are and meds are going. On that issue I should know more next week.

I'll likely update again sometime next week,
Thanks for stopping by, and if you wore anything purple today THANK YOU!


Monday, May 13, 2013

Day-to-day with MPS; Odds and ends.

I talked to my GC today while at ERT (via email ironically though in the same office) and we are going to slow the infusion to either 4 hrs for the drug itself to infuse (is just under 4 hrs lately due to reduction about a year ago in the overall fluid/saline volume) or a bit slower. Because I seem to be retaining fluid on and off (up and down weight gain by as much as 5-7 pds, from 112pds to 124pds (I realize that is more than 5-7) so our increased/extra vial(s) are now the exact dose, usually, not extra. I see Cardiology next week so we'll see what he suggests as far as the seeming fluid retention, it usually isn't extremely noticeable just semi-puffy legs by days end.  Breathing has been somewhat improved, yay! I think the Aldactone and then the doubled lasix helps.

See the post later in this update, written from last week but I see Dr.Bragg on Weds for the headaches and just to make a plan regarding symptoms now vs whenever we do the valve replacement(s) and associated blood thinner be it soon or con't to post-pone for the very reason of blood thinners will complicate all future surgeries after  the cardiac surgery. I talk to some of the (few) MPS adults who take blood thinners post their own valve replacements and how many surgeries they need but are never done now bc their drs are (I guess) afraid of dealing w these and surgery especially more elective surgeries. In one case I don't think the surgery (in my opinion) is particularly elective but that is totally a patient and their providers choice and what symptoms one can or will live with. Not mine  to give a say on, though I sure do care and totally know how the person feels. Ugh at MPS!

Otherwise have been going back and forth w UofMn trying to schedule the study dates, bc the neurosurgeon has to re-program the shunts after the fMRI that is done per this study and i've had so much going on we've had a few issues finding dates that work for the study researchers, myself and the neurosurgeon! Shooting for mid-June this time. We'll see if it works for all of us! :)

Ever since I was finally diagnosed 8-9 years ago i've really tried to be an open book on many aspects of my life with MPS, especially bc I really wished when I had been diagnosed I had, had someone whose issues I could have read about and (in some ways) compared myself too. I guess i've wanted to be/hoped to be a source for new or  really, even older (and I get lots of those messages/emails!) MPS families or adults and then after I started dealing w the shunts and their on-going frustrations I had wished someone out there would write about their experiences with shunts, again didn't really find anything. I never found "that somebody" but here again have gotten so many messages from people saying how grateful they where for the incredibly detailed info regarding stuff my drs told me, my experiences with hydro, surgeries, scars, you name it I tried to really share it. I am actuallly still amazed how many people also seemed to really want the nitty gritty details about both MPS and especially about shunts both in and out of the MPS community. It has always been somewhat cathartic for me to write about my experiences (easier than talking about it, which I don't do often w anyone) and then the information is there for others to see "Wow, this really is normal" or "someone else gets it" ( get that alot).   So why do I write the above? Because I REALLY wish some other MPS adult would write about their experiences, I really like information, it is how I am able to deal with this crappy disorder and it also helps me to learn, too. I would give just about anything if someone else would write what their heart surgery was like or their shunt surgery (I don't know  if there are any other adults my age or older who are shunted, though some I suspect should be tested) or even simpler things like how they deal w insurance, drs, life and finding a balance? Anyone!?!? :)

ps: I don't worry to much about finding a balance bc I can't leave MPS behind, it follows me and is just a part of me so no-sense in trying to pretend it isn't me and I am not it... But still I do wonder how others find their own form of "balance"? For me it is just cont'ing to do the things I like to do around all the things MPS thinks I should do..

See below, thanks for stopping by,


*This was written mid-to-late last week.

I've written a couple other articles and blog posts this week so haven't been here writing since last week. I had asked the RN (Stefanie) at NORD if they would write anything for their NORD blog regarding MPS Awareness day and she in turn asked me if I would like to submit something. I wrote that article monday while at ERT (fitting) and then also had an article to write for the MPS Society 'Courage' newsletter regarding my trip to Boston so also wrote that partially sunday night then finished it while at ERT (infusion). I am waiting to see if either needs corrections (likely) for space purposes or just general editing. I was super amped to write the one for NORD especially so I hope it turned out ok! I will share the link or article here next week and on the fb page for the NORD blog submission. **The NORD article is being edited for space and so will be run in the next coming week or so. I may post an "un-edited" version here sometime this week. Also I encourage everyone to post regarding May 15th, Awareness day to the NORD fb and twitter (if you use that medium) pages!

I also am writing a submission for the Global Genes Project blog as a  'Guest Blogger' for MPS Awareness Day (May 15th, wear your purple!) but have to start that yet. ***I actually never got to writing this yet as the past week has been pretty hectic and tiring so I will do that sometime in the next weeks.** I agreed to watch Zander this Sat night and sunday we are having a cookout at my sister's (God grant me the energy for this wknd, given the headaches and feeling super tired) and tomorrow (fri) my sister and I had previously decided to get-together and go look for flowers for my Mom's Mother's day present. Even though I don't feel the greatest I really enjoy having Zander here so am looking fwd to this wknd.

I asked Dr.Bragg about moving up the appt w her so I could quiz her some about the headaches (knowing we may not be able to do anythng) and almost more importantly so I can talk to her about the future Coumadin; (blood thinner) which will make future surgeries more difficult (not impossible just alot more planning) once it is commenced post-valve replacements down the road; I fup w Cardiology in 2 weeks so she suggested I meet with her early next week. I do like Dr.Bragg (and her staff) and we get along well but ooh my gosh I feel about like I live there! Atleast we have had a on/off break the past 1 1/2-2 months since the last revision. I also am incredibly grateful she is willing to email as it does make comunicating alot easier.

Last I am working on the referral to the PMR (physical med rehab) dr that Dr.Bragg and her nurse both refer pts to alot and really like. This dr is out of network w my primary insur so I will see her using my secondary insur as I do w my Physical Therapist and Pain Mngmt dr if all works out. My PCP is maling me the referral and I will put this together with the records I have from my former PMR dr at FMLH and submit the records by weeks end if  all goes well.. Really, all I should have to do is take the records out of their appropriate binder/file and scan to the computer + fax everything.

Thursday, May 2, 2013

It's been a year (for the 2nd shunt)... adjusted the setting..

Have to start this by saying that I realized last night it has been just over a year since the 2nd shunt was placed after we did a 5 day lumbar external drain the week before (in addition to the VP shunt which was in place) + Dr.Bragg had let me go home for about a week to let the external drain incisions heal, her thought was we could probably lower the risk of infection if we didnt place the 2nd shunt in the same admission, immediately after or during the external drain removal. Anyways, just over a year ago is when we did that 2nd shunt placement, after I think it was a 3-4 day in-pt trial of the lumbar EVD and after about a week (5 days I think) of then being in-patient from the LP shunt placement. By the next day or 1 1/2 days later could barely move the nerves in my low back and hip where so overly irritated I was in agonizing pain, I was doubling and tripling up on pain meds at home in addition to ice/heat and nothing was helping. I called the Neuro-Resident on call that night who told me I could either come in through the ER or see Dr.Bragg in clinic the next morning, I opted (semi-stupidly bc of
how badly I felt, would have been better off I guess going in through the ER bc once I was then admitted to the floor from clinic I had to wait for Residents to put in Dr.Bragg's order's which took the nurses hounding them over and over for several hours + finally calling Dr.Bragg who got it done.) Becausae I had opted to wait and have my Dad take me to her clinic the next morning which minus having to wait bc the Resident had never passed on the message to Dr.Bragg and she was in surgery or something she saw me as soon as she was going to get to her clinic. From her clinic I was re-admitted back to the floor, a PCA pain pump started, the Pain dept consulted and she talked to a colleague of her's + I then spent the next month in-patient as we adjusted the shunt settings, tried to the let the overly-irritated lumbar area heal (the lumbar catheter had been tunneled alot further than otherwise is usually done and was sitting up in my thoracic spine which may also have played a part if I am remembering right) and we did various different med treatments that she, her colleagues and my Pain Mngmt dr considered some of which worked well and some which where there own (mostly adverse nightime) issues. Definitely glad it is now vs a year ago!
                           X-ray of the VP shunt (Codman programmable) valve

Today I saw Dr.Bragg's NP to have the VP shunt turned down in the hopes this will dampen the headaches (pressure), vision issues (ugh!!!!!), and on/off other issues that have been occuring the past few days increasing though ironically today wasnt to bad of a day after I got past this morning. Sue and I had a bit of trouble w the Codman valve adjuster as it is a bit tricky to get it at the right setting and sometimes takes a few tries though Dr.Bragg (and I had forgotten she does this) showed Sue a more fail-proof way to prevent the setting from not going correctly which worked well the 2nd time we tried adjusting the valve after the 1st Xray. I went in w the valve at 50 and am now at 40 with the lowest it could go is down to 30 though bc we almost always have occluded once we went down as low as we could turn the shunts I doubt we would go that low. I hope this works and I really wish we could figure something out, I realllyyy like feeling even semi-human when the shunts are working "pretty-well" though "really-well" would be completely awesome, though I guess keep dreaming, right!? Someday, maybe! I got the appt changed to next Thurs to get my lenses on my glasses strenghtened (again) which hopefully this shunt adjustment will help vision to but I do think the shunts have had such an impact on vision that the lenses just need to be stronger (which i've posted about this before I think but
the Optometrist had said he thought they needed to be stronger but did'nt think I would wear the glasses if he made them as strong as the reading says they should be. Whatever the case may be I need to do something!

That's about it, I left the fup appt for mid-June but Dr.Bragg in an email earler this week had asked me to let her know how this went/keep her updated so if it goes ok will keep the appt then (though may change it back a week due to another appt in Milw) or otherwise her Secretary and Nurse are both really easy to get ahold of/easy to work with which is nice atleast bc I feel like I am forever talking to them!
Thanks for stopping by,


Wednesday, May 1, 2013

Awareness Day, Headaches, etc.

In a couple weeks it is May 15th, National MPS Awareness Day, a day celebrated all over by individuals and families w various forms of MPS and ML. Other than handing out 'Team MPS' pins to family, friends and providers I don't have any plans other than cont' to share information here and on the fb page but it's nice that a day like this exists for not only MPS but on other day's for other disorders to be represented as well and for awareness to be raised near and far! :)

I actually just completely don't get it, don't get what is with these shunts, why they work, albeit even if imperfectly atleast I still feel better for periods of time and especially after this last revision for the past 5 or so weeks). Atleast mornings werent very bad and nights werent very bad (minus breathing but that I try to sleep upright and on the couch as much as possible) and the little symptoms I had where more of a "background annoyance", I could live with them for now. Grr!!!! Dr.Bragg has said all along if I needed anything to let her know and especially last week she was out part of the previous week/last week due to her Mom passing away (I can't imagine) but when she was back said if I needed anything she was back. I called monday and asked her Secretary, Anna if I could have the shunt reset down a notch (at 30, go down to 20) sometime this week and so we are going to do that on Thurs. one of the NP's (Sue or Emily) and I pray it works, Please!!!!!  I know Cardiology and PCP are hesistant about any un-needed surgeries and Dr.Bragg I think is intimidated (?) by the cardiac and airway issues although I do think if it came down to it, which it better not, I so want it not to that with careful fluid management and coordination they would allow a shunt revision. I have wondered all along, if we had such good luck w this Codman-VP valve would the same work better in the TPL shunt (than the current Strata valve) at keeping both shunts working? I may not have had complete symptoms resolution but this valve is the closest i've felt to good in a long while minus of course the external drains. Again, I just say Grr!! Stayed tuned I guess..

I am seeing the Eye doc again next week (local) to get the lenses on glasses adjusted, the ones I just got, they do make a great difference but I think what he said about needing to be stronger (he didnt think I wuld wear them if he made them as strong as they needed to be right away) is true. I am sure part of it has to do with the shunt-pressure headaches.

I also scheduled the Hand surgery  with the Hand surgeon which I think I mentioned last time my PCP wants wants Dr.E (Cardiologist) to do part of the pre-op and she will do part weighing in on endocrine pre/post-dosing, etc. That then is scheduled out to June 5th which is fine and i'll actualy be doing the surgery completely awake, able to be sitting up some during and with a nerve block. On a side note my PCP also commented in the same note that  w the future coumadin dosing this would likely be a oral med (totaly didnt know it came as an oral med, that is AWESOME news atleast in a otherwise crappy med!) and for planned surgeries they would switch out the oral med to injections starting 5 days before any surgery but she said it is manageable. If yah have to take a good with a bad atleast it isnt injections all of the time and doesn't sound like the injections are the worst, perhaps unlike the solu-cortef (cortisol) intramuscular injections which are quite un-pleasant and not particularly pleasant feeling! Those I have to do w any colds, fever, nausea/throwing up or "stressful" situation which to me just means anything out of the ordinary health wise.

The doubled lasix dose hasnt been going all that well, the side effects are something next to impossible between making your hair and skin feel like straw (already feels like this much of the time) although the aldactone seems ok. I am wondering if at fup in a few weeks maybe we can try backing off the lasix and upping the aldactone, is worth asking.. I definitely am not a super fan (or fan at all!) of lasix!

I start back to PT today, has been a nice 1 1/2 month break, though I do basic, simple stretching at home.
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