Definite Hyper POTS

What Is Hyperadrenergic POTS?
 

Written By: Kimberly Swanson


Hyperadrenergic Postural Tachycardia Syndrome is under the canopy of a more broad condition called, "Dysautonomia". What is Dysautonomia? It is a term used to describe an overall defect, flaw, breakdown or failure of the Autonomic Nervous System. Many conditions can be fit under this canopy of Dysautonomia like: Autonomic Neuropathy, Pure Autonomic Failure, PD POTS, Hyper POTS, Multiple System Atrophy, Baroreflex Failure, Reflex Sympathetic Dystrophy type 1 & 2, Fibromyalgia, etc.

 

Hyper POTS: This type of POTS has a strong familial link. "There is evidence to suggest that Hyperadrenergic POTS is a genetic disorder involving a mutation in a protein that recycles norepinephrine in the spaces between nerves the intrasynaptic cleft" (Grubb BP. The fainting Phenomena (second edition) Blakwell/Futura Press, Malden MA 2007). "This in turn leads to excessive degree of norepinephrine serum spillover in response to a variety of sympathetic stimuli thereby producing a "hyperadrenergic" state that appears similar to a Pheochromocytoma" (Medscape Today - The Postural Tachycardia Syndrome: A Concise Guide: Classification). People can have this condition genetically, but never realize it. There has to be a trigger to bring them out like: a car accident, a virus, emotional trauma, surgery, endocrine problems, infectious disease or connective tissue disease; any condition that leads to a deconditioned or debilitated state. For example; let’s say someone was in an auto accident and broke their leg. This patient would be in a lot of pain. Due to their pain; their sleep would become disrupted, eating habits would change and their activity level would be drastically different. Those three changes alone would be enough to bring out their underlying condition of Hyperadrenergic POTS. It's usually passed from mother to daughter, but men can also be affected. Hyper POTS is generally seen in people of Nordic decent with blond hair and blue eyes (Dr. Blair Grubb). Norepinephrine can affect any/all of the body's systems that are automatic in nature (meaning: you don't have to consciously think about doing it) like: blood pressure, heart rate, thermoregulatory system, digestion, mental processes (cognitive abilities and emotions) and musculoskeletal system. When a patient with Hyper POTS stands; their BP AND heart rate increases. The heart rate rises 30 beats per minute or to a heart rate greater than 120 beats per minute within 10 minutes of upright position. The symptoms of Hyperadrenergic POTS are:

 

Chest Pressure, Syncope, Near Syncope, Motion Sickness, Heat Intolerance, Daily Headache / Migraine, Appetite: overactive or none/early satiety, Red burning face (severe flushing), Nausea, Extreme fatigue, Weakness, Constipation, Diarrhea, Muscle Cramping, Eye Symptoms: sore/blurry vision/watery or dry eyes, Light Sensitive, Noise Sensitive, Balance Disturbances, Lightheaded, Hot/Freezing Temperature Disturbance, Vomiting, Body jerks, Heart in throat feeling, High Blood Pressure, Bowel Incontinence, Can’t handle stress/good or bad, Rapid Heart Beat, Heat Surges, Feeling Shaky (Internally and/or externally), Insomnia, “Body” Anxiety (adrenaline) feeling it flow, Hand tremors, Shortness of Breath, Excessive Sweating, Difficulty Communicating/forming thoughts, Clenched teeth, Slurred Speech, Uncontrolled / Unprovoked Emotions: Depression/Anger/Crying/Frustration/Anxiety, Urinary Retention, Adrenergic Urticaria, Medication Sensitivity and Reflex Anoxic Seizures.

 

The treatments for Hyperadrenergic POTS are: to find the right alpha-beta block(s) to control the high BP and heart rate (like Catapres and/or Labetalol), an anti-depressant to control the re-uptake of norepinephrine, a good sleep hygiene plan complemented with an effective sleep medication to reach stage 4 sleep (the restorative stage of sleep) and exercise to burn off the excess norepinephrine and restore deconditioned muscles - should complete at least 20 -30 minutes of aerobic workouts/3x per week. There are some anti-depressants that can make you MORE symptomatic so ask your doctor about this before taking an anti-depressant.

 

Hyperadrenergic patients will have this syndrome the rest of their life. That's the bad news, but there is good news. Once the patient finds the right combination of medications, is getting into stage 4 sleep and is exercising; their syndrome will become manageable. They will never be normal; but they will be productive and have fulfilling lives. It can be a long road to find the right treatment plan, but hang in there and keep trying; it will pay off eventually.

 

Most literature on Hyperadrenergic POTS says it’s relatively uncommon, but if we put it in perspective of being the same as Fibromyalgia; we realize it’s far more common than we once thought. There are approximately forty million people world wide with Fibromyalgia. People with Fibro don’t need to know they have Hyperadrenergic POTS to be effectively treated, but Hyper POTS patients need to know they have Fibromyalgia in order to understand and treat their condition.

 

My hope is to make a bridge between these two syndromes to properly educate patients, their families and physicians on how to make a cohesive treatment plan.

 

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