Poetry for POTSies

A friend recently reminded me of one of my favorite poems from my childhood that my second grade teacher, Mr. Markey, used to read to us in the most animated way possible.  Shel Silverstein's "Sick" is one of the many funny poems from his fantastic children's poetry book, Where the Sidewalk Ends.  Upon reading this poem for the first time in about 20 years, I had to laugh because this character sounds like a dysautonoma patient. My commentary is in blue.

Sick

"I cannot go to school today,"

Said little Peggy Ann McKay.
"I have the measles and the mumps, (these were really hives from MCAD and parotid gland swelling from Sjogren's)
A gash, a rash and purple bumps. (delta-storage pool bleeding disorder, more MCAD and those purple goosebumps are a Midodrine side effect)

My mouth is wet, my throat is dry, (trench mouth is really a candida infection, probably caused by persistent dry mouth - dry mouth and throat are caused by low parasympathetic tone failing to stimulate salivation)`
I'm going blind in my right eye. (she had a grey out/pre-syncopal episode)
My tonsils are as big as rocks, (latent EBV infection)
I've counted sixteen chicken pox (it's not pox, it's more hives from MCAD)

And there's one more--that's seventeen, (that one is a Lyme, Babesia and Bartonella infected tick bite)
And don't you think my face looks green? (she's going to puke because of her gastroparesis)
My leg is cut--my eyes are blue-- (poor wound healing from EDS and that bleeding disorder, and evidence of northern european ancestry which seems to make her more prone to this stuff)
It might be instamatic flu. (or pheochromocytoma?)

I cough and sneeze and gasp and choke, (glossopharyngeal nerve hypersensitivity)
I'm sure that my left leg is broke-- (from all that klutzy fainting)
My hip hurts when I move my chin, (myofascial pain - it's all connected)
My belly button's caving in, (IBS/gastoparesis, or just the scar from her amyloidosis stomach fat biopsy)

My back is wrenched, my ankle's sprained, (EDS double whammy!)
My 'pendix pains each time it rains. (not her appendix; it's recurrent autoimmune pancreatitis)
My nose is cold, my toes are numb. (Raynaud's and sensory small fiber neuropathy)
I have a sliver in my thumb. (happened as she slid down wooden stairs on her tush to get herself a glass of water, because she couldn't stand up)

My neck is stiff, my voice is weak, (EDS, kyphosis and more glossopharyngeal nerve stuff)
I hardly whisper when I speak. (acid reflux)
My tongue is filling up my mouth, (anaphylaxsis or hereditary angiodema)
I think my hair is falling out. (hypothyroidism or vitamin deficiency - if it's the hair on her legs, it's severe small fiber neuropathy)

My elbow's bent, my spine ain't straight, (more EDS)
My temperature is one-o-eight. (oh shoot, she's got a PICC line infection)
My brain is shrunk, I cannot hear, (CCSVI?, hearing loss is due to temporary effects of cerebral hypoperfusion)
There is a hole inside my ear. (the one and only normal thing about her)

I have a hangnail, and my heart is--what? (hangnail due to fragile EDS skin and she was just about tell tell us about her upright tachycardia)
What's that? What's that you say?
You say today is. . .Saturday?
G'bye, I'm going out to play!" (malingering or it must have all been in her head!!!!!)

------

Now don't everyone get all mad at me for the ending.  I think we have to take a look at ourselves and laugh sometimes.  We really are a bunch of hot messes.  Every single one of those blue comments is something that happened to one of my POTS friends, and many of them have happened to me.  Sometimes, when your body really is falling apart or not cooperating the way it should, a good sense of humor is all you have to get you through the day.  I hope this post made you smile and laugh.  I had fun writing it.

Dysautonomia Awareness Month Tweet-A-Thon - Oct. 24th!

Rally the troops! The Dysautonomia Awareness Month Tweet-A-Thon is tomorrow, Wednesday, Oct. 24th. We are mass tweeting to get increased media coverage and recognition from the World Health Organization.

Here's a website that explains how you can participate.  This will only take 2 minutes:
http://www.dysautonomiainternational.org/page.php?ID=126

Please feel free to re-post this to other dysautonomia groups and blogs.

POTS Research Study - Enrolling Now!

Calling all POTSies ages 14-29! Your participation in a POTS research survey is needed. The research is being conducted by super-smart POTS patient and future doctor Amanda Ross, Dr. Julian Stewart of New York Medical College and Dr. Peter Rowe of Johns Hopkins. We need LOTS of people to participate, and the best part is you don't have to leave your couch.

Click here for more information on the study.

Skin Biopsies for POTS Patients - worth it?

If you want the short answer, yes, I think skin biopsies looking for sensory and sudomotor abnormalities are worth it for some POTS patients.

The long answer...
I'm not a doctor, so you should discuss this with your own physician.  I am a POTS patient who was able to figure out the underlying cause of my POTS because a chance skin biopsy performed on me revealed autonomic neuropathy.  This led me to push my doctors to find the cause of the neuropathy, since I was sure the autonomic nerve damage was causing the POTS symptoms.  We found the underlying cause of my nerve damage (and autoimmune disease, Sjogren's Syndrome), treated it directly with IVIG and now I am doing awesome.  We have used skin biopsies to monitor the progress of my treatment.  We have seen the nerve damage heal over time because we are treating the root cause of the nerve damage, rather that just masking symptoms with the traditional POTS medications.

Why are skin biopsies even relevant to POTS?
Mayo Clinic has two different studies showing that at least 50% of POTS patients have a peripheral autonomic neuropathy.  It does not seem to be a full body widespread autonomic neuropathy in most patients.  One reason we know this is because, on the whole, POTS patients do not have cardiac autonomic neuropathy, which is seen in about 20% of diabetic people who have diabetic autonomic neuropathy.  That is a more serious form of autonomic neuropathy.  Of course, POTS is serious, but the neuropathy involved with typical POTS doesn't appear to impact the heart.  Our tachycardia is not caused by nerve damage to the heart.  It is essentially a healthy tachycardic response to other stresses on our vascular system (low blood volume, increased norepinephrine, venous pooling in the legs and stomach, etc.).

I believe that all patients should try to learn as much about their own manifestation of POTS as possible.  You can then try to tailor your therapies to what you find out.  For example, if you have EDS, you would make sure that you take extra precautions during exercise.  If you have autonomic neuropathy, you would make sure you are not taking any medications that could make this worse (some medicines can cause neuropathy, others can make the symptoms of it worse).  Also, if you have confirmed autonomic neuropathy, it means your doctors should be helping you look for the cause of the nerve damage.  If your doctor won't do that, then you need to find a new doctor.

Not everyone who has autonomic neuropathy will be able to determine the underlying cause of the nerve damage.  However, to me, it is unacceptable for physicians to dismiss nerve damage as "idiopathic," which means "of an unknown cause," until they have done a careful and methodical search for a potential cause of the nerve damage.  In my own experience, and from what I have heard from other POTS patients, physicians rarely look carefully for the cause of autonomic neuropathy in POTS.  Perhaps the patient has had a few simple blood tests checking for common things like diabetes or Lyme Disease, but after than, it seems like many doctors give up and send the patient home with an "idiopathic" diagnosis.  This is not fair to patients.  If a cause can be determined, there is a good chance that the patient will be able to directly treat that cause.  This can help them prevent further nerve damage, stabilize the damage that has already been done, and perhaps prevent future damage.  I am proof that if you treat the autonomic neuropathy directly, you can feel much better.

Additionally, I have heard from many patients who were told they didn't have neuropathy by a neurologist who only did a quick physical exam and an EMG test.  EMG testing cannot detect damage to small fiber nerves.  EMG only tests large fiber nerves.  A normal result on EMG testing does not mean you don't have neuropathy.  it just means you don't have large fiber neuropathy.  We already know that most POTS patients don't have large fiber neuropathy, and that about 50% of them have small fiber autonomic neuropathy.   Similarly, and MRI will not detect small fiber neuropathy.

What do skin biopsies test for?
In the context of POTS, there are two different types of skin biopsies.

The first is called an ENFD (epidermal nerve fiber density).  This is a older form of skin biopsy that is used to look for damage to the small fiber sensory nerves in your skin.  If you have shooting nerve pains in your feet, and sometimes your hands, tingling, burning, pins and needles... you probably have small fiber sensory neuropathy.

The second is called SGNFD (sweat gland nerve fiber density). This is a newer form of skin biopsy that is used to look for damage to the small fiber sudomotor nerves in your skin, that innervate your sweat glands.  These sudomotor nerves are part of the sympathetic nervous system, and they are considered autonomic nerves.  When the SGNFD biopsy is done, they can also look at the small fiber sensory nerves at the same time.  Interestingly, the SGNFD test can reveal sensory nerve damage even if the EPNFD is normal.  It is fairly common to have sensory neuropathy and autonomic neuropathy in the same patient, although sometimes only one type of nerve is impacted.

You can use these tests to see if you have small fiber nerve damage in the first place, and then if you do, you can have follow up biopsies over time, one year, five year, etc. to see if your nerve damage has improved or worsened or stayed the same.

Is this the same thing as a QSART?
No, but the SGNFD does look for something similar.  QSART stands for Quantitative Sudomotor Autonomic Reflex Test.  QSART involves applying a small amount of acetycholine to your skin and then passing a very small electrical current through it, and then recording how well your sweat glands work.  If the QSART is abnormal, you most likely have an autonomic neuropathy, but the QSART does not directly measure your nerve damage.  QSART was invented by Dr. Low at Mayo Clinic before the SGNFD skin biopsy was commercially available.

Where can I get an SGNFD skin biopsy?
Any doctor can order a SGNFD test kit, from anywhere in the world.  That is why the SGNFD test is so great.  You don't have to wait months or years to get into an autonomic specialty clinic.  You can just have your local physician order the test kit from Therpath.  The doctor doesn't even have to be a neurologist.  He or she can very easily perform the skin biopsy.  It is not rocket science.  Then the lab that makes the test kits will send the results and an interpretation back to you and your doctor.

Are skin biopsies reliable?
Yes, studies by reputable autonomic experts have shown that the SGNFD is more sensitive that the QSART for the evaluation of small fiber autonomic neuropathy, as well as small fiber sensory neuropathy.

Do skin biopsies hurt and will they leave a scar?
A little bit, but only a pinch for a second or two.  Your doctor can give you a small injection of Lidocaine or another numbing agent just before taking the biopsy.  The size of the biopsy is only 3mm across by 3 or 4 mm deep.  It only takes a few seconds.  I have had a total of six skin biopsies.  Some with Lidocaine, some without.  Personally, I think the injection of the Lidocaine hurts more than the biopsy itself.  After the sample is taken, your doctor will put some gauze and tape, or a regular bandaid on the biopsy site.  It will form a scab the size of a freckle and heal in a few weeks.  Of my six biopsies, only one has a visible scar, and it really looks like a white freckle.  No one notices it except for me.

October is Global Dysautonomia Awareness Month!

Dysautonomia International has declared October to be Global Dysautonomia Awareness Month!

They have created this ribbon graphic and are encouraging everyone to use it as their Facebook profile photo for the month of October.  You can also put this image on your website or blog, or use it as part of your e-mail signature for the month.

There are also lots of great events going on around the globe and online.  Please visit www.dysautonomiainternational.org for more information, or if you would like to host an event in your neck of the woods.

Autoimmune basis for POTS - new research from Mayo Clinic!

New research out of Mayo Clinic shows autoimmunity in POTS patients:

Autoimmunoreactive IgGs from patients with postural orthostatic tachycardia syndrome.

Mayo Clinic has hinted in several of its prior POTS related publications that POTS may have an autoimmune basis.  I believe this is the first study that directly investigated this subject.  This is very exciting news.

As a patient advocate, I have spoken with literally thousands of POTS patients about their symptoms.  While I'm not a doctor, I have noticed that the vast majority of them have autoimmune tendencies.  I have POTS as the result of a confirmed autoimmune disease.  I know many, many other POTS patients who have confirmed autoimmune diseases too - Sjogren's, Lupus, Rheumatoid Arthritis, Scleroderma, Celiac, Hashimoto's Thyroiditis, Myasthenia Gravis and more.

When I hear patients describing frequent rashes, skin changes, Raynaud's, symptoms of small fiber neuropathy, sensitivities to gluten, or a family history of other autoimmune conditions, that sets of alarm bells in my head.  THIS PERSON PROBABLY HAS AN UNDIAGNOSED AUTOIMMUNE DISEASE!  Of course, telling a fellow patient that doesn't really help them, and it could scare them for no reason, since I can't know for sure.  I usually encourage them to see a good neurologist and a rheumatologist, if possible.

I eagerly await additional research from Mayo Clinic on autoimmunity in POTS patients.  If anyone at Mayo is reading this, or any other interested researchers, I really want to see a trial of IVIG therapy on POTS patients with confirmed autonomic neuropathy and a suspicion of autoimmunity.

Since I started IVIG one year ago, I went from being bedridden to almost normal on my activity level.  I had a mild dilated left ventricle cardiomyopathy.  After a year of IVIG, it is not showing up on my cardiac MRIs or echos, so it seems to have resolved.  I just had my one year repeat skin biopsies, and I would bet good money that my sudomotor and sensory small fiber density has improved.

What a POTS patient should know about Sjogren's Syndrome

I have POTS as a result of autonomic neuropathy that is caused by an autoimmune disease, Sjogren's Syndrome.  Not everyone with Sjogren's developed POTS, and not everyone with POTS has it because of Sjogren's.  However, there do seem to be quite a few people who have POTS caused by an underlying autoimmune disease.  I have met over 50 POTS patients who have Sjogren's Syndrome and I have been asked by hundreds of other POTS patient how they can get tested for Sjogren's. These are important things you should discuss with your doctor, but here is some info that you may find helpful.

First things first, do I have neuropathy?
As a POTS patient, I would only worry about Sjogren's as a possible underlying cause if you have neuropathy.  About 50% of POTS patients have a partial autonomic neuropathy according to multiple studies conducted by Mayo Clinic.  The autonomic neuropathy seen in POTS is often seen in the peripheral small fiber autonomic nerves.  These nerves are closely associated with small fiber sensory nerves.

Sometimes, the small fiber sensory neuropathy is much easier to diagnose than autonomic neuropathy.  This is because sensory neuropathy has easy to recognize symptoms like burning, tingling, creepy crawly, stinging or stabbing pains in the feet, sometimes extending to the lower legs and hands.  In some people, this pain is worse at night.  Sometimes sensory neuropathy also causes numbness.  However, not everyone who has sensory small fiber neuropathy notices numbness or pain.

How can I get tested for neuropathy?
If you have any of these pain or numbness type symptoms neuropathy, as many POTS patients do, you may want to ask your doctor to give you a physical exam to check for sensory small fiber neuropathy.  You should also ask your doctor to examine you for autonomic neuropathy.  Most doctors will try to send you to an autonomic specialty center to get this done.  It's great if you can get there, but if not, do not fret.

Any doctor can order a simple skin biopsy kit to test your small fiber nerves for sensory or autonomic neuropathy.  There is a company called Therapath based out of New York City.  They are very legitimate and are associated with highly respected neurologists and pathologists at the finest research/teaching hospitals in New York City.  There website contains a plethora of useful information, such as:
an overview of small fiber neuropathy (sensory and autonomic) and it's common causes;
a great explanation of the Sweat Gland Nerve Fiber Density test, that looks for small fiber sensory and autonomic neuropathy;
a "how to" for medical professionals, on how to take the skin biopsy sample; and
Information on how to order the test kit.

I have neuropathy, so now what?
If your doctor determines that you have some form of neuropathy, you should then ask "WHY?"  Many doctors are quick to tell the young POTS patient that their neuropathy is "idiopathic," meaning of an unknown origin.  While it is true that we can't always determine the root cause of neuropathy, it is wrong to label it "idiopathic" until a thorough investigation has been made, to rule out the known causes of neuropathy.  There are many, many different conditions that can cause neuropathy. Some of them are discussed on my prior post, "What Causes Autonomic Neuropathy?"  Also, even if your doctors can't figure out the cause of your neuropathy right away, it is common to figure it out within a few years of getting diagnosed.  Sometimes new symptoms arise, or the neuropathy progresses in a certain pattern than indicates a particular cause.  Just because they can't figure it out right away, doesn't mean you should lose all hope in figuring out the root cause.

Wasn't this a blog post about Sjogren's?
Yes, now we get to the part about Sjogren's Syndrome.  A good neurologist will test for many different things to try to get the root of your sensory and/or autonomic neuropathy.  One of those things should be Sjogren's.  Sjogren's is the second most common autoimmune disease in the world, right behind rheumatoid arthritis.  It impacts an estimated 4 million people in the US, however about 75% of those people remain undiagnosed.  On average, it takes 9 years from the onset of symptoms to the time of diagnoses. This is because physicians are not very well educated on how to diagnose Sjogren's.  Interestingly, Sjogren's is female predominant, just like POTS.  About 80-90% of Sjogren's patients are female.  About half of all Sjogren's patients show signs of autonomic dysfunction (lightheadedness upon standing, fainting, GI motility problems, etc.).  And more than half of the Sjogren's population has neuropathy of some sort.

Sjogren's is a systemic autoimmune disease.  It can impact any system and any organ in the body, from your toe nails to your brain.  The most common symptoms of Sjogren's are fatigue, joint pain, muscle pains, dry eyes or mouth, trouble swallowing.  However, each patient is unique, and not everyone who has Sjogren's has these symptoms.  Sjogren's is the result of an overactive immune system.  The pathology of Sjogren's if far to complex to explain in a blog post, but put simply, in Sjogren's, some of your B-lymphocytes produced in the bone marrow get programmed incorrectly.  The B-lymphocytes stick around too long in the body, and then they do two things.  First, they start producing naughty antibodies that can damage your organs.  Then the B-lymphocytes, instead of recycling themselves like they are supposed to, stick around too long in the body and begin to invade tissues where they don't belong, like your salivary glands, GI tract, blood vessels, spinal cord, joints, etc.

Common misunderstandings about Sjogren's:
You have to be older to get Sjogren's - WRONG!
Many doctors think Sjogren's only impacts post-menopausal women.  While the average age of diagnosis is in the mid 40s, there are many, many younger people with Sjogren's.  I am one on of them.  I had an acute onset of neurological symptoms at age 31, but looking back at my medical history, it seems that I have been having mild symptoms of Sjogren's since about 3rd grade.  There are journal articles discussing pediatric Sjogren's, and how it tends to present a little differently in younger patients, with the dryness symptoms being less severe and the neurological symptoms being more severe at the onset.

You can rule out Sjogren's with an antibody blood test - WRONG!
Many doctors use the ANA, SS-A and SS-B blood tests to diagnose Sjogren's, and if a patient is negative or normal on those test, they will tell the patient they do not have Sjogren's.  This is flat our wrong.  You cannot rule out Sjogren's with a blood test.  The current research on Sjogren's shows that less than 30% of Sjogren's patients have the SS-A or SS-B antibodies.  If we only used blood tests to diagnose Sjogren's, we would miss about 70% of the people who actually have it.  It is likely that there are other antibodies associated with Sjogren's that haven't been identified yet.  In fact, there is evidence that alpha-fodrin antibody may be associated with some of the neurological damage Sjogren's causes, but that test is not commercially available in the US yet.  Additionally, there is something called seronegative Sjogren's.  The medical literature on Sjogren's notes that the SS-A or SS-B antibodies may never form, and may actually come later in the disease in some patients. There are also other antibodies involved with Sjogren's, like the

There is also data indicating that many Sjogren's patients present with small fiber sensory neuropathy as the first obvious symptoms of the disease (and as we discussed earlier, autonomic neuropathy is commonly associated with sensory neuropathy).  Being that neurological symptoms may come first, antibodies may come later, or never at all, and the average patient takes 9 years to get diagnosed, I think that many POTS patients with neuropathy might actually have undiagnosed Sjogren's.  I think doctors are missing the boat on this, because the average 20 something year old POTS patient who doesn't have any abnormal antibodies, doesn't fit into the mold of the 60 year old lady with antibodies they think of when they think of Sjogren's.

You have to have dryness -WRONG!
Doctors always associate Sjogren's with severe dryness.  Yes, dryness of the eyes, mouth, skin or vaginal areas are considered hallmark signs of Sjogren's.  However, you do not have to have severe dryness to have Sjogren's.  And, if you don't think you have dryness, you might actually be a little drier than normal, and not realize it because that is the way you have always been, or because it is so mild.  I would be a good example of this.  I have very mild dry eye, so mild, that I didn't even notice it until my eye doctor tested me for it.  I never would have complained about dry eye as a symptom, because my eyes weren't bothering me.  The only hint I had, was that after wearing contacts for 15 years, I had to switch to glasses - and that happened 2 years before the acute onset of my POTS symptoms.  I just assumed that the contacts were bothering me after wearing them for so long, because contacts can scratch up your eyes a little bit.  I also thought it was from reading to much at work and putting strain on my eyes.  Nope, it was Sjogren's doing it's thing before I even realized there was a problem.  Likewise, I don't have any dryness in my girly parts, and I don't have any dryness in my mouth.  The only thing I notice about my mouth is that, if I sleep with my mouth open at night, I tend to wake up with cotton mouth.  That's pretty normal and plenty of people who don't have Sjogren's do that while sleeping too.  My point is, you don't have to have severe or any obvious dryness to have Sjogren's.  My rheumatologist, who is one of the world's best Sjogren's experts here in New York, told me that the dryness from Sjogren's can develop over time as the disease progresses.  Rather than waiting for it to happen, I am on a preventative medication regime.

So how can you rule out Sjogren's?
After having had every test in the book, here is what I would do.  I am not a doctor, and you are going to have to talk to your doctor about all of this, since there aren't tests you can do on your own.  You may want to show your doctor the latest diagnostic guidelines for Sjogren's, as of April 2012. from the American College of Rheumatology. Many doctors still rely on the prior guidelines, which are also known as the American European Consensus Classification Criteria.

1. Identify any relevant family history.  Do you have any relatives with Sjogren's?  Do you have any relatives with other autoimmune diseases?  Do you have any relatives with thyroid problems or Raynaud's or unexplained neuropathy?  Sjogren's is not directly inherited, but autoimmune diseases as a whole tend to run in families and it is common if you have Sjogren's, to have another relative with it.  However, you still could have an autoimmune diseases if there is no family history.  You could be the first one to screw up the gene pool, or, more likely, someone in your family in the past did have an autoimmune disease, but back in the old days the medical knowledge was sparse that people didn't get accurately diagnosed.  You dear old great granny Elma who had horrible teeth and lots of rashes may have had Sjogren's or some other autoimmune disease, but they didn't have a name for it back then, and the definitely didn't have any effective treatments for it.

2. Get the antibody blood tests.  For Sjogren's, that usually means ANA, SS-A, SS-B.  Other markers in inflammation that are not antibodies include Sed Rate, C-Reactive Protein, and complement levels (C3, C4, C5, etc.).  Even if all of these come back normal, it doesn't rule out Sjogren's or anything else. They are only helpful if they come back super positive.  If you have an extremely elevated SS-A and lots of clinical symptoms, then you probably have Sjogren's and don't have to go on for further testing.  If the antibody testing does not confirm Sjogren's, proceed to the next step.

3. Tear and saliva flow testing.  Most commonly, the Shirmer tear test or the Lysamine Green eye drop test can be performed by your eye doctor. This can reveal dryness that you may not even notice on a daily basis.  You can have salivary flow measured a number of different ways, including a scan of your largest salivary gland, the parotid gland, using a radiotracer.  Even if these tests are normal, that still doesn't rule out Sjogren's.  Proceed to the next step.

4. Get a minor salivary gland lip biopsy.  This is considered the gold standard test for diagnosing Sjogren's Syndrome.  If you have neuropathy and POTS, do not let your doctor's "rule out Sjogren's" until you have had this test.  Doctors are reluctant to order it because it is invasive.  However, if you are like me, you are so sick that a little biopsy poke on your inner lip is well worth it to diagnose or rule out a serious autoimmune disease that could be causing your symptoms.  If I was feeling great and not sick, then I wouldn't want a biopsy, but when times are tough, you have to do whatever it takes to get yourself figured out.  For me, the biopsy was done under sedation, only because I was getting a stomach fat biopsy to rule out amyloidosis at the same time (that's another blog post...), and that biopsy is deeper, so they sedated me.  Many people can do the minor salivary gland lip biopsy with just a little novocaine or lidocaine injection in the mouth.   The wound felt like a bad canker sore for about a week or two.  It was tolerable.  I was able to eat and talk.  If it really bothered me, I would spray some sort throat numbing spray on it, like Chloroseptic.  I had a fat lip for a few days, and then a little puffiness.  But it went away in a few weeks.  I also had a small numb spot on my inner lip where the biopsy was taken from.  This is because there are many sensory nerves in your lip.  That healed over time, because the nerves can grow back.  There is no visible scar left from my lip biopsy.

The reason this test is used to diagnosed Sjogren's, is because it shows whether or not your lymphocytes (white blood cells) are invading your salivary glands.  That is one of the ways in which Sjogren's attacks the body - misbehaving lymphocytes infiltrate into tissues where they don't belong, like your salivary glands, tear glands, blood vessels, nerve fibers, etc.

If you have this biopsy done, you may want to have two separate pathologists from different hospitals interpret the slides, and you want both of them to be pathologists who have experience diagnosing Sjogren's.


Conclusion
I know this is a long post, but this is fairly complex stuff.  I hope this info is helpful to some of my readers.  If any of you are diagnosed with Sjogren's in addition to your POTS, please let me know.  I am keeping track of how many of us have Sjogren's.  Once we have a large enough group, I'd like to show it to the POTS researchers keep in touch with.  My gut instinct is telling me that there are more of us POTSies with undiagnosed Sjogren's than they anyone realizes.  I would like Sjogren's screening to be standard for all POTS patients.

Leaving a systemic lifelong disease like Sjogren's untreated could lead to dire consequences, but treating it early and aggressively can prevent problems down the road. You know the old saying... an ounce of prevention is worth a pound of cure.

Find a Dysautonomia Awareness Month Event Near You!

Get ready, because October 2013 is the first Global Dysautonomia Awareness Month!  Awareness events are being hosted by Dysautonomia International and other dysautonomia related organizations around the globe during the month of October.

Check out the Interactive Global Dysautonomia Map to see if there an October awareness event near you.

If you don't see an event listed for you area, consider starting your own.  It doesn't have to be a big fancy event.  It can just be a small "POTS Luck" dinner or a backyard BBQ, a Halloween party at your friend's house or if you have friends who run 5k races (very common in October), you can ask them to run for "Team Tachy."  The purpose of these events is to raise awareness, so be sure to invite people who don't already know about dysautonomia.  Print out educational brochures or information from the Dysautonomia International website and hand it out at the event.

If you decide to throw your own event, contact events@dysautonomiainternational.org so they can list your event on their global map.  They can also help get your press coverage from your local newspapers.

Hey POTSgrrl fans...

I will continue my POTSgrrl blog, but I am moving the majority of my POTS education and advocacy efforts to Dysautonomia International, a new non-profit I founded with some other patients and physicians to raise money for autono

mic research, physician education and increasing public awareness. Our Facebook page is up and our website will be up any day now. 

"Like" us on Facebook to get updates on the latest dysautonomia news and research:
https://www.facebook.com/DysautonomiaInternational

Stay tuned for our website launch in the next few days.  If you like POTSgrrl, you are gonna LOVE the Dysautonomia International website.  The website will have accurate medical information, tools patients can use, resources for physicians and researchers, and unlike my POTSgrrl blog which has been text only, the website will actually have graphics.  Woohoo, fancy...  :)

The importance of proper Vitamin D levels in POTS patients

A new case report has been published regarding a 37 year old woman whose POTS symptoms were greatly improved after calcitriol supplementation.  Calcitriol is the hormonally active form of Vitamin D.  Vitamin D is normally converted into calcitriol in the kidney, but in some persons with a 1-α hydroxylation defect, this would not occur.  Here is the link to the article:

1-α hydroxylation defect in postural orthostatic tachycardia syndrome: remission with calcitriol supplementation.

Low Vitamin D levels are very common in North America and northern Europe, but it seems to be a universal problem amongst almost every POTS patient I have spoken with that has had their Vitamin D levels tested.  I wonder if the autonomic experts at Mayo and Vanderbilt and elsewhere have tested large groups of POTS patients for the 1-α hydroxylation defect, or looked at the portion of our genes associated with this process.

If you have POTS and you haven't already done so, ask your doctor to test your Vitamin D levels during your next visit.  In the meanwhile, make sure you eat plenty of healthy foods that have Vitamin D and that you get at least 10-20 minutes of sunshine on your skin everyday (sunscreen and clothing blocks Vitamin D production).

I have been on 1000 I.U. of a Vitamin D3 supplement, twice daily, for over a year, because my Vitamin D levels were very low when my doctor tested them.  I also make sure to get plenty of sunshine on my skin, since your body can produce it's own Vitamin D when your skin is exposed to natural sunlight.  Despite a healthy diet, supplementation and adequate sunlight, my Vitamin D levels still hover around the low end of the normal range.  I am going to bring up this case report to my doctor at my next visit and see what she thinks.

There is extensive medical literature on the role of Vitamin D in autoimmunity and overall immune system health - so much that I should probably dedicate an entire blog post to that topic.  Stay tuned...

Enroll in POTS/OI and EDS Research

This post is something I learned about from the lovely Kristina, who runs the Defy Gravity blog.  If you have POTS or another form of orthostatic intolerance and EDS, please check this out from the University of Washington:

Hypermobile EDS with postural orthostatic hypotension - search for identification of large kindreds.

Few disorders are more challenging than hypermobile Ehlers-Danlos Syndrome (formerly known as EDS type III) when trying to identify cause. Defining a population of patients with a clear clinical phenotype is an essential first step. We are actively recruiting individuals with hypermobile EDS and postural orthostatic hypotension (changes with blood pressure on standing or known abnormalities in autonomic testing) to recruit families with multiple affected family members with hypermobile EDS with or without autonomic alteration. The nature of the families and the number of individuals available will determine how each study is done. We will need detailed clinical information, may ask affected individuals to be seen in the Genetic Medicine Clinic at the University of Washington, and collect a blood sample to complete the study."

Contact information: Dru Leistritz, MS, CGC (dru2@uw.edu)

New Mayo Clinic Research on POTS

Check out the new Mayo Clinic study on POTS:

A Prospective, 1-Year Follow-up Study of Postural Tachycardia Syndrome

This is the first time someone has followed a cohort of POTS patients prospectively for a year to see if they improved.  Interestingly, their neuropathy and heart rate did not really improve, but their orthostatic intolerance seems to have improved somewhat.

Mayo notes that, just like in their prior 10 year retrospective study, about 50% of POTS patients in this study have a limited autonomic neuropathy.  The note that they study was designed to exclude individuals who had another known cause of autonomic failure.

I would be VERY interested to know what tests Mayo ran on these patients before ruling out other known causes of autonomic failure.  I want to know this, because I assume Mayo is doing an excellent job of ruling out known causes of autonomic neuropathy, so those are the tests that other POTS patients who have neuropathy should be asking their local neurologist for.  I am a firm believer that, if you have autonomic neuropathy, you should not accept a diagnosis of POTS until your neurologist has taken great care to rule out the known causes of autonomic neuropathy.

I hope Mayo follows this group of patients for more than a year.  We need a 10 to 20 year propsective study.  Do they all eventually recover?  Do they relapse after a period of wellness?  How do they fare during or after pregnancy?  Do any of them get worse?  Do any of them eventually get diagnosed with an underlying condition?  Does the autonomic neuropathy eventually improve?

I am thankful for the researchers at Mayo who have been involved in this study, and for the patients who were dedicated enough to return to Mayo for their 1 year follow up studies.

Salt, With a Side Order of Salt

Salt is very out of fashion these days.  The government tells us that salt is bad.  Most doctors tell us to lower our salt intake.  Eat less salt!  Avoid a high salt diet!  Sir, please step away from the salt shaker before your head explodes!

Salt does not deserve the evil reputation is has been assigned in modern times.  Salt is literally the "salt of life."  Without salt, we would die.  The ancient Romans valued salt, paying soldiers partially with salt - a salarium.  Thus, a man "not worth his salt" is not deserving of his wages for the day.  This is where we derive the word "salary" from.  So when your boss pays your salary, just think, you could be getting some of that in salt!

Salt was not just used as a currency.  It was used to flavor and preserve foods.  The word "salad" comes from the Roman practice of salting leafy green vegetables (delicious by the way!).  Even going back as far as 6000 B.C., ancient communities that were lucky enough to have a salt production facility often prospered and saw a large growth in their population and wealth.  So let's embrace salt and restore it to it's honorable position in the front and center of the spice rack.

Salt is also great for very modern POTSies.  Most POTS patients can benefit from increasing their dietary salt intake.  Before adding substantial amounts of salt to your diet, you should check with your physician, because there are some forms of POTS and other health problems that would make it unwise for you to increase your salt intake.

How much salt should a POTSy get?
I don't have the magic answer on this.  In theory, I would say as much as you need to help regulate your blood pressure and blood volume to a healthy enough level, so that it improves your symptoms, but not so much that is causes other problems for you.

The medical literature on POTS gives a few different suggested ranges, anywhere from 3-10 grams of salt per day.  Keep in mind that sodium is not the same thing as salt.  Each teaspoon of typical table salt contains about 2.3 grams of sodium.  Each gram of salt contains .4 grams of sodium and .6 grams of chloride.  Again, I would encourage you to speak with your doctor about how much sodium you should get in your diet.

While 10 grams a day may seem like a huge quantity of salt, it really isn't that much sodium.  10 grams of average table salt would give you 4 grams of sodium.  Accodring to the US Center for Disease Control, the average American consumes 3.3 grams of sodium per day.  So consuming 4 grams isn't really that much more than the average joe is consuming.  However, if you have POTS, hopefully you are ecting healthier than the average joe, which means less processed junk that contains all of that salt.  So you if you are eating fresh, natural, not-so-processed foods, you will have to put a little effort in to it to get your 4 grams of sodium per day.

Supplements or Salt Shaker?
You can purchase several different types of salt supplements. Nuun tablets and Thermotabs are popular, but really don't contain that much sodium.  Nuun tables have about .36 grams of sodium per tablet and Thermotabs have .18 grams of sodium.  You would have to take a large quantity of these supplements in one day in order to rely on them exclusively for your sodium needs, but that probably wouldn't be a good idea, since they also contain potassium, and you don't want too much of that.

You can also buy Sodium Chloride tablets.  They usually come in 1g tablets.  They can really irritate your stomach, but some people tolerate them without any problem.  Be sure to drink them with plenty of water and take them with food to minimize the risk of stomach irritation.

I prefer to obtain my 4 grams of sodium per day through a good old fashioned salt shaker, and a cupboard full of high salt, healthy foods.

What Type of Salt?
Be cautious about consuming excessive amounts of iodized table salt.  Regular table salt is iodized.  This can lead to iodine-induced hyperthyroidism in some people.  When I was first sick, we knew I had low blood pressure, so my doctor's encouraged me to consume as much salt as I could, to help increase my blood pressure.  I was using iodized table salt.  I developed flushing, hives, itching all over my body and a host of other symptoms - my doctor thought I had mastocytosis or MCAD (Mast Cell Activation Disorder).  But a young resident at Cornell figured out that all of that iodized salt intake was probably causing these symptoms.  Sure enough, when I switched to sea salt, that does not have added iodine, those symptoms disappeared shortly thereafter.

Iodine is an essential nutrient.  It was added to most of the world's salt supply in the 1920s to prevent goiter.  Goiter is an enlargement of the thyroid gland causing by a lack of iodine in the diet.  If you eat a healthy balanced diet, you shouldn't need additional iodine.  Plenty of regular foods, like eggs, spinach and milk, contain iodine.  Sea salt has trace amounts of iodine in it as well, as iodine is naturally occurring in sea water.  Saltwater fish and sea vegetables (kelp, seaweed, nori) are also a good source of iodine.

High Salt Food Ideas:
Take a cue from the ancient Romans and salt your salad.  A good quality olive oil and a heavy dousing of sea salt on top of baby spinach or mixed greens is delicious.

Broth - store bought chicken, beef and vegetable broth usually contain large quantities of sodium.  Many also contain MSG or other chemical additives, but if you have time to make a homemade broth, it is very easy.  And you can make large batches of it at once and freeze individual sized portions.  Then you can add as much salt as you'd like. Broth actually tastes great with ridiculous amounts of salt in it, and if you add too much salt, you can just as some more water to mellow it out.  I am known to enjoy a cup of chicken broth in a mug in the morning, rather than a coffee.

Soy Sauce/Tamari - Tamari is just soy sauce that doesn't have wheat in it (great for gluten free people). Both contain tons of sodium.  Don't limit your soy sauce to just Chinese take out.  Soy sauce is great with steamed veggies, rice, fish, chicken, beef, turkey, gravies and my favorite, with sunny side up eggs and asparagus.

Nachos - I know you are thinking, how are nachos healthy?  Well, the nacho chips themselves are probably the least healthy part of the meal, but they aren't that bad, and they are high in salt.  Top them with black beans, shredded chicken, salsa, tomatoes, black olives, lettuce, cheese and sour cream. Add a little salt to each ingredient as you make the platter.

Cheese - if you can tolerate dairy, cheese is an excellent source of sodium, and adding more salt to cheese can make it taste even better if you don't overdo it.  I love dipping a small wedge of cheddar into coarse sea salt.  The crunchiness of the salt goes well with the creaminess of the cheese.

Pickles - pickles are basically just cucumbers doused in salt and herbs.  Some brands of store bought pickles have preservatives and chemical additives.  Homemade pickles are very easy to make.  There are endless recipes online.  And when you are done marinating your pickles, you can even drink the juice.  Some people like to add a little sugar to it and partially freeze it, like you would for a frozen margarita.

Mashed Potatoes, Baked Potatoes, Baked French Fries or Sweet Potato Fries are a great way to sneak in lots of salt.  I have never had mashed potatoes that tasted too salty.

Eggs hide salt very well too, and I know I promised to keep this list healthy, but we can't talk about eggs without mentioning that bacon has tons of sodium in it and nothing goes better with eggs than bacon (and a prior item, cheese!).

We'd love to know, what are your favorite high salt foods?

Compression Stockings: The Good, The Bad, The Ugly

Many POTS patients have been advised by their doctors to try wearing medical compression stockings to help their orthostatic symptoms.  For some reason, many patients are reluctant to try them.  I know I was at first too.  All I could imagine was some old granny with thick beige colored itchy knee highs  rolled halfway down her jiggly calves, only held up by cankle fat.  I may have been sick, but I was still vein enough to not want to look like that.

My first attempt at compression stockings was while I was admitted to a large hospital in NYC in 2010.  My doctor really pushed me to try them.  I tried the ones the hospital had on hand.  They were hospital white, thick, itchy and worst of all, they had latex in the rubber that wrapped around the leg band (they were thighs highs).  Not exactly a good experience.

My wonderful husband wanted to help me anyway he could, so he walked a few blocks down to a drugstore and bought me three pairs of compression stockings - thigh highs, pantyhose, black, nude and all in different sizes.  I think he just bought the first three pairs he saw on the shelf.  They were very expensive, so I only kept one pair that I thought fit me.  I later learned they were the wrong size to work effectively, but at least they were comfy.

Then I started to do a little research and talk to other patients about compression stockings.  I learned some great tips:

Knee highs aren't going to do anything for a POTSy.  Most of us have blood pooling in our upper legs and stomach area too.  If you put knee high compression stockings on the average POTSy, they get fluid retention or blood pooling just above the top of the stocking at the knee.  Not helpful, and not a good look.

Thigh highs are better than knee highs, but only really look good on skinny girls and marathon runners.
Thigh highs are better than knee highs for POTSies, but if you have a healthy layer of thigh fat as most women do, medical compression thigh highs will leave a weird dent in your thigh at the top of the stocking.  If any of you vixens have worn some sexy Victoria's Secret thigh highs and though you looked great in them, keep in mind that this is not the same thing.  Medical compression stockings are REALLY tight and tend to make you feel and look like a stuffed sausage.   If you have normal female thigh jiggle, this dent looks weird under pants or skirts.  If you are skinny or have rock hard quads, this won't be a problem for you.

Full length pantyhose are the most effective for POTS.  Since they provide compression to the whole lower body, including the belly area, they work better than the thigh highs and knee highs.  Some POTSies are worried that compression on their belly area could worsen nausea, reflux or further impair gastric motility.  One way to deal with this is to roll them down to below the belly.  I have done this many times after eating, since I have some motility problems too.


Open toe is the way to go!  I love wearing flip flops and you can't wear them with regular pantyhose.  Most compression stocking brands, and definitely the Juzo Soft brand, comes with an option for closed toe or open toe.  Even the thigh highs come in open toe now.  Besides flip flops, open toe stockings are easier to put on and take off that closed toe stockings.  They usually give you a slip to put over your foot, which makes the stocking slide on much easier.  Once you have the stocking on, you simply slide the slip out of the toe opening.

Not all brands or all lines within a brand are equal.  Most people dread the thought of itchy, scratchy, sweaty compression stockings.  Me too.  I tried many different brands and different lines within each brand.  My favorite line is the Juzo Soft line, sometimes called Juzo Soft 2000.  Second best is Jobst Opaque.  I also like the stockings from Rejuvahealth.com.  The Juzo Soft stockings are very soft, and they seem to breathe pretty good.  I have worn them for 2 years though the summer heat without a problem.

They come in colors and fun prints now!  You'll probably want your first few pairs to be a skin-toned color, but after you have a few of those, why not have some fun and get a pair of paisley or tie-dyed stockings, or ballerina pink.  The Juzo Soft line comes in a bunch of solid colors like blueberry, plum, pink, green, red, yellow, etc.  They also have seasonal prints like tie-dye.  Rejuvahealth.com comes in paisley, colored animal prints, and a few other fun prints.

Cut out the crotch of you brand new $100 stockings. What? Yes, that's what I said. OK, I know this is gross.  You don't want to think about this.  But any of you ladies who has ever had a yeast infection (um, probably all of you at one point or another) would do ANYTHING to avoid dealing with that awfulness again, wouldn't you?  So just trust me on this one.  Cut out the crotch of you brand new $100 stockings and let your lady parts breathe so you don't end up brewing any funky stuff down there.    Compression stockings are made from various synthetic fibers.  They don't breathe very much and they are very tight.  This creates ideal growing conditions for candida (yeast) overgrowth.  Add to that, that many medications can cause yeast overgrowth, and people with various medical conditions are more prone to yeast infections, particularly people with weakened immune systems.  So just cut the crotch out.  Make the cut when you are NOT wearing the stockings.  Cut out the crotch area inside of the seams, leaving the seams intact, and at least a half inch of fabric between the cut and the crotch seam.  This will help deter any runs in the stockings.  I have done this to 12 pairs of stockings and have not had one run in my stockings caused by this.  I have had runs start at the heel from walking barefoot outside with my stockings on - oops!

Compression stockings are for men too.  Most companies that make medical compression stockings offer a few styles meant for men, with a little extra room in "the region."

Try out different compression strengths before you decide they are not for you.  I have heard some patients say "compression stockings did nothing for me," but they only tried the 10-15mmHg compression, which is very minimal.  I have heard other patients say "oh they were so tight my legs went numb and cold," but those people had bought a size too small and had the 30-40 strength compression, which is really, really tight.  I would encourage people to get fitted properly for compression stockings (take the measurements in the morning when you first get out of bed).  I would also encourage you to try the 20-30mmHg first.  If they feel way too tight, then try either a larger size or a lesser compression strength.  If they feel like they are not helping at all because they are too lose, try a smaller size, and if that doesn't help, then try a stronger compression.

Insurance will often pay for them.  Before ordering them, check with your insurance company.  Sometimes they will cover medical compression stockings if you have a script from your doctor.  For POTSies, it may be easier to get your doctor to write Orthostatic Intolerance or Hypotension as the diagnosis on your script, since the compression stocking company and the insurance company probably have no clue what POTS is.  Since stockings can be well over $100 a pair, it is worth it to make a few phone calls to see if they will cover them.  If they are covered, find out how many pairs per year you are entitled to.  My insurer had no limit; they said it just had to be within reason.  So I order a new 6 pairs every 6 months.  The insurance company probably has a list of approved durable medical good providers that you will have to use if you want them to pay for the stockings.  Just ask that company to order the stockings you want.  Most of them have large catalogs they order from, so you shouldn't have to be limited to only what they advertise online or have in their store.

Comparison shop online.  If insurance won't pay for them, search around and comparison shop online.  I have found the same pair of stockings on one website for $160 and then on another website for $95.  Also keep in mind tax and shipping costs when comparing prices.

Buy one pair at a time until you find the perfect stocking for you.  Don't order 6 pairs of a size or brand you have never tried before.  You could end up wasting a ton of money if they are not comfortable or don't fit you.

Wash them everyday.  I originally thought that I should wear them for 2 days before I washed them, but  then I read the washing instructions and it said to wash them after every wear.  Your skin has natural oils that break down the elastic fibers in the stockings if they are not washed every time you wear them.  I wash them in a pillow case on the gentle cycle with my regular laundry.

Compression stokcings are most effective when you put them on just as you first get out of bed in the morning.  The longer you are sitting or standing upright before putting on your stockings, the more time your blood has to pool in your lower legs.  Blood pooling can actually cause a shift of fluid out of your veins and into the surrounding tissues, which can worsen low blood volume or low blood pressure if you are already dealing with that.  Putting your stockings on first thing in the morning avoids this.

NEW GENETIC RESEARCH ON POTS

New research sheds light on the role of genetics in POTS: 

Epigenetic Modification of the Norepinephrine Transporter Gene in Postural Tachycardia Syndrome

Previously, a mutation in the norepinephrine transporter gene (SLC6A2) this gene has been implicated in POTS in one family in the U.S., but despite testing thousands of other patients, the same mutation was not found in other POTS patients.

Recently, researchers in Australia decided to look at the amount of norepineprhine transporter (NET) in POTS patients, and found that, even though the SLC6A2 gene mutation was not present, POTS patients tended to have a deficit of NET compared to healthy controls.   NET is a protein that controls the re-uptake of norepineprhine form extracelluar spaces (spaces outside of cells).  Seeking to understand why POTS patients would lower amounts of NET, researchers re-examined the expression of the SLC6A2 gene in POTS patients and healthy controls.  They discovered that, while the SLC6A2 gene itself was normal in POTS patients (other than the one family previously mentioned), the POTS patients with reduced NET levels had an increase in chromatin modifications.  Chromatin is the combination of DNA and proteins that allow DNA strands to wind up into tiny coils in the nucleus of a cell.  Each human cell contains 1.8 meters of DNA, but when chromatin does it's job, that 1.8 meters of DNA gets wound up into a tiny little package that is less than .01mm in size, and fits neatly inside the nucleus of the cell.

Modifications in chromatin are known to cause changes in the way a gene is expressed.  There is an entire field of genetic research dedicated to understanding changes in gene expression caused by mechanisms other than changes to the DNA itself.  This is called epigentics.

This is really exciting research.  If this study can be applied to the larger POTS population, in theory, it could lead to the development of a blood test to diagnose POTS some day, and perhaps even therapies aimed at correcting the abnormal chromatin structure. 

AUTONOMIC TESTING 101

Many POTS patients e-mail me asking me what tests they should ask their doctor to run when their doctor suspects they have POTS.  I am not a doctor, but I have had many different autonomic tests.  Here are some of the tests commonly performed on patients with a suspected or known POTS diagnosis.  They are not all considered "autonomic" tests, but many of them can be used to evaluate the functioning of the autonomic nervous system.  Some of the tests are used to rule out other diseases, some are to determine how severe the POTS is and some are to help determine the cause of the POTS.  Not every patient needs every test, and not every doctor is aware of the availability or usefulness of each test.  By posting information on these tests here, I am NOT suggesting that every POTS patient needs every one of these tests.

Here is a good instructional video on some of the autonomic tests from the lab of Dr. Peter Novak, a well regarded autonomic neurologist in Massachusetts.



Tilt Table Test
This is probably the most common test giving to people suspected of having POTS.  The tilt test, sometimes called HUT for "heads up tilt," is a non-invasive test.  Sometimes, your doctor may put an IV line in your arm to deliver medication or draw blood during the test, but this is not always done.  Try to avoid an infusion of a stimulant drug just before they tilt you.  Some doctors do this to speed up the test, but it is not very pleasant and not needed to diagnose POTS.  

You start the test laying flat on a table.  They will put a blood pressure cuff on your arm and some electrodes on your test that record your heart rate.  Sometimes they put a 'beat to beat' blood pressure monitor on one or two of your fingers.  Your doctor will strap you to the table, usually with velcro straps.  This is just to protect you from falling in case you faint during the test.  They don't want you falling off the table and getting hurt.  Sometimes they strap you with your arms down at your sides.  Sometimes they strap you with one or both of your arms out in a T shape, like Jesus on the cross.   If they are going to give you medication through and IV line, it is usually given at this time.  Once they have your baseline laying down heart rate and blood pressure (BP) recorded, then they tilt you upright - your feet towards and ground and your head towards the ceiling.  I have heard some people worry about being flipped upside down during a tilt test.  There is no upside down part of a tilt test.  They usually tilt you upright fairly quickly - to mimic the act of going from laying down to standing.  The machines you are hooked up to are going to be recording your heart beat constantly and your BP.  Usually the BP is taken every minute, or constantly if you are hooked up to a beat to beat BP monitor.

They usually tilt you to just about 70-80 degrees - so you are not fully upright.  This is to prevent you from using your leg muscles to stand up, which can interfere with the test results.  It is very important not to wiggle or use your leg muscles at all.  Although it can be a little scary, just try to relax and remember you are strapped to the table so you aren't going to fall.  Tilt tests can last from a few moments (if you pass out right away like I did the first time I had one) to 45 minutes (if you don't pass out at all like I did during my 3rd tilt test).  The point of the test is not to make you pass out.  It is to see how your heart rate and blood pressure respond to the change in position.  Many people do not pass out during the test.

It is important to note that you can have three different results on three different tilt tests - and you could end up with three diagnoses.  This happened to me.  POTS is finnicky and the symptoms may not be consistent from day to day and hour to hour.  Also, any medications or high salt/fluid diet you are on can influence the test restults.  Be sure to talk to your doctor at least two weeks before the test to see if they want you to go off of any of your medications.  If you are trying to confirm or rule out a POTS diagnosis, you do not want to be on any meds that would skew the test results.  Sometimes they want to see how well your POTS meds are working, so they ask you to stay on your meds before the test.  This test is usually performed by an EP Cardiologist.  EP stands for Electrophysiologist.  This is a cardiologist who specializes in the electrical system of the heart.


Supine & Upright Catecholamines
This test can often be performed during a tilt test, although a tilt machine is not needed to complete the test.  This test consists of two blood draws taken about 30 minutes to an hour apart - one in the supine (laying down) position and the other in the upright (standing) position.  The blood is then evaluated for epineprhine, norepinephrine, renin, vasopressin, dopamine, aldosterone and various other hormone and neurotransmitter substances involved in the control of the autonomic nervous system.  Not every lab looks at each of the substances listed.  Some only look at one or two of the substances.  It is important to ask your doctor which substances he/she will be checking for, as you do not want to have to repeat the test at a later date if you can avoid it.  This test is very useful in the evaluation of patients with suspected "Hyperandrenergic" POTS, who commonly have extremely elevated levels of norepinephrine.

It is very important to follow specific procedures for this test, otherwise the results will be skewed.  The catheter to draw the blood out of should be insterted, and then the patient should be allowed to lay down calmly in a quite and dark/dimly lit space for at least a half hour.  The reason the needle is inserted a half hour before the blood draw is because the pain from the needle stick is enough to spike the patients hormone levels, which would alter the test results.  Any agitation, pain, loud noises or distractions could elevate the patients norepinephrine levels, or other substances tested.  The point of the first blood draw it to get blood form the patient in a relaxed state.

After the first "relaxed" blood draw is taken, then the patient should attempt to stand up for about 30 minutes.  Since this is difficult for many POTS patients, the patient should stand for a s long as possible, or sit up and stay busy, sometimes a pattern of sitting and standing can be used since standing for the entire 30 minutes is difficult for many patients.  The patient does not need to be in the calm/quite space at this time.  In fact, putting the patient in a "normal" stress situation is probably helpful, as the point of the second blood draw is too see how much the patients's hormone and neurotransmitter levels change after a half hour of upright activities.


QSART
QSART stands for Quantitative Sudomotor Autonomic Reflex Test.  This is a non-invasive test, although it does involve a mild stinging or zapping sensation on a small area of your skin.  They place electrodes on your lower leg and sometimes your arm as well.  Then a very small and safe electrical current is put on your skin and the electrodes record how much your skin sweats in the area of the electrodes.  A deviation from normal can indicate problems with the autonomic nervous system.  This test is usually performed at an Autonomic Laboratory, which can be hard to find.  Check the research hospitals nearest to your home to see if they have an autonomic lab or at least a good neurology department that may have the equipment needed for this test.  Keep in mind that this test only check one tiny spot on your skin, so if it comes back normal, that does not rule out autonomic neuropathy entirely.  Some people have a patchy distribution of neuropathy, and that may be harder to pick up with this test.


TST
TST stands for Thermoregulatory Sweat Test.  This is like a full body deluxe version of the QSART.  As of early 2012, this test is only offered in three hospitals in the United States - Mayo Clinic in Minnesota, Cleveland Clinic and University Hospital, which also happens to be in Cleveland, down the block from Cleveland Clinic.  For this test, you have to take off your clothes and put on a small disposable bathing suit (bikini for the ladies).  You are then covered with a powder called Azithrin Red.  This is the same powder used to stamp "USDA" on raw beef in the United States.  And yes, after this test your will look like a piece of raw beef.  Once your skin is covered in powder, you lay down in a glass box that is about the size of a large refrigerator, except it is really hot in there.  They take photos of your skin with the powder on, and then the slowly turn up the heat until you begin to sweat.  They take more photos of your skin as time progresses, to view the shape and pattern that you sweat in, and also the amount of sweat you produce.  Your heart rate and body temperature is monitored to make sure you are not overheating.  You can see the test technician the whole time you are in the box.  In fact, at Cleveland Clinic they have photos of tropical beach scenes around the box and they play some relaxing Hawaiian luau music to help keep you calm.  They don't want you to sweat because you are nervous, because that could mess up the test results.  Once the test is done, you get to take a shower and scrub off the powder.  If you cannot shower yourself, they will get a nurse to assist you or let you have a wheelchair to use in the shower.  The powder turns blackish purple after it comes in contact with your sweat, which is why you will look like a side of rare beef when you are done with this test.  Try as you might, you are probably not going to get all of that powder off in the shower, and even if you do, any place that got sweaty gets stained with the dye.  Forehead, armpits, back of your shoulders... mottled black and purple.  I highly recommend wearing black clothes the day of this test and having a long sleeve cover up to wear so when you walk out after the test, people don't like at you like a diseased purple monster.

This test can help reveal patterns of hypohydrosis (lack of sweating), hyperhydrosis (too much sweating), delayed sweat responses, unusual sweat patterns (for example, no sweat on upper body and normal sweat on lower body) and certain neurological conditions.  The doctors can sometimes even identify where a spinal cord or brain lesion is, based on the sweat pattern.


Valsalva Maneuver
This is a fairly simple non-invasive test.  Your doctor will give you a tube to blow into.  Sometimes the tube has a pressure gauge on it and you are instructed to keep the pressure above a certain number.  Your doctor will be monitoring your heart beat and blood pressure before, during and after you blow into the tube.  Your doctor may make you breathe in and out very fast a few times or hold your breath during this test.  The results are used to help evaluate your autonomic reflexes.


Small Fiber Nerve Skin Biopsy
There are two different types of skin biopsies that can be done on to evaluate the patient's small fiber nerves.  One looks at the small fiber sensory nerves, and the other looks at small fiber autonomic nerves.  These are both very minor 3-4mm skin biopsies that just feel like a pinch.  The generally don't require stitches or leave any numbness.  A good pathologist can count the number of nerve fibers present, and note whether there are any damaged fibers or other unusual features in the biopsy tissue.  About 50% of POTS patients are known to have autonomic neuropathy.  Many doctors are aware of the skin biopsy that looks for small fiber sensory nerves, but there seems to be less awareness of the availability of small fiber autonomic nerve analysis.  Here is a five minute educational video for doctors on how to perform the skin biopies from a company that produces the test kits.  The actual biopsy is performed just after minute 2:30.  As you can see, it is very small.


Nerve Biopsy
This is very different than a small fiber nerve skin biopsy.  During a nerve biopsy, a doctor cuts out a portion of a patients large fiber nerve and exams it for any abnormalities.  The nerve is often taken from the foot of shin area, and this procedure can leave a sizable area of numbness or even muscular weakness after the procedure.  A nerve biopsy is usually only considered once a Nerve Conduction Study(NCS) or Eletromyelogram (EMG) has shown an abnormalities in large fiber nerves.  Large fiber nerve involvement is uncommon in POTS, although some patients may have an underlying neurological condition that is causing their POTS, and sometimes that underlying condition can involve large fiber nerves.


Muscle Biopsy
I include this here simply because so many patients confuse the various forms of biopsies.  A muscle biopsy is not a routine POTS test.  However, it is sometimes used to evaluate patients for mitochondrial diseases, neuromuscular disorders and to determine the cause of weakness.  Muscle biopsies can show abnormalities in the nerves that innervate muscles (motor nerves) or abnormalities in the muscle tissues. This is an important distinction.  The test should not be done on a muscle that was subjected to EMG testing within the past month, as that can impacts the results.


Upper Endoscopy
While not truly an autonomic function test, dysautonomia patients who have severe or disabling GI symptoms may be advised to have an upper endoscopy.  Prior to the test, the patient usually has to fast for a certain period of time - less than a day usually.  This is important because if there is food in your stomach your doctor won't be able to see things clearly down there, and it is also dangerous to eat or drink prior to being sedated, because it increases your risk of vomiting and choking on vomit. If you are told not to eat or drink before this test, follow those instructions carefully. During this test, the patient is given a sedative to make them drowsy, and the doctor places a long thing tube in the mouth, down the throat, through the esophagus and into the stomach.  The tube has a small camera and a light on the end of it, so the doctor can look for ulcers, inflammation and other abnormalities in the stomach.  Sometimes the doctor will take a small biopsy of the stomach using the endoscopy tube, to make sure the stomach lining is normal when viewed under a microscope.

After the test, sometimes you can have a mild sore throat from the tube they slid down into your stomach.  Just drink plenty of fluids and maybe eat soft foods for a day or two, like mashed potatoes or soup.



Colonoscopy
This is essentially the same thing as the upper endoscopy described above, except the doctor takes a slightly different tube and places it into the anus and up into the colon and intestines.  This is sometimes done to rule out various causes of GI distress and to obtain biopsies for things like Celiac Disease (although most doctors prefer a small intestine biopsy to diagnose Celiac, which is usually obtained during an endoscopy).

You are sedated during this procedure and it does not hurt.  Before this test, most patients are given something the night before to drink to help clean out their intestines.  The most common cleanser given is called GoLytely.  It is clear and viscous and sometimes they have flavor packets you can add to it to make it more tolerable.  Either way, it's not fun to drink.  I couldn't have the flavor packets because they had something in them I was allergic to, so I had to drink mine straight up.  I found it easiest to cover a glass with saran wrap and stick a straw through it (so I wouldn't have to smell the liquid).  It tasted so gross that I was vomiting it back up, so my doctor let me suck on a watermelon Jolly Rancher candy as I drank it.  I have also heard of some people drinking it with clear apple juice.  I would ask your doctor first.  And once you start drinking the cleanser, you do not want to be far from the bathroom.  Find your comfy PJs and be prepared to hang out in the bathroom for the rest of the night.  

Gastric Emptying Study (Gastric Emptying Scintigraphy)

Many patients with POTS and other forms of dysautonomia have impairments to the movement of food through their GI tract.  This is called gastroparesis.  This test helps your doctor determine whether you have a delayed movement in your GI tract.  During a Gastric Emptying Study, you eat something solid (like a scrambled egg), and something liquid (like water or broth), that has been laced with an radioactive substance, called a radiotracer.  Then the doctor or radiology technician places a machine similar to a Geiger counter over your stomach to detect the amount of radiotracer left in the stomach.  The levels are usually measured at 1, 2, 3 and 4 hours.  If 10% of the food is still left in the stomach at the four hour mark, the patient is usually diagnosed with gastroparesis.  This test can also be used to detect food moving too quickly through the GI tract.

Barium Swallow and Modified Barium Swallow

During a regular Barium Swallow, you are told to fast for a period of time before the test.  Then you are given something to eat that has some barium on it.  Barium shows up on x-rays very well, so this test can be used to see if food is moving properly though your GI tract.  During the regular barium swallow, they x-ray your stomach area.  

During a modified barium swallow, they use a moving x-ray image called video-flouroscopy to watch you chew and swallow gooey/chewy foods, crunchy/dry foods, liquids and sometimes sugar pills that have barium on them.  They are looking to see if your tongue, throat, esophagus and lower esophageal sphincter (valve at the top of your stomach) are functioning properly.

Breath Test

Some doctors use a breathe test to determine how well the stomach is functioning.  The patient eats a meal containing a small amount of a radioactive isotope.  Then breath samples are taken to determine how much of that isotope is present in the carbon dioxide the patient exhales.

Esophageal Motility Study (Esophageal Manometry)
During this test, a doctor places a thing tube into the nose, through the back of the the throat
and into the esophagus, then down into the stomach.  Once the tube is in place, you are asked to swallow.  The tube measures the pressure inside your esophagus.  This can be used to check if your esophagus is working properly to move food and liquids down to your stomach.


Blood Tests
In addition to the supine & upright catecholamine tests described above, here is a sampling of some of the more common blood tests doctors perform on patients suspected of having POTS.  Most often, these tests are used to rule out other illness as the underlying cause of the patients POTS symptoms.  Sometimes, the tests are used to help identify which treatment options would be most beneficial for a POTS patient.

CBC  - complete blood count - this looks at a patinets red blood cells, white blood cells, hematocrit, etc.  This would help a doctor look for signs of infection, anemia or other hematological abnormalities.

BMP or CMP - basic or complete metabolic panel - this looks at electrolytes, which are often off balance in POTS patients, particularly potassium, which is often low.

Mono/Epstein Bar Virus - about 50% of POTS patients develop POTS after a viral infection, and since Eppstein Bar is one of the most common human viruses, and is also known to cause symptoms similar to POTS, this virus is commonly tested for.  Keep in mind that almost 95% of humans have been exposed to Epstein Bar by the time they reach adulthood, and once you have had it, your body will continue to produce IgG antibodies to it probably for the rest of your life.  A positive IgG does not mean you have an active Epstein Bar infection.  There are four or five different tests for the Epstein Bar virus that are required to decipher between a current infection, a recent infection, or a long ago infection.  You should probably see an experienced infectious disease specialist if you or your doctor suspect Epstein Bar or mononucleosis.

Lyme Disease and associated co-infections like Bartonella, Erlichia and Babesosis - when ticks transmit enough saliva to transmit a disease, they rarely only transmit one infectious agent.  Most ticks have numerous pathogens in their bodies, and as such, it would be rare to find a patient who has become sick from a tick bourne illness who has only one type of infection.  Lyme and it's common co-infectious can be notoriously hard to test for and diagnose.  The standard blood tests for Lyme, both the Western Blot and the ELISA, are notorious for high false negatives - that is, they will return a negative result, when the patient actually has a known case of Lyme Disease.  If you suspect Lyme or one of it's co-infections, I highly recommend finding a reputable LLMD (lyme literate medical doctor).  LLMD is not some official designation, so any doctor can claim to be an LLMD.  Probably the best way to find a good Lyme doctor is to call your local or state Lyme Disease patient alliance.  They will know who is good at diagnosing and treating Lyme, and who isn't.

Mycoplasma - there are several species of mycoplasma bacteria that cause infections in humans, the most common of which is Mycoplasma pneumoniae - the bacteria that causes "walking pneumonia."  While most cases of walking pneumonia are experienced as an annoying chest cold, but not as severe as a case of true "pneumonia," a small percentage of Mycoplasma pneumonia cases can lead to neurological impairment.  Mycoplasma pneumonia has been implicated in the onset of POTS, and it is treatable with strong antibiotics if needed and if it is caught early enough in the infection.

B12, Homocysteine, Methylmalonic Acid - these tests are used to make sure your B12 levels are normal, and to make sure your body is able to properly utilize B12.  B12 is important for proper nerve health, and if your B12 is low, it could be the cause of you POTS symptoms.

CoQ10 - This is an essential vitamin-like substance that is found in all cells in the human body.  It is required for energy production within the cell and plays and important role as an antioxidant.  Normal levels of CoQ10 are important to overall health.

Vitamin D (D2 and D3) - this is low in most Americans and Europeans (those residing in far northern latitudes), and is linked with an increased risk for infection, autoimmune diseases and even some forms of cancer.  While low Vitamin D is probably not the cause of your POTS, correcting any deficiency you may have could help you feel better overall.

Lead, Mercury, Cadmium, Arsensic (organic and inorganic) - these are commonly tested to rule our heavy metal poisoning.  Even if you have no known exposures to these substances, it is actually quite common to have elevated levels of these substances if you live in an industrialized nation.  My cadmium was high, although my doctor told me this was just the same as everyone else who lives in New York.  Cadmium is in air pollution and it rains down into our drinking water and lakes and streams, and that is how it ends up in our bodies.

Immune System Related Tests
Sed Rate and CRP - these are tests that look for general inflammation in the body.  If they come back positive, it does not tell the doctor what specifi disease or inflammatory process is occuring, but it does give a general clue that there is some inflammatory process occuring - wheter it be from an injury, inflammation, infection, allergies, autoimmune disease, etc.

SS-A and SS-B - These are antibodies seen in about 70% of Sjogren's Syndrome patients.  Sjogren's can be the underlying cause of POTS.  However, even if your antibodies are negative, that does not rule out Sjogren's.  Only a minor salivary gland lip biopsy can rule it out for sure.

RF - rheumatoid factor.  Rf is seen in several autoimmune diseases, some of which can cause autonomic neuropathy and in turn POTS.

ANA and ENA - this are also antibody tests which can be positive from various conditions, some of which are autoimmune.  ANA can also be elevated after infections.

Lupus Band Test - this is a minor skin biopsy used to evaluate a patient for Lupus, and autoimmune disesae that is known to be associated with some cases of POTS.

Complement Levels - your doctor can test various complement levels, like C3, C4 and C5, to see if your immune system is under an abnormal influence, possible from an autoimmune disease.  And elevated or reduced complement level does not confirm a specific autoimmune disease, but if gives your doctor a hint that something is going on with your immune system.

Paraneoplastic Panel - this is a panel of several antibody tests to look for neurological antibodies associated with rare cancers.  These cancers are hard to detect on scans, but often the antibodies they trigger cause neurological damage before the tumors can be seen.  In the US, this test is usually performed at your home lab and then sent off to Mayo Clinic's lab in Minnesota.



This is only a partial list. There are many other tests your doctors may perform to evaluate you for POTS and to try to identify the underlying cause of your POTS.