What is POTS and What Do You Do After Diagnosis?

You’ve been having debilitating symptoms for a while now, and either someone told you about dysautonomia and POTS, you’ve come across it in your Google searching, or your doctor has just diagnosed you with it. Your first question may be what to do next. That’s an important question, and one with quite a few possible responses. First, let’s get into the basics, because this all may still be new to you.

What is dysautonomia?

Also known as “autonomic neuropathy”, dysautonomia is the dysfunction of the autonomic nervous system (ANS). The autonomic nervous system controls all of our body’s “automatic” functions, things that we don’t even think about. This includes heart rate, pupil dilation, digestion, temperature control, kidney function, breathing, and more. Since it affects so many organs and parts of our bodies, we may need a plethora of different specialists. This is why a diagnosis of dysautonomia can feel overwhelming, but know you’re not alone. Every person with dysautonomia is different, so you may not have all of the symptoms someone else may.

There are multiple forms of dysautonomia, some more common than others. Below are all the types that fall under the dysautonomia umbrella. There are at least 15 types of dysautonomia. These include (but are not limited to):

• Postural Orthostatic Tachycardia Syndrome (POTS) – I’ll go into the description of this later, as this post is specifically about POTS.
• Neurocardiogenic Syncope (NCS) – also known as vasovagal syncope, it’s the most common form of dysautonomia, affecting tens of millions of people worldwide. NCS causes those with it to faint, maybe multiple times a day, or maybe just a couple of times in their lifetime. 
• Multiple System Atrophy (MSA) – a fatal form of dysautonomia that occurs in those over 40 years of age. It is a neurodegenerative disorder, and leaves the patient bedridden within a couple of years.
• Familial Dysautonomia (FD) – the rarest form of dysautonomia, only affecting about 350 people worldwide. Mostly found in those with Ashkenazi Jewish decent, it’s a genetic disease that can cause an insensitivity to pain, unstable blood pressure and body temperature, absence of tears, inability to suck or swallow, and more.
• Inappropriate Sinus Tachycardia (IST) – this form of dysautonomia is said to affect about 1.2% of the population. Those with IST have unexpectedly fast heart rates (over 100 beats-per-minute) with minimal physical activity. Symptoms include weakness, chest pain, dizziness, and more. IST is a diagnosis of exclusion, making sure there could be no other cause for the tachycardia like medications, fever, infections, heart failure, etc. 

How can you get dysautonomia?

There are multiple ways one gets dysautonomia, but the most common ways are from an infection (flu, Epstein-Barr (EBV), mono, COVID, etc), autoimmune disease (Sjögrens’ Syndrome being the most common), pregnancy, concussion, trauma, diabetes, Ehlers- Danlos Syndrome, Parkinson’s, and more.

What is POTS?

POTS stands for Postural Orthostatic Tachycardia Syndrome. Those with POTS have an increased heart rate of at least 30bpm (beats-per-minute), or 40bpm for adolescents upon standing. Over 80% of those with POTS are women, mostly of child-bearing age. It affects at least 1-3 million Americans, and 1 in 100 teenagers. Teenagers seem to be more likely to grow out of POTS than adults.

Symptoms include shakiness, exercise intolerance, fatigue, shortness of breath, syncope (fainting) or presyncope (feeling faint), chest pains, lightheadedness, dizziness, shortness of breath, sleep disturbances, and much more.

How do you test for POTS?

The standard for testing is still autonomic testing and a Tilt Table Test. I’ve personally had 3 tilt table tests, and while they’re not fun, they’re an important diagnostic cool. If your doctor does not have access to a tilt table, they can do what they call a “poor man’s tilt table test”, which is taking your blood pressure and heart rate while you’re standing for at least 10 minutes.

A tilt table test is used to “trigger” your symptoms. It will measure your blood pressure, heart rate, along with an ECG (electrocardiogram) during the test. An IV will also be placed in your arm. You are strapped onto a table while first lying down to get your base reading. Slowly the table will tilt up to 60 to 80 degrees, and there is a plate at the bottom of the table called a footboard, for you to stand on. You will stay in this “standing” position for up to 45 minutes, while trying to remain as still as possible. The doctor may choose to give you either nitrates (to dilate your blood vessels and cause blood pooling in your lower extremities), Isoproterenol (will speed up your heart rate to mimic the “fight or flight” response), or Clomipramine (alters the levels of brain hormones such as serotonin, might cause you to faint). For my tilt table test, I had the IV, but I didn’t need any of the medications because my heart rate went high enough on its own.

Autonomic testing may include the QSART test (also known as sweat test), a thermoregulatory sweat test (TST), deep breathing test and Valsalva Maneuver. 

After Getting a POTS Diagnosis

Even though it took a few years for me to get my POTS diagnosis, getting diagnosed was the easy part. I say that because treatment is not easy, nor is finding the correct doctor to treat POTS. To date, I have been to over 8 cardiologists alone. Many doctors just do not understand POTS, unfortunately, and there are no FDA-approved treatments or medications.

It’s not hopeless, though. There are treatments that work for many people, but it will take a lot of patience and trial and error.

It’s also important to remember you are not alone. There are literally millions of us going through the same thing, and many more undiagnosed, searching for an answer to all their symptoms. The chronic illness community can be very helpful, and they’re always there for you.

I also highly suggest going to Dysautonomia International. They’re the leading non-profit organization for dysautonomia, and are always funding new research. They provide tons of information and a list of recommended doctors all over the world, which is extremely helpful.

There are a few medications and that are commonly used, and those include salt tablets, beta-blockers, Midodrine, Fludrocortisone, and a few more.

There are also a few things you can do and change in order to try to manage symptoms. Keep in mind, that’s the only treatment available, symptom management. 

• Medical-grade compression stockings or socks – this helps reduce pooling in the legs and feet, and pushes the blood back up to the heart. Some people also find help from the compression garments on Amazon, and don’t require the medical grade. The difference is in the compression. Anything under 20mmHg is considered non-medical, and anything over (typically 20-30mmHg) is considered medical-grade. 
• Abdominal binders or compression underwear – this does the same thing as compression stockings, and is helpful if you get blood pooling in the stomach. If you have a pair of Spanx or another similar brand, you can try those and see if you get any symptom relief. For me personally, I can’t wear them for too long or I often start to feel nauseous towards the end of the night.
• Increase sodium intake to 3,000mg to 10,000mg per day – we tend to have low blood volume with POTS, and a high salt diet helps increase our blood plasma.
• Drink 2-3 liters of water a day – this is hard for me, as too much water makes me nauseous and “sloshy”, but I try to drink as much as I can.
• Besides water, you can also try drinks with electrolytes.
• More frequent, smaller meals.
• Salty snacks – my favorites are pickles, (black) olives, pretzels, nuts, and canned soup. Can also get regular sodium chicken/beef/vegetable broth, soy sauce, deli meats, etc.

My #1 Tip After Getting Diagnosed

Find a doctor who is empathetic, who you trust, and who understands you and your illness. This may mean trying out different doctors, but like I said, I’ve been to many over the years in order to find one who “gets it”.

My treating doctor is a neurologist. POTS affects the heart rate (along with other things), but it is a neurological condition. While cardiologists typically perform the Tilt Table Tests, treating doctors are often neurologists as well. I personally think it’s important to have both a neurologist and a cardiologist, however I am currently without a cardiologist. One of the largest hospital systems in my area, which I had about 85% of my doctors through, has stopped taking my insurance as of January of 2022. For this reason, I need to find a new cardiologist (and about 10 other specialists).

In my experience, cardiologists rarely understand dysautonomia and POTS as well as neurologists do. I’ve been to 8 cardiologists at this point, and not a single one has fully understood it or known how to treat me. That’s not to say all cardiologists are this way, I know of many people who have luck with theirs. It’s why I’m still on the lookout for the right cardiologist, because I know how important it is for me with my POTS and my HSD/hEDS diagnosis.

Just don’t give up if you go to a few, or 8 like me, and don’t find the right one. There’s always another one out there who could be the answer. When you get diagnosed with a chronic illness, you quickly learn to be your own advocate. If the doctor doesn’t feel like the right fit, you are under no obligation to stay with them, even if they are the doctor that diagnosed you. Trust your gut.

Comorbidities with POTS

There are a number of chronic illnesses that tend to go along with POTS. Some are more common than others, but it’s important to know about them because they may describe some of your symptoms that are not explained by dysautonomia. This was the case for me, and thanks to the POTS community, I was able to learn about these comorbidities, ask my doctors about them, and start the process for diagnosis and treatment.

The Trifecta

The most common comorbidities with POTS (and dysautonomia in general) are what doctors call “the trifecta”. This includes a collection of 3 syndromes that often plague a lot of us, including myself.

• Dysautonomia, including POTS.
• Ehlers-Danlos Syndrome (EDS) or Hypermobility Spectrum Disorder (HSD)– The explanation of both of these get a little tricky, and you’ll notice I use both when describing my own diagnosis. I’ll get into that reasoning at another time, but here’s a basic explanation of the two. Ehlers-Danlos Syndrome is a group of genetic connective tissue disorders. Symptoms can greatly vary, depending on both type of EDS and person, but it affects the function and production of collagen. A lot (but not all) of those with EDS have hypermobile joints, subluxations (partial dislocations), full dislocations, stretchy and “velvety” skin, abnormal scar healing, hernias, and much more. There are 13 types of EDS; the most common is the Hypermobile type, which is also the only type of EDS that has yet to have the genetic mutation discovered by science. Because of this, diagnosis is based on exclusion of other possible factors. It is typically still diagnosed by a geneticist who uses something called the Beighton Score. I will get into more of this and HSD in a separate post.
  Hypermobility Spectrum Disorder has 4 types (localized, generalized, historical, and peripheral), and is diagnosed if you have hypermobile joints (past what is considered normal), but not enough points from the Beighton Score for the full EDS diagnosis. HSD can be either inherited or not (for example, dancers and gymnasts may have an acquired form). Like EDS, subluxations and dislocations are possible, and some of the other symptoms are as well. Since this diagnosis is still only a few years old, it is a little complicated. Again, I’ll get into that another time. However, it is treated the same as EDS would be. It can be just as problematic and painful as EDS, and there are a lot of similarities.
• Mast Cell Activation Syndrome (MCAS) – this is a condition in which the patient suffers from multiple episodes of hives, flushing, swelling, anaphylaxis, difficulty breathing, low blood sugar, severe diarrhea, and more. During these episodes, mast cell mediators are released. Often the episodes are due to an unknown cause and trigger. For this reason, diagnosis is tricky, and many doctors don’t know much about it. It is typically diagnosed by an allergist or immunologist who is knowledgeable on MCAS. Treatment often includes giving the patient both a collection of over-the-counter medications that manage symptoms, which include antihistamines.

Other common comorbidities that are outside of the trifecta include:

Migraines, fibromyalgia, gastroparesis, Chronic Fatigue Syndrome (CFS)/Myalgia Encephalomyelitis (ME), Raynaud’s, Irritable Bowel Syndrome (IBS), autoimmune disease, Small Fiber Neuropathy, (SFN), and more.

 

 

*Please keep in mind I am not a medical doctor. None of this is meant to be medical advice, or be in place of any doctor. I do not suggest trying any of these things without consulting with your doctor. I am only providing my personal knowledge based on my decade-long battle with POTS. I’ve had 3 Tilt Table Tests at this point, and have tried almost every treatment available to me. What may not work for me, may do wonders for you, and what may not work for you, may work for me. These suggestions are just based on my experience, so take it with a grain (or two) of salt (since salt is so important for us!)