Looking at my schedule a “new” 18-year-old patient was next. The name seemed familiar.

 

My office manager said that she had been my patient the first five years of her life but then the family had moved out of state. They recently moved back to this area. The young lady needed some immunizations for college and wanted to come back to this office. After almost 13 years I remembered her and her mom very well. Mary had grown into a mature and healthy young lady, and mom had not changed much over the years. We all laughed when I was doing my exam and mom asked, “Can I still see Elmo in her ears?” as this would always make them both smile.
 

The history revealed that the only medication she was on was fludrocortisone. This had been prescribed for a condition called P.O.T.S.

 

About a year ago Mary was recovering from infectious mononucleosis. For months after the diagnosis she was very fatigued, would become dizzy and light-headed and almost faint whenever she stood up. At times she had palpitations and would occasionally get pounding headaches. Initially she was told that this was all part of mono and to just give it time. There was also a concern that she could be depressed. However, she saw a psychologist who said that she was NOT depressed and not at all anxious. One episode occurred when she actually blacked out and fainted after getting up suddenly to answer the phone. This generated a 911 call. She was taken to the ER by ambulance. The ER doctor was an experienced and astute physician who did some measurements of her pulse and blood pressure in various positions. He suspected that she might have P.O.T.S and referred her to a Cardiologist. More specific tests were done that confirmed P.O.T.S. The Cardiologist made dietary changes and prescribed fludrocortisone. She has been fine since.

 

P.O.T.S is a circulating blood disorder caused by a disruption of the autonomic nervous system.

This system is a part of our nervous system that regulates critical functions of which we cannot consciously control, such as heart rate, blood pressure, sweat and body temperature. P.O.T.S is more common in females, over 85% of the child bearing age, 15-to-50 years. It is estimated to occur in 1-3 million people in the United States. Although it was “coined” P.O.T.S in 1993, this disorder has been around for many years but under different names.
 

Normally when we stand up after sitting or lying down for a short time, the blood tends to pool in the lower half of the body.

To insure that the blood supply to the brain remains adequate, the heart momentarily speeds up, and the blood vessels constrict. This is a result of the release of the hormones epinephrine and norepinephrine (fight or flight). Once the blood flow has been adequately restored the heart rate slows back down to normal and the blood vessels relax.
 

In P.O.T.S, when one stands up the blood vessels don’t respond to the hormones that should cause them to constrict.

 

The blood continues to pool in the lower half of the body despite the heart rate increasing. As the brain is not getting sufficient blood, more epinephrine and norepinephrine is released. This in turn may further increase the heart rate and may cause tremors, palpitations or shakiness. These symptoms may be exacerbated when one takes a hot shower, is upright for a long period of time, or is just being active on a hot day.

No one knows the actual cause of this syndrome.It is known to occur after pregnancy, surgery, trauma or viral illnesses such as mono. The myriad of symptoms seen with P.O.T.S can be a high or low blood pressure, an increased or decreased heart rate, dizziness, fatigue, light headedness, “brain fog”, headache, chest pain, tremors, shakiness or blurred vision. One may also have a gravity dependent reddish-purple discoloration of the legs from the pooling of blood which resolves when the patient lays down.
 

To make a diagnosis of P.O.T.S, there should be an increase in heart rate of 30 beats a minute within 10 minutes of standing up in adults.

 

For children it would be an increase of 40 beats per minute. One can check a pulse and blood pressure in the horizontal position, then have the patient sit up or stand while repeating the BP and pulse at 2, 5 and 10 minutes. The astute ER physician did just that. There are more sophisticated tests that can confirm the diagnosis. One is the Tilt Test whereby the patient is strapped on a table that can maneuver in different positions while vital signs are taken. This is what the Cardiologist did to confirm the diagnosis.
 

Each case of P.O.T.S presents differently and uniquely.

 

Therefore the treatment varies from case to case. In general the various treatments include proper hydration (drinking 2-3 liters of fluid a day), increasing salt in one’s diet, proper dietary changes, compression stockings and physical therapy. There are medications that can be used including Fludrocortisne which enhances sodium retention in the kidney. Midodrine improves blood vessel constriction, and beta blockers help regulate and control the heart rate. It has been well documented that alcohol and caffeine can worsen the effect of P.O.T.S.

 

A point worth mentioning is that many patients with P.O.T.S are often mis-diagnosed as being depressed or overly anxious. This has been shown to not be the case. In fact one study showed that the incidence of depression in patients with P.O.T.S was actually LESS than the general population.

 

The severity of symptoms ranges from merely a mild “head rush” when one stands up to being so fatigued that one cannot work or carry on daily functions of living. It typically responds well to appropriate treatment. In many cases it gets better with time and may go away. Needless to say, P.O.T.S is a very interesting and intriguing syndrome that has been around for a long time. But at least now it has a name and can be successfully treated.