May 18, 2011

What causes POTS in the first place?

Well, it seems like there are probably 1000 things that cause POTS.  POTS is really just a convenient name we give to a long list of symptoms.  However, the technical criteria for POTS is usually stated as an increase in heart rate of 30 bpm or more upon standing, and over 120 bpm, and then there is some disagreements as to whether the diagnostic criteria should include "without significant orthostatic hypotension" or not.   But as POTS patients know, nobody who has POTS only has just an increase in their heartbeat upon standing.  It seems like there are many more symptoms that are commonly found in POTS patients, but they haven't all made the list for the diagnostic criteria.  It's much easier to tell someone "I have POTS" than to say "I have tachycardia, erratic blood pressure, autonomic dysfunction, syncope, tingling feet, diarrhea, dizziness, shortness of breath, etc., etc., etc...."

But maybe we are not doing ourselves any favors by labeling ourselves as having POTS, because it doesn't really signify which of the many symptoms that go along with POTS each of us may have, and it doesn't signify what the cause is of any of the symptoms.

As soon as your doctor says you have POTS, you should starting asking "why?" and "what is causing it?"  It's possible you will never know, because there are lots of things modern medicine simply hasn't figured out yet.  But, there are lots of things your doctors can look for to try to determine the underlying cause of your POTS symptoms.

Generally speaking, here are some of the things that can cause POTS.  Of course, I'm not a doctor and I'm probably leaving out many important things, but this is just something to help you understand how many things could possibly cause POTS.

-anything that can damage your autonomic nervous system
-endocrine abnormalities
-severe deconditioning (just like NASA astronauts get after prolonged spaceflight)
-infections and post-infectious/Chronic Fatigue like illnesses
-genetic diseases
-autoimmune and autoinflammatory diseases
-Ehlers Danlos Syndrome and connective tissue diseases
-physical traumas, like head, neck and spinal injuries

Just by pure luck, my doctors had done a skin biopsy looking for an allergic autoimmune disease called mastocytosis.  When they looked at my skin biopsy, they didn't find mastocytosis, but the found that many of the nerves that control small blood vessels and my tiny hair follicle muscles (think goosebump muscles) were damaged and dying.  The pathologist said I had "profound autonomic neuropathy."

As soon as my doctors said I had autonomic neuropathy, the first word out of my mouth was, "why?"  This was not a metaphorical or philosophical "why?" - like a "why me God?" type of question.  This was me wanting to know as soon as possible WHAT was causing my autonomic nerves to die off.  I assume they don't spontaneous combust, so we can rule that out.  I'm pretty sure I don't have the Black Plague, so we can rule that one out too.  Besides that, just about anything can damage your nerve cells.

For example, you can get neuropathy from diabetes, toxic chemical exposure, vitamin deficiencies, infections, autoimmune disorders, genetic diseases and physical traumas (like a neck injury or surgery on your spine that went wrong).

I had blood tests to check for diabetes - nope, but I did have signs of reactive hypoglycemia, which is common in neuropathy patients.

We are all exposed to hundreds, if not thousands of chemicals on a daily basis  - usually in trace amounts and we usually aren't even aware of it.  I have had repeat exposure to various pesticides, coal dust and soot, probably arsenic since I'm an avid gardner on Long Island, NY (we have very high background levels of arsenic in our soil) and my dad was exposed to Agent Orange in Vietnam before I was born.
I had lots of heavy metal blood and urine tests.  Nothing abnormal except for some higher than normal arsenic levels in my urine, but not my blood.  My doctor suggested that could be because my body was ridding itself of past arsenic build up because I was losing so much weight when I first got sick.

I had two physical traumas that may or may not have anything to do with my getting POTS.  First, I wacked my tailbone so hard that my spine felt like it got pushed into my brain - that was a bad snowboarding fall a few days before I started to get dizzy and sick feeling.  Second, about 6 months before I got sick I experienced really bad heat stroke when I was climbing the Cape Hatteras Lighthouse in the Outer Banks on a 108 degree day.

I was tested for many, many infectious diseases.  Negative for Lyme, Bartonella, Babesia, Mono, CMV (cytalomegalo virus), AIDS 1 & 2, Hepatitis, TB, Giardia, Parasites and many others. I was IgG positive for a prior infection with the Eppstein Barr virus (which most Americans have been exposed to by age 30).  I also had high IgG levels of Mycoplasma pneumoniae, which is bacterial "walking pneumonia."  My doctors have disagreed with each other on whether a high IgG with my symptoms was a current infection or a past infection, since IgG levels usually represent past infections.  I don't know who to believe, but Mycoplasma pneumoniae infections can cause nerve damage and POTS.

I doubt I had any vitamin deficiencies at the onset of my illness since I never had any GI problems before all of this, and I ate a fairly healthy diet, and I was "well fed" which is a nice way of saying I was a little chubs.  However, even a few days of diarrhea can leave anyone with vitamin deficiencies.  After a few m months of severe GI issues I had lost 60 lbs. and I started have blood tests showing low potassium, low iron, low Vitamin D, low calcium, etc.  So those vitamin  deficits could have played a role in causing my neuropathy, but we don't really know for sure.  I am now taking lots of vitamin supplements and my levels are back to the low end of the "normal range."

So that leaves us with autoimmune disorders and genetic deficiencies.  Well, I am no expert in either field, but here is what I have been discussing with my doctors.

-Celiac Disease - a genetic autoimmune disorder in which your body produces an antibody when you wat foods containing gluten.  Gluten is a protein found in wheat, oats. barely, rye and some other grains. The antibody produced then attacks your GI tract, which can lead to malabsoprtion problems (and then vitamin deficiencies), and sometimes those antibodies can attack your nerve cells, causing neuropathy.  There are several antibody tests for Celiac, but you have to be eating gluten for those tests to work.  If you are already eating gluten free, you need a genetic test for celiac.  I am still trying to get that one, because my antibody tests were all negative, but my GI symptoms got much better when I stopped eating gluten.

-Crohn's Disease and Ulcerative Colitis - these are separate diseases, but very similar symptoms and both can lead to nerve damage and POTS - mostly because of inflammation and vitamin deficits.

-Ehlers Danlos Syndrome - this is a genetic disease that involves a problem with collagen production.  There are several forms of it, but the most well known one is connected with joint hypermobility and very stretchy skin.  It seems like many POTS patients have this, but it can be very hard to diagnose.  Cleveland Clinic doesn't think I have it, and I agree, even though I have hypermobile hips and shoulders.

-Amyloiydosis - a disease in which the body improperly stores proteins in various tissues throughout the body.  Some forms of this are genetic.  I am being tested for this at Cleveland Clinic this week since my dad has mystery neuropathy too.  First they are doing a stomach fat biopsy to look for the offending proteins, then they are doing the genetic test to look for the gene mutation that causes it.

-Sjorgen's Syndrome  - an autoimmune disease in which your body's white blood cells attack your tissues.  It can lead to dry eyes, dry mouth, painful intercourse, and lung, heart, and kidney inflammation.  There are antibody tests used to look for it, but a lip biopsy is the definitive test used.

-Lupus-is an autoimmune disease in which your body's immune system can attack any of your own tissues.  This causes inflammation, pain and damage.  Lupus can be fatal, although most people who are under medical treatments can lead a normal long life.

So these things are just the tip of the iceberg, as far as answering the question, "what causes POTS?"  There are so many things that cause POTS, and it is so important for you and your doctors to try to figure out WHY you have POTS.  It could mean the difference between getting proper medical care and making a complete recovery or suffering for the rest of your life (or even dying) from a rare autoimmune disease.  No matter what, don't give up. Keep looking for answers.  Even if the best doctors in the world can't figure out what's causing your POTS right now, there is always hope that a new disease will be discovered, or a new treatment will be found that can help you someday.

1 comment:

  1. My POTS became symptomatic after going off birth control pills at age 51. I began having abdominal pain and constipation 3 months after stopping them. I was diagnosed with Pelvic Congestion by CT scan and had an ovarian vein embolization. Two weeks later the pain returned. I lost 16 ponds over 9 months. At 5'8" I was down to 110 pounds. I opted to have a hysterectomy because I was desperate to stop the pain. My surgeon only took one ovary because she did not see evidence of any reason to take both. My pain continued 8 wks postoperative. A second opinion was sought from another GYN. She looked at me and said"You look like someone with POTS". Checking my heart rate sitting and standing, she told to find a POTS Dr. In the meantime, I started taking my BC pills and started feeling better. I was slowly getting better. After 6 months back on them, I am doing a lot better. None of my doctors can explain what this is all about. I continue to search for answers am taking the BC pills with baby aspirin daily. I keep well-hydrated and exercise often.