Friday, July 26, 2019

Surgeries, Procedures, Surgeries... but progress

General Surgery - bilateral inguinal (basically groin) hernia surgery w/mesh is in about a week (8/1) at St.Mary's Hospital in Madison. I will admit it makes me a little nervous having no idea who the anesthesia team will be but I can only pray they will have done their research on my case AND will heed my Primary drs notes + past anesthesia notes re airway!
I did see my PCPs pre-op note (I get copies of many of my drs notes to keep in my own records) and she did a really good job emphasizing the airway difficulty issues as well as emphasizing the cardiac-fluid concerns and that the endocrine (cortisol) dosing has to be followed amongst other stuff. I am always grateful she is so thorough.

Pulmonary - Bronchoscopy scheduled (8/8, Froedtert Hospital, where my Lung dr is). This as I think I posted in my last update here is to flush the area where the lung nodules are growing or try to flush the one area where 1 of the nodules is growing as they are not able to be biopsied directly by bronchoscopy and then take that fluid and run labs if I remember right to see if they can figure out what these nodules are caused from. It is not my Lung dr doing the procedure (apparently only some Lung drs doing interventional procedures; who knew!?!) so this to I will pray the dr.and anesthesia drs on my case are capable and careful! My Endocrine dr. has sent over her steroid dosing recommendations for this procedure to my pulmonary team.

Peds Neurosurgery Appt  - Halellujah! We have a date folks!! Last wk I was in Mn for the final longitudinal MPS I study tracking memory/cognitive function in us MPS I pts. - I have to preface what I am going to write by saying I semi jokingly said to the person doing my testing (I had had her last yr as well) that they needed to change up their tests as I literally have most of the stuff memorized and soooo borrriing, lol). Well, lol it didn't matter this year that i've done those same tests (literally the same tests) for probably going on 5 yrs if not even longer than that as I apparently did very badly on some of the parts and testing (oops) and what they saw is indicative of changes these drs see in patients with my genetic disorder who either have un-diagnosed hydrocephalus or as is my case have treated hydrocephalus that is not being well managed. 

This needless led to not only the Study dr (head Neuropsych dr., she's very nice and a asset to our MPS I Community) laying out her concerns but she also reached out to the Metabolic Genetics dr (who I know reasonably well, this is the dr I spoke with at NIH a little over a year ago and have known for years through the UofMn though he is not my dr). He in turn called me and we spoke after he had spoken to their Peds Neurosurgery dept (the Neurosurgeon i've seen there a few times has apparently left clinical practice and doing administrative work but the Nrsgn who took over for him is a former Resident at UW so she was familiar with (remembered) my complex case when the Genetics dr talked to her I guess.

I flew home that next day and saw my Primary dr for pre-op a couple days later. She reached out to my Nrsgn at UW expressing her concerns and the UofMn drs concerns amongst all the other concerns and other of my drs who have concerns with these on-going weight and other changes. This in turn I heard from my Nrsgns office and had an appt with him today right after a quick brain MRI.

Honestly I was surprised how well the appt went but he agrees we need to do something and asked did I have an opinion which shunt may not be working right?  Honestly if I had to guess i'd say the LP Shunt but at the same time I honestly have very little idea. So the plan is to schedule surgery which we have; he was wanting to do it already next wk but b/c I have the hernia surgery next Thurs and it is at Dean (St.Mary's) and then I have the lung-bronchoscopy procedure the week after (Froedtert) I asked if we could postpone it a little.
 I actually reallllyyyyy want to feel better but I also know that much anesthesia and steroids so close together not to mention I imagine the hernia surgery recovery may be a little uncomfortable i'd rather just wait atleast a few wks. so we settled for Aug 28th.
This does mean having to get a new pre-op with my Primary dr for that surgery but honestly it is worth it to do that again to have a 2 wk break between the hernia surgery and bronchoscopy (1 week between these 2)  and then 2wks between those 2 things and the shunt surgery revision. Even this all is waayyyyy toooo much but still better than 1 right after another after another! Atleast I have a PCP who is remarkably easy to like so she's not the worst dr to have to see (but like anything if could have would have been better to not have to do this again =)  ).

Re the VPL Shunt surgery Dr.Iskandar said if this wasn't succesful then we'd plan to look at the LP Shunt via surgery but he'd talk to his partners and come up  with a plan how best to approach that shunt and area given the pretty significant difficulties we've had in the past and what to do with the shunt to try to make it function better. If we do have to do this I will ask (beg, lol?) him to please move the valve and reservoir which for the past few years have sat on my low back (a very present bump that sticks out like a sore thumb and super uncomfortable when I drive or when I walk for longer periods) to my side which right now sit on my low back and are sooo freaking uncomfortable  when I drive or sit certain ways. It would be amazing to have those 2 things back on my side where they pretty much never caused any issue!
I'll update again when there's something to update including with the heart monitor results. I did see the monitor report on mychart but I don't really know what it means (was very different than past ones but may well be innocent changes).
Thanks for stopping by,


Saturday, July 13, 2019

New Apt., Pulmonary (On-going Lung changes), Mn Study.. Other med stuff..

Move to new Apt.
I finally moved to my new Apt about 2wks ago. - I like (surprisingly) being back in BD and nice to close to multiple (better) grocery stores + a hop/skip (less, lol) to the hwy to get to Madison and really only about 15more mins to get to Milw. so that's still not bad either. My cats have finally settled in and I have new critters here to (squirrels and birds =) ). My nephew loves all the kids at this Apt complex (is much bigger but also much quieter here) and my backyard is pretty big so Z has lots of area to run around and explore and bird hunt (+ the other kids of which he already made friends with a few last wknd).
Albeit it's only been about 2 wks but when I 1st moved to this new Apt and still on occasion I think about all I went through while living in Juneau (in BD now). All the surgeries (3 Open Heart surgeries, 40-some shunt/spine surgeries, 3 hand surgeries, and probably something I am forgetting). Several of my nieces and nephews where born while I lived in Juneau, I started teaching Sunday School at St.John's there (7 yrs I think this yr!?!!?) and now I coordinate or run the SS program (I have plenty of people I can turn to for back up on this and my SS Teachers are great if I ask them to help with something or something we need to do).
My Grey was just a kitten - a baby teeny, tiny kitten when I lived there and her Mom (my Ally cat, I had to have her put to sleep last yr =/ ) was a tiny, stray i'd just brought in right before I moved to that Apt. - I found Dr.Bragg and my current PCP amongst several others on my current time while living there.
The many trips to Milw for ERT (Infusion) and then later to Madison for my weekly infusion from that Apt. - The many times my Dad (or my brother) would have to come and help with my cars (I had atleast 3 while I lived in Juneau - the 02' Saturn, The Grand Am', and 01' Alero, then my last 11' Aveo  before buying my current 16' Chevy Cruz. A car I swore i'd never spend that much $$ on! Oy, but I like it and don't generally mind the payments to much. I have it almost 1/2 paid off 6mo in so that's a bonus (so grateful for the part time work I have. that to I started 7 yrs ago while living in Juneau., in fact I was in-patient when the Foundation contacted me about possibly working for them after my previous work (APF) had just recently shut down. - So many memories!
It kind of makes me wonder what all will transpire while at this Apt. - More good memories and changes I hope amongst all the medical stuff and hopefully no losses to my medical team! 

Pulmonary, I had the 3mo CT Scan follow up (Lung dr, Team monitoring the nodules that have grown the past year or so on CT Scans and 3mo ago the Radiologist reading the report recommended these be repeated again at a 3mo interval to monitor any changes).
Anyways so my Pulmonary (Lung) dr., messaged me back a few days after the scans and the 2 nodules had grown again albeit they are still very small. Because of the area they area growing in (L upper lung lobe and R infrahilar nodule) - Honestly I have pretty much no idea what it means other than they are still small but the changes where 7mm 3mo ago to 8mm now and 5mm 10/15/18.  The R nodule  measured 14mm (axial series) and 11mm up to 18mm (craniocaudal dimension) 3mo ago
previously 13mm. Are you confused yet? Lol, me to! (laughing b/c for real). There are other nodules apparently per the scan but unchanged so those aren't of concern.

It is very possible these 2 growrths are infection related to the humira I take.
So the plan is 1. 2 specialized urine tests to check for fungal infections (my Primary dr. put in the order for my Lung dr and will get these done next wk when at infusion).   2. Dr.Biller feels a bronchoscopy (basically a scope of lungs) is also warranted though b/c the area these are in is very hard to access they plan is to instead start with a saline flush of 1 of the areas and then test that flushed saline via lab to check for infection.
If those are negative I am not sure what happens next but will worry about that all when/or if needed (possible things could be but less likely cancer, infection, and may have been 1 other thing).
 Right now there's just enough other things to deal with!

UofMn AND headed to UofMn tmrw for the longitudinal MPS I study to so enough other things to get through 1st.  (I think this is the last visit) finish that study up. Basically a 1/2 day of realllyyyyyyyy boring testing. Like mind numbingly boring testing!
But if we as MPS I Adults don't do it then how do we expect Drs/Researchers to ever understand our brain and memory issues? So thus I have done the study the past few years and will do the last study visit. Hopefully honestly this information will be useful anyways for the new drug my PCP and the BioTech company are working to get approval for. While in Mn i'll see a few of my friends so that atleast is a fun part.
Bay Beach Amusement Park               
     Before the upcoming hernia surgery (Aug. 1st), and Pulmonary-Bronchoscopy I am going to my most very favorite place with family. I have pre-op late next wk but then convinced family we should go to Bay Beach (Amusement Park, Green Bay) this yr. We haven't been there in a year or 2 and I absolutely LOVE that place so am super excited!!! I am like a kid in a candy shop when it comes to carnival type rides which is what Bay Beach is (rides, rides, rides!!). Can't wait!!
General Surgery, Hernias  - This has been a little difficult bowel wise though is this related to the hernias? Guess won't really know till after the surgery and even that will likely take atleast a few days as I am sure it will be atleast some painful and all the air they place in ones bowel (I think? Given it's an open surgery?) with surgery and anesthesia meds will likely screw up bowels even more. Such joy. I can hardly wait. I seriously pray just about every day if not every day (amongst other stuff and not always just asking God for help but thanking him to) that something be sorted out soon with atleast one of my issues. The bowel stuff? Headaches (over a year later and no closer to an answer. Still waiting on this stupid intracranial pressure monitoring device =0/ )  or other issues but those 2 are the top things at the moment and the most affect on my daily life. I pray this surgery helps the stomach stuff.

Cardiology - Heart monitoring
I have been wearing a newer heart monitoring device (similar to the Zio heart monitor i've had at other times)  the past 2wks though have to wear it about 5 more days as there where about 5 days the monitor came off and I had to wait for new patches to adhere the monitor to my skin with. The Zio definetly works easier AND has a button on it you can just press if there are any episodes. This one does now have that and so you have to carry a little paper booklet around which I have not multiple times (like when I was moving). Kind of annoying! Makes ya appreciate those things that are easier though. =)
Thanks for stopping by,