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!
Thanks for stopping by,
Erica
