Thursday, November 28, 2013

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!


Wednesday, November 27, 2013

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!


Friday, November 22, 2013

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,

Thursday, November 14, 2013

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,

Thursday, November 7, 2013

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,