Thursday, February 1, 2018

Fevers, Peds Neurosurgery; Shunt taps. Speaking at NIH-Rare Disease mtng

I've been having fevers during infusion for last few months but these have started  occurring outside of infusions at times now. I had talked to my Primary dr to give her a heads up awhile back (I really wasn't to concerned but my Infusion Nurse would mention it every wk and ask if I felt ok). My Primary dr then raised concern for possible Port (access device) or shunt infection. Blood cultures came back negative for the Port and we think they likely will for the shunts to but I am praying so!
 We all as I think I may have previously wrote about feel it's probably pretty unlikely but at the same time the fevers are coming from some where and continuing for months + started before I started the Humira injections (which can lower ones immune system).

I had asked my former Neurosurgeon if she would be concerned about these fevers and she said yes, given our past history with slower growing infections that these fevers could be caused by a CSF (Spinal Fluid) infection and she felt we needed to tap the shunts + test fluid to be sure.
A few wks back my Primary dr had talked to my Neurosurgeon and shared her concern + asked him to tap the shunts and test CSF (Spinal fluid). At the time he had agreed to it but then opted to wait. It's now some wks later and this is still occurring so after another message Neurosurgeon had agreed to the shunt taps and was done earlier today, Thurs (God help me, this wk was initially really quiet and then ended up being  1 appt after another after another Tues through Fri all an hour at minimum 1 way drive.). I am tired!

Thankfully after the shunt taps and removing some fluid I felt good for awhile and was AMAZING the immediate hours after - I literally got a ton of errands done before then having to drive back to BD and go to Physical Therapy. After PT I ran a couple more small errands and went to my Parents for supper. SOOO NICE!!!! I SOOOO freaking wish my Neurosurgeon could understand that difference!
 It's not just subtle either but a HUGE difference when that extra CSF is off! My Physical Therapist said to me (as he does when he can tell I felt good) "I can always tell by your eyes, they are brighter when you don't have a headache!"  which is also a comment Dr.Bragg used to make.
 Aghhh I want to feel that good ALLL the time!!!! How do I convince my Neurosurgeon of this and to look past the fact I do feeling bad really damn well (unfriken fortunately I think sometimes! I don't know how to show him as I can't whine and complain and carry on?!?!?

 I saw Audiology Weds (to fix my 1 hearing aid, part of the end just needed to be put back together and  Flast for the week is  INR for Cardiology. That atleast was finally in-a safe (high safe actually!) range when tested Tues so Cardiology dropped the dose a little and wanted to test again Fri. I am HOPING it will be good so I don't have to try and figure out how to get it tested while in San Diego next wk! The level is I'm guessing still fairly higher range as during the shunt taps today I was bleeding all over - it was a spinal fluid-blood leaking mess!

Lastly, I don't think I wrote about it here last time but I was asked by Sangamo (Biotech/Pharma company I spoke at in San Francisco back in Oct) to speak at the NIH in March for a Rare Disease mtng. I'll be the Patient rep on a 4 person panel talking specifically about gene editing/gene therapy.
 The other 3 members a physician (Dr.Whitley from UofMn), the CEO of Sangamo  (Dr.Macrae) and another Reseacher. I'll have just 5mins so I've written a tentative speech and will be working with someone from Sangamo on editing and getting this set although the person I mainly work with at Sangamo seemed to really like the speech as I've written it. =)
It is  realllly neat and an honor to have the opportunity to speak at the National Institutes of Health (NIH)!

I am headed to the WORLD Mtng Sunday although watching the weather here and in Chicago, a but nervous! Hoping the snow holds off and my flights do not get delayed!!!!
Will update soon,
Thanks for stopping by,


Friday, January 19, 2018

Good ole' shunts... Frustrating as ?? Provider communication..

Sharing a bit from my latest post (see below) those who follow this blog may have read the prior update.

If you've read my last post you'll know what's been going on already but after having saw my PCP the other day (for something unrelated to the shunts but I asked her about the symptoms since there) she talked to my Peds Neurosurgeon (Dr.Simpson typically will only call Providers of mine if she has a concern or something she needs to verify say for a surgery). She messaged me after the Appt to let me know she had called him and related her concerns for possible infection - especially given being on the Humira. She and I both agree/agreed infection risk is unlikely but b/c of the relatively newly started Humira (which can lower immune system/ability to fight infections) she wanted to rule out an infection.
I know blood tests for the actual Humira study last wk per my Study Team where all normal so it really was just concerns b/c of the shunts, the heart issues and the shunts + have had new un-explained low grade fevers the past few days and then fevers on and off during my infusions the last few months (I can't remember how Dr.Simpson explained how infusion could stir up if there is a infection laying dormant and thus bring out the short term fevers but it made sense when she said it).

Non the less so after she talked to Dr.Iskandar (Peds Nrsgn) the other day the Appt with him was moved up as symptoms had been downright AWEFUL Weds night and Thurs. He agreed w/her about doing shunt tap, testing CSF just to be sure and rule out a infection. That Appt was today and I guess I rarely if ever go in to these Neurosurg. Appts expecting anything BUT I atleast hoped we night just tap the shunts atleast rule out for sure if there is or is not an infection. I really do agree there may not be BUT there also is little other reason to explain these symptoms. Just once it would be nice to rule in or out a shunt issue quickly?..

None-the-less we wait and see and believe me i'll pray it just goes away. Per a MyChart msg from my PCP Dr.Iskandar did call her and update her after the Appt. so if nothing else atleast was listening to what I said in part while there (which was in a way a wasted trip, if he wasn't going to tap the shunts I really shouldn't have needed to go). Non-the-less I guess if nothing else he asked me a question about another of my Providers (Cardiology) and why did I think that dr would be upset (or whatever word he used) with him. To that I honestly didn't really know what to say but communication, it really comes down to my Providers and b/c I am a complex (really complex) case and 1 issue almost always has an impact on any other given issue I deal with (be it shunts to heart or shunts to blood thinners, shunts to endocrine issues, and so on, etc). Key really I tried to emphasize is it's really important he be willing to work with the rest of my Drs., pick up the phone or message them if we're dealing with an issue that might impact their issue or a surgery.. Was a fairly long but good conversation. I try to not do this but today when he'd try to stop what I was saying to make a comment (for instance that he understood) I cut back in without trying to be rude and re-emphasized how important this was, that communication really has to happen to make care better between him and other of my Providers and make issues less complicated. Given he called Dr.Simpson after the Appt he atleast was listening. He also asked that I tell him when I thought he needed to/should talk to 1 of my other Providers which I admit is really outside my comfort zone, I don't entirely always know when he should but together we can try to figure it out. I guess I'm just used to others of my Providers that do it automatically and let me know after.

My PCP asked that I let Cardiology know what was going on as I'd gained 4pds overnight (stomach I think, has been really swollen) and she increased not only the Colchicine (an anti inflammatory type drug) + the Lasix (water pill that keeps fluid off for heart purposes) which I had cut the dose a few months ago she upped back to 60mgs 2x a day for a few days and Cardiology agreed with. Cardiology Nurse (she's amazing, always grateful for the good Drs, Nurses on my Team!) just asked that I update them on Monday and they'd check INR (blood thinner dose) Tues per usual. That I suspect will be a mess as I haven't been eating much (I am on Lovenox already anyways).

Hopefully above all else these symptoms just will settle!!!! I feel bad and to boot was going to get my nephew this wknd and feel bad for not doing that. - I am VERY sure Z is disappointed and I am disappointed to not have him. =/  Hopefully next wknd, I miss him.

Lastly my study dr. (Humira, Dr.Polgreen) asked me to keep her updated, she per her email may opt to hold the Humira dose next wk till we get these symptoms sorted out if it has not calmed. I am praying all will have settled. I actually think in small ways the Humira is helping (see my last post). I am usually really hesitant to be sure about something like a placebo vs real drug but this time I truly think it may be the real drug. LOL, i'll really think I'm crazy if at wk 16 it's not!
Will try to update again soon.
Thanks for stopping by,


Past few weeks to Tues -
Shunts - so good, so bad and so ?
Late last wk, beginning of this wk I couldn't help but smile to myself as it's really seems like I may be on the Humira (vs placebo) - obviously I won't really know till week 16 but for several reasons I suspect it's actual drug. Anyways 1 of the 1st things I've noticed is instead of waking up 3-4 or more times a night (which when the shunts are being good that 3-4 time a night for me is actually relatively good. When the shunts are being an issue I often wake up 5-6+ times a night to go to the bathrm. Anyways so the past wk or so up till mid this week I had on average every night but 1 woke up only once during the night!! That was/is awesome!
Of ourse my body being my body that couldn't last to long. A few days ago the pressure (headaches)  that has been getting worse (definitely not as bad as sometimes but definitely worse) was pretty bad and bladder/low back/abdomen where seeming super irritated and incredibly uncomfortable. It didn't seem to matter what I tried I could not get the abdominal/side pain and radiating pain down my L leg to go away for the life of me and so minus going to a Appt I'd already had scheduled with my Primary dr (I guess good timing given these new issues =/ ) I have not done much the past 2.5days.

PCP Appt - Neurosurgery issues
Since I was seeing her anyways I brought up these new symptoms to my PCP who is concerned there might be a shunt infection going on. The risk of this is pretty small but she's still concerned and wants to rule it out b/c I've had low grade fever on and off for months during my infusions (I don't remember how she explained the possible relation but at the time it made sense if there where in fact an infection). Those fevers + starting the Humira a few wks ago (which can lower ones immune system) and the overall symptoms Dr,Simpson opted to call my Peds Neurosurgeon after I'd left her office yesterday.
 He in turn apparently agreed with her we should tap both shunts and test the Spinal fluid just to be sure. That Appt was originally next wk but b/c the past 1.5days have been exceptionally bad - last night being realllyyyyyyyy baaadddd Neurosurgery moved the Appt up to tmrw (Fri) morn.
I am really not psyched at still another Appt this wk but hopefully is an easy Appt and can figure things out or get an idea what might be going on whether shunts or not. I am really grateful my Primary dr., called and talked to Dr.Iskandar on my behalf. She's one of my favorite drs on my Team (and I genuinely like my entire Team) as she spends way more time than she'd have to on my care, simply making many things less difficult. Anyone in the MPS (or chronic illness) world knows dedicated drs can make a world of difference!