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“At first it was hard for her to get her mind around the fact that a simple virus could do so much to her.” New York Times article

The New York Times has a clever feature every month called “Diagnosis” where a doctor presents a mystery illness and asks readers to guess what it is.

The latest involved an active and high-performing 21 year old female college student who got a cold, shook it off but felt tired and out of it. A couple of weeks later she got out of bed – and hit the floor.

collapse

Whatever happened – it was quick and it was devastating

She made it to the bathroom where she saw a pale, ashen grey-lipped face staring back at her in the mirror.  Two hours later she was in the emergency room. She didn’t realize it but her life as she knew it, was over.

It had been a rough year and a half for her: two back surgeries followed by whiplash and headaches from an auto accident.  She was taking a pain-killer, a sleep medication and an antidepressant to help with her headaches and her sleep. Now, after a seeming mild cold, she was unable to stand without feeling like she was going to black out.

The next day, still at the hospital, she’s felt better except when she stood up – and her pulse skyrocketed to 140 beats/minute. She goes on to see a neurologist, gets a CT scan done and more.

All her medical records are posted on the article and the author, a doctor, takes the readers step by step through the process. It’s fascinating process. Then she turns it over to the readers. What does the patient have?

An amazing array of possible diagnoses pour in that if anything gave me some compassion for complex choices facing doctors. There was Marfan Syndrome, carotid-cavernous sinus fistula, stroke, migraine, chiari formation, conversion reaction, Lyme, anxiety, central nervous system infection, posterior encephalopathy syndrome, cerebral hypotension, Susac’s syndrome, reversible cerebrovasoconstriction syndrome, serotonin syndrome, sinus venous thrombosis, cerebral autosomal dominant arteriopathy, NMDAR encephalitis. pituitary tumor, temporal lobe epilepsy.

One doctor says she’ll eat crow if the patient doesn’t have a cerebral spinal fluid leak. Another self-proclaimed world expert on dizziness says she must have serotonin syndrome.

It’s a sophisticated crowd yet it takes 300 replies before someone gets what many of us already know: she has postural orthostatic tachycardia syndrome (POTS).

Postural Orthostatic Tachycardia Syndrome (POTS)

This young lady was lucky. The fact that she was taking an anti-depressant and anti-anxiety drug and had been seen at a pain clinic after an auto accident would have lead many doctors  to conclude that her problems were psychological.  After a careful examination of her records, however, the young doctor attending her noticed that her heart rate had skyrocketed when she was asked to stand in the emergency room. Her blood pressure, however, didn’t drop. That meant she wasn’t dehydrated – and it meant the doctor had no idea what was going on.

The doctor knew enough, though, to give her a tilt table test and curious about what was going on, accompanied her to the test. The results of the test introduced her to a new diagnosis: postural orthostatic tachycardia syndrome.

Could you have POTS without knowing it? Let’s take a short look at POTS.

Symptoms

According to Dysautonomia International the symptoms of POTS include: fatigue, headaches, lightheadedness, heart palpitations, exercise intolerance, nausea, diminished concentration, tremulousness (shaking), syncope (fainting), coldness or pain in the extremities, chest pain, shortness of breath, gastric motility problems and many others.

POTS ranges in severity from relative mild to being severely disabling. Dysautonomia International states POTS affects between 1-3 million people in the U.S., most of them women.

POTS Occurs When

  • A sustained increase in heart rate of greater than 30 beats per minute occurs within 10 minutes of standing
  • Those aged 12-19 years require an increase of at least 40 beats per minute
  • These criteria may not apply to those with a low heart rate when resting

While a tilt test is preferred a home test can be diagnostic.

 The Home POTS Test

  1. Lie down and rest.
  2. Measure your heart rate and blood pressure.
  3. Stand up and measure both after 2, 5 and 10 minutes. Be careful – you may get dizzy or faint.
  4. If your BP drops more than 20/10mmHg within 3 minutes, it’s not POTS.  It’s OH and that requires a more detailed neuro work up, as it’s usually caused by something worse than POTS

Mayo Clinic Introduction to POTS

Finding Help

If your home tests suggests you have POTS (or if just your blood pressure drops precipitously which means you have another form of orthostatic intolerance) contact your doctor. If he/she doesn’t know about the disease -as they very well may not –  look for a specialist.

Prognosis

The NY Times article indicates the young woman knows that POTS is not going to go away.  Some POTS patients do very well with treatment. Others, however, do not and there is no known cure for POTS.

A young woman’s post in the comments section of the NY Times article demonstrated how life-altering even well-managed POTS can be:

While I am very glad to hear that this young woman was diagnosed so quickly, I also want to make it clear that her story did not end with that diagnosis. Though the basics of POTS are known – a malfunctioning autonomic nervous system which causes orthostatic intolerance and manifests in dizziness, migraines, joint pain, pounding heart, inability to sleep, depression, anxiety, chest pain, etc. – there exists no cure.

Lifestyle changes such as increasing salt and fluid intake, wearing compression stockings, gradually increasing exercise in addition to medication trials can mitigate some symptoms, but many POTS patients are unable to ever reach a stable and symptom free life.

Personally, I suffer daily from pain and discomfort due to POTS and I have lived this way for eight years. Now, a year after my diagnosis and in the care of one of the nation’s few POTS specialists at the Mayo Clinic in Arizona, my health is very bad.

Every day I battle myriad symptoms and the desperation and depression that come with living your teens and twenties under the cloud of a chronic illness. I am an ambitious and relentlessly driven person, and yet I had to repeatedly withdraw from college due to my illness. Even though I eventually graduated, which itself was constantly in doubt, I am not yet able to work and am back with my parents.”

Low Diagnostic Rates and Funding

The NIH does not include either POTS or orthostatic intolerance in the 250 or so diseases and conditions for which it tracks funding levels. There’s a good reason why. It’s only funding six POTS-associated studies to the tune about $1.5 million dollars a year.

POTS has got a couple of things going against it. It was first described in 1993 and it mostly affects women. It fits in a class of disorders that often affect women, are pretty common and cause a lot of distress but don’t usually kill. The NIH essentially ignores all these disorders.  They include POTS, fibromyalgia, ME/CFS, interstitial cystitis and others.

Check out this POTS Cheat Sheet  from Dysautonomia International 

Ten Facts Doctor Should Know About POTS

1. POTS is a disorder of the autonomic nervous system. Approximately 50% of POTS patients have sudomotor neuropathy, and 20% have cardiac dropout on MIBG scans, which may be due to cardiac autonomic neuropathy.(1-4)

2. POTS is not rare. POTS impacts an estimated 1,000,000 to 3,000,000 Americans;(5-10, 30) 80-85% female, most commonly women of childbearing age.(7,8,11,12) POTS occurs around the world, but estimates are not available for other countries.

3. Research indicates that POTS may be an autoimmune condition. Antibodies targeting alpha1, beta1, and beta2 adrenergic receptors, muscarinic3 receptors,(12) g-AchR receptors,(3,13) and numerous cardiac lipid raft proteins(14) have been identified in POTS patients. Preliminary studies are underway exploring the use of IVIG in POTS.(15)

4. POTS or POTS like symptoms can appear secondary to other autoimmune conditions, such as Sjogren’s syndrome, lupus, antiphospholipid syndrome, celiac disease, ankylosing spondylitis, sarcoidosis, Lambert Eaton myasthenic syndrome, myasthenia gravis, and mulitple sclerosis.(9,12,16-21)

5. POTS can cause symptoms throughout the body including tachycardia, palpitations, chest pains, lightheadedness, fainting, nausea, fatigue, gastroparesis or rapid gastric emptying, migraines, blood pooling in the extremities, Raynaud’s, tremulousness, sleep abnormalities, cognitive impairments and more.(9,12,22)

6. The majority of POTS patients are hypovolemic despite adequate hydration. Standard blood and urine tests may not detect this hypovolemia, as the patient is typically deficient in plasma and RBCs. Blood volume analysis with a radio-tracer can be used to evaluate a POTS patient for hypovolemia.(23)

7. POTS is often misdiagnosed. The average time to diagnosis is 5 years and 11 months.(24) 85% of POTS patients are told it’s “all in their head” or given similar psychiatric labels prior to receiving a POTS diagnosis,(24) but research shows that POTS patients are no more likely to have psychiatric disorders than healthy controls.(25,26)

8. Most teens do not “outgrow” POTS. A 10 year retrospective study of Mayo Clinic’s pediatric POTS patients found that 60% had improved symptoms but still had POTS, 20% had recovered, and 20% had worsened or stayed the same since the time of diagnosis.(27)

9. The severity of POTS symptoms varies. Approximately 25% of POTS patients are so disabled that they cannot work or attend school.(9) The disability seen in POTS has been compared to COPD and congestive heart failure.(9)

10. Patients with suspected POTS should be referred for autonomic function testing including tilt with heart rate variability, Valsalva and QSART. In some cases, supine/upright catecholamine testing, blood volume analysis with a radio tracer, and skin biopsies to screen for sudomotor and sensory small fiber neuropathy may be used. An investigation should be made to search for underlying or contributing conditions such as autoimmunity, Ehlers-Danlos syndrome(28) or mast cell disorders.(29)

Resources

More POTS Blogs From Health Rising

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