Medical Testing Update: Part 1

I’m not entirely sure which is worse:

Having every test comes back normal and

you have no idea why you feel so horrible everyday

-or-

The sudden onset of one abnormal test after another,

slowly taking away any hope of you ever feeling better.


I’ve dreaded  writing this post, mainly because I still have so many more questions than I do actual answers at this point, but the final test results should be revealed at my follow-up on Wednesday and I figured that there is no time like the present. Well, there’s that, and also the fact that I want to share these findings in their entirety and explain exactly why I’ve been so quiet lately.

So much to say and not enough time or energy to do it all at once.

So this post will be broken down between a few different parts.

So much has gone on in the last couple months. These days, I don’t know what is connected to what anymore, particularly when it comes to my symptoms. I always knew that my diagnosis wouldn’t be a simple one, but it seems like every week now the doctors add one more new problem to the list. It’s been a lot to take in, especially because life keeps throwing in additional added stress that I honestly could do without currently. I haven’t had much time to think about anything, let alone process what this all means. I honestly haven’t even been able to research these results like I normally would, thus being left with so many unanswered questions. The doctors may lose it when I walk into my follow-up appointments with the two-page list of questions that I’ve already put together without even really trying.

If you remember from my earlier posts “Welcome to the new age” and “Oh doctor, doctor…”, tests had just come back, confirming I had three vascular compression syndromes (Superior Mesenteric Artery Syndrome, Nutcracker Syndrome, and May-Thurner Syndrome) as well as POTS (Orthostatic Tachycardia Syndrome), and I had just started care with a new cardiologist, who just so happened to notice some irregularities in my earlier cardiac tests from back in 2005, and again in February 2014 (but the results were dismissed by two different doctors for being “insignificant”). Anyways, he decided to order some follow-up testing before referring me over to the dysautonomia research clinic at the university’s medical school for treatment, hoping they’d also be able to refer me to one of the very few doctors in the country that treat the vascular compression disorders. I had honestly believed going into this round of medical testing that these tests would be (again) a waste of time, as they have been “insignificant” for 10 years now. I knew, though, that once these tests were finally completed that I’d be able to begin treatment for [at the very least – one part of] my illness. Obviously I obliged. I also had one more test that my GI doctor had also ordered, but figured that the non-stop medically testing was almost truly over. Finally!

But I was wrong… so very wrong.


Initially, the cardiologist ordered two tests:

Echo1. Echocardiogram with contrast.

 An echocardiogram is a test that uses sound waves to create pictures of the heart. This test is done to evaluate the valves and chambers of the heart from the outside of your body.

An echocardiogram can help detect:

Abnormal heart valves

Abnormal heart rhythms

Congenital heart disease

Damage to the heart muscle from a heart attack

Heart murmurs

Inflammation (pericarditis) or fluid in the sac around the heart (pericardial effusion)

Infection on or around the heart valves (infectious endocarditis)

Pulmonary hypertension

Ability of the heart to pump (for people with heart failure)

Source of a blood clot after a stroke or TIA

Contrast Used: Agitated Saline Injection

 Agitated saline solution administered via intravenous injection provides air microbubble contrast in the right heart. The air microbubbles are short-lived and diffuse into the lungs when traversing the pulmonary circulation. Therefore, the microbubbles enter the left heart only in the presence of a right to left intracardiac or extracardiac (pulmonary arteriovenous) shunt. Saline microbubbles are therefore helpful in examining the right heart and identifying shunts or holes in the heart.

2. Holter Monitor (24hr)

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A Holter monitor is a machine that continuously records the heart’s rhythms. The monitor is worn for 24 – 48 hours during normal activity. Electrodes (small conducting patches) are stuck onto your chest. These are attached by wires to a small recording monitor. You carry the Holter monitor in a pocket or pouch worn around your neck or waist. The monitor runs on batteries. While you wear the monitor, it records your heart’s electrical activity.

Holter monitoring is used to determine how the heart responds to normal activity. The monitor may also be used:

After a heart attack

To diagnose heart rhythm problems

When starting a new heart medicine

It may be used to diagnose:

Atrial fibrillation or flutter

Multifocal atrial tachycardia

Palpitations

Paroxysmal supraventricular tachycardia

Reasons for fainting

Slow heart rate (bradycardia)

Ventricular tachycardia


Like I said , I’ve had these tests before and I really wasn’t too worried about them.

Hell, the last time I even had a holter monitor on (back in 2005), I had to wear that baby for an entire month… in the middle of summer… in Florida… and you can’t get it wet… at all… even with sweat… So, 24 hours in Colorado – no problem. I’d say my symptoms were low-average on the day that I had the monitor on. I kept thinking to myself, of course, you’ll have a semi-good day for the first time in a long time BECAUSE you’re being monitored. I did have my usual tachycardia and dizziness, plus one episode of syncope, but nothing I would call spectacular or extraordinary, though. I did, however, have one very loving foster dog concerned about why I had wires and a box attached to me, so he was by my side non-stop throughout the entire day.

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Anyways, the next test was the echocardiogram. The saline contrast was definitively the most interesting part of the test- at least from a patient’s perspective. I was disappointed that they had me facing away from the screen when they actually injected it, but you can feel the bubbles as they move through the body and to your heart. Plus the second nurse was fun to watch, as she had no choice but to demonstrate good coordination skills when “swishing” the saline solution back and forth between the syringes, all while holding my IV catheter in at the same time since my veins refused to cooperate that day.

When the tests were done, I was told they’d be back in a few days and that the doctor would give me a call. Although, typically it’s the nurse who calls -not the doctor- but that’s beside the point. I went on my merry way and didn’t think twice about it again.

The next morning, as I’m still asleep from being up late due to sickness the night before, I see a missed call from the hospital and it shows that I have a voicemail. I press play and realize it was the cardiologist HIMSELF calling. Uh, oh… I immediately call back, but have to leave a message as he in currently in with patients. Well, crap!

Still, I didn’t expect the doctor to have found anything of significance anyways, so I let it go out of my mind and got started on the rest of my day. That afternoon, the nurse from the cardiologist’s office calls (I guess the doctor was still busy with patients) and says that the doctor wanted to make sure that someone called me with the echocardiogram results that had come in earlier that morning, although they were still waiting for the holter monitor results. She then tells me that the test showed that I didn’t have a hole in my heart, which is what was originally expected based on the previous cardiac results. Good, I think myself… another normal test.

For once, I don’t want abnormal tests – not when it comes to my heart, anyways.

Even more so because I watched my mother die from a heart attack at age 49.

But the nurse wasn’t done. Oh… She goes on to say that, unfortunately, it looks like there is still some pericardial effusion (which was also seen on the Feb 2014 test). More importantly, though, it looks like your heart is failing… *Excuse me, did you just say thatmy heart is failing?!?!* Well, your ejection fraction is less than 50% on the left side, which means your heart is pushing out less blood than it should be. The doctor wants to order a couple more tests if that’s okay.

*Of course it’s okay…

Am I really going to say no

directly after you just told me that

I’m going into heart failure.*

I’m connected over to the scheduling department at the main hospital to schedule an exercise stress test. They also have already started on the pre-authorization process for an MRI of my heart as well… with contrast. (Sadly, no bubbles this time around, just gadolinium). The hospital tells me that I’ll get a call soon, once my insurance approves the test, but the doctor wants this test done ASAP. I hang up the phone, in shock.

Did she really just say my heart is failing? I heard that correctly, right? 

I walk out to the deck and sat down quietly. The first thing I do, of course, is start Googling everything I can: “heart failure”, “low ejection fraction”, “heart failure, left side”, “low ejection left side” “causes of low ejection”…

Ejection-Fraction

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Just then, my husband walks out. He always has such good timing.

“What did the doctor have to say?

I pause for a moment, not sure what to tell him 

-or-  

if I can tell him at all…

Can I even say it out loud? I’m not sure.

“They need to order more tests because my heart isn’t pumping correctly, something about the left side and the ejection fraction.” 

I don’t want it to be a big deal, ya know.

At least not yet.

Not until I see the results and it’s all matter-of-fact.

“What did they order?”

“An exercise stress test and an MRI of my heart…”

My husband works in health insurance.

He knows how hard it is to get MRI’s approved because of the expense.

Typically, there has to be history and prior tests.

Unless there’s a legitimate reason the patients needs an MRI,

as opposed to other diagnostic tests.

“So what does this mean?”

“It means that my heart is failing…”

And with that, there’s nothing much more to say.

Of course, now I am worried. And starting to get scared. But what can I do but wait?  Besides, maybe it’s not that bad and they are just being cautious. It is the heart, after all. I try not to work myself up over it.

A few friends stop by that night and we act like everything is normal, because -ultimately-  it is relatively normal for me. I’ve lived with this condition for years, remember. Tests showed abnormalities 10 years ago.

10 YEARS! 

Plus, we’re still waiting for the holter monitor results. There’s no way they’ll come back any worse, especially when I wasn’t even that sick during the recording time.

But I was wrong, so very wrong… again.


To Be Continued…

Shatter every window till it’s all blown away

Living with a chronic illness is like surviving a bad storm.

Some storms blow in faster than others, almost unexpectedly.

The winds start to pick  up,  sweeping everything that you once knew away.

Some storms come in as a light drizzle, clouded by gray.

While others bring in the darkness, turning the day into night.

And you’ll never know what will be left behind once the storm is over.

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I was numb after I received the call from the cardiologist’s office.

Maybe I was just in shock. Or possibly in denial. I’m not sure.

It took until today to finally hit me, though.

And it felt as if I had been stricken down by a large bolt of lightning, riveting from the sky.

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Usually, abnormal test results don’t bother me. They typically lead to answers, bringing us closer to a diagnosis. More often than not, I pray for abnormal results. I wasn’t, however, prepared for what came back in the echocardiogram. It wasn’t good news.

With every word spoken, I watched my hopes and dreams  slowly wash away.

What does this mean as far as my current diagnoses?

How will this affect treatment?

What’s going to happen with my life?

There’s so much I have left to do and say.

Things that I want to fix.

To accomplish with my life.

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 Is it really as a big deal as it sounds? More than likely, yes. But nothing is definitive until I go for additional testing and confirm some more things. However, I can say that this news has given me the most doubt and fear  I’ve ever felt since I started my search for a diagnosis.

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It wasn’t until today, though, that the storm really started to roll in.

I didn’t want anyone to know how worried I really was, so I kept it all deep inside. As this morning went on, it was just one little thing after another. Over and over. The pressure inside me began to build until there was nothing left to do but explode.

The anger and sadness came out of nowhere, like a tornado in the sky.

I just want this all to be done and over with for good.

I don’t think I’ll survive through any more hard blows.

I can only hold out in this storm for so long.

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I’m not sure what to do at this point.

Or how to really handle this type of news.

I still haven’t told my family as of yet.

I’m not even sure whether I should tell anyone all.

How do you even bring up something like this?

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Would anyone even care? 

Would I be treated differently?

Will everything change?

Maybe. But maybe not. 

While I do know there are people out there who love and care for me more than I probably know,

I also know the way it hurts when someone that I love doesn’t seem to have the time

or the interest to know what is going on with my health and my illness.

 It was just today that I heard that someone close to me said that I’m addicted

to both stress and being chronically ill.

Don’t get me wrong, these words cut me pretty deep,

but I wonder if maybe it’s a result of putting myself out there for the world to see.

Knowing what I know now,

in addition to the people who make it abundantly clear they don’t care anymore,

Do I tell them now anyways or wait for them find out on their own?

It’s always the uncertainty in these types of decisions that scare me the most.

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Plus I don’t have any real answers at this point regarding what can be done about it, if anything at all. 

So maybe it’s best to keep it a secret, at least for now.

Or until  I get some more clear cut answers.

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For now, I just need to keep my focus

and not let my emotions get the best of me this time around.

My list of things I need to do is still a mile long,

and there is no time left to waste.

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It’s just too hard to see past all the rain sometimes.

But in the end,  I know that I can face the truth in what lies ahead for me

and that there will be some kind of light that follows shortly after the storm.

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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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