Monday, July 30, 2018

Sometimes it's just hard.. sometimes it's depressing w/out being depressed.

Just let me start this off by saying I am not depressed I am not overly worried and I will be fine I just have a lot on my mind the last week or so and have not been feeling good ALOT. Most people would have zero clue or some who know me might be able to tell a little but I seriously have not been feeling good.
 Some is usual stuff (which is incredibly sad to even consider that!), like headaches albeit they just keep getting worse, so mornings really suck and every morning I have to admit I wake up and I wonder is this every going to be better again? Am I ever going to find  a Neurosurgeon who will believe me and help me again? It is truly depressing (again I am not depressed the whole issue is just depressing, that does not mean I am depressed. I'm not).
Some nights in the middle of the night when I wake up and get up to take a med for nausea or something else to try and fall back asleep I wonder what have I done that I have to feel this bad? That I can't find a dr again who will believe me (I otherwise have a really great team of Providers, I am grateful for my PCP and all she does and the rest of my Providers minus Neurosurgery are great).
 It is depressing. I know it's not me but that doesn't mean it's not hard day after day after day and honestly the fact that vast majority of people who see me have no clue. I am glad I can hide it but sometimes I guess maybe it would just be nice to have someone who could see that underneath the smile and the sometimes crabby mood and the tiredness it's not actually about me being crabby or in a bad mood or about me not wanting to talk to you or whatever people might think (I over think these things) but it is about it takes to much energy to that I don't always have.

Catch me later in the afternoon and it's a different story. It's like a shade is pulled up, my head clears, the pressure goes away and I feel a lot better. I am not always a lot less tired but I can function a lot better b/c my head isn't in as much pressure...
Add to this now for the past month or maybe it's even been a couple months now, I can't even remember anymore it all blends together after awhile but the breathing/fluid stuff had/has picked up and is an issue again. Thankfully my Primary dr is really great and often without making me come in she helps manage these things and figure out what might be going on.

In the case of this swelling/breathing I did end up going in to see her and she in turn called and talked to my Cardiologist (Heart dr) but before even talking to him (she was persistent took her a week or so to get ahold of him albeit he is a great dr and we both really like him) she had re-upped my 1 water pill dose (gets fluid off from heart/lungs) and was considering a few things.
She also reached out to my study dr for the Humira as that can cause heart failure issues (we knew this going in to the study but my team felt the risk was small and potential benefits pretty good for my case. The Humira really has been good for my spine-nerve issues to calm this.
Needless  being completely honest (and very negative but seriously I think I'm a little bit allowed albeit I say that kind of smiling and shrugging) it has SUCKED being back on this high dose Lasix and then I am also still on the other water pill, a longer acting 1 2x a day that gets fluid off over a longer period. This means managing how much I drink, somedays I do better than others and I don't have a set amount I can drink but I try to not over do it. It also messes with some mineral levels though I do already take potassium and magnesium which are 2 it can affect.
For now my Team is letting me stay on the humira and I see my Cardiologist in a couple weeks right before I have the 4th carpal tunnel release on my R hand so guess will see then.
I get some swelling yet as the day goes on in my ankles but stomach has been a lot better. The truly ironic part is overall my actual body weight is down by around 7pds before and since starting back on this higher dose. So I was retaining fluid but also losing weight (I'm very sure some of that is shunt related, I often only eat 1 meal a day).

So I guess I just pray and keep praying somehow I could find a new Neurosurgeon who'd be willing to take on my case and to really think outside the box as that's what Dr.Bragg did and it's the only way my shunts can be successful. God has a plan, right? I just need his help waiting.

4th Hand Surgery - moved up to Sept 10th
In the meantime the 4th hand surgery on R hand was moved up to Sept 10th (not sure why they moved it up but not complaining as that hand is something so painful. I should do better and just wear the splint. I think I may this week while traveling but I will be happy when it's just done and over again!
For that surgery everything is set minus seeing my Cardiologist the day before (just to be sure per my PCP to make sure Dr.Earing doesn't have any concerns but anesthesia is already being done under MAC so monitored and least amount of fluids will be used and is least harsh surgery approach on cardiac system.
We are waiting on cortisol stress dose recommendations from my Endocrine dr but if for some reasons those didn't come (would be very unusual) my PCP said she plans to use what she received from this same dr for the last hand surgery about 4yrs ago. I LOVE that my PCP is that organized!

Lastly I am traveling to San Diego later this wk, I get botox injections tmrw with Dr.Caldera at UW then ERT (Infusion) at Dean on Tues and fly out of Madison on Weds to SD for the Conf.
I have a slew of mtngs with various pharma/biotech peoples throughout the conference then have a talk I am giving on Thurs night (Transition to adult care) + other stuff i'll have going on and the talks i'll listen to off course. This is our National MPS Conf but this year is actually an International Conf so will be families, Drs and researchers from many countries there. Should be good!

Thursday, July 19, 2018

Cardiology; surgery. Humira; questions

Hopefully just a quick update but my Primary dr., messaged this morning (Thurs) that she had gotten ahold of and talked to my Cardiologist.  (I am so grateful I was given her name 5 or 6yrs ago; she's persistent and never seems to give up no matter how much is going on with my case). I am grateful despite her having known little to nothing about my MPS she in fact did learn and will ask if she isn't sure about something. I so appreciate she's never afraid to reach out to other of my Providers and always willing to learn or figure something out!

Dr.Simpson  and my Cardiologist spoke, she shared with him what's been going on and he seems to agree with her that I am dealing with fluid issues and that going back up on the Lasix (gets fluid off) was the right decision.
He per Dr.Simpson feels due to my mitral valve and the issues we're having is due to fluid sensitivity due to the mitral valve problems (ya know, the 2nd valve we replaced approx. 2.5yrs ago. No one is quite sure why my body has done the quirky things it's done with this valve or in general why my heart issues are so significant for an MPS I Pt.)
 Initially Dr.Simpson, Dr.Earing per her message wondered if it had some to do with increased fluid in the Humira I get every 2wks but that is an injection not an infusion so is very little additional fluid. This will stay the same and nothing will change with the humira injection or study I am in thankfully.  There's back and forth question if the humira is causing these issues but so far team has let me stay on it, I think b/c I have had good changes/affects from it even if there is some question of possible cardiac involvement/side effect (I don't think anyone is entirely sure yet if the Humira is causing these changes).
The weekly enzyme replacement infusion I get is also 1/2-to1/4 the total volume other MPS I Pts get so that's not the issue or cause of the fluid overload..  My body as Dr.Bragg (former Neurosurgeon) used to say just doesn't do normal - my body reads the 1st line of the manual then makes it's own rules (shrugs).

So for now we're just staying on the increased Lasix dose, Dr.Earing was apparently reassured I'd just seen Dr.Simpson but Dr.Simpson wanted me to see Dr.Earing or 1 of his colleagues before the hand surgery on the 10th if possible. I fwd'd that to Jane, his Nurse and she was able to make an appt the 9th so we'll go from there.  
Pre-op for the Hand surgery is with 1 of the NPs in Dr.Simpson's clinic which will or could add an extra layer of confusion but I also know Dr.Simpson is pretty cautious and likely will make sure things are squared away and what's needed is in place.
 I've let my Endocrine dr know so they'll fwd their pre/post surgery cortisol mngmt plan to my Hand surgeon and my PCP prior to surgery.

Pulmonary, yesterday (Weds) Dr.Biller wanted to see what Dr.Earing thought 1st but thought we should probably re-do the PFTs (breathing tests) and maybe a CT Scan (Dr.Simpson wasn't sure we'd need the CT so I'm not sure). Per Dr.Biller's request I was to let her know when Dr.Simpson/Dr.Earing spoke and she'll schedule the other stuff I guess.

Hopefully it all works out ok and comes together! Otherwise I travel to San Diego in a couple wks for the International MPS Conf., I am hoping I am feeling a bit better but wouldn't be the 1st time I guess I traveled when I wasn't feeling great if not. After that I go to MN mid-August and later in Sept to L.A to finish out the Humira study.
Will update when I know something new.
Pray for good answers, meds to work and this to resolve if possible!
Take care, God bless,


Tuesday, July 17, 2018

Humira, Heart, newish symptoms.. ? Seeing Pulmonary, Hand surgery, etc

For the past how ever many weeks (I can't even remember maybe a month or could be longer now, maybe it's been more, it all blurs together after awhile) I've been dealing with intermittent swelling that gets worse as the day goes on and by end of day my ankles/legs and stomach get pretty swollen despite overall I've actually lost weight (going back up on the Lasix has helped this a lot which is so good but sucks if i'm being honest even if it's being really negative about it actually as I am tired of that med and just don't want to need it).
 I've had increase in breathing difficulty though this is nothing that would be apparent to someone else as it's just a difficulty getting a decent/regular (?) amount of air without having to think about it. Hard to explain I guess but it's frustrating though as it's very similar to before the heart surgeries. I do think this hot humid weather isn't helping but even when inside it's not all that great so I think there's more to whatever is causing this.

It has been ridiculously hot/humid here and from the start of the Humira study the bigger concern was  being sure this drug didn't cause heart failure in my case (an issue we dealt with and managed before my 3 OHS but has been stable and not an issue really since). I shared pictures of the intermittent swelling w/my PCP then saw her a couple weeks ago as she was/or is  concerned the symptoms I am experiencing may be heart failure acting up or heart related and possibly related to the Humira.
 At this point if it is she thinks we can manage with the Lasix as long as my symptoms improve which after she upped the Lasix dose back to the 60mgs 2x a day I had been on up till about 6mo or so ago symptoms have improved atleast some. Dr.Simpson requested my Cardiologist input (I talked to his Nurse last wk as I was getting INR results from her and she was going to pass Dr.Simpson's notes to Dr.Earing albeit a week later and she (Dr.Simpson) has had to leave a few more messages.
Jane had emailed the INR results on Tues last wk (was super low on 2 different occasions last week but then the level was just above range and slightly high on Tues and then today (a week later) was high again  (Coumadin could drive a person to drink its insane!).
 I'm not sure why Dr.Simpson isn't hearing back from Cardiology, its weird actually, usually my Cardiologist is great about returning providers calls and yet nothing?  For now I am continuing the Humira and we just hope we can figure something out, hopefully Dr.Simpson can get a response from Dr.Earing?
My Study dr had asked me to let her know what he (Dr.Earing) said so I just have been giving her what little updates I have; it's all a little confusing being completely honest with 3 different drs involved albeit all 3 are very good and I know we'll figure it out at some pt. Soon would be great though b/c seriously right now it makes me feel a little nutty! I know I should trust myself,  I KNOW my symptoms are real and the issues is real but when you continually have to rely on others to help it IS hard incredibly hard for me.

What I really want is to not need the Lasix (I hate that med) and yet to be able to stay on the Humira! I'd gladly give up just about any of my other meds minus a couple that have clear impact/help for me but the Humira has made a really big difference in the spine-nerve bladder stuff and that's so incredible after so many years! I wish this breathing/swelling stuff where just simple and Cardiology would return my PCPs call. I completely get that Drs are busy but it's hard when you don't feel good and I guess as i've written about before I think I have gotten even worse at trusting myself since the whole (kind of) mess with my Nrsgn and his note questioning that I understand him. I just want to feel good once, to be able to breathe normal, to not need all this med to get/keep fluid off and all the other meds on top of the headache/shunt stuff. I am tired I guess of it all albeit that never means I am giving up, just that at this point it is frustrating.

 My Study dr has also been weighing in and is involved + they all are figuring out what if anything we need to do. -- Hopefully NOT discontinuing the Humira! There are some meds I take I couldn't say for sure how they help but the Humira I can say exactly the ways it does and it's been such a relief. Say a prayer my Team can figure this out and we finally hear back from Cardiology I guess!?
 Pulmonary - normal f/up but good timing (see if she can help)
I also see my pulmonary dr tmrw (Weds) at FMLH so we are hoping maybe she might have some thought or insight and maybe be able to help. My case just seriously never seems to be clear so I never get my hopes up but I still do hope Dr.Biller might be able to help somehow? My PCP thought getting her opinion was worth it especially since I was seeing Dr.Biller anyways.. She's been part of my Team since I was 1st diagnosed with the MPS I so she knows me well.
Seeing Peds Neurosurgeon - UofMn
On 2 separate notes I am seeing the Peds Neurosurgeon at UofMn when I am there in a few wks to get his opinion. I don't know that I am overly optimistic but I hope he can weight in and maybe give some advice on symptoms/the pressure-headaches (shunts). Is worth a shot.
 Giving A Talk on 'Transition to Adult Care'
On 2nd note I have been asked to give a short talk on 'transition to adult care' at the International MPS Conf., which is the conference this year hosted by the US MPS Society but including all countries MPS Societies. Should be good! I hope I am feeling better by then but if not I guess wouldn't be the 1st time I traveled when not feeling that great.
Hand Surgery date moved up:
And a 3rd note, the hand surgery which has been scheduled for Sept was moved up today to Aug 10th. The Hand Clinic called needing to change the date and the 10th was 1 of a few dates they had which I could do. This will be my 4th surgery on my R hand but a new surgeon.   Odd that in needing to change the date they could actually do sooner but guess that's ok.
 Only bad thing is the pre-op is not with my PCP and she is sooo familiar with my history and issues so has always been phenomenal at rounding everything up before each surgery. The (new to me) NP I see instead will have a bit of figuring out to do but I guess we'll figure it out.
 I've already left a msg for my Endocrine dr atleast so that pre/post surgery cortisol dosing will get sent to Hand surgery for the surgery.

I'll try to do an update sometime soon when/if we hear from my Cardiology Dr and also with how the Pulmonary (Lung) Appt went.
PS as a quick side note in just a few days it will be 5yrs since the 1st of the 3 OHS. Amazing actually all that's happened since then. 5yrs ago I certainly never expected would be like this although the OHS's really did help, just I guess not as much or maybe not for as long as I would have hoped? I'm not exactly, entirely sure given the continual seeming changes.
Thanks for stopping by,