Quick Update On The New Year

Hello, Everyone.

Sorry that I’ve been M.I.A. lately, but (as per usual) everything has been hectic once again. However, I wanted to check in with you all and provide some updates as to what’s been going on in my life and with my health.

First off, thank you to everyone who has followed along with my blog this past year and to all the new readers who have spent time browsing and viewing everything I have put up on the blog (especially those from the Nutcracker Syndrome Group Facebook Page- and thank you to Margreta for her wonderful research skills and for finding/linking to my page). I have a lot of great information currently saved that just needs to be edited and posted, so hopefully all of that will be up live on the page here soon. Also, this week is super exciting because it’s the 7th Annual Wear Purple Day Event for SMA Syndrome Awareness but more about that later (see details below).

As most of you know, right before the holidays I took a short break from medical testing, doctors appointments, and school. 3-weeks off?!?!?! While I was hoping to be super productive and get so much done during this time, I sadly spent almost my entire break in bed as I was knocked down hard by an extreme flare that -even now- I’m still not completely over. It’s been an awful couple of months for sure and most of my days have been spent sleeping rather than doing much of anything else. Quite disappointing. Then with school and appointments starting back up, I already feel like I am so far behind on everything.  Not to mention, I’ve  had a couple hard blows from some of my doctors and with my disability claims already this year. So, needless to say, 2016 has been far from a perfect start. Likewise, it hasn’t been overly exciting either.

At the beginning of January, I submitted the last of the documents that were requested for converting my short-term disability plan into long-term, although my STD claim ended the first week of December and I have yet to hear anything in regards to a decision. I do know that they sent my case for medical review and I’m guessing that’s not a good sign in terms of getting approval. I knew when I received the other denial letter from my first STD case that they were setting up to deny my long-term (or, at least, it seems like it anyway). Financially it’s been tough, but somehow we’re making it through – at least, for now.

Also, I’ve had some follow-up appointments with my both primary care physician and my gastroenterologist, as well as continuing the usual weekly appointments with my therapist. My bloodwork and urinalysis were showing some irregularities at the start of December, so we re-ran those labs hoping to see if there had been any changes  that could perhaps lead to a diagnosis for the underlying skin/allergy/systemic disorder that nobody can seem to figure out. Like usual, though, my tests came back normal; except for finding protein and urobilinogen on my urinalysis and the low vitamin D levels and a decreasing eGFR rate in my blood serology. However, the good news is that my amyloidosis protein serology came back negative and my cortisol levels (which were low over the summer)are within normal limits again. My primary care had ordered an ultrasound of my left kidney last week to make sure nothing else was going on, but the hospital informed me yesterday that my scan was normal as well. So no more answers, but no change in diagnosis at this point either.

My GI appointment is a long story, so I’ll have to save that for another time. In fact, I plan to use it as an example for my follow-up post to The Good Patient (Part 1 of 2) – The Bad Patient. I bet you can see where I am going with this. Also, I have some other things I want to discuss in regards to my psychological evaluations and appointments in therapy, but that will have to wait until I have more time as well. The next few weeks are hectic, as I have more labs and medical tests ordered. Next week I am scheduled for more blood screenings, a 24-hour impedance and pH study (since I couldn’t complete the first one), and an anorectal manometry – how miserable, really. I’m also waiting for the hospital to call so I can schedule an MRI Abdomen/Pelvis (which I have not had before) and then I go for a tilt-table test on March 1. After all, of that is said and done, I will then follow-up up with my PCP, GI, and Cardio doctors to discuss whether or not to move forward with the surgery that the vascular surgeon recommended for the Nutcracker Syndrome. I’m sure more things will come up between now and then, but that’s the plan so far.

Aside from all that, it’s just making it through another semester in school and learning to  somehow manage my symptoms, but that is becoming harder as time and time goes on. Does that sound like I’m doing enough to you? Some days I think so; other days I feel terrible for not doing more with myself – oh the dreaded guilt of chronic illness.

Finally, as I mentioned at the start of this post, this Thursday – January 28th- is the 7th Annual Wear Purple Day to promote SMA Syndrome Awareness. It’s an online event, but everyone (both patients and non-patients alike) are encouraged to join the event via the SMA Facebook Page (linked here) and post fun pictures of you or anyone else wearing the color purple that day in support. For more information, you can either send me a message through my blog, Facebook, or email accounts or you can message the event page directly. Please share with your friends, family, co-workers —> everyone and anyone that you know. Too many SMA Warriors have been lost this year and it’s important to bring awareness to this condition. I’m hoping to be posting videos, photos, and research both here and on the Undiagnosed Warrior Facebook Page starting tomorrow.


[Taken from the 7th Annual Purple Day for SMAS Page and written by Marge Reed (for the 4th Annual PURPLE Day- 28th Jan, 2013) and (slightly) adapted for the 5th Annual PURPLE Day, on the (28/01/2014), by Maria McMillan and again (updated for our 6th Annual event from 28/01/2015 to 30/01/2015) and updated for our 7th Annual PURPLE Day, Jan 28th to 31st, 2016.]

THE STORY BEHIND PURPLE DAY:

"On January 28, 2010, my friend Samantha Mina traveled 800 miles from Virginia to check into the Florida Mayo Clinic for SMA Syndrome.
An innovative SMA Mom Maria McMillan decided, before Samantha's departure, to ask friends and fellow survivor families around the globe to wear purple that day to show their support for SMA Syndrome sufferers. With hundreds of RSVPs and dozens of uploaded photos of purple-clad people, the first annual Worldwide SMA Syndrome Awareness Day was born.
Then nearly 1000 people attended during each 2011 and 2012, praise the Lord!
Indeed, the past couple of years were marked by leaps and bounds in global SMA Syndrome education and when the documentary style TV show "Mystery Diagnosis" scouted Samantha and featured her story on the Discovery Health Channel on 83010 the survivor support group swelled to 1200 members.

At 8th January 2014 our Awareness group now has 1508
members!

As at 4th December 2014, our Awareness Group now has 1756 members!
*As at 18th December 2015 our Awareness Group now has 2113 members*
We had almost 1400 attendees for our 4th Annual PURPLE Day, Jan 28th, 2013! (Inclusive of 'Non-Facebook attendees).
So, to celebrate all the incredible progress in SMA Syndrome Awareness that has been made thus far and to encourage its continuation in 2014 we invite you to participate in the 5th annual worldwide SMA Syndrome Awareness day on Tuesday January 28 2014 (to Thursday 30th January, 2014), to allow for global time differences, by simply wearing a purple article of clothing and/or a purple ribbon, or a whole purple outfit, if you wish! Please feel free to take a photo of yourself to upload to this page.
And now, we invite you to attend our 6th Annual worldwide SMA Syndrome Awareness Day on Wednesday 28th January 2015 to Friday 30th January, 2015 (to allow for global time differences.
We had 3991 attendees for our 2015 event!
*And now, we invite you to our 7th Annual 'PURPLE Day' from Thursday, Jan 28th to Sunday, January 31st, 2016*
Thank you for sharing our 2016 event both on Facebook and in the 'real world'.

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(Once again, you can join the event here)

 

 

 

 

Feeling kind of fragile lately, I know only I can save me now…

“Fragile, Quiet
Feels like I’m dying
Crystal waters
Pulling me under
My rage is like thunder”

(Jennifer Marks – Lyrics Below)


Lately,

I feel the universe is testing me

To see if I will break…

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What do you do when you can’t do it all?
When everything is extremely important and none of it can be eliminated?
And all of it is time sensitive?

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 It feels like it’s never going to end.

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But so much has happened, and is happening

in both my life and my health.

I’ve been waiting for a free moment to update,

but I don’t even know where to start.


No Sleep September 

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Wasn’t able to sleep for weeks all through September, although it seemed to run into the first week of October. No idea why, I just suddenly became “nocturnal”.  I would be wide awake until about 10 am before I would then crash out until about 5 or 6 pm. I tried forcing myself to stay up, hoping I’d fall asleep early, but even that didn’t work. Again, I wouldn’t get tired to 10 am and would be “stuck” awake for 2-3 days. It was awful.

I am FINALLY back to sleeping at night, but now all I want to do is sleep. No amount of caffeine seems to fix my tiredness. I talked to a few of my doctors about it and none of them really seemed too worried about it except for my therapist. I guess we’ll see how the next few weeks go and bring it up again at my follow-up appointments, but I’m really worried how drastically my sleep patterns have changed.

The ups and downs with doctors

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Gastroenterology

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I had a follow up with my GI doc mid-September. After meeting with vascular surgery and the lack of medical evidence from the last few rounds of GI testing, I assumed we were pretty much done. Boy, was I wrong.

Despite reviewing the update from the vascular surgeon, she wanted to order even more testing. Excuse me, what? I wasn’t happy, to say the least. Especially because a few of the ones she ordered were ones I’ve already had in the past that all came back normal, not to mention that every GI test involves some sort of pain or torture.I also really don’t want to do them again, but she wanted to see if there is any change between then and now, especially with confirmation of Superior Mesenteric Artery Syndrome (SMAS) now. Plus, she talked with the vascular surgeon and convinced them that I needed to redo the upper GI series/small bowel follow through, even though he said it wasn’t necessary. This test, out of all the medical tests I’ve ever had, has been nemesis since early childhood. Even now, I cringe thinking about it.

Really, though, I have been having medical testing multiple times a week since February and frankly, I’m just tired. But if I decline to do them, then I am a “noncompliant patient” and it could affect my disability claims. *Sigh* There’s no winning, so here I am, yet again.

Vascular Surgery

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I was excited for my follow with the vascular surgeon with how well our consult went the month before and was ready to get a game plan going to treat the vascular compression syndromes. Originally, I was scheduled for an afternoon venogram that day to measure the pressure of blood flow in the veins that were compressed and see if they could confirm a diagnosis of pelvic congestion syndrome. I should have known that the plan could potentially change (especially after he changed his mind on needing additional GI testing), but he seemed so confident at our consult that I didn’t even give it a second thought. Wrong again.

A week or so before my appointment, I received a call from the same hospital asking to schedule with Dr. So-And-So. I had no idea who Dr. So-And-So was. His receptionist explained that he was a GI surgeon that my vascular surgeon called to set up a consult with because he will be assisting in my surgery. MY SURGERY?!?!?! It was news to me, but I scheduled anyways. I think to myself, FINALLY! We’re going to do something. I was both excited and petrified at the same time, especially given the potential severity and risks (depending on which type of surgery they were looking at doing).

I started making lists of things to get myself ready, reading about the pros and cons of each procedure, etc. I even made it Facebook official:

September 22:

I just got a call from the GI surgeon at University. The Vascular Surgeon called him to see if he could assist with surgery, so I have my consult end of October. Looks like we’re going in to fix the SMAS, Nutcracker, and May-Thurner all at once.

I’m petrified and ecstatic all at once. I’ll have to wait until all testing and consults are done to see if they are stenting the veins or completely “rerouting” them. Huge difference in recovery time. From what I hear full recovery isn’t guaranteed or it can fail entirely, but this is my only shot of ever feeling better.
Hoping for surgery around Christmas.

Essentially, overplanning like I usually do. Then I realized, I didn’t receive the document about prep instructions for the venogram in the mail as I expected. I decided to call back to the hospital to see what I needed to do. Good thing I called, though, because they didn’t schedule me for a venogram like I was told during my consult. It was just a follow-up appointment. Um, okay… I thought maybe they found something different during their medical conference, where they were going to review my scans again to make sure nothing had been missed. I was even more anxious for the follow-up now.

The day of the appointment, we drive the hour and a half up there and we’re almost late because traffic was horrible. I hadn’t slept well the night before at all, but I just really wanted to know what was going on with surgery. For some reason, I just had a really bad feeling  – it wasn’t until the appointment was over that I figured out why. We meet with a student, who takes the basic info about how I am doing and how literally nothing had changed since our first meeting together.

The doctor comes in a few minutes later and says that he thought all my testing that was recently ordered through my GI doctor (at another hospital, I might add) would have been done by now. Nope, we JUST got it scheduled. He says there’s nothing to really go over until all that’s done. So, then this appointment is a waste of time? Basically – Sorry that no one called to verify. I’m speechless. I ask him why we needed the tests run again – just to see if there is anything else it could be. Wait, are we NOT sure anymore? Well, we know that the compressions are there, but we don’t want to make it worse with surgery if something else could possibly be causing your symptoms. What happened with doing the venogram? I’m okay with scheduling that but if the tests show something else, then we can pursue that first. Then why are you sending me for a surgery consult with your colleague, who is supposed to assist in the surgery that  you don’t want to do because it could potentially make my symptoms worse? 

I didn’t say the last part, of course, but I left my appointment both angry and frustrated yet again. I’m so tired of doctors changing their minds on what they want to do. So far, all they keep doing is running medical test after medical test, not providing any treatment.It just feels like a giant waste of time. I came home and vented about this on one of the support group pages I follow and I’m pretty sure that  someone pinpointed exactly why this doctor changed his mind so suddenly. It’s happened to her too, multiple times. Because of the complexity of the surgeries, on top of the fact that it would be multiple interventions at a time and with more than one doctor performing, they get “scared” to “make it worse”. They begin stalling by ordering more tests or try this medication, even though it’s already been done – just to buy time. To me, it makes sense, although I can never prove it.

My venogram is actually scheduled now for December 3 after my continuous testing supposedly ends. We’ll see if that even happens. If it doesn’t, I don’t know what I’ll do at this point.

A new Primary Care doctor

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In the midst of all the other doctor drama, I also had to begin my search once again for a new primary care doctor. If you remember from early posts (And it’s a sad pictureSay something, I’m giving up on you, I know the shame in your defeat or Oh doctor, doctor), finding a GOOD primary care doctor has been the biggest challenge in my quest of finding a diagnosis. There’s something in this town that make doctors… oh, what’s the word?  oh yeah… WORTHLESS. Which is why I travel so far to see specialists, despite living in a relatively large city. But seeing as my past PCP disappeared out of nowhere, I had no choice but to find a new primary care physician (although to be honest – I only stayed with her out of convenience, despite that she worked in the worst medical office consisting of the rudest staff to I’ve encountered to date).

Earlier in the year when I had tried to find a new doctor, I originally called a clinic that ended up not taking my insurance, but the receptionist who answered actually was on the same insurance plan. She told me to try one specific clinic because they were great and she had felt my frustration with quite a few of the other doctors in town. Glad it’s not just me. However, after the fiasco with the few offices I had tried back in the Spring, I was too worn out to try and find a new doctor, so I never called them. Luckily, I kept their number, though.

I was so worried about having to go through this process again and I really worked myself up about seeing a new doctor. But ultimately, he turned out to be better than expected. The clinic was both clean and nice. The receptionists were friendly and organized as well, which was a huge difference from the last practice. The wait wasn’t too long and the doctor spent a great deal of time with me, learning about my diagnoses, comparing “expected symptoms and treatment” to my own personal experiences, and he actually did an exam. He asked me if there was anything else I needed him to do, to which I replied I had short-term disability and work paperwork that I needed to have filled out because they wanted me back at work following this appointment (which had been stressing me out over the last few weeks, to say the least).

What amazed me, though, was the fact that he refused to fill them out. Instead, he wrote them a letter saying that I am really sick with multiple complicated diagnoses and it could take a while to get a treatment plan going before I will be well enough to work again. I was in shock. He believed me! I can’t even put into words how grateful I am for him taking the time to sit down with me and listen to what I had to say. He scheduled me for a follow-up in a month just to see how things are progressing with my specialists and ordered my general, yearly bloodwork to make sure nothing is missed along the way. I’m so glad to finally have a good doctor that is also close to home.

Work and disability

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Off the advice of my therapist, I decided to start my social security disability claim over the summer just in case I wasn’t able to get back to work as fast as I had originally thought. It was a good thing I did since I currently have no expected return to work date now. So far, the process hasn’t been too bad. However, a few weeks ago, I received a letter in the mail stating I was being sent for a psychiatric evaluation. I was stunned and worried. Did they think it is all in my head? Do they think I’m lying about my symptoms? why are they doing this? Needless to say, I was worried. I talked with my therapist about the neuropsychologist that was doing my mental comprehensive exam with, and she, unfortunately, didn’t have the best news for me since she was familiar with him. Great. Again, I overprepared myself with tons of documents and information to bring to the exam, especially since she told me to bring proof of a diagnosis so he doesn’t label me as having a somatization disorder. But, overall, I think the appointment went well. Despite the fact that he wasn’t exceptionally outgoing or friendly, he seemed to identify with my answers to his questions and stated that I was very pleasant and intelligent. So I am hoping this appointment helped my case, instead of hurting it. Still, I worry about my future without disability at this point, especially considering that the number of appointments and testing don’t look like they’ll be decreasing any time soon.

Later in the afternoon, following my appointment for the CE exam, I received a call from my manager at work. As soon as I picked up, I knew something was wrong. He conferenced in a woman from the human resources department and instantly I knew – I was getting fired. I held back my tears as they advised me that since I had no expected return date that they would need to let me go because of business needs. I was heartbroken, but I understood. Still, it didn’t stop oncoming fear and panic inside me. My thoughts were racing through my head. I felt like I couldn’t breath.

What am I going to do?

I’ve never been fired from a job before.

How is this going to affect my short-term disability claim?

Or the long-term disability claim that was just initiated the day before?

How am I going to live? or survive?

We need to renew our lease next month…

What am I going to do?

I was beside myself. More importantly, I was ashamed. Up until now, I could still say I had an amazing job and that I was just on medical leave. Now I’m just unemployed. I have worked consistently since the age of 15, no matter the symptoms but only started having real trouble with maintaining work this past December when symptoms got out of control. I felt like a failure. Luckily, though, I am still eligible to continue my short-term disability benefits and finish my long-term disability application since I was on medical leave during my termination. Still, so much more has been lost than gained and it’s been difficult to remain positive.

To top it all off, getting fired has added even more work for because now I have to write  a letter to each and every state asking to return my insurance licenses so that I don’t receive fines for not continuing education or fees, in addition to the separation paperwork, returning equipment, moving and changing all of my policies. And remember, I still have all of these doctors appointments and medical testing scheduled multiple times a week, maintaining my current STD claim, finishing up the paperwork and processing for both SSDI and LTD, and finals for school. I’m about to pull what little left of hair I have left out of my head, I swear. But somehow, like everything else, it will get done. I’m just not sure how to do it all exactly, but I have no choice but to find a way.

change-is-imperative

And last but definitely not least, 

I received yet another diagnosis.

But seeing as this post is already too long, on top of the fact that I am exhausted from all that has been going on, I’ll update this information with more specific posts on some of the medical testing I’ve been going through. Thank you all for listening and allowing me to vent about everything going on. In the meantime, I’ll try not to let all of this bother me,  although that is easier said than done. I just need to focus and get it all done the best  that I can. Really, it’s the only thing I can do.

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Fragile by Jennifer Marks

There’s a hole at the bottom of this cup
I want to fix it so I can fill it up
There is sand where the flowers used to be
I was happy then and you were here with me

Feeling kind of fragile lately
I know only I can save me now
I’m not hoping
There’s no use praying
I know only I can save me now
Feeling kind of fragile

There is dust in the seat where you once sat
Time is cruel it’s too stubborn to turn back
My heart’s so heavy it will never float
I’m holding on, cause I’m afraid to let go

Feeling kind of fragile lately
I know only I can save me now
I’m not hoping
There’s no use praying
I know only I can save me now
Feeling kind of …

Fragile, Quiet
Feels like I’m dying
Crystal waters
Pulling me under
My rage is like thunder

Feeling kind of fragile lately
I know only I can save me now
I’m not hoping
There’s no use praying
I know only I can save me now
Feeling kind of fragile.

Follow-up With Cardiology

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The Conclusion from Medical Updates Part 1Part 2Part 2.5,

Part 3: The Cardiac MRI, &  Part 3: The Exercise Stress Test


Although preparing for any type of doctors appointment is stressful enough for almost anyone, it is even harder when you have a chronic illness, or even worse – multiple chronic conditions.

There is always so much preparation and pressure that goes into getting ready for each appointment:
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However, it’s entirely worse when you know you are  awaiting abnormal tests results.

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My list of questions for my cardiologist had gotten so long,

I was afraid to even present it to the cardiologist:

  • Medication questions:
    • Should I take the beta-blockers in the morning or evening?
    • Will I stay at this dose?
  • Abnormal test result questions:
    • Could this condition be genetic? Is it associated to a particular gene or chromosome?
    • Could the cardiac issues be caused by the vascular compressions or vice-versa?
    • Could I still have P.O.T.S. or are my symptoms caused by the cardiac issues?
    • Is there any way to know the cause of the pericardial effusion?
    • Was there a blockage in the arteries?
    • What is the treatment?
    • Why did I only show an abnormal heart rhythm after exercise?
    • Should I continue to salt-load and water-load, even though that is against cardiac treatment but good for P.O.T.S.?
      • Should I still wear the compression socks?
    • Why did these abnormalities not show on any EKG?
    • Do I have sustained or nonstained ventricular tachycardia?
      • Is the tachycardia polymorphic or monomorphic?
    • Is there any evidence that I’ve had a heart attack?
    • Could the ventricular tachycardia be causing the low ejection fraction?
    • Do I have diastolic or systolic heart failure?
    • Could the cardiac issues be causing the gastrointestinal symptoms?
  • Other questions:
    • Will I still go to the Dysautonomia Clinic at University Hospital?
    • Should I get a second opinion from vascular surgery?
    • Could any of this be related to neurotransmitters or hormones?
      • particularly, catecholamine and cortisol levels?
    • What s the likelihood I have an infiltrative disease or autoimmune disease?
      • Amyloidosis?
      • Lyme?
      • Sarcoidosis?
      • Other?
  • Most important question:
    • What is my prognosis?

I had visions of the doctor literally picking me up and throwing me out of the office, shortly followed my “scroll” of questions. I highly doubted that it would actually happen, but you never know these days. It’s was a lot to ask. I typically try to keep both my medical concerns and my list of questions to five or less each, respectively. However, I felt this was super important and I needed to know. I opted to take my chances.

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The night before my follow-up appointment with the cardiologist I could barely sleep.

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There were too many things running through my mind. Do I have everything I need? What am I forgetting? What do I do if he says [this]? What if they tell me [that]? I literally drove myself crazy just over thinking everything. I knew I needed sleep more than anything. It was an hours drive again and I was scheduled first thing at 7:30 a.m. I think I finally fell into sleep somewhere around 3:00 a.m. before my alarms went off at 5:00 a.m. I was too anxious to need much sleep anyway, the adrenaline kept me awake that morning on the drive anyway.

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We arrived at the clinic that morning, early as usual.

It wasn’t long after we checked in that we were escorted back to the exam room. From there, though, we had a wait, which is unusual for this clinic.  We wait in silence as 5 minutes go by, then 10,  and 20. You could feel the anxiety and tension build up in the tiny exam room as we waited on the cardiologist. I could see my husband getting impatient, it’s all over his face. I, myself, feel like I’m going to explode inside. I don’t dare to breathe. Just when I can’t take it anymore, the doctor walks in.

I take a deep breath. My palms are sweaty. My heart is beating. 

I just want to get this over with.


results

Blue=My notes

Red = Abnormal results

The Echocardiogram With Agitated Saline:

Physician Interpretation:

Left Ventricle: No regional wall motion abnormalities noted. The left ventricle
cavity size is normal. Left ventricular systolic function is mildly reduced. Normal left ventricular diastolic function with normal LA pressure. Left ventricular wall thickness is normal. There are false chords noted in the left ventricle

[Had to look this up, as I had no idea what it meant: “Left ventricular (LV) false chordae tendinae (false chords) have been implicated as a source of idiopathic left (IL) ventricular tachycardia (VT). However, it is unknown whether pretest bias contributes to an apparent association with disease. The purpose of this study was to determine the prevalence of false chords on direct inspection of the LV endocardium”.]

Right Ventricle: Global RV systolic function is normal with a tricuspid annular
plane excursion of 1.76 cm. Right ventricular size is normal.
Left Atrium: The left atrium is normal in size with a left atrial volume index of
10 ml/m2.
Right Atrium: The right atrium is normal in size. Eustachian valve seen in the
right atrium (normal finding).

[Of personal note here, I looked up to see why this would be noted like this and I found this:

“Eustachian Valve: It is a remnant of a fetal structure that directed incoming oxygenated blood to the foramen ovale and away from the right atrium.  

Incomplete regression of this structure results in a thickened ridge at the IVC/RA junction, which can occasionally be thick enough to mimic thrombus or a right atrial mass on echocardiography, cardiac CT, or cardiac MRI.

A thickened Eustachian valve may also interfere with placement of an atrial septal defect or patent foramen ovale closure device.

The Eustachian valve can be seen in the 4-chamber view or the bi cavil view of the right atrium; it is seen in approximately 25% individuals, at the junction of the IVC and right atrium. It appears as an elongated, membranous, possibly undulating structure. Usually it is of no physiological consequence, but can be confused with an intracardiac thrombus, cause turbulent atrial blood flow, complicate IVC cannulation or serve as a site for endocarditis formation”.]

Aortic Valve: The aortic valve was not well visualized. No evidence of aortic
regurgitation is seen. No evidence of aortic valve stenosis.
Mitral Valve: Trace mitral valve regurgitation. No mitral valve prolapse is noted.
Tricuspid Valve: Grossly normal. Unable to estimate Right Ventricular systolic
pressure due to inadequate or absent TR Doppler signal. There is trace tricuspid
regurgitation.
Pulmonic Valve: The pulmonic valve was not well visualized. No pulmonic valve
stenosis. Trace pulmonic valve regurgitation.
Vessels: IVC is normal in size with normal inspiratory collapse suggesting a normal
right atrial pressure (3) rnrnHg.
Aorta: The aortic arch was not well visualized. Aortic root is normal in size. No
obvious coarctation of the aorta noted by 20, Doppler.
Pericardium: No definite echocardiographic evidence of hemodynamic compromise. There is a moderate pericardial effusion localized near the right ventricle.
Shunts: There is no obvious right to left shunt at rest, with cough, or Valsalva on
agitated saline contrast examination.

The Holter Monitor:

1. Sinus rhythm, predominantly sinus tachycardia, with rates between 61-190
bpm and average rate 101 bpm .
2. Supraventricular ectopy: One isolated PAC in 24 hrs.
3. Ventricular ectopy: One, isolate 7 beat run ~f monomorphic ventricular
tachycardia with irregular rate 169 pm at 11:59 AM; otherwise, no other
ventricular ectopy.
4. Longest R-R was 1.2 seconds during sinus arrhythmia.
5. Symptoms of “fatigue, faint, abdominal pain, dizzy, chest pressure, chest
pain, flutter, chest tightness, pre– syncope,” and patient events all correlated
with sinus tachycardia, and in particular, the  symptom of “pre-syncope” correlated with sinus tachycardia 185 bpm; with the  7 beat run of monomorphic ventricular tachycardia was asymptomatic.

The Exercise Stress Test:

Summary:

1. Fair age- and gender-adjusted exercise capacity.
2. No evidence for exercise-induced ischemic ECG changes at the level of
exercise achieved.
3. Normal HR response (patient held Metoprolol for 48+ hours prior to exercise,
normal BP response. Target heart rate was achieved.
4. Pulse oximetry readings were greater than or equal to 95% on room air
throughout -the study.

Note: The baseline ECG reveals sinus tachycardia, rate of 107 bpm. ST-T shifts of ischemia or ectopy noted.

The Cardiac MRI w/ and w/o Contrast:

RESULT: Cardiac MRI
Clinical History: Pericardial effusion, cardiomyopathy. Evaluate LV function
delayed enhancement pattern
Technique: Following initial axial haste images, cine and dark blood images were
obtained in short axis, and vertical and horizontal long axis. 20 ml of ProHance
were administered intravenously, without adverse event. Immediate images were
obtained for perfusion. Delayed images were obtained in all 3 planes to evaluate
for delayed hyperenhancement. VIBE sequence was additionally acquired through the
lungs.
Cr: 0.95
eGFR: 72

The National Kidney Foundation (NKF) suggests only reporting actual results once values are < 60 mL/min (they state normal values as 90-120 mL/min). An eGFR below 60 mL/min suggests that some kidney damage has occurred.

KIDNEY DAMAGE STAGE DESCRIPTION GFR OTHER FINDINGS
1 Normal or minimal kidney damage with normal GFR 90+ Protein or albumin in urine are high, cells or casts seen in urine
2 Mild decrease in GFR 60-89 Protein or albumin in urine are high, cells or casts seen in urine
3 Moderate decrease in GFR 30-59
4 Severe decrease in GFR 15-29
5 Kidney failure <15

Findings:
Survey images of the mediastinum show normal heart size. There is no pathologic mediastinal adenopathy or pleural effusion.
Atria: Right and left atria are normal in size and contract normally.
Right Ventricle: Right ventricle is normal in size. Globally preserved systolic function is preserved. No wall motion abnormality.
Left Ventricle: Normal size and wall thickness. Globally preserved systolic
function without wall motion abnormality.
Pericardium: Small pericardial effusion without evidence of constrictive physiology.

Perfusion images show: Homogenous perfusion without focal abnormality.

Delayed hyperenhancement images show: No delayed myocardial enhancement. Apparent focus of increased signal intensity seen at the lateral aspect of the base appears most consistent with a focus of epicardial fat when comparing to SSFP images and four-chamber and short axis sequences ( four-chamber series 3 image 59, short axis series 57 images 1 through 3) .

Left ventricular ejection fraction: 59%
End diastolic volume: 84 ml
End systolic volume: 34 ml
Stroke-volume: 50 ml
Cardiac output: 4.2 liters per minute
Left ventricular myocardial mass (at ED): 59 g
Right ventricular ejection fraction: 51%

IMPRESSION:
No delayed myocardial enhancement to suggest infiltrative cardiomyopathy.
Preserved LV systolic function without wall motion abnormality.
Small pericardial effusion without evidence for constrictive physiology.


INTERVAL HISTORY:
Nichole returns following her initial visit with me on 07/08/2015. At that time, we had performed an echocardiogram to assess for LV size, systolic function and possible pericardial effusion. This revealed the presence of a moderate size effusion without clear evidence of hemodynamic compromise. Also surprising was the presence of borderline reduced LV systolic function.. Based upon these findings, a cardiac MRI was run and revealed preserved LV systolic function, EF 59%, with normal left ventricular end diastolic and systolic volumes. RV ejection fraction was also normal at 51%. There was a small pericardial effusion which was circumferential and without clear evidence of septal shift or other stigmata of constrictive physiology. Also surprising was the presence of what was identified to be a 4-beat run of wide complex tachycardia which occurred at a rate of 169 beats per minute at 12:00 a.m. There were no associated symptoms. There were multiple entries recording complaints of fatigue, faint abdominal pain, dizzy, chest pressure, chest pain, flutter, chest tightness and presyncope, all of which were correlated with sinus tachycardia. Based upon these findings, we start Nichole on metoprolol XL 25 mg daily, which she initially felt somewhat more fatigued and dizzy on, but since that time has adjusted to. She has also made a more concerted effort to use volume and sodium loading, for which she feels better overall from a POTS standpoint. She continues to report left-sided chest pain, which is not necessarily positional in nature.

REVIEW OF SYSTEMS:
Positive for weight gain, fatigue, loss of appetite, chills, dizziness, nosebleed, shortness of breath, chest pain, palpitations, near fainting/fainting, leg cramps, abdominal pain, diarrhea, constipation, nausea, vomiting, blood in stool, rash, itching, nighttime urination, snoring, back pain, muscle aches and joint aches. Comprehensive review of
other 12-organ review of systems is otherwise negative.

ALLERGIES:
Epinephrine caused adverse reaction.
IMPRESSION:
1. Pericardial effusion in the context of presumed autoimmune disorder not otherwise specified, possibly lupus with negative antinuclear antibody (ANA), undergoing further evaluation with [Immunology]. Pericardial effusion does not appear to be associated with constrictive physiology by echocardiographic criteria. This is likely chronic in
nature. I cannot exclude the possibility of chronic pericarditis as a contributing cause to her chest discomfort.
2. Probable postural orthostatic tachycardia syndrome (POTS), complicating #1.
3. New onset wide complex tachycardia, possibly ventricular tachycardia. I cannot exclude atrial dysrhythmia with aberrancy tolerating beta blocker therapy, with associated preserved left ventricular (LV) systolic function by magnetic resonance imaging (MRI), which is gold standard data for ventricular volumes and function.
4. Prior history of superior mesenteric artery (SMA) syndrome and May-Thurner syndrome.

PLAN:
1. Referral to Dr. [Vascular Surgery] at the University cardiovascular center for a second opinion.
2. Consider initiation of low-dose ibuprofen. I will discuss this plan of care with Drs. [gastroenterology] and [immunology] to ensure that this is appropriate from their perspectives.
3. Continue beta blocker therapy for ventricular tachycardia versus supraventricular tachycardia (SVT) with aberrancy with monitoring symptoms.
4. Return to clinic in three months’ time for clinical reassessment.
5. Repeat echocardiogram in three months’ time for reassessment of pericardial effusion, particularly should we initiate nonsteroidal anti-inflammatory drug (NSAID) therapy.
6. Avoid prednisone therapy due to potential provocation of a relapsed pericarditis.
7. Collaborative care with Drs. [gastroenterology] and [immunology].


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The good news: My heart is not failing like they originally thought on the left side. In fact, my ejection fractions are higher on the left than the right.

The bad news: Looks like there are some valvular problems with blood flow, but only mildly. Also, there are a few structural and/or congenital abnormalities which have led me to some further research that is quite interesting, but I won’t post until I’ve got a few more pieces together and have talked to the doctor about it.

Finally, there’s no significant answers or treatment plan at this time. At this point we are continuing medications as previously prescribed. Both the GI and the Immunology doctors gave the go ahead to start 200mg Ibuprofen twice a day (although right before this test I was on 800mg ibuprofen one time a day for bleeding following my endometrial ablation – which means it probably won’t reduce the pericardial effusion, but we’ll see).

The cardiologist does not want to start me at the dysautonomia clinic until he is sure that the effusion is not contributing to my POTS symptoms, although he doubts that it is. He still firmly believes I have P.O.T.S. or some form of dysautonomia, but he doesn’t want to send the referral just to get sent right back over something he should be handling on his own anyway. Still no idea what could be causing the pericardial effusion, but the cardiologist continues to believe it is something autoimmune related (due to its chronic nature), despite what the immunologist now says about having zero indications for autoimmune disease (even though that wasn’t what she told me) but not sure if we’ll ever find it if it is. Did a random skin biopsy on my arm last week when I had a “vasculitis-type rash”, but it came back inconclusive as well. I should know more when I get the report when I get the sutures out of Tuesday.

The cardiologists did, however, give me a referral for a second opinion from vascular surgery at University. I think he has some ideas on some of the research I am contemplating as well, but he won’t say at this time. He put my order in as “Urgent”. The new hospital called a couple of days later to schedule, but I couldn’t get the other hospitals to send records and scans over it time for the appointment. We rescheduled with them for this upcoming Tuesday. I’m hoping they can shed more light on the impact of the compression disorders or, at the very least, believe they exist (which they do).

It’s been a whirlwind couple of weeks as far as medical stuff goes. While I’m tired and ready for a break, at least we’re getting somewhere and I’m not going to die like I thought after the first few phone calls from the cardiologist’s office. My heart is not “normal”, so it’s not good news but it’s not bad either. So far, the Metoprolol is not helping the tachycardia, but I am still on a low dose and I really need to call the doctor and see if we can do an increase. So while this is the end of the chaos with cardiology, at least for now, it’s only the beginning for so many other new doctors and appointments.

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Oh doctor, doctor, I must have gotten this sick somehow. I’m going to ask you a series of questions, and I want them answered on the spot, right now.

Patience has never been a virtue of mine, as many of my friends and family can attest to.

But I feel that I have been extremely patient with my doctors, maybe more than I should be.

I wasn’t planning to do an update until I received the final confirmation from my doctors,

but it seems like that won’t be happening anytime soon,

so I figured that no time is better than the present.

It’s been just about a month since my abnormal tests came back. I have left multiple messages for both doctors, one every week, hoping for a callback or at least an idea as to whether or not I need to come in for an appointment to discuss the test results in person.However, all I got was silence. I literally felt my blood boiling each day that went by that I didn’t receive a call. 

I’d whine about how:

“They’re delaying my care.”

“Sure, just because I’ve been sick forever, no reason to rush or anything.”

“I don’t know how doctors can get away with not calling when tests are abnormal.”

And, of course, the classic:

“I’m going to die before they ever figure out what this is.”

I was feeling sorry for myself, to say the least. I felt alone, abandoned, and lost as to what to do next. It was really starting to mess with my head. I hated being this way but I hated how these doctors were making me feel even more.

I felt like they were completely disregarding everything I have gone through

in my search for a diagnosis.

The years of life I have lost due to being sick.

The hours spent driving to appointments.

The amount of time in hospitals and doctor’s offices.

The high levels of radiation and all the discomfort in medical testing.

The countless pills prescribed, which often caused more side-effects than actual relief.

The procedures and surgeries, which also didn’t fix my disease.

The friends and family I lost, because they didn’t understand.

My career goals and aspirations that have been placed on hold.

Everything I once was and what I could be.

My whole life is on hold!

And here, right in front of you, are these abnormal tests

that provide answers to my chronic, undiagnosed illness

that I’ve fought so hard, for so long, to find

and you can’t pick up the phone to call me? 

Maybe I am being impractical,

but a month spent waiting seems way too long.

That’s a month to complete more testing or get second opinions.

Four weeks of treatment or management.

30 days of my life lost, left waiting.

FINALLY, a nurse from my GI, Rheumatologist/Immunologist, and Cardiologist’s office called, but she only caused  more confusion and frustration than before. She tells me my fecal cultures came back normal, which I already knew. She then says my cortisol levels were low (again, I’ve had my test results for weeks) and the doctor would like me to rerun it to be sure. She’s going to mail the paperwork so I can at least have my blood drawn in town, instead of driving over an hour. Great, thank you. She also tells me to stop taking my lupus medications… wait, what? The GI doctor and the Rheumatologist/Immunologist agree that I should stop taking them, because I’m not autoimmune. Um… ok. How come she originally, as did all the other doctors, think it was seronegative autoimmune disease? Specifically, Lupus?  I’m not in the mood to argue, I’ve been feeling terrible. I ask if I stop them forever, she says yes and the conversation is over.

She calls again yesterday, just to tell me to stop taking the Lupus medications… again. Yes, you told me the day before. Both doctors think it may be causing my painful and urgent diarrhea.Um, I have had on and off urgent diarrhea for… like… ever. But ok. So it’s not because it can’t possibly be autoimmune? I’m confused. She asks if the medication has helped. I said I’m over the initial side effects. I’ve had constipation for the past few days, so don’t think it’s causing diarrhea, and I haven’t had any extreme photosensitivity as in the past weeks, but still having outbreaks of rashes. I don’t know what is causing what anymore. My symptoms are too random and sporadic. She says to call next week or the week after, let them know if being off the medication makes me feel better or not, and then we will restart it if it’s not the problem. Wait… so let me get this straight? Now you’re taking me off it, to see if it’s having side effects I have had forever, only to restart it and have to adjust to it once again? Are you kidding me? Well, it may be affecting your cortisol levels, so they want to see if I stop it if it’ll change my blood test.  I am beyond confused and frustrated at this point. I went and redid my cortisol blood test this morning, so I guess we’ll just wait and see what that tells us.

Still haven’t gotten a call from the vascular surgeon. My CT Angiography was the one I was MOST worried about and  I fretted every night about him not calling me to discuss the findings. . Finally fed up, having left four messages now, I called and scheduled an appointment to discuss. I don’t know whether or not I trust him to treat at this point, given the lack of respect of promised phone calls with no answers, but he may just not know what to do or say about the finding. This is the doctor who didn’t believe in Nutcracker Syndrome at all for his own, valid reasoning, but admitted my original CT Scan showed the most convincing case of Nutcracker Syndrome that he has seen in over 30 years. He ran the CTA to “prove him wrong”. On the order form for the test, he even wrote “to exclude Nutcracker Syndrome”, instead of “evaluate for Nutcracker Syndrome”. He was really convinced I couldn’t have it, it’s too rare, and most vascular surgeons don’t think it’s a real thing.

Well, guess what? I proved him wrong. Not only that, they found two more (even rarer)  vascular compressions. The radiologist noted both May-Thurner Syndrome and Superior Mesenteric Artery Syndrome, although the Nutcracker Syndrome is the most extensive. So perhaps, maybe he is lost as to what to do or say at this point, I don’t know. But I’d rather him tell me that if that is, in fact, the case, rather than be silent about it. But I have an appointment now, so he can’t ignore me. So we’ll see how that goes.

I also had my consultation with cardiology last week. It had gone way better than expected and I really liked the doctor. He not only listened to me, he caught things other cardiologists had  missed in the past, and had my notes completed (and accurate) by the end of the day. I was fearful for this appointment, as I have not had the best luck with cardiologists in the past. They always say they hear a “murmur” or “valve issue”, order tests, and then call me crazy. This has happened on multiple occasions, both in my teens and early twenties. So you can see how I’d be nervous about going straight into an appointment saying “I think I have POTS syndrome and so does my neurologist and the immunologist (although she seems to have forgotten EVERYTHING she told me in my last appointment, so maybe she doesn’t think I do anymore, who knows)”. I show him the letter from the neurologist and my ‘poor man’s tilt table test’ results. He says that it looks like I have POTS, but he wanted to have some “orthostatic fun” in the office just to see. He measured my heart rate and blood pressure while laying down, sitting up, and standing.  Sure enough, my blood pressure dropped really low and my heart rate increased up to 150. Yep, he’s pretty convinced that it is POTS, but because of the missed information in previous cardio tests, he wants to rerun them again just to make sure it’s not something “easier” or misdiagnosed.

In my echocardiograms from 2005 and 2007,  he noticed that there was what he called “abnormal electricity” shown, but the EKG didn’t catch it, so it was dismissed. It happened again in my 3D echo from last year. Also, the 3D echo from 2014 showed I had pericarditis, which is a typical sign of autoimmune (particularly Lupus), but, of course, need to rule out other possible causes as well. And finally, my halter monitor from 2007 showed abnormalities and heart beats exceeding 160 bp, which was also dismissed during that time. So he ordered a 3D echo again, to see if the pericarditis has cleared on its own or if it’s gotten worse. He also wants to evaluate the possibility of a hole in my heart (which many people are born with, although it usually clears up on its own as you get older) since they can’t confirm the cause of my hypoxemia, other than the mild sleep apnea that was confirmed through my sleep study last year (although he doesn’t believe that is what is causing it, because again, it was very mild and happens sporadically during the daytime as well). So I’ll be back in the hospital tomorrow to complete all of these cardio tests. If  all the differential diagnoses are excluded, then he will be referring me to the dysautonomia clinic for further treatment, but was comfortable enough to put down Postural Orthostatic Tachycardia Syndrome as an official diagnosis. Again, something I have been saying since I started looking for answers. Finally! So we’ll see how testing goes tomorrow and I guess go from there.


So what does this all mean?

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It’s more than just ONE cause, obviously.

There are multiple conditions feeding off of one another, 

making my conditions not only rare,

but also complicated to treat and manage.

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Here’s what is on the table (so far):

Dysautonomia:

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More specifically —> Postural Orthostatic Tachycardia Syndrome (POTS):

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Nutcracker Syndrome:

“The nutcracker syndrome is quite a rare condition. It is due to the compression of the distal segment of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta (also called left renal vein entrapment).  This syndrome needs treatment when symptoms are disabling” (Hartung, O., 2009, p. 246).

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Superior Mesenteric Artery Syndrome (SMAS):

“Superior mesenteric artery syndrome (SMAS) is a digestive condition that occurs when the duodenum (the first part of the small intestine) is compressed between two arteries (the aorta and the superior mesenteric artery). This compression causes partial or complete blockage of the duodenum. Signs and symptoms may include abdominal fullness; bloating after meals; nausea and vomiting; and abdominal cramping that may be helped by lying in certain positions.” (NIH Office of Rare Diseases, 2014)

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May-Thurner Syndrome:

“May-Thurner syndrome (MTS) is caused when the left iliac vein is compressed by the right iliac artery, which increases the risk of deep vein thrombosis (DVT) in the left extremity. DVT is a blood clot that may partially or completely block blood flow through the vein. Even though DVT itself is not life-threatening, the blood clot has the potential to break free and travel through the bloodstream, where it can become lodged in the blood vessels of the lung (known as a pulmonary embolism). This can be a life-threatening condition” (ClevelandClinic.org)

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Symptoms and Causes of Low Cortisol Levels:

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A lower than normal level may indicate:

  • Addison disease, in which the adrenal glands do not produce enough cortisol
  • Hypopituitarism, in which the pituitary gland does not signal the adrenal gland to produce enough cortisol
  • Suppression of normal pituitary or adrenal function by glucocorticoid medications including pills, skin creams, eyedrops, inhalers, joint injections, chemotherapy

Other conditions for which the test may be ordered include:


Is this a final diagnosis?

Knowing how thing have gone in the past, it’s  highly doubtful.

Maybe there is more to the story…

Maybe there is less significance…

Only time will tell.

Again, I am left waiting…


Now if only I can get all my doctors organized and working together, maybe I can clear up what this all means and what needs to be done next. Surgery? Medications? Again, who knows… 

While I DO know for sure that NONE of these diagnoses will ever be “cured”, I’m hoping we can at least find a way to manage everything so I could hopefully live a semi-normal life again.

I still have to do a hydrogen/methane breath test next week, as well as upcoming appointments the week after with dermatology (to run biopsies on my skin rashes, hopefully to “catch” the autoimmune disease that’s hiding in my skin) and a follow-up with the vascular surgeon. Plus the results from the cortisol testing I did today and the cardio tests tomorrow. Let’s hope we can get the pieces put all together and figure out what’s next as far as treatment goes.

I guess I’ll just have to wait… something that is unfortunately becoming entirely too common at this point, but at least we’re getting somewhere… slowly. 

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