Post-op Info, Plans, up-coming Shunt revisions

Talked to Dr.Bragg this morning, and just said to her how surprised I was by how uncomfortable/how much discomfort there has been since this shunt revision yesterday (Fri), the discomfort is just WOW! She said she wasn't to surprised unfortunately as really this surgery was more in-depth and more manipulating than is a normal, typical shunt revision.
She must have talked to my Cardiology Team as she said they do not recommend the Lovenox injections as a bridge between now and surgery the 14th. Instead they recommended to her I re-start Coumadin today and check-in with them on Monday (I suppose to say when I should next do INR Labs). Due to how high the INR blood level was when I came in they also recommended to her to start back on 5mgs/7 days a week vs 6mgs/7 days a week.
The plan was to release me today, while the head incision is just something incredibly uncomfortable I also am not doing anything else special here so no sense really to stay. I am not sure now though if I will get discharged today or now will it be tomorrow as I am on the PCA Pain pump since surgery and typically the docs like to wean this for 24 hrs before releasing pts. I am not entirely sure what is going on on that front yet.

Regarding Fup as Dr.Bragg goes on vacation sometime this week (I think it is, then comes back the day before my surgery) she wants to keep the Telfa dressing stapled over the surgical site until late next week. I'll fup with one of her NP's then on Thurs or Fri, they will check the site and remove the dressing.
Regarding the upcoming VP and Thoracic Shunt revisions Jan 14th, Dr.Bragg said she would want to admit me the day before again in order to check INR levels, any other Labs and she wants a CT scan to be done in which she uses this 'mapping' to load on to their Stealth Guidance machine in the OR. This machine is then used to help better visualize the small areas she works in and better position the VP shunt especially since she will be going back in to a area she previously had placed a shunt and which we had a lot of placement issues with at the time.

As far as the VP shunt she will use the same type of valve (Codman Hakim) as we've had good results with this minus the catheter wearing down the skin. In the case of the exposed catheter she feels this has to do w the Hakim valve, the skin that grew under the exposed catheter (a phenomenon she said she hadn't seen before) and the tip of the valve originally rubbing against the skin over time wearing it down. The skin that then began to grow under the catheter caused the tip of the valve or catheter (can't remember this part for certain) to be pushed up and in time wear down the skin creating the exposure. This took place over about a 9mo time as that valve was placed last March I believe.
Regarding the Thoracic Shunt she is thinking of using a newer valve her partner has had success with called a 'ProSA' programmable Valve. This is a smaller profile valve that she plans to (I think I remember this right) replace in the same location as the current Hakim Valve but she feels this will be a lot less noticeable with movement or sitting back and thus less uncomfortable. Dr.Bragg did say with this valve it is more of a pain to re-program as it does also require Xray to re-program after imaging (for instance MRI) and she said the re-programmer is different than anything else she has used. When they go to re-program the valve she said it is similar to clicking a pen, the tip has to touch the middle of the programmable valve in-order for proper re-programming to occur. Interesting!?! I just hope this valve works atleast as well if not even better than the Codman Hakim did!
For now I am on 2 IV antibiotics in-patient and will likely go home on the oral versions of these, the one i'll stay on right up till surgery.

One thing i'll have to ask about is if the NP's/Dr.Bragg can work with either my CHW Genetic Counselor or more likely my Genzyme Case Management Team in order to have the Aldurazyme shipped to UWHC that I would otherwise, normally get infused on 1/13 (the day before surgery).

I'll update again when I know something worth updating about,
Thanks for stopping by!

Erica

ProSa Valve

#28 Today's VP Shunt Surgery

This morning Lab-INR was a little high (not sure if it was higher than yesterday's) but the Neuro Team opted to do 2 Plasma bags over a quicker period (1-2 hours verses 4 or more hour infusion) in order to try and bring INR down to a more appropriate-less bleeding risk range for surgery.
The plan is to cover the open incision via (per the Resident) loosening the skin around the open VP shunt site/exposed shunt catheter and try to pull this over and I assume stitch that in place? Sounds a little on the gruesome (skin an indian anyone?) side.. Hopefully the Neurosurgery Team wont need to shave much hair/this loosening the skin wont affect hair growth... I know that's a vane thing to be concerned about but when you have so little control over many things related to your body it is nice to have a few things you can still manage!...

10:00PM 12/26-
So surgery was successful from what I glean from my Dad (talked to Dr.Bragg post-surgery) and the Nurses/Anesthesiologist who all updated me and in the case of Anesthesia stopped by my rm in post-op care. I'll update when I talk to Dr.Bragg in the morning though one thing the Staff and my Dad told me was Dr.Bragg was surprised by and had never seen where an exposed shunt catheter when they go to remove it has skin growing under the catheter (vs over I guess which would keep the area closed). I think they all said Dr.Bragg felt this might have something to do w the underlying MPS but I don't remember that part for certain and will ask tomorrow.
What I do know from Dr.Bragg as she stopped by my in-patient room late Friday morning after her other surgeries where done is that 1. she planned to use the previous VP shunt incision along the valve site (several inches long) and then take the exposed catheter and "under-mine" it by lifting and stretching skin (not sure if I re-explained that correctly), tuck the catheter inside what I think she explained (but again cant remember for certain) a pocket of sorts and then pull the 2 incision sides together and stitch it up. The incision is covered by a fairly large, stapled on Telfa sponge so I cant tell how big the area is.. It does look like they shaved a minority of surrounding hair which is/will be/would be great!
She plans to as I've written here before con't to treat the formerly exposed areas with antibiotics and Jan 14th we'll go back to the OR. Right now she (Dr.Bragg) is considering whether to bridge this next 2 1/2 (ish) weeks with injection Lovenox (is done twice daily) which offers fairly consistent INR level as the med only lasts 12 hrs. Otherwise she will restart the Coumadin, we'll have to con't daily blood draws until INR is back in-range and till I could be discharged.. Per the Cardiology NP getting INR back in-range can be more difficult when Vit K or Plasma has to be given to bring down a pre-surgery, high INR # though I cant remember why she said that was.

When Dr.Bragg does move the VP shunt valve and proximal catheter the 14th she plans to use the same shunt catheter tract (not sure if that's the right word to use) as the areas where the catheter feeds down to my abdomen/'T' connector have worked well and not a lot of options where else we could try. The valve will be at the back of my head again I guess (circa Dec/Jan 2012/'13 when we had bc of the shunt infections moved the VP shunt to a parietal location. I'll update on that down the road.. She plans to use the same Codman Hakim programmable valve (has worked pretty well, not perfectly but when the 2 shunts where working pretty well I did feel a lot better than I otherwise had in a long time! =)

I don't really know much else so will update when I do,
Thanks for stopping by!
Erica

Surgery; Exposed (VP) Shunt site

I was re-admitted to the Neurosurgery floor today (Thurs) in-order that Labs (specifically/especially INR which was just a tad high at 1.8 per the Nurse), Chest Xray, Urine culture and Exam. Otherwise I have just been wandering the hospital some early this afternoon (had to check-in at 1:00) and then hanging in my room. A good friend of mine came to visit tonight for several hours which was lovely. One of her kiddos also has a shunt and complex shunt issues so she completely gets all this

Surgery is scheduled at 12:30/12:50 (somewhere in there) tomorrow under General Anesthetic to cover the exposed VP shunt catheter site. The exposed site is located right next to the programmable valve which Dr.Bragg said last weekend is usually a little more difficult of an area to close. I don't know actual details of what she will do yet but she still plans to cover this open area, followed by continuing treating with antibiotics and in several weeks (Jan 14th) move the VP shunt location although I don't know if that means just moving the distal location or if she plans to move the entire shunt to a different site? Either way the surgery is 2-part in a sense, several weeks apart.

I know this is short but thanks for stopping by, prayers appreciated!
I'll update when I get a chance,
Erica

Merry Christmas! (released, re-admit Thurs for Friday surgery)

Merry Christmas!!
Sorry for no update regarding what is going on with the exposed (VP) shunt catheter. See below for the details regarding getting released this past Sunday but Dr.Bragg had decided I could go home on Sunday instead of Monday and she would bring me back on Thurs and then surgery is still Fri. I have to be back at UW at 1:00 on Thurs to be re-admitted to the Neurosurgery floor and then they will likely do labs to check INR (blood thinner level) and any other testing needed pre-surgery. The actual surgery Fri atleast as last planned was to be under General Anesthetic (meaning intubation).
Dr.Bragg wasn't yet sure on Sunday when I talked to her what she was going to do to temporarily cover the exposed catheter so I don't know those details, she often thinks over these surgeries right up till surgery in order to come up w a best plan. I am hopeful since I will have to go back in again Jan 14th for the actual surgery to move this shunt location that I will be able to get INR up fairly easily (post-surgery) and get out maybe as soon as Monday? I honestly don't know what she is thinking... This surgery was just a temporary cover in a sense so that the catheter isn't continue to be exposed to the outside and thus germs have an easy entrance site.. At the same time she said if she where to move the shunt right away in this surgery from their experience there has been a higher long term infection risk.. thus why she is doing this in 2 parts.

I have just been glad to have been home for a few days though I have to admit I felt more than a bit frazzled everyday since in trying to get the things done I needed to! Monday included Labs, ERT at CHW, pack everything for my parents today (we celebrate Christmas present opening Christmas Eve morning), My sister, Mom, nephews and I also decorated Cookies Monday night at my parents. Then when I finally got home in addition to packing up the presents I cleaned my Apt some (seems like tree needles EVERYWHERE!). Today after getting home from celebrating Christmas Eve morning/early afternoon at my parents and then later running errands (picking up meds, store, etc) I did some packing for Thurs re-admit, took down my tree and a bunch of other inconsequential things but that I needed to take care of! It didn't help that I forgot my planner and paperwork at home Monday that I wanted to do at ERT so I was working on that stuff in-between the past few days too!

Tonight I was able to go to my Church's Kids program (parochial school PreK-8 grade) which was lovely and so nice to be there in person versus watching a Christmas Eve service on TV like last year!


Written Sunday Dec 22 Alright so the now new plan is (found out mid-Sunday afternoon) that I get to be released Sunday evening on oral Clindamycin (antibiotic) instead of IV Clindamycin and a day earlier than they had planned due to there being the oral version of this antibiotic available. This in turn means I will go to ERT at CHW (Children's Hosp.) Monday morning per normal schedule vs being infused at UW, in-patient. Since my INR had continued to increase despite no increase in the Coumadin dose Dr.Bragg and the Pharmacist wrote an prescription for the injection Lovenox but don't expect i'll actually need to use it. I have to do a INR blood-lab test Monday while at CHW. Instead of admitting me the morning of surgery as formerly planned Dr.Bragg is also now planning with her team to direct admit me to the Neurosurgery floor Thurs. at which time they will do Lab draws to re-check INR. If this level is still to high they can then give a med to counteract the blood thinner and which makes the blood at a more surgery appropriate blood (clotting) level.

I'll update sometime Thurs with any details I learn regarding Fri's surgery.
If you believe in prayer, please pray that 1. surgery goes well and easy. and 2. That I get released from the hospital before Dr.Bragg goes on vacation (not sure when that is)!!!
A Prince was born to save OUR sins!
Erica

Talked to Dr.Bragg, Home for Christmas, A (tentative) Plan

This is copied from my latest fb update, a couple friends where asking for updates so I posted this there and copied it to here verses re-writing a completely new, but virtually the same post.
So the news is.... drumroll please (lol!)...
Per Dr.Bragg, Her plan is to let me out on Monday and have me come back on Fri. Part of the reasoning for waiting till Mon to get released is we wait for the INR (blood thinner level) to come down to a therapeutic (surgery appropriate?) level.. then bridge w injection Lovenox till Fri. They also are watching the CSF culture, have to arrange the various things and want to make a temporary cover of sorts for the open shunt (catheter) area.
I'll as of right now probably get out Monday for Tues-Thurs w/IV Antibiotics and the Lovenox injections (to bridge) and Neurosurgery will cover the open area over the shunt valve. Dr.Bragg said this is a harder area to go in and cover in surgery as it is right over/next to the valve so she is formulating the best initial treatment plan for when I go home and especially for surgery on Fri.
I'll come back on Fri for surgery (general anesthesia) to cover the open catheter via graft or some other method and then in a few more wks of IV Antibiotics likely after which I will come back on our already scheduled OR (surgery) date Jan. 14th to completely move the VP shunt. We'll put off the Thoracic shunt move to a future, to be determined date. Ironic since that is the shunt that most bothers me! I know we need to prevent any infections from occurring in this open VP shunt site though. =/

So that folks is how 1 surgery in Jan. now becomes 3 surgeries but STILL I am going HOME FOR CHRISTMAS!!! CSF cultures so far have been clear! INR level when they first tested my blood level yesterday was lower than it was when tested this morning (meaning the blood thinner level has further to fall before it is in the "lovenox/bridging safe range". This med typically takes 2 days for a result to show up so what was tested out this morning was a result of what I did or didn't do that affected the Coumadin from 2 days ago.

While waiting in the ER last night Joan and I where talking about the things that are hard to decipher. I was talking about how hard it is to me to pick up on people's jokes, especially when the headaches are happening and how sarcasm usually goes over my head. There are so many things that (especially) people say that are so hard to interpret when the headaches are a little bit worse or the other symptoms have picked up! Joan (Sunday School Co-Teacher, great friend) and I while waiting in the ER last night to go up to the Neurosurgery fl where talking about figuring out sarcasm and if people are being sarcastic funny or sarcastic-hurtful. I rarely get this sort of thing and have just come to the conclusion lately when symptoms are worse I have an even harder time deciphering people's meanings when not black and white! Not a big deal but does make for some aweful funny "in my head thoughts or even questions to other people sometimes trying to figure out what something means or what someone meant! =)

Anyways, that is I think about all I know,
Will keep everyone updated,

Erica

VP shunt visible, Admitted via ER at UWHC-Surgery.

Just a quick post- Several weeks ago I had an Appt to go and get my haircut but at the almost last minute had called and cancelled it as I wasn't feeling well (headahces, ironically) and so I called the other day and went instead today (Fri). Anyways, my hairstylist today asked me while cutting my hair if I had had a shunt revision on the VP shunt recently of which I haven't and and she commented something along the lines of "oh this looks different, something is different with it". I didn't think to much about it till a little later and so I (as best I could) looked at the area and noted the shunt catheter appeared to be sticking out of incision and visible in about an inch area!
Not entirely knowing what to do (my brilliance) I posted on fb asking if others have experienced this which a few commented asking if I was sure it wasn't scar tissue (very sure!) or something normal.. Dr.Bragg commented that no, it isn't normal and unfortunately she thought I needed to head to the ER. =/
Anyone who remembers last year knows this is particularly disappointing bc her and I spent last Christmas with me under her care due to the shunt infection and the virtual entire month of Dec. in-patient!
Needless, I finished up at the store and called my Dad after talking to Dr.Bragg's Secretary (who Dr.Bragg had already talked to) and headed back to my Apt to pack a bag. My Co-Sunday School Teacher was meeting me at my Apt to get the supplies I had for our group-Sunday School (Christmas) meeting which after our lesson, having to do w the nativity scene we where making nativity banners w felt and dowels.

While meeting Joan at my Apt I could then give her the gifts (ornaments) we had bought for our kids. She it turns out ended up coming w me to the ER in Madison and spending from around 6-11/11:30 hanging out w/me while the ER did it's thing and we waited for a room on the Neurosurgery floor. She is AMAZING, how many friends seriously would take the time to go w a friend and spend their whole Fri night sitting in a ER?

The Neurosurgery Resident came by and did a Shunt tap (off the VP shunt) once I was in a room on the Neurosurgery fl to test for/rule out CSF infection. The Lab person did 2 blood draws to check for/rule out blood infection and the Nurses are coming back in a bit to access the Port in order to start IV antibiotics. The plan is to let the INR level trend down over the next few days and then do surgery I guess to replace or revise the VP shunt. I am not entirely sure what the plan is yet and won't know for sure I don't think till Dr.Bragg comes by (I assume) in the morning. I just pray I am healed and out of here by the time she goes on vacation!! I don't think she will do the Thoracic Peritoneal shunt while I am in-patient this time both b/c she doesn't like to mix 2 different areas/surgeries in one and then not know for sure what worked and 2. I imagine she will want to wait and do the TP shunt as scheduled in Jan.-I am more than ok with not doing both right now!

1. The part of the VP shunt cathether that is visible.
2, 3. Joan and I goofing around w 'balloon' gloves' (see the sign)

Tomorrow my Mom, Sister and I had planned to decorate our Christmas cookies (late this year) so it seems it will be another Christmas not doing this. Last year my Mom brought the frosting (she makes homemade) and the sprinkles to my hospital room along w cleaning supplies (to clean before we decorated) and then we went ahead and did it anyways. I am hoping maybe we will do something like that again this year? Guess tomorrow will tell!
I will update when I know anything more.

Hopefully the CSF and blood labs come back clear and hopefully this surgery whenever it is goes well and as well we are able to get INR back up to a therapeutic level fairly quickly post-surgery!

As disappointed as I am to be back here, spending it seems Christmas here again ( =/ ) I do know God has a greater purpose and so I have to put my trust and Faith in him!
Thanks for stopping by,
Erica

Cardiology-Holter monitor, Headaches suck

I talked to the Cardiology NP today (Weds night) and again this morning (Thurs) and they are doing a 24 hr Holter monitor (a continuous EKG) along w the EKG (remember the post I wrote a few wks ago saying I didn't think we'd have to do either of these things!? Remind me to shut my mouth in the future!) ;)
Dr.E (Cardiologist) will take this monitor results to the EP (heart rhythm) dr. then.
The recently increased Bisoprolol dose didn't do much to slow heart rate down so Dr.Earing's thought or plan was to make a new medication recommendation, likely from what Nancy (the NP) said a different beta blocker. They likely will call early next week with the Holter results and new med recommendation.
The EKG was fine and heart rhythm was normal (not exactly un-expected since it is a 6second snapshot out of a 24 hr day!) but the NP did say Dr.E was at least happy in that brief period my Heart rate (HR) was in the high 70's an improvement from immediately post both heart surgeries (July, Sept) where heart rate was high 170's, then high 120's while still in-patient and has ranged in the 80's-90's to low 100's (125 typical highest I think) since the last few months minus a few longer-higher periods.

Even when HR is running along at a faster clip I don't stop and take my pulse to see how fast it is as it doesn't change anything so those are just measurements from Cardiology Appts and from at ERT (infusion). HR overall doesn't get a whole lot faster or slower when at Cardiac Rehab.

---

I turn the Holter monitor back in tomorrow afternoon. The reasoning as I explained in the update after the Cardiology Appt a few weeks ago behind Cardiologist wanting a slower heart rate is so the heart has more time between each heart beat and thus (if I am remembering right) the heart gets more time to rest between each beat and isn't working as hard.
This week has been another chaotic week of appts betweem Monday and Weds being in Menomonee Falls for Cardiac Rehab, Tues at ERT (CHW), today also at CHW for the EKG and Holter hook-up and then tomorrow is Pain Mngmt (normal) Fup followed by dropping off the completed Holter at CHW. Friday I am getting my hair cut and that is it besides actually getting normal errands done!

Sometimes I think (seriously) that a hand drill and a straw would make a better shunt than does the current shunts I have. I mean don't get me wrong the 2 shunts I have worked reasonably well actually for about 6 mo (as a whole) and even though they weren't perfect it was still better than almost any other shunt system i'd had in the past. The past week and especially the past few days though has been quite aweful and I feel aweful-with mornings being the worst by far.
Not only do I have trouble falling asleep (getting comfortable) but then when I do wake up in the morning I am tired and I feel as if I have a ton of bricks inside my head. This isn't a new issue it has been a common refrain over the past years especially for anyone who reads this frequently!
Anyways I really think someone in the medical community ought to come up with a hand drill patients could use to drain their own fluid when our shunts are being imperfect and we have to wait for surgery for other reasons (holidays, family preferences (I don't think people have a clue how this feels), etc. I don't wish to be in-patient over the holidays either as I really enjoy all the traditions we have (baking/decorating cookies, shopping, making decorations w my nephew, decorating and wrapping the presents I find (I love wrapping presents), etc. Sunday School and our Christmas-Group Party, being with my fellow SS Teachers, and so much more. I do wish though there was a way to improve how I feel as I literally spend atleast 1/2 the day, sometimes much more than that feeling as if "I got run over be a reindeer".
Aaagghhh I HATE shunts-actually mores specifically as imperfect as they are I am grateful for shunts, I HATE hydrocephalus and MPS! This time has been so weird, so all over the place ie the symptoms are my classic bad in the morning, somewhat improve as the day goes on but then some days some of the other symptoms are worse and some days a little better.
I always feel crazy though, seriously when I see people later in the day and I feel "more fine" and I am sure I look pretty fine so they have no idea what a lot of the day is really like! Not to mention you keep on keeping on w the things you have going on but sometimes looking back it is like "holy s***, did I really make it through all that!?" - Not exaggerating on that!

Anyways, next week is Christmas, I am not in the hospital this year and I have all my gifts, bought, wrapped and tagged! I love Christmas for it's true reason though, Our Savior's birth and being with Family!
Thanks for stopping by,
Merry Christmas!
Erica

Endocrine, PMR (Physical Med Rehab-Botox), Cardiology

Cardiology

The Cardiology Appt went well with nothing to big coming up (didn't really expect anything to) just a few adjustments and otherwise all current cardiac meds (Lasix, spironolactone, beta blocker) will stay the same as "you look good and are doing pretty good so I don't want to upset the apple cart right now" I believe where Dr.E's words.
Overall Heart function is at the low end of normal so they want to keep the heart rate lower than it is with Dr.Earing doubling the current beta blocker (bisoprolol, which is used commonly for CHF, BP and less commonly for rhythm) dose. If heart rate with this increase where to last longer than 10min and especially 20min stretches at a time in the 80', 90's or higher he (Cardiol.) mentioned he would probably go to a different, stronger class of meds. His reasoning for this is that he wants there to be more time in-between each heart beat for filling and relaxing (blood, oxygen I think they explained) so the heart isn't working so hard so often. His explanation for why this is was both it was an issue prior to the heart surgeries, the MPS likely factors in and the heart surgery can often cause short term damage which needs to re-wire itself. If it where to change or worsen he would add the different med class and do an EKG + have me come in sooner (I cant imagine it will) but otherwise I see them for an Echo and Fup in 3 mo. and keep them updated in-between. They also call with each INR/lab work result so while we don't see each other we also don't escape each other.
Otherwise INR was in the high-normal range so this will be re-checked the 1st week of the new year (around New Years) and their hope is it will trend down between now and then as they'd like it to really be in the 2-ish range (2.5-3.5 is ideal range). When we do have to stop the Coumadin (see below) a few days before the shunt/spine surgery in mid-Jan., if the INR level is closer to the 2's there wouldn't be as far for the range to have to fall when I would go off of Coumadin leading up to surgery and wouldn't be as much bleeding during surgery then I think. The higher the INR before I stop the Coumadin the farther it has to fall and the less likely it is to be very low leading in to surgery meaning bleeding would be worse during I guess. (did that all just sound as confusing as I think it does? No matter how many times I re-type it, it doesn't come out sounding any clearer/better!)
Because I have a newer tri-leaflet valve and no other risk factors (previous clots, a 2nd mechanical valve) I won't have to bridge with Lovenox injections apparently before this up-coming surgery (per newer ACC recommendations) which is a nice thing! Dr.Simpson my PCP had been planning on doing the lovenox (is newer recomm's) so I passed that information along to both my PCP and to Dr.Bragg. I won't complain!

Endocrine, Physical Med Rehab (Botox)
The other 2 appts besides Cardiac Rehab where Endocrine on Tues., which those labs take a few days to come back (cortisol, thyroid, other hormone levels, etc.
Yesterday I saw the new Physical Med-Rehab dr that Dr.Bragg and especially her Nurse had recommended. Dr.Caldera is also at UW and i'll see her using my secondary insurance but she agreed that cont'ing with the botox was warranted and she would con't with the areas I previously had done + may recommend doing further botox in other areas but would like to review the previous notes further.

She seemed to have a good idea of my past shunt surgery history and was aware of the heart surgeries as the reason why I had previously had to reschedule seeing her and somewhat aware of MPS affects. Because of the holidays and other things going on we opted to wait till the end of Jan to do the next round of botox injections since it was last done in June (so just a few months outside the normal 3 mo pattern I typically do). She commented if I felt it needed to be done sooner to please let her know and she would make it happen which she seems to not mind working with other providers if need be and interested in the overall picture.

The other (much sadder) news this week has been on Sunday my Dad and I went and visited my Grandpa and he was doing really well (well for Grandpa and how he has been up and down over the past 2 or so years). but then Monday he had went drastically down hill. By Tues night most of us family where at the Assisted Living home to say good bye, later Tues night Grandpa passed away.
I am both sad about losing someone who meant so much to me growing up but also I know he is in such a better place, free of the earthly pains and restored to a full and glorious body. I can't argue with that so while our earthly sorrow is great, Grandpa's heavenly gain is soooo much better!
The funeral services are Monday night, i'll likely drive up there from ERT (infusion) in Milw. One of my closest friends (from sun school, we both teach) texted me to ask for arrangement information as although she has never met my Grandpa or most of my family other than my Dad she wants to try and come to the services to offer support. I know she has to work all day so just knowing she cares means more than I can even say! I am very grateful to have such friends as she!!
On a completely different note, Sunday I am going with Church to the Seminary where there is a Christmas Concert being put on. My other Sun School Teacher (who I teach with) and I are riding together (bus) and should be nice, afterwards (or maybe it is before I am not entirely sure) the group stops to eat. I love Christmas music and especially Christian Christmas music so am looking fwd to this.
I know it may seem odd to jump from talking about the loss of my Grandpa but I feel like with the knowledge of knowing he had God's grace and forgiveness I truly do believe he is restored to a heavenly home, one in which all who believe will one day also gain and so much better than anything we could have here on earth!

God's blessings, remember in everything God does have a reason,

Thanks for stopping by,

Erica

10 Things I am thankful for...

A friend of mine posted a similar list of her 10 things she is grateful for (her son has MPS II-Hunters, another form of my disorder).

No particular order:

1. The family I have who are there when I need them to be there, especially my parents! Family and friends who come to visit when I am in the hospital and my parents and sister, the ones who take the time on Holidays for us to be together.

2. My nephews and nieces, especially the ones who love to come visit or spend time together. :)

3. For a space to call my own, for my car and for the work I have with Gene Spotlight which affords me the ability to have extra spending money outside of the disability income. I am grateful to work for GSF as well b/c it allows me the opportunity to peak in to what is coming as potential treatments for MPS I!

4. For providers, especially those who email or text message and who care enough to answer my questions about the care they give. For the providers who are willing to work with other specialists on my 'Team' and who believe me despite when things are not always black and white-textbook.

5. I am grateful to not be spending this Holidays in the Hospital (!!) and I am also grateful we will be able to move the 2nd (Thoracic) shunt back to it's former location in Jan!

6. I am grateful for my Church family, most especially for my 2 fellow Sun School Teachers (obviously there are more teachers than just the 3 of us but I consider these 2 particularly close) who I consider great friends, who have visited while I have been in-pt and who I never feel like I have to apologize for having an opinion.


8. As much as I sometimes don't want to go I am thankful for Aldurazyme and I am thankful there are potential drugs on the horizon to compliment what Aldurazyme doesn't help.

9. Specifically regarding the MPS I am thankful for my Primary Care dr who despite knowing nothing about MPS when I started with her has learned. I am thankful for my Peds Neurosurgeon who has stuck by my care through many hills and valleys- always seeming willing to try and think of another approach or thing we can try. I am thankful for my Pain Mngmt dr who gave me back my life when chronic pain was affecting my daily life significantly and no one else would really help. I am also very thankful to my Cardiologist and his Team who made the 2 open heart surgeries go smoothly despite some set backs. I am thankful for his skill in ironing out the complications following the 1st OHS and then knowing what to do to help the issues/symptoms following the 2nd surgery. As well I am grateful he and Dr.Bragg (neurosurgery) each have a good team who answer calls, they both answer emails and all 4 of these drs have worked together through all of the many surgeries. Last, I am grateful to my Endocrine dr, she reaches out to the rest of the team and they to her in order to make sure the cortisol issues are well managed pre, during and following surgeries.

10. I am grateful I don't need someone else to tell me what I need to do, that as much as I dislike it I am able to pick up the phone on my own and call whomever I need to. I am grateful I can make my own decisions and I am grateful that although I am stubborn I also get along really well with the vast majority of people I meet! - I know there are people who dislike me and I am ok with that too!

What are you thankful for?
Thanks for stopping by! - Happy Thanksgiving!

Erica

A range of things, Happy Thanksgiving!

I had Labs today for Cardiology due to the CoQ10 addition and Jane, Dr.E's Nurse called with the results. The INR was to low at 1.75 whereas goal range is 2.5-3.5, they will re-check this and other labs next Thurs., a week from now prior to Cardiology Appt fup.

The Pre-Op Appt for Dr.Bragg's surgery with PCP is set for early Jan., with her (PCP) plan being to contact Cardiology and get their 'ok' on her pre-surgery bridging plan. From what my PCP shared her plan is to bridge with lovenox injections for 3-5 days prior to the shunt/spine surgery + stop the Coumadin during that time and nothing the day of surgery. Lovenox/Coumadin are then re-started at Dr.Bragg's discretion the day of or following surgery and monitored daily till labs are back in range.

I talked to Dr.Bragg's Nurse today and she (Dr.Bragg) hadn't decided on a valve yet for the surgery which after Lisa said it didn't surprise me to much. I said to Lisa, often she (Bragg) will come in to pre-op the morning of surgery and talk about how she was still thinking over what she was going to do or change the night before my surgeries and what she finally decided on. Sometimes Dr.Bragg wouldn't have decided until she was in surgery and then make a decision. She knows her stuff so I trust any decisions she makes. I do like information ahead of time, it helps me deal with things but I also know taking her time and thinking things over is Dr.Bragg's style so it is fine not knowing.

Next week is another week of pure craziness, I am not entirely sure when i'll get Cardiac Rehab in (usually Mon, Weds, Fri and I almost always already miss Mon's due to ERT) but maybe they will let me come to an earlier or later class on Thurs as I will be in Madison Weds. (seeing the new physical med rehab dr., does the spine-muscle botox) and Thurs see Cardiology. On Monday I am going in to CR after infusion to their 2:30 class whereas I normally on Weds/Thurs am in the 1:00 class so maybe will do the same or earlier 11:00 class Thurs. I will never get done with CR at this rate as there is always some other Appt conflicting w the Cardiac Rehab times! Anyways, Monday is ERT (infusion), Tues is Endocrine, then meeting a friend for lunch, Weds the PMR Appt in Madison and Thurs is Cardiology. Hopefully all this will calm down soon, right now seems like a lot bc of Cardiac Rehab and 3 and 6 mo fups w several drs. - Is CRaZy!

Anyways, I guess that's all that is new on the MPS/medical front. Thanks for stopping by - Happy Thanksgiving!

Erica

Shunt (tentative) scheduled, Bits of other things..

We are looking at mid-Jan for the Shunt surgery with the date likely to be Jan 14. From what I understand (i've emailed Dr.Bragg to clarify, i'll try to update this if I got anything wrong) the plan would be to move the valve over, still tunneled in the thoracic spine but not as centered I think? With the Peds General Surgeon (or that was her thought last week it is always possible she will change her mind and either do it solo or use someone else) move the distal, draining end back out of the peritoneum and to pleural space again. Potentially use a different valve though this is one of the parts I am least clear about as her Secretary couldn't say if she'd had time to talk to the Shunt rep or not about our issues.

I talked to my PCP (mychart messaging, similar to email) and she commented back regarding what she would recommend w/the Coumadin dosing leading up to this surgery (any surgery apparently), though i'll also have to see her sometime before that surgery for pre-op. ( "Usually for bridging we have you stop coumadin 5-7 days prior to surgery and start Lovenox 3-5 days prior to surgery and hold the Lovenox morning of surgery. And then after surgery do coumadin and Lovenox until inr at goal again. When we get closer to the date I will ensure I talk with your cardiologist to make sure they are agreeable." - Dr.Bragg has essentially said the same things especially about post-op INR management and has always just commented she would use that time to do any adjustments to new valve (programmable settings) and initiate/wean from PCA pump. I am not entirely sure if post-op is done the same for this type of surgery as it was w the heart surgeries but there instead of lovenox injections they did heparin via the IV and Coumadin then tested INR every morning till in-range.

I did talk to the Cardiology Nurse and got approval to start CoQ10 but need to do Labs for INR (blood thinner level) mid-next week instead of waiting till the following weeks Cardiology Appt. (labs would be done then too) as CoQ10 can affect Coumadin dosing.
I also just emailed the Cardiology NP to ask if we can switch to brand name Coumadin so that the inactive/active ingredients never change whereas with generic Warfarin the inactive ingredients change anytime the supplier/brand changes at the Pharmacy which I think is affecting the issues. Most meds generic isn't an issue but there are a few that it can pose problems for some people. I do take another drug as brand name though it is also available as a generic due to the generic not having worked as well and the prior auth's required for this where fairly straight forward. We'll see. :)

This last Sat, I did go canvassing with St.John's for a new start-up WELS Church in Mount Horeb. The experience was fun and nice to be around Church family for that day. As well although the weather was drizzly so we didn't run in to many people while leaving the brochure in doorways it was still interesting to learn the various "do's and don'ts" of canvassing and hear the history of this new Church and the fill-in Pastor we worked with. I would do it again.

We had our monthly Sunday School meeting Sunday night to go over Dec. and Jan's class lessons which also was informative (as usual) and so nice to be around others who share the same convictions that I do. I can't explain what it means to have this 'family' but it has been a good thing. Non of us are perfect but we share a bond in a way despite all of our very different paths in life I guess, maybe? I know 2 of the Sun School Teachers have become very good friends despite our age differences and I feel like I've been able to lean on them in a way during times when all 3 of us are dealing w our own struggles (both lost either a husband at a younger age or a daughter who was then my age) but we still (I think) make each other laugh and not forget we all are only human.

I have been pretty busy when at home trying to track down information for GSF, working on some information for the Church Committee and putting together this weekend Sun School lesson in-between having had appts everyday but today. Tonight I am meeting friends in Sun Prairie for dinner! :) Looking fwd to seeing them!

Otherwise med stuff has really centered around Cardiac Rehab this week other than Infusion which continues to go fine. I have sloowwllly been increasing speeds on the treadmill which I usually walk for about 20mins and then do the spin bike in-which I've been slowly able to increase range. I think I've said it before but I like the treadmill better than the spin bike though both are not the most-comfortable on mid-spine! The older guys and the ladies in class are funny to chat with and between them and the 3 Nurses/EP's who float around monitoring all of us, making suggestions the time does typically (thankfully!) go fairly fast.

Tomorrow Sara, Mom, Zander, Bella (nephew and niece) and I are going shopping and then Z is spending the night here. I teach Sun School Sunday morning before Church so Z will come along for that. I have an Advent craft (Advent starts after Thanksgiving but we don't have SS the Sun after Thanksgiving) and the snack we made for the kids for this week so hopefully he does atleast as well as last time! Often when Connie one of my good friends and I teach on the same Sunday we'll then sit in church together and Z seems to like her. :)

Thanks for stopping by,
Erica

Neurosurgery (post-heart surgeries) Fup

Busy, busy week again of medical appts., always so fun (lol, joking, seriously!) but otherwise other things going on to and a good enough week. :)
I saw Dr.Bragg today which was a planned appt some months ago and we've communicated in-between. Last I saw her (a few wks after the 1st heart surgery, not long before the 2nd) was mid-Aug before the 2nd heart surgery. Anyways our plan was always tentatively to put the Thoracic Shunt distal (draining) end back in to the pleural space sometime around 8-12 wks post-heart surgery. It will likely be closer to 4 months post-the heart surgery when we revise the shunt unfortunately given the holidays, which I say unfortunately ONLY bc the stomach spasms are a pain, literally. We may be able to move the valve to a less "noticeable" area ie an area the valve would be less apt to move either further over on my thoracic spine closer to the side or further down. Otherwise I will be happy to go about holiday traditions on the outside, granted the nurses and my family made last years holiday's 'enjoyable' despite being in the hospital including some of our traditions we adapted and did while I was in-patient and the nurses decorated my room BUT I am happier to do these things on the outside! :)
Some of the things we talked about where moving the valve as mentioned above, trying a different, smaller in profile valve I think still a Codman brand but I also can't remember this part for certain. Dr.Bragg was going to talk to her Rep in the next week about some options she thought we might try, see what that Rep thought/recommended and asked me to talk to Cardiology to make sure they have no qualms about her planning this surgery (I also see them the 1st week of Dec.) and any recommendations they might have. She also talked about our having to bridge with heparin prior to the shunt/spine surgery and then they would resume the Coumadin post surgery while cont'ing to bridge w the heparin until levels where back to therapeutic.
Last she mentioned she has been working with a newer Peds General Surgeon and would ask him to help with disconnecting/removing the current 'T' connector connecting the 2 different shunt ends so they drain as one in to the peritoneum. The General Surgeon would also help her place the newly disconnected Thoracic shunt in-to the pleural space given the probable scarring from the 2 recent heart surgeries and to prevent the lung from collapsing I think (it partially collapsed when 1st placed in this location last Dec.). I'll call her Nurse in about a week and see what Dr.Bragg found out + I imagine we'll schedule surgery at that point. I am hoping we can do it shortly after Xmas (maybe the following week) maybe before or right after New Years? We'll see..
Just will be glad when it is OVER!

Tomorrow 2 of my fellow Sunday School Teachers and I are doing the initial part of our T-day cone cornucopias for the kids and then we'll finish them after our monthly Sunday School meeting Sunday night (decorating/filling/wrapping). Sat., I volunteered with Church to go and do canvassing in Mt.Horeb for (I think it is) a new WELS Church starting up there. The weather sounds like it should be decent if it doesn't rain and I think it should be a good and interesting experience. :)

The only other thing I thought was interesting that I didn't mention last week was at the Cardiology appt Dr.E and Nancy (NP) mentioned how loud the heart beat is when they take BP/listen and commented they thought maybe the swelling around the heart has went down significantly causing it to be "quieter"? Before the heart surgeries the heart specifically the L atrium had been severely enlarged so things are improving! Awesome!

I'll update when I know more from Dr.Bragg/her nurse next week. Thanks for stopping by,
Erica

Cardiology 2 month Fup, Other Appts..

This week has been chock full of appts but really today's was the only one that was a fup. I saw Cardiology (NP and Cardiologist) for one month follow up from Surgery 2 months ago. We discussed a potential med for fast heart rate but decided it's liveable and we can always re-visit the issue at next fup if is an on-going "annoyance". Really the surgery healing continues to go really well, pain is pretty much non-existant since a few weeks after surgery. Heart rate is incredibly annoying, semi-fast(tachy) often but still intermittent. Otherwise all seems well with the new valve, VSD closure and the areas around the valve that where enlarged and fixed in surgery.
I fup in 4 weeks and then if things are going okay still Dr.E will set the next fup at 4 month out from that. Right now we are about week 8 or 9 (the 2nd heart surgery was 2 months ago on 11/3).

On a separate note Dr.Earing is involved with our 'Adult Congenital Heart' group and has brought up about doing another 'patient education night'. He brought this up at the end of the appt today as it's something we've been talking about since the ACHD Picnic a month or so ago. He wants to go ahead, schedule this, have the topic based around the new healthcare laws/reforms and how this will/may affect ACHD pts. He will send the information out to myself and the group leader (Patty who started the group) and we will in-turn send the information out to the ACHD community who are part of our group and get feedback. - One thing I just thought of now is would it be informative to ACHD pts for Dr.E to have pts with both forms of private (employer, privately bought) insur., a patient with Medicaid and a patient with medicare share their experiences getting ACHD care? Something i'll run past Dr.Earing in the near future to consider for the meeting.

All Cardiac meds are the same and dosing will stay the same, we talked about lowering or stopping the Lasix (takes fluid off) or the Spironolactone (a longer acting med that works similarly to Lasix) but because I've had issues with fluid retention and pleural and pericardial effusions following the 2nd heart surgery and am doing well on these doses now Dr.E and his NP thought we should leave these meds alone. We'll re-check kidney levels (and other Labs) at the Dec Appt and re-visit whether or not to lower/stop either of them again then. I honestly hate these meds (and when I am going somewhere for instance Sat w my Sister and Mom) I just don't take the morning dose and on infusion days I take the morning dose either very early or if I am going to get home early enough (rare) i'll take it early afternoon then the 2nd dose in the early evening vs having to try to find bathrooms constantly while out. Occasionally I also just skip one of the doses altogether. The meds do make a big difference in fluid retention and thus how I feel so as much as I dislike them I also can't complain to much about them-a catch 22!?

The Coumadin (blood thinner) level was just under the therapeutic range (2 mo after surgery we finally got it back to the 1st surgery dosing) but b/c the level was close enough to the recommended level (2.5-3.5 in the 1st 3 months)the dose will stay the same (6mgs 5 days/wk, 4mgs/2 days wk.) and instead of weekly Lab checks I can go until the next Cardiology Appt in 4 wks without having this drawn as long as nothing adverse occurs. - Adverse things would be: abnormal bleeding/bruising/bloody noses, getting sick/antibiotic use, surgery or anything related. I'll take that break - thankfully it isn't very hard getting the labs drawn since I can just walk over to CHW's Lab when I am at ERT (infusion) any needed Monday if not before a Cardiology Appt but still a break is nice!

I guess the only other semi-new things are the Thoracic Shunt seems to be moving even more or doing something as by the end of the day or more especially when I am sitting forward for any reasons that area on my mid-spine is pretty uncomfortable. Unsure why but a pain in the arsh! I see Dr.Bragg anyways next week to talk about placing this shunt back to drain in the pleural space (if we can given her concern there could be permanent scarring in the pleural space from the OHS-pleural tubes.) so I guess it isn't a huge deal right now.

Last but not least are a few mundane things. I guess my PCP would like an Audiology consult per the Genzyme-MPS I (ERT) protocol but gave me le-way on when to do this as long as I would do it sometime in the next few months. I figure if I scheduled it sometime in Dec or Jan would be around 2 yrs since the last time this was done. Ive not had this testing through Dean before but have some options where I would have it done and have the order to schedule when it works for me. I see the new (recommended by Dr.Bragg's Team) PMR dr at UW in Dec (for Botox) in addition to the Cardiology Appt., normal Cardiac Rehab Appts., Neuro-Endocrine fup and usual monthly Pain Mngmt fup but I also imagine there is never really any good time to do new appts.

ERT was switched to Weds this week due to the Nurse being sick Monday which worked out ok as I had to come to Milw for Cardiac Rehab anyways. Since the rest of the week was pretty crazy I just used Monday to get a little caught up on other things, work on some things for GSF (not that I had much luck with what I was trying to do/find!) and otherwise not to much.
Cardiac Rehab is going ok, we've slowwwllllyyy been increasing the levels of the different things I do and seems to be going ok. I've gotten to know some of the other pts there (older or much older people) so that makes the time on the machines go a little faster or otherwise I use my earphones and watch TV. I am not entirely sure how long this program goes on for but it seems to vary patient to patient, progress to progress.
Sunday School is going well, all of our kids sing on Sunday and otherwise I have just been alternating between Bible Bingo (which the kids love) and bringing in various craft ideas. Joan and I (we alternate weeks and group teach once a mo) have a pretty easy going group of kids-all girls right now. That's about all, thanks for stopping by,
Erica

Old news (sort of), New news, Catching up..

Dr.Simpson my PCP is wanting to schedule an appt due to muscle spasms in stomach (likely shunt related given previous history of these prior to our having moved the Thoracic shunt to the pleural space last winter and pre-the heart surgeries (having had to temporarily re-locate this shunt to drain in the abdomen where the 1st (VP) shunt lies) and due to muscle spasms in other areas. I've been having muscle-type spasms in L side (fingers, calves/leg, foot and oddly last in mid-side/upper stomach). These all tend to be short lived but repeatedly occur other than the abdomen/stomach spasms which go on for hours several times a wk. I think PCP perhaps wants to get a better idea of what it all is, i'll humor her I guess. I had just sent her an email-mychart asking if she had any thought on how I might be able to prevent it when she suggested seeing her given the longevity of some of the issues and how the shunt related spasms have been an on/off issue. Since I am still semi-fairly new to her (just over a year now) while she has learned a lot about my history and MPS she also still tries to stay very involved in what various specialists are recommending, adding potential recommendations and looking in to options. ------------------------------------------------------------------------------------------------------------------------- I emailed a bit w Dr.Bragg (Neurosurgeon) again last week, I had asked her in my initial email if she thought when we where able to move the thoracic shunt back is there ever a way to tunnel the valve of the shunt so it sits more on the side (more muscle/tissue/fat) so it isn't as noticeable (moves) when I might bend down/twist or sit back. There I s some concern there could be permanent scarring from the multiple heart surgeries, done in short order and thus the many chest-pleural tubes so will be a problem we'll have to sort out first. If there is no permanent scarring from the 2 heart surgeries and repeated chest tubes- 3 the 1st time, 4 the second time we'll move on. She did email back to say she was willing to consider other options for where the valve is secured/tied down (right now sits basically over the rib area/spine area between my spine and side and VERY noticeable when I move as it also moves. It isn't about a cosmetic issue but about the discomfort the valve places! Not an awesome feeling, not painful just not awesome feeling! I will see her sometime in mid-Nov (in 2 wks which worked best w my schedule) as this is coming up on week 7 or 8 post-the 2nd heart surgery and Cardiology has said we have to wait till closer to week 12 plus she would like to wait to around that timeframe to (3 month mark or so) in order that any scarring or issues from the chest tubes may be healed. I'll likely call and schedule next week for later in Nov. and we'll figure out a plan and schedule from there. <--- Obviously I was writing this in multiple parts! ------------------------------------------------------------------------------------------------------------------------ On a different note I was writing most of this from the National MPS Conf in San Antonio (is held in a different city each yr) and spent some time talking to a Genzyme-Registry person. I asked her if she 1. knew of any other way the registry could be updated w our info from say surgeries, bigger issues if our Genetics drs/team arent doing it and 2. would pts ever be able to access this info in the future? - Apparently Genzyme has roled out a newer format to their other Lysosomal-enzyme treated Registries and will be doing the same in late 2014/early 2015 for MPS I. In the newer formatted Registries any of our specialists will be able to get a passcode and be able to upload their info related to us (say a surgery or outcome) to keep the Registry better updated on all MPS I pts as right now they have very poor compliance w just Genetics drs having access.. I really think my Cardiologist and Endocrine dr would update their info as they are very interested in MPS I info and would then get info in return, in general. I tend to think Dr.Bragg might also from time to time be willing to update shunt/surgery info or have one of her staff and for that matter my PCP likely would to. I think it will be a good thing for access for all of the MPS I community! Those of us MPS I pts who have drs participating will then also be able to get a summary of our submitted info (can't remember this part for sure) compared to other MPS I pts or perhaps it will be just a summary of what was submitted on us. No-less it's a start and I think will be good for information for the MPS I community as more articles can then be written and shared... -------------------------------------------------------------------------------------------------------------------------- Otherwise things have been somewhat quiet; I was initially sitting at the San Antonio airport writing this waiting to fly home but am now at my Apt finishing. The Conf was fun - not much info if any for adults but nice to see friends. It is good the info is there for families w younger kids and hopefully someday there will be topics/speakers for adults to. Was interesting to see the ones you think are friends but w all the fb drama avoided me(and on the other hand how many made comments about what was going on supporting me/saying they know I would never do what was said); honestly im not sure I mind bc people who know me really know me! I shared 2 joined but separate rooms w Allison (Gene Spotlight) whom I work for, we each had our own bathrooms, each had double beds, our own mini-fridges, etc. We generally just left the door between our rooms open other than when we where changing or showering and had a great time catching up, talking. Either talking back and forth while we where each in our own rooms or sitting on each others beds chatting and going to the various sessions together. Thurs night before the MPS I-attenuated syndrome breakout session we met up w a friend of hers who lives in Tx and had driven down and went down to the San Antonio riverwalk to walk around and eat which was fun and nice. I'll try to attach a few pics, I didn't take a ton but took a few - i've attached a couple. :) ------------------------------------------------------------------------------------------------------------------------ I have a few appts (Cardiology for instance) coming up again in the next few weeks (have just mostly had routine things like Pain Mngmt fup and Cardiac Rehab in the past 2 weeks) and con't Cardiac Rehab and so if nothing else will update after those. We've cont'd to do labs for the Coumadin (blood thinner) weekly right now and are slowly tweaking the dose back up, Cardiology suspects it will semi-even out soon. I have labs again before ERT on mon. but right now the dose is 6mgs 5 days a week and 4mgs 2 days a week (M,F). ---------------------------------
Thanks for stopping by,
Erica

4th week Post-Op Cardiology Fup Appt

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

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

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

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

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

Erica

2nd post-op Cardiology Fup, Fluid

So this should be a fairly quick update. I saw Cardiology today (no tests other than Labs this week) and went ok. There continues to be fluid around the heart and lungs but my stomach and legs are pretty much back to my normal and weight wise I am back down to 116 pds (I varied between 112-118 pre-heart surgeries) so that is good, is nice that my pants fit again!

The big concern right now continues to be what causes this cough and so Dr.E (Cardiologist) would like an ENT consult (yay, hooray) so they can make sure it isn't an issue with either the voice area (cant remember how he worded it, maybe windpipe?) being partially paralyzed in the last surgery or reflux. None of us (NP, Cardiologist or I) really thinks it is these things as I've never had an issue with reflux (but i'll start a temporary protonix again for a week just to make sure, again not awesome to me) and the other issue would have been most likely to show up right after the 2nd surgery and this cough didn't start until about 12 days post-op and after I was home. We know it isn't a cold so the likely cause is just the fluid around heart and lungs and waiting for that to keep coming off both areas. If it got worse I have to call, hopefully it won't. Not sure I can take worse!

**I just thought of this so came back in to edit and in fact didn't even say anything to my parents as I didn't think of it (being I am at their house yet) but Dr.Earing did say today if the fluid didn't come off on it's own with the large diuretic doses they would likely have to go in and remove it I guess via a needle. He was totally against this for (I think) infection reasons  and I was like there is no way in hell anyone is jabbing some big arsh needle in my chest not to mention multiple times if I am awake (I imagine maybe they could use sedation/anesthesia) but like I said this would be total last ditch and not something he is leaning towards at this point. Come on fluid and GO AWAY!!!!

Labs for blood thinner have been all over the place since being discharged this last time; while they where often to low prior to the 2nd surgery so we kept having to increase the Coumadin dose, this time the levels have been to high in all but today's case (4 different labs) causing significant bruising in 2 of the cases and last week a milder, less spread out bruising. Today's lab was back to, to low so the Coumadin dose has been increased and Cardiology will re-check labs (if no issues, God willing) before next Thurs appt and Xray.

Otherwise Cardiologist is happy with this heart surgery out come and thinks energy will slowly come back. Part of my being so much more worn out this time is they believe and I can understand my body just not having had time to recover from the 1st surgery and it being so in-depth and long. I had the option to put off Cardiac Rehab for a few weeks (I really do feel kind of crappy) due to this cough as he was somewhat concerned it would worsen with that but he also gave me the option. I figure we can see how tomorrow goes and since I will only go once this week and once next week due to schedule issues on my part it shouldn't be to much overwhelming and I can always post-pone if needed. In that case the NP just said to let her know and she would talk to the Rehab Nursing staff.

That's about it, I am 3 weeks out from surgery, other than pain from this cough I am not feeling to bad (other than legs are something awful tired and feel very worn out when I walk) but still I am driving, doing my own thing and should be able to go back to my own Apt very soon. I want to clean the carpet but otherwise not much reason otherwise to not go back. :) I taught Sunday School this past Sunday for the 1st time this yr (our 2nd wk) and we have 6 girls and 1 boy. I had 2 of the kids in SS last year, 4 of the other girls in VBS and 1 new girl - all seem to have liked it despite my coughing to no end. :) Should be a good year.

Thanks for stopping by,

Erica

1st Post-op Cardiology Fup, Fluid everywhere

As fairly easy as the 1st heart surgery was this one has literally felt like it is kicking my butt, not only tired but short of breath with stairs or semi-longer distances, swelling (10 pd+ gain over a few days though thankfully seem to be getting that off fairly well now.)  On top of that loveliness have been dealing w and very un-successfully trying to control since the weekend a progressively hacking (dry) cough that feels like it is going to rip my chest wires apart. Super uncomfortable. I feel miserable is what it comes down to and have to admit even though I know it is wrong to do so have questioned why does one thing after another have to keep piling up? Can't I just feel ok for awhile?   - Late last week I think after I got discharged from CHW there started pretty extensive swelling and bruising with the bruising going from the sternum incision down the entire L side of my upper body and then going across the abdomen. The swelling was in my stomach (think few months preggo look) and ankles/legs  and cont'd to get worse Fri so I finally called the Cardiology Nurse.

  I  Talked to Dr.E's (Cardiologist) Nurse on Fri and initially they increased meds and I had to call Dr.Earing on Sat. morning. He at first wanted me to go to the ER but  I was able to convince him we should just watch it for another day on the new doses. Sunday I called again per his wishes and he adjusted another med + instead of seeing them Tues I saw them today (mon) after ERT which made for a  long day! Chest and Abdomen Xrays along w an Echo showed there is Fluid building up around the heart (there was a small amount when I was discharged but this has increased some, as well as fluid around the lungs and throughout the abdomen. So far since I was able to get some fluid off in a few days w the med adjustment we are  just watching this and will stay on the increased diuretic (gets fluid off) meds which are Lasix 40mgs 3x's a day and Aldactone 25mgs 2x's a day.
Labs also showed that the INR (blood thinner level) is also very high so does explain a lot of the bruising. This med dose was decreased from 6mgs 5 days a week, 4 mgs 2 days a week to 2 mgs until Thurs when they will re-check labs and if needed adjust dose. I can do those labs while I am at an outside but connected hospital. With the last heart surgery this Coumadin (blood thinner) dose was the opposite when I came home with us having to increase the strength at each weeks Echo/Cardiology Appt but likely has to do w the surgery and other things going on + not as great of an appetite due to this cough.

I follow up again with more Labs next Tues and Appt with Dr.Earing + the NP and not sure if they will tack on an Echo at the end again.

The week that I was discharged from CHW the Anesthesiologist stopped by my room again and has asked me if I would take some pictures for her to use. She also asked me if I would be interested in a Therapeutic Yoga program that is run at the same center she goes to for Yoga as she wondered if this wouldn't help some with overall body discomfort/joint issues. I said I was (why not I guess) and so she is going to get back to me with that info. I do PT and starting Cardiac Rehab but figure anything that might help is worth at least looking in to at worst it wouldn't be something i'd be interested in and at best would be useful. She wants to use the pics I've put together and am taking over the next 7 days (mouth opening as she suspects based off the 2 surgeries and having done both anesthesia intubations that having had ERT the day before surgery the 1st time helped w jaw opening vs the 2nd time I had missed the day before due to the holiday and so it had been a week). The other pic is the MPS chromosome tattoo.  I don't know if she will end up having time but she said she  would still like to write an article of her findings and what she used with these 2 heart surgeries and though she might not get a chance to do it till later in Fall she asked me to send her  a copy of these pics. If she is able to write the article that will be good for the MPS community and is non-identifying so no one would know who it was written about.

I'll try to update at least after next weeks Cardiology Appt.
Thanks for stopping by,

Erica

Cardiac Surgery - Take II (Finally, An update from last weeks surgery!)

 I apologize for no update since my Mom left off last Weds/Thurs updating my progress post surgery. I've meant to write a note sooner and just had no motivation or real energy to sit down and do it. I've not spent much time on my laptop mostly I guess due to being tired/lack of sleep and just plain putting off this and a few other things I should to get done!

Surgery itself according to the Anesthesiologist took about 10-12 hrs (I cant remember which) this time and was much more complicated than was expected/hoped for (The Surgeon had hoped to only have to re-seat the mechanical aortic valve) due to the issues going on and scar tissue. They had to re-do everything that was done In the last surgery 7 wks ago. As far as scar tissue they where amazed to note that this was a closer equivalent at my being 6 weeks post-op to someone who had, had cardiac surgery at say 3 years old and was then having revision surgery again 10 years later!? I guess in thinking about this after I heard the information I wasn't maybe entirely surprised as my Mom reminded me when I had called her that night that with Dr. Bragg's shunt surgeries she to would often comment how quickly the shunt tubing and pieces where scarred. It was always a similar issue w the shunts being so scarred, so early she typically couldn't visibly see them without dissecting down (something like that) vs in a average patient at say 6 weeks she would be able to identify the shunt location upon incision and  remove it. This makes me think then that it has to be something with the MPS storage affecting how our bodies deal with devices or foreign material? In one way I guess it is good in that it means said equipment is less likely to come lose after a few weeks, maybe?   The other comment I heard was that there was a large loss of blood (Dr.Taylor, the Anesthesiologist stopped by a bit earlier tonight) Dr.Taylor wanted to share a few things plus ask a few questions. She did share that the blood loss with the cardiac surgery a week ago was equaled to my near total body volume being lost and thus replaced with blood products.  Wow, at the amount of blood loss! I also don't think I've ever had to get blood/product transfusion with any of the very many other surgeries in the past including there was minimal blood loss w the 1st cardiac surgery last month so this was a first. Between this loss plus the heart/Lung machine the Anesthesiologist at least wandered if these 2 combinations but especially the Heart/Lung machine would explain the more difficult recovery this time? Last time I believe very little blood was lost and though I don't know the specific amount of time spent on the Heart/Lung Machine this time I  wonder if an increased time = increased loss of the synthetic enzyme (replacement, aldurazyme) so nothing to help my body really fight as hard? The Anesthesiologist felt the almost certain complete loss of the synthetic enzyme would have played a part and especially given I had already missed last weeks dose (due to Labor day we would have done it at clinic on Tues, the day of surgery) so less even available to begin with? I did get ERT here on the floor this week (so missed just a 2 wk span) which was complicated but my GC managed to get it done! The 'enzyme/bypass potential issue in MPS I pts was something the Anesthesiologist  wanted to explore and I think was going to either talk to or send her findings to my Cardiologist. I am going to email her to see if she would share anything she finds if she does look in to it.

Another issue the Anesthesiologist brought up to me was given how difficult this recovery was (multi-factorial likely) she wondered if she hadn't been wrong about it being better to bring me off of the intubation and sedation at post op day 1 (Weds) vs instead leaving the breathing tube in for 3 days like the 1st surgery 7 weeks ago? Given how much better I sailed through the recovery once I was awakened completely and extubated the 1st time around (better pain control didn't hurt the 1st time either) I am curious if she is right about waiting some? Her thought was maybe it is better to give the MPS body (specifically my body) a longer chance to recover and a little healing time first? She felt this particular surgery due to it's long length  would have put extreme un-due stress on the joints/muscles/spine (especially c-spine which is fused), the shunts/hydrocephalus, the Endocrine system (steroid need) and overall affects. She said she also wondered if giving an extra dose of the Aldurazyme before any MPS I pt has cardiac surgery and goes on by-pass could make a difference as there would be essentially extra enzyme there for the body to deal with regardless of the by-pass filtering. She I think was also going to talk to my Cardiologist about this. Hopefully we wont need to find out for the future but is really nice to know how much time she (Anesthesiol) put in to my care, how much fup she did post-op (stopping by daily through Fri night and again this week) and that even though she doesn't have to she wanted to help both now and if needed (hopefully not!) make a difference in out-comes in the future.
Last but perhaps most memorable was this Anesthesiologist when I was lying on the OR table and they where hooking up all the monitors was talking about my MPS I 'chromosome tattoo' and how my Surgeon had taken a picture of it the last surgery. She wondered if I would mind signing a HIPPAA release form for her so she could take a picture and potentially write an article related to her/my experiences with these 2 cardiac valve surgeries and the TEE Anesthesia and she thought the chromosome tattoo would be a nice addition/pictorial for any article. I hope she does write an article as I think it is sorely needed! I've shared her letter/summary here and on fb previously for other MPS Families to potentially use as a draft in order to get copies of their own or their child's Anesthesiologist impression but feel there are sorely needed more medical journal articles on MPS and cardiac issues and cardiac issues/anesthesia!

There has been strong talk of was the valve issues this 2nd time being caused by Bacterial Endocarditis due to the look of it once I was opened up on the table but no official consensus could really be made on this. The Cardiology/Cardiovascular teams initially intended to send me home on 6 weeks of 3-4 time daily IV (hours long) antibiotic but have now opted to do an Oral Med and watch the valve closely. I will fup with them as soon as Monday.

Otherwise the worst issue has been I don't have the energy I had with the 1st surgery but everyone feels this is likely due to this 2nd 're-do' surgery taking so long (10-12 hrs), being on by-pass so long during that as well as flat on my back during the surgery (hard on an MPS body) and that the healing process is just going to take longer till I notice probably even subtle differences and my body can catch up. Breathing has also been an issue but this is also felt to be due to my body adjusting to the new heart function/the surgery and will likely take a few weeks to feel like I don't have to struggle to breathe or more aptly feel like I am suffocating in trying to get air. My oxygen level has been at or right near 100% and Blood pressure hasn't been to out of normal if at all while pulse is pretty high compared to normal but that is being treated with a different beta blocker for now so it really should be just a 'give it time to heal' thing!

Lots going on! Should still be able to get out of the hospital tomorrow (Weds) which is awesome! 2 friends of mine are coming to pick me up and our plan is to go get lunch and ice cream before we head home. We all 3 also have a Sunday School meeting tomorrow night so I am hoping I can either go to my Apt once we get home or hang with one of those 2 till the meeting starts later (6:30).. We'll see how it all plays out!

On a side note my Cardiologist must have asked my Cardiothoracic Surgeon to call me and he did this afternoon, in amongst 1/2 a doz Residents being in my Hospital room but was still a good talk.
Thanks for stopping by,

Erica

Erica is having a very rough time of it.  I can only hope and pray that she will be back posting on here very soon.  God Bless You Erica!  Mom loves you with all of her heart and soul.

It is still Mom updating tonight.  Erica had a fairly uncomfortable day today.  Breathing tube out but everything else still on and in.  They are changing her pain meds tonight so hopefully she will get some rest.  She did get to drink a little soda this afternoon and I think it tasted pretty good!  One day at a time and hopefully -- moving forward all the way this time!

Erica's surgery went good today.  Totally did everything over.  Doctor feels confident he got it right this time.  Will hope and pray day after day that this time the outcome is 100% successful.  Long day so will update tomorrow -- or even better -- may be Erica will update.

Cardiology Fup, OHS- Aortic Valve re-replacement, Last wk's Fup w Neurosurgery.

Well, wow? What a few days filled with way to much news? Yesterday I saw Cardiology for the 2 week fup, Labs and repeat Echo (ultrasound of the heart). Not the best news. The Echo continues to show increased leaking (perivalvar) around the new mechanical valve and is to the point now where the leaking is severe.  Dr.E (Cardiologist) said he could hear the difference even more so while listening to heart beat/rhythm and pulses in wrists/feet where much more palpable as well he had concerns due to my hands being so cold - to that I just said if they would turn the AC down a bit we all might not feel like we where artic frost! Kidding aside this is crappy news and not what I wanted to hear although at the same time I said to him and Nancy (NP) that I also didn't know what to "wish" for? No changes but know they expect there will be worsening as it had continued to and just a matter of when? Or wish for changes but know it would mean having to go back in and do most of the open heart surgery (cracking chest, bi-pass, anesthesia, new healing, etc) again fairly soon (next week already it turns out) but then know at least we would be only a fair amount of weeks away from being able to place the current Thoracic Peritoneal shunt back to it's former (pre-cardiac surgeries) location in the Pleural space? No-win really and not a decision that ultimately no matter was or would have been up to me anyways. I guess I got my "decision" made for me by my body anyways although I admit I was pretty surprised when the CV Surgeon's Secretary called me already Thurs afternoon, not long after I had left the Appt and said they where scheduling it for Tues!? I had tried bartering with Dr.Earing to wait and do the surgery at minimum in mid-Sept (not thinking they would rush it) or even mid-Oct. due to a bunch of stuff going on with Sunday School (planning, a 1-day Conference, meetings/Canvassing, etc.) I think he (Dr.E) just thought that was funny that I wanted to wait as far out as Oct. =/ I thought it seemed like a good plan albeit all the longer we'd have to wait on the shunt business!?

The Aortic Valve replacement-revision is set for Tues., next week, Sept 3rd. The plan is to  take out and re-seat the new/leaking valve to get rid of the post-surgery issues or if they feel it would be better they will put in a new mechanical valve and whatever else if anything needs to be done. . What they do depends what they find once I am on bi-pass and opened up. Surgery is scheduled to last from 7:30-1:30.
The Pre-Op Appt  which included an Appt w the NP and CV Surgeon's PA, Chest Xray (see that faint glowing, green light in the distance?) and Labs + was to include an EKG. Surgery will be at CHW again and I requested plus was able to get assigned the same Anesthesiologist as last time so that at least is good given she is familiar w my airway now and she was very nice as well very well recommended by my Cardiologist and the Pre-Op NP last time and both where glad she was going to do my case this time.

Did you know that scar tissue at least in heart surgery peaks at week 5/6 post-surgery? I would have guessed if not told that it would be more like 3-6 months post-surgery! This part could make surgery a bit more difficult/longer but they are familiar with my anatomy, I am stopping the Coumadin (blood thinner) as of Sat. and they will have blood on hand if needed. - In the mean time I get to walk around w these very nifty (note sarcasm) white blood product-ID bands all weekend. Yippee! I strongly dislike the feel of these sort of things so am wondering if there might be a way to remove them and re-anchor without being to noticeable. I've not had a chance to look to hard at this yet.

I've heard from numerous Cardiology/Cardiovascular Providers over the past 2 days at Appts how they are amazed at my ability to " just role with the punches" and how so many of their staff comment they like working with me because I never get upset at them for having to give bad news. I am glad actually that people will share this sentiments b/c while I rarely share how I feel about stuff on the outside it does frustrate me that we have to do this again. Not frustrate in a way that I feel it is anyone's fault just more that I feel tired of the MPS sometimes, of so little knowledge about our issues and that when drs do encounter our issues how come there isn't a place they can go and publish or just share that info for the collective good of the MPS community? I know there is Genzyme's Registry but that is only for enrolled providers (typically Genetics  I think) and even then it seems few providers share what they see at least mine don't because they don't have time. I wish there was a way for our specialists to share what they see or find in us pts to help other providers? Non-the-less my frustration is calmed a bit (perhaps selfishly) knowing Providers think I am doing an ok job and that they like working with me apparently because I do know like anyone there ARE times I am likely not  all peachy and sun-shiny nice and i'd like nothing more than to punch a wall!


Last but not least I saw Dr.Bragg last Thurs for (approx.) 1 month follow up  and I can tell she feels bad about the new cardiac issues though she made the comment (purely joking) something to the effect of "I've not worn your parents in to new complications occurring?" - One of the many things I like about her, she can always put a light spin on a crap situation. Her support and her updating her team + the D6-4 Nurses after the last surgery + I imagine this time (she reads/sees my fb) is nice as those Nurses are in a way like another form of family.

And really, truly last! Last night after Church the  Pastor (around my age, married w a awefully cute 1 1/2 yr old daughter) invited myself, my Sunday School Co-Teacher and another friend over to his  house which was nice. I cant explain it but these are amazing friends and I never have to be anything special around them, they may not totally understand the MPS but both Joan (co-teacher) and Pastor S. are always there to visit in the hospital and Joan and I and another of our Sun. School Teachers have a great time when together either their visiting when I am in-pt, we often do lunch these days after Church when we are all there on Sundays or at our Sunday School meetings. Love them - given I am not much for hanging out with people or going out they really are fun to be around! =)

All for now, since I likely I won't update before Tues's surgery i'll have my Mom update this and fb on Tues and as needed throughout the week. If I am sedated again,  if the same plan as last surgery 5 weeks ago is followed Mom will do short updates till I am able again.
Thanks for stopping by,

Erica