Thursday, January 18, 2018

Shunts, uncertainty... Humira and other stuff

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!

Lovenox / Coumadin -
I've been back on the Counadin (oral blood thinner which we went back to and replaced the injection Lovenox I had been on for a little over a year).. - Thankfully my Cardiology Team is pretty great and especially the Nurse (Jane) with my Cardiologist is infinitely patient as we've definitely had to do a lot of tweeking, testing, adjusting, re-testing, re-adjusting with the Coumadin dose. Honestly I don't even really get annoyed with this med as I am just grateful to (for the most part) be off the 2x daily Lovenox injections.
When INR is below 2.0 (2.0 and above is a somewhat safer range, my ideal range with the 2 mechanical heart valves is 2.5-3.5) then Cardiology adds back the twice daily injections. In these cases I end up having to re-test again later the same week BUT again it's still better than being on Lovenox every day 2x daily forever! I'll find some positive in the difficulty of this med =)

PCP Coumadin Mngmt:
My Primary dr., also has been a great help in the Coumadin/INR mngmt as has my Infusion Nurses - the 'Care Everywhere' system which allows my Providers at any given health system in WI (so Dr.Simpson at Dean can see Dr.Earing's Cardiology records at CHW and he can see her and other of my Providers records at Dean, UW, etc.). This means when Dr.Simpson orders the INR (She has a standing order the INR blood draws) which I then get done when getting accessed for Infusion, she automatically gets the result (as do I on MyChart). She'll typically msg if she has any concern + I call and leave a msg for Cardiology + Jane calls or emails to let me know what if anything I need to do with my dose and when to test again. Maybe sounds complex but this set up works really, really well!

Humira Study - Good Teams
Lastly Dr.Polgreen from the Humira Study called earlier to check in on how things are going, work out arrangements for the beginning of Febr when we'll both be traveling to the same (WORLD) Mtng and I also will have to dose that wk with Humira. She'll be driving down to SD so offered to just bring the drug w/her and we'll meet up that Tues of the mtngs.
For study purposes she gathered some info on any changes/issues going on and other related study questions. I have to say though she and her Study Coord., + other Team members have been really good to work with! I guess I knew this to some degree as Dr.Polgreen has always been a great resource and always willing to answer my questions or questions my Neuro Endocrine dr had and I know a lot of MPS Families that really like her but it's still always nice when your working with a Team and they go out of their way in little or big ways to make things a little easier!

It sounds like per an email from my main contact at Sangamo that the Rare Disease Mtng at the NIH is going ahead. They are still working out details about my speaking but Cristeen  just said Sangamo would work with me on what would be needed before the mtng (March 1st). Seems should be an interesting day. I'll try to share the schedule for 3/1 in the next update if I remember.

Will try to update after tmrws Peds Neurosurgery Appt or soon.
Thanks for stopping by,


Friday, January 12, 2018

Humira (MPS I) Study update; Various other updates.

I've been meaning to finish this for a week or so now so one of these times I really will get it done. That said just a myriad of things going on but also has been (sort of) slower lately. A lot of really cold weather has meant more days at home (my Apt) and honestly I haven't minded that much. I haven't been feeling the best headache (pressure really) lately and some episodes of lightheaded-like feeling though these are typically shorter in nature so being at home, just getting caught up on things here has been ok

Humira study - PMR Dr. / Zebra cases -
The 2nd dose of the Humira was a little delayed (by a week due to a issue with the lab company that comes out and does the labs needed for this study) but that was finally re-arranged earlier this week after a few delays and I was able to give myself the 2nd dose of Hmura (or placebo) Weds night. The 3rd dose will then be in 2 weeks from now. I am genuinely glad to be doing this study and equally glad how optimistic or hopeful my Team is that it may help..
I have to be honest I'm curious with the Headaches and not entirely sure how it could help those but for now just living day to day with this issue as they have slowly been coming back... but again day to day on all things MPS and even days when it's extremely frustrating (not every day just some days =)  )  it's all a balance and I refuse to let any of it stop my keeping on living.

I guess if I hope anything from this study it will be that  the Humira might help the spine inflammation and that in turn help us the times when we need to re-position the shunts (perhaps help calm the arachnoiditis and in turn secondary scarring) or just plain help spine in general. If it helps joints in general and my Physical Therapist and I be able to keep the work he does lasting longer term (what he does already works amazing) would be really, really great! to. Overall I am just glad to be in this on the drug (or placebo) now and that the dr (Dr.Polgreen) running it is a really great dr to work with as is her Study Coord. Alison, the Study Coord., has been really great overall and this past 2 wks especially sorting out the lab stuff and Fed Ex shipment related issues. Makes being in a study a TON easier when your working with a good team! 

I know for the Humira study in general Dr.Polgreen has funding for 3 more Pts so I am glad to see it being able to help more Pts in my community and to see final results. I think (but can't remember for sure) they are giving a talk on this study at the upcoming WORLD Mtng which if so should be interesting. To date I believe there have been 5 Pts so far besides myself.

Heart - Heart Rhythm:
Just a really quick update on this, the Amiodarone which we started a few months ago.
Heart Rhythm is still some off but certainly better than it had been - ironically this improved right about the time we did the last Heart monitor but I won't complain. I will say it's SUPER annoying when it still does it's runs of (seems like) excessive skipped beats and weird beats but atleast it's less than it was pre-Amiodarone. I do think the Ivabradine still helps actual heart rate (keeping it slower).
Endocrine and PCP have been comitoring thyroid levels as the Amiodarine can mess with this and per labs done several times now it appears the med may be having some affect but 2 of the last 4 lab checks done the levels where back in a reasonable/good range.

Headaches - Pressure.
These 4 or so months since we did the last shunt surgery where I felt really, really good and I felt pretty darn human (as human as my body lets me anyways =D ) have been really nice. It is never any less of a bummer when the headaches (pressure) comes back and other symptoms in turn also come back. I'm so not ready to deal with this again and the endless having to (basically?) argue that I'm not making the symptoms up, that I really don't want to be asking for help (I haven't bothered calling my Neurosurgeons office yet, in reality his Secretary and Nurses are very nice but I just can't stomach having to try to plead/argue my case yet). I feel discouraged writing this (not a feeling I try to wallow in much) and have just been ignoring it as much as possible but I also know it's affecting other things. SOO FRUSTRATING are these shunts and more than that the pressure!!!! I thankfully have a Team of Providers who are good and even my Neurosurgeon, he's good it's just he totally doesn't seem to get that I never want to be asking for his help and in my case, my MPS makes it much different than the vast majority if not all of his other complex cases.  Like always frustrating! I am glad Dr.Bragg cont's to help but man I miss her being here!

RegenX-Bio / Sangamo Studies:
This post is already getting way to long so i'll just say I am working with both companies and will try to update next time. Both studies seem promising to our MPS I Community though!

Running for Rare Team - 2018 Runner
I am paired up with my runner for the 2018 Boston Marathon. I don't know if i'll make it out to the actual marathon this year but I am hoping so. I am in the process of trying to coord., appt with my Metabolic Genetics dr (Dr.Bodamer) out there for that same wknd but still have to figure out travel and all the expense that goes with that wknd.  It seems per Dr.Bodamer's Secretary he may be out of the office that wknd so we'll see. **** Update on this Dr.Bodamer is out of the office so I really don't know what to do yet. I want to go but don't know about the costs and then I'd have to go back out again likely sometime this year to see Dr.Bodamer as I really want to see whats going on with the Whole Exome testing results and other studies he has mentioned. Just unsure at this point.
 Honestly I have been considering asking my PCP about switching back to a Genetics dr. locally - I'm not sure about this but will talk to my PCP and it's possible I could still work with Dr.Bodamer but just need to figure things out I guess.
The National MPS Society has  medical travel assistance program and PSI also helps some but it's still some I have to pay out of pocket so need to figure that out and what the total cost would be this year given would likely mean 2 trips.
 I DO really want to but we'll see! The biggest is hotels out there for the marathon can be extremely expensive. Dr.Bodamer wanted me to do Neuropsych testing w/1 of his drs (testing that's routinely done in us MPS I Pts., I've done it at UofMn many times over the years but not in recent years) but that dr apparently only sees Pts certain days and I don't want to stretch out my time out there even longer at even greater expense whenever I do make the actual apt sometimes this year.

 My runner this year though seems so neat, we've really hit it off; She works at Genzyme so knows some of the folks I know and of course other runners from the Team. In general the Marathon wknd is just a weekend that can't quite be explained! :)
Lots to figure out I guess.

Will try to update soon. I scheduled to see my Primary dr next week I believe it is so will maybe figure some of this decisions out with her.
Thanks for stopping by,