Jun 29, 2012

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.

Jun 25, 2012


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. 

Jun 15, 2012


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 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 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.

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.

B12HomocysteineMethylmalonic 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.

Jun 4, 2012

Dear Doctors-An Open Letter to The Medical Profession

Dear Doctors,

I am writing all of you this letter because I have some "stuff" I need to get off my chest.  I know, I know; you probably don't want to hear another patient whining about some perceived mistreatment.  As an attorney, I get plenty of calls from people looking for a new lawyer because their old lawyer supposedly "wronged" them and they want me to help them get revenge and sue their old lawyer.  I don't usually take these people on as clients, because in most cases, they were not wronged, they just lost their case for other reasons. Well, don't worry, this isn't a revenge letter.  Think of it more as a pep talk.

I want health care reform, but not the type they are fighting about on Capital Hill.  I want health care reform in the way we educate our doctors and the way we treat and respect our patients - and that is MUCH easier to implement than anything they are discussing on Capitol Hill.

First, I want medical schools to give all you of a broader perspective.  I know the medical field is becoming increasingly specialized, as each field becomes more technical and our collective knowlegde in a particular fields becomes more complex and in depth.  However, most of you will not be Harvard or Mayo Clinic front line researchers - you are going to see real people with cardiovascular systems AND gastrointestinal systems, so you need to know about BOTH and how they interact!  I do not want any of you telling your patients, as one of my prior GI doctors told me, that you don't want to hear about my fainting, only about my GI symptoms.  This refusal to listen to symptoms related to organs or systems outside of his specialty lead to an almost two year delay in my diagnosis.  Believe it or not, my fainting and GI symptoms were caused by the same thing - autonomic neuropathy (yikes, a third discipline - neurology!) and it was all caused by a systemic autoimmune disease (paging rheumatology...).  I spent two years bouncing from specialist to specialist getting misdiagnosed with ailments in each specialists' field.  Rare was the doctor who looked at my symptoms or tests results outside of his or her field, but the one who diagnosed me accurately (POTS/autonomic neuropathy secondary to Sjogren's Syndrome) did just that.

Maybe from your first day of medical school you were 100% certain you wanted to be a cardiologist, but that doesn't mean you should snooze your way through infectious diseases and genetics, because chances are, many of your patients are going to have more than just a heart problem.  Some of them may have a heart problem because of an infection or a genetic disease - so please pay attention!

I also want medical schools to make you learn more about rare diseases - all those "zebras" you hear about, but are told not to look for "when you hear hoofbeats."  Rare diseases individually may be rare, but as a whole, it is not that uncommon to come across a patient with a rare disease.  The National Organization for Rare Disorders (NORD) notes that 30 Million Americans have a rare disease.  That is 1 out of every 10 Americans.  And keep in mind that your patients with rare diseases will be much less likely to identify their illness themselves.  Someone with broken arm usually doesn't need you to tell them they have a broken arm, but someone with a rare disease, one that the general public has never heard of, will really need your help to figure it out.

I also want medical schools to drill it into your brains that YOU DONT KNOW EVERYTHING AND THERE IS NO SHAME IN THAT!  Far too many of you are telling patients, "there is nothing wrong with you" when what you really mean is, "I do not know what is wrong with you."  If you encounter a patient with a long list of medical complaints and you can't find anything wrong with them, just confess that you can't figure it out.  Your patient will appreciate your honesty.  Then you can try to get them to see a doctor who can perhaps help them figure it out.  Some of you are already wise enough and confident enough in your own abilities to admit when you don't know something - and I applaud you for that.  I wish your peers would adopt this common sense approach.

Law schools teach lawyers that we should only take on cases that we have the experience to handle, and if we get in over our head, we are supposed to hit the books to gain competency in that subject or bring on an additional attorney who has the relevant experience.  For example, as an attorney who usually practices environmental law and policy, I would not take on a murder trial involving the death penalty.  It would be professionally irresponsible of me to do so.  But if asked to represent that murder suspect, I would not tell him he does not have a legal problem simply because I didn't understand it and didn't know what to do to help him.  Without hesitation, I would tell him that I am not qualified to help him, and then I would call the best criminal lawyer I know and ask him to take on the case.  Why is the medical profession different?  Why do so many of you tell the patient there is nothing wrong with them, just because you can't figure it out?  Don't you think that's pretty egotistical? Let's be realistic.  I'm pretty sure with all the specialization going on in medicine, there is no way even the most intelligent and sophisticated doctor could know everything there is to know about medicine.

Similar to the "there is nothing wrong with you" label, is the "it's all in your head" label.  Some of you are far too quick to dismiss your patients' health complaints as being just some mental issue of their own creation.  I find that quite amusing, since now that you are all so specialized, you probably don't have a great deal of expertise in psychiatry or mental health issues.  If you are a infectious disease specialist, would you diagnose a patient with cancer after a brief conversation about their symptoms?  Of course not! You'd runs some tests, maybe send them to an oncologist, run some more tests, and then maybe you'd confirm or rule out cancer or let the oncologist do it.  Unless you are a mental health expert, why would you make a mental health diagnosis before you really took the time to figure out if that is the actual problem?  I was told by several doctors in the first few months of my illness that all of these awful symptoms I was telling them about were just because I was anxious and/or depressed.  Without knowing me (they had literally spent maybe 5 minutes with me) they were surmizing it must be because of stress at work, or because I did not have a child yet, or because of marital problems or because I was co-dependent or just wanted attention.  None of these could be further from the truth.  I was on vacation in VT having the time of my life snowboarding and hanging out with friends in the outdoor hot tub when I suddenly woke up sick one morning.  I had (and still have) an awesome job doing exactly what I love (environmental law).  My wonderful husband and I were enjoying our double income no kids (DINK!) lifestyle for a few more years, living it up at our ski house in VT on winter weekends and as diehard surfer beach bums on summer weekends.  And anyone who knows me knows that I am a fiercely independent woman.  I went half way across the US and then to Europe without my parents in 10th grade,  Mexico with my boyfriend in 12th grade, and then I left my boyfriend back home to go away to college, and moved away from my husband to attend law school.  I definitely don't rely on others to tell me what to do.  In fact, my worst fault is probably that I have a hard time listening to ANYONE tell me what to do.  My penchant for resisting authority figures is probably how I ended up being an attorney in the first place.  I have a coffee mug at work that says "well behaved women rarely make history."  Funny enough, someone also bought me a plaque that says the same thing to hang above my desk.  Maybe my friends are trying to tell me something...

So, this is probably the thing that upsets me most about the medical profession - the rush to judgement that it must be all in the patient's head if the doctor can't figure it out after a few standard blood tests.  To be fair, it's not all of you.  Most of the doctors I saw did take me seriously and did try to help me.  But I am furious at those few that told me it was all in my head, and then proceeded to give me medications that I did not need and were in fact quite dangerous for the underlying condition I had, that they didn't bother to figure out.  I am furious because in my desperation to feel better, I let my guard down and trusted them and took those god awful medicines and almost started to believe that all of this was in my head.  They almost made me lose faith in myself, which is worse than all of the physical symptoms I have had combined.  Thankfully, I was emotionally strong enough, and smart enough, to see that those naysaying doctors were the ones who needed their heads examined, and perhaps their medical licenses as well.  I picked up the pieces of my shattered self after they made me teribbly sick with just a few days of their dangerous pills, and I moved on, to find a doctor who actually knew what he/she was doing and actually cared.

If this had just happened to me, it would be easy to dismiss as one bad doctor and one unfortunate circumstance - just bad luck on my part.  Well, sadly, almost every single POTS patients I have ever spoken with or read about had the same experience with numerous doctors.  I even read one journal article (yes, believe it or not, people who did not go to medical school are smart enough to find, read and understand peer reviewed journal articles) that found 97% of POTS patients had been misdiagnosed as just having anxiety and/or depression prior to their correct diagnosis of POTS.  How can there be hundreds, if not thousands, of doctors dismissing very serious health problems, like syncope, tachycardia over 150 bpm, crashing low blood pressure, severe GI symptoms and more, as all in the patients head?  I know how - because most POTS patients are young women who "look fine," and there is some residual sexism still left in medicine, even if we don't outwardly refer to it anymore.  Misdiagnosing 97% of the 500,000 to 1 million Americans currently living with POTS (80% of whom are women, most of whom are under age 30) sounds not too far removed from those horror stories you hear about Victorian women in the 1800s being thrown in a padded room for "hysterics" because they complained about menstrual cramps.

And hey, I'm not saying you're never going to see a patient who has a serious anxiety disorder and/or depression, and I'm not saying that out of the 500,000 to 1 Million people with POTS, none of them have anxiety or depression.  But peer reviewed literature (which I really wish you would read when patients like me bring it to you) makes it clear that POTS patients are no more anxious than healthy controls.  And sure, you will probably get a few patients now and then who are actually hypochondriacs or maybe even some that have Muchausen Syndrome or another factitious disorder - but these are supposedly just as rare as the physiological "real" rare diseases you seem to have such a hard time diagnosing.

Many times when I went to the ER, I would end up with a well intentioned but clueless resident and even though all I needed was some IV saline for my orthostatic intolerance and hypotension, I was usually offered a Xanax because they thought I was "just having a panic attack."  I was not, and I knew it, so I would refuse the Xanax.  Then I would have to defend my decision to the doctor about why I didn't want to take it (and thus was labeled as an uncooperative patient in addition to a panic attack patient).  A little fact you ER docs need to learn about panic attacks, and I'm sure they must have covered this in medical school, maybe in the fine print in the back of the abnormal psych textbooks - blood pressure does not drop to 60/30 during a panic attack!  Panic attacks result in INCREASED blood pressure.  Many, but not all, POTS patients have really bad POTS symptoms when their BP drops too low, and this is when they are most likely to end up in your ER.  Please learn to distinguish this and stop handing out Xanax like candy - you've already got enough of America addicted to that awful stuff!

Next, I'd like medical schools to require you to pass a "bedside manner" test.  You should not be allowed to practice medicine unless you have achieved and can maintain a certain level of bedside manner - even if you have to fake it.  Your score in this area should be generated by the actual patients you treat during your residency.  If enough of them think you have a terrible bedside manner, you should not be allowed to practice medicine on real patients.  Back to cadavers for you, or better yet, become a lab researcher so you don't have to see real people everyday.

For some reason, a good bedside manner is considered an "old fashioned" concept in medicine.  Why is that?  Have patients suddenly started asking for rude doctors?  Does the latest research show that the patients of obnoxious doctors' recover quicker than do patients' of polite doctors who enter a room with a smile?  I'm not asking for much here.  I don't need a new best friend or drinking buddy.  All you really have to do is respect your patients, or at least have the decency to act like you respect them.  You should not interrupt their questions.  You expect them to listen to you, and thus you should listen to them.  You shouldn't roll your eyes at them, even if they ask a stupid question.  You shouldn't begin looking at your watch 5 minutes after the appointment starts.  If you are a senior doctor or a Professor working with young residents, don't scold them for being polite.  A young doctor was placing barium in my mouth for a test and she accidentally spilled it on my cheek and my shirt.  She politely apologized and grabbed a tissue to help me clean it up.  Her supervising physician sternly scolded her, right in front of me, "don't ever apologize, it makes you seem unprofessional!"  Really? When did basic manners and politeness becomes unprofessional?  In fact, in any other profession, if you dropped sticky white goo all over a client or customer's shirt and didn't apologize, it would be considered extremely unprofessional.

I don't want you to think that I am angry with all doctors.  I am not.  In fact, I have the best General Practitioner anyone has ever had on the face of this planet and I really adore her.  Her awesomeness makes up for a least half of the obnoxious, narrow minded doctors I had to deal with over the past two years.  I just want the medical profession as a whole to do a better job in dealing with real people.  Stop treating symptoms and start treating people.  And I would appreciate it if you could learn more about those medical "zebras." We are a herd 30 million strong, and someday, you or someone you love may be one of us.

That all for today's pep talk.  Next time, I will be giving your medical colleagues working for the insurance industry a "pep talk" (but just between you and me, I would prefer to give them a beating).