Wednesday, June 19, 2019

General Surgery; moving fwd w/ surgery. Compassionate Access drug...

Just a update - surprising I am writing this for once the same day as an appt! Anyways a few things to update - both the ultrasound/General Surgery f/up and then a update on the compassionate access drug.

General Surgery Appt / Ultrasound
This Appt went well - the ultrasound Tech told me right at the ultrasound appt she confirmed there where 2 inguinal hernias on the ultrasound but thankfully the scan was super fast and I was actually able to stand up while she did this so much easier on my head and my body!

I saw my General Surgeon in clinic (love that her Secretary was able to schedule this f/up right after the ultrasound!) and she confirmed what the Ultrasound Tech told me. After going over her concerns and what would/could possibly be problematic with the surgery approach she laid out that she would in fact use mesh for this open (vs laparoscopic) surgery approach to give the best chance of these hernias not recurring due to my underlying genetic disorder (and our high propensity for hernias).
Surgery will be at the hospital vs at the out-patient center but likely should still be able to go home the same day. My LP Shunt (drains to peritoneal (or essentially abdomen) shouldn't be an issue and especially b/c she is doing the open surgery approach.

Surgery itself is scheduled for Aug 1st so I should be all moved and settled I would hope in my new Apt! I love that this Surgeon is well aware of some of the issues she may face and that are concerns for my case like airway, the need for cortisol replacement and my being on humira which she/her team have already spoken to my Rheumatologist and have a plan in place for the humira dosing. She sounds like she'll be a great advocate for the need for the cortisol should anesthesia give any issues (and my endocrine dr and PCP always do a great job sending this info over to any Surgeons I have).

Compassionate Access Drug
I again can't say a lot but the new goal per my PCP at an appt today (whirlwind day, 1st the ultrasound then General Surgery and the PCP Appt at a different clinic/site) would be to do a 5-6mo trial of this alternate drug as that's apparently when pts. notice most improvement if they are going to if I remember what she told me right. 
She's figuring out dosing and infusion time for my case to avoid the same fluid overload we deal with w/Aldurazyme when I was getting it in the total 250ml volume dosing over 4hrs (my dose now is 150ml over 6hrs with the same actual med dose based on weight). I forget if there was something else but the other thing they'll figure out is with this other drug there's increased chance/risk for blood sugar increases/spikes I guess due to how the med is mixed or given (I don't remember exactly why this was but do remember a few friends having this issue in the study) but Dr.Simpson said 1 potential way they avoid this (if I remember right which may be a bit questionable we talked about ALOT today! =)  ) is eating candy/food at various times during. I kind of laughed when we had talked about this and said once I get past the 1st few hours of the morning at infusion food isn't usually an issue. Sometimes I just munch a little, other times I eat more but I always pack a variety of snacks and almost always some food or another for a lunch (PBJ (1 of my favorites!) or a salad or if I've brought something from when at  my parents) to eat and typically almost always have some candy in my purse or bag. 

Dr.Simpson also said she had reached out to and talked to my Cardiologist (Dr.Earing) and Neurosugeon (Dr.Iskandar) and both signed off on my doing this drug as an alternate trial to what I receive now every wk. I do not remember what exactly concern Dr.Iskandar asked Dr.Simpson about, if there was an increased infection risk maybe (?) but she seemed to have gotten past that and was happy with both signing off. 

Right now Dr.Simpson just needs some final paperwork from the study person we both met a few wks ago and per an email earlier tonight from the CEO is sounds like that would be sent to Dr.Simpson next wk. Once the paperwork is submitted to the FDA the CEO just asked that I (or Dr.Simpson) let him know and he'd label the drug vials for my case. SO HAPPY!!! Literally a month ago we didn't think this was going to happen!!!
                                                Visit at Infusion from Genzyme Patient Liaison
Even in the toughest times God is ALWAYS with us and always has a plan. His timing is not always my timing but then sometimes he just works in mysterious ways and we get fantastic surprises to! I am so excited as honestly the shunt/headache stuff and other stuff has been sooo trying at times I just needed something positive health wise (and today it came in spades, even the general surgery stuff, it sucks needing a surgery but I am grateful to have a surgeon I completely feel confident and comfortable  and who I feel confident is a team player in this surgery + looking out for all my issues not just the part she'll directly work on).
Although I never stopped trusting that God had/has a plan this all just reinforces how good and gracious our heavenly father is.  I am so grateful for my faith and for all the people God places in my life.
Thank you to those in my family, my providers who never give up even when it's sometimes tedious or not clear and for my friends who well some of you just get all this like few other can!
Thank you,


Monday, June 17, 2019

General Surgery Appt (possible surgery), Cardiology Appt, etc.

General Surgery
Sorry for not updating sooner from the other weeks update but I saw the General Surgeon a couple wks ago who like my PCP suspects the swollen area in my abdomen/groin is a hernia but wanted to do a ultrasound 1st just to rule out it isn't enlarged lymph nodes 1st. That is scheduled for Weds and then I f/up with the General Surgeon right after (glad they could be coordinated together! I liked this Surgeon and her Nurse for that). She just said if it is hernia and requires surgery she'd opt to do a open approach versus laparoscopic due to my being on blood thinners (lovenox especially) and needing to stay on these right up till any surgery is done. 
Obviously a laparoscopic approach is less invasive so that would be a bummer but I guess will just see what becomes of the ultrasound and appt on Weds.

Compassionate Access Drug 
In the mean time my PCP heard from the company re the drug and things changed + paperwork is being submitted. I don't remember what I updated last time but the drug person for that company came by infusion a few wks ago and met with my PCP + my PCP then sent her down my way. The drug Rep thought it was possible we could have approval as soon as July. Would be awesome!

Last wk I taught VBS at Church which went really well and was fun. I taught 5th and 6th grade again this yr (I teach 3rd/4th for Sunday School but had my choice for VBS again) and really is neat to teach a different grade. Many of the kids where/are my former SS kids and/or kids I had in prior years for VBS so went well. I have TERRIBLE issues with names but have a few work arounds for my aweful memory and my 3 helpers where also great this yr (1 of which is 1 of my regular (pre-k/k) Sunday School Teachers so she knows how I do things and to we know each other well. I rescheduled everything but infusion so was a nice week with a nice 
break from appts!

Cardiology Appt
Saw Cardiology the other week and was a good appt to. Usual just watching various things but overall stable and no med changes. His biggest concern is the not being able to be on regular coumadin (blood thinner) and needing to be on lovenox instead as that isn't actually recommended or approved for long term for mechanical valves but overall it really did seem to be an okay appt.
Zander's 13th Birthday
Last this Sunday we celebrated my nephew's (13th!) Birthday and my Dad's birthday as well as of course Father's day for my Dad. Was a fun weekend. One of my closest friends (fellow SS Teacher :) and 1 of  Z's favorites) came as well as a bunch of family to his party which my parents held at their house. Was a nice day albeit a tad chilly for being June!  =) 

                       Happy 13th my bird and tree loving kiddo!

I am probably forgetting something but will try to update after Weds  General Surgery Appt.
Thanks for checking in,