Living with dysautonomia

A CNS reader writes: Did you know that October is Dysautonomia Month? Dys- what? you would ask. Another disease awareness? Sick and tired of this already. But please keep reading, for you might find it useful even if not today, for it may affect you or your children, teenage girls especially, tomorrow, and you would spend months trying to figure out what is wrong unless you are aware.

It is estimated that up to three million people in the US alone have one or another form of dysautonomia, Postural Orthostatic Tachycardia Syndrome (POTS) in particular, while European data is largely unavailable. One in 100 teenagers is deemed to have POTS (source: Dysautonomia International). The symptoms are usually dismissed as anxiety by doctors.

Before I continue to my personal experience with the diagnosis and explain what POTS is, let me show you some perspective.

Birth and death: 500,000 BC until today:
Born-109 billion
Dead-102 billion
What was the leading cause of death until 1900?
– INFECTION!
What are the prevailing diseases and causes of death after 1900?
– Cancer, autoimmune, autonomic, cardiovascular, neurological and metabolic.

So what is dysautonomia?

Dysautonomia is an umbrella term used to describe several different medical conditions that cause a malfunction of the Autonomic Nervous System. Your AUTOPILOT! The Autonomic Nervous System controls the “automatic” functions of the body that we do not consciously think about, such as heart rate, blood pressure, digestion, dilation and constriction of the pupils of the eye, kidney function, and temperature control.

Dysautonomia is not rare. Over 70 million people worldwide live with various forms of dysautonomia. POTS is a variant of dysautonomia and more people have POTS than other forms of dysautonomia.

Now I will describe in a very simplified way what happens to a person who has POTS and why it is so easy to dismiss it by doctors, even though diagnostics protocol has been established for several years already and is updated annually. I believe ICD-12 code is coming specifically for POTS. That is a biggie!

So when a healthy person stands up from a supine or sitting position, his brain automatically sends a signal to his lower body blood vessels to constrict, otherwise gravity will pull blood into lower extremities. It happens automatically. When a person with POTS changes position, his lower body blood vessels don’t constrict for some reason, blood gets pulled into his lower extremities, estimated upper body blood volume is reduced by 30-40%.

The brain senses that not enough blood is being supplied to the vital upper body organs, including the heart, and sends signals to the heart to pump faster, which goes into an overdrive trying to pump blood into the vital organs. Paradoxically, blood pressure remains normal and if this continues, the brain, to prevent vital blood loss, constricts its blood vessels, causing the person to faint, but it is life saving since once on the ground, blood flow through the body restores on its own.

So four things happen upon standing in a person with classic POTS: blood pools into his legs, the heart starts running a marathon, blood pressure remains normal and person either faints or feels that he is going to faint (usually he or she either sits down or lies down to prevent fainting).

So why is it so hard to diagnose? Well, syncopes (fainting) and pre-syncopes can have many many many reasons. There are two types of syncopes that doctors are aware of, but not POTS. Orthostatic hypotention syncope and vasovagal syncope are not variants of dysautonomia.

But POTS causing syncopes is easy to diagnose if one knows what to look for. Let’s start with the fact that the majority of people who develop POTS are young, vibrant and active individuals, many were athletes before this syndrome hit them seemingly out of the blue. They don’t look sick and the very first thing a doctor says is, “But you look so well! You can’t possibly have anything,” and brush it off to anxiety.

If a doctor, any doctor, properly examines a person, he would find out, on the very first visit, that his heart rate goes significantly up in a standing position and remains high, going even faster the longer the person stands without any movement. If a doctor looks at his legs, he would notice they turn purple.

Now let see what really happens (my experience) when a person decides to see a doctor because he/she always feels as if he/she is going to faint.

I need to mention that when the heart rate is elevated, even significantly, but in a normal sinus rhythm, you don’t feel it as you would feel palpitations or arrhythmia, so you have no idea your heart rate is elevated. Secondly, you don’t really pay attention to the change of your legs’ skin color, if you notice it at all. You just feel something is wrong, you feel near fainting all the time, but continue to ignore it. But it is becoming harder to work, study and live normal life.

I went to see a doctor only after I started experiencing such severe shortness of breath that making a few steps without sitting on the ground became impossible.

So, I went to a doctor, an internist, employed by one of the Cayman hospitals (that doctor is in a private practice today). My vitals were checked while I sat in a chair, stethoscope to my heart, followed by a verdict: all is well, you look good, probably anxiety, take anxiety pills, but we”ll run blood work just in case, and head to the MRI. The fact that “good looking” to me is unable to make few steps, finish a meal or simply talk due to shortness of breath is completely ignored.

I have to add that at that time I wasn’t feeling like that all the time. Episodes would come and go.

My second visit to the doctor: all blood work, and MRI results came back normal. Maybe I should see a psychiatrist?

Meantime, I already had rwo ER visits. They run tests. Keep in mind all are performed while I was in a supine position, so all are normal. It is only when I stand up that everything goes haywire.

Visits to the internist continue. The unspoken message is, it is all in my head. Not once were my vitals were checked in a supine, sitting and standing position consecutively. Had it been done, I would have been diagnosed right away.

On my third ER visit I was admitted and Dr Nelson, bless his heart, had decided to perform tilt table test on me, sending me to Miami with a nurse. This test is now available at Health City Cayman Islands. The diagnosis was made: Classic POTS.

I returned to Cayman and it was just the beginning of my long journey living with POTS. Little did I know what lay ahead of me.

My internist continued to treat me as it wasn’t a big deal. I changed doctors. Today I would have asked her, let’s suck 30-40% of your blood volume from your upper body and then you tell me how you feel.

Unfortunately there’s no treatments for POTS, just managing symptoms. Some recover, some have it easy, some are bedridden and home-bound. But the good thing is that today, unlike five years ago, there are established diagnostic and symptoms management protocols. There’s the Dysautonomia International Foundation that is pushing for more research. You can learn all you need to know from their site.

There are still very few doctors who specialize in POTS/Dysautonomia, but the number is growing. Primary care providers simply educate themselves at the start when treating dysautonomia patients instead of referring them to “specialists” who also know very little.

For unknown reasons the number of children/teenagers having dysautonomia is growing dramatically. A viral infection, a vaccination, an anesthesia, head trauma, and many other reasons could disable your “auto pilot”, your ANS. Adults, males and females can also have POTS. It is estimated that more people have dysautonomia than multiple sclerosis and Parkinson’s disease combined.

I hope my dysautonomia diagnosis experience would helpful because, sadly, it remains an “exotic” animal for many doctors. They either know very little or nothing at all about it.

October is Dysautonomia Awareness Month. The colour is turquoise. If you see it, you know now what it stands for.

If you live in the Cayman Islands and there is something about your life that you’d like to share with us, email info@caymannewsservice.com

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