11/6/2020 by Share Your Story

Understanding the Care and Symptoms of Dysautonomia

Several people in our audience live with variations on this neurological disease, so here's a primer on types and symptoms of dysautonomia.


Understanding the Care and Symptoms of Dysautonomia

Introduction

Hello readers, it’s your editor, Jim here. We’re going to try understanding dysautonomia together in this article. Fair warning, you’re going to be running into a lot of scientific terms. Don’t be discouraged. We’ll explain as we go and at the end of the article we’ll include a glossary and some other tools to help this all make sense, including some practical comments from patients. Our goal is to leave you with a clear enough understanding of the origin and symptoms of dysautonomia so that you could explain it very basically to someone else.

What Is Dysautonomia?

Dysautonomia is a disorder of the autonomous nervous system (ANS) that leads to the failure of the sympathetic and parasympathetic parts of ANS, which control involuntary body functions:

  • Heart rate
  • Blood pressure
  • Breathing
  • Digestion
  • Temperature
  • Hormonal and bladder function
  • Sexual function, etc.

How Rare Is Dysautonomia?

Not rare at all! It affects about 70 million across the globe and may be congenital (present at birth), develop gradually over time, or appear suddenly at any age. It affects men and women equally.

What Causes Dysautonomia?

When the autonomic nervous system does not communicate with the rest of the nervous system the way it is designed to, dysautonomia can be the result. What causes the ANS to not communicate correctly is unknown. Unless it is caused as the result of another condition (that would be called “Secondary” dysautonomia) the dysautonomia is called “Primary”. If the primary dysautonomia is known to be hereditary (inherited from parents) then it is considered “Hereditary”.

Hereditary dysautonomia:

Also known as “Familial dysautonomia” or Hereditary sensory neuropathy type 3; Hereditary sensory and autonomic neuropathy 3; HSAN 3; HSN 3; Riley Day syndrome.

Passed on through a family’s genes, familial dysautonomia can reduce a person’s pain threshold, affect tear production, and ability to regulate body temperature. It is commonly diagnosed among Ashkenazi Jews.

Primary dysautonomia:

Develops without any underlying cause.

Primary Dysautonomia can lead to a number of other conditions:

Neurocardiogenic syncope (NCS):

The most common form of dysautonomia that can lead to fainting spells as frequently as several times a day or as infrequently as a few times throughout life.

Postural orthostatic tachycardia syndrome (POTS):

Disruption of blood flow due to POTS can lead to fainting, chest pain, or shortness of breath when a person tries to stand up.

Multiple system atrophy (MSA):

Common among those over 40 years old, MSA is rare but can be debilitating and even fatal. Disease features mirror those of Parkinson’s disease, which can lead to misdiagnosis. MSA can cause incontinence, heart rate issues, low blood pressure, and erectile dysfunction.

Pure autonomic failure:

This type of dysautonomia causes a drop in blood pressure with a change in position from sitting to standing up, leading to dizziness, fainting, chest pain, tiredness. Sitting or lying down can help relieve these symptoms.

Secondary dysautonomia:

May develop as a consequence of another disease that the individual may be suffering from, such as –

  • Diabetes
  • Parkinson’s disease
  • Muscular sclerosis
  • Rheumatoid arthritis
  • Lupus
  • Sjogren’s syndrome
  • Sarcoidosis
  • Crohn’s disease, ulcerative colitis
  • Celiac disease
  • Charcot-Marie-Tooth disease
  • Chiari malformation
  • Amyloidosis
  • Guillain-Barre syndrome
  • Ehlers-Danlos syndrome
  • Lambert-Eaton syndrome
  • Vitamin B and E deficiencies
  • Human immunodeficiency virus (HIV)
  • Lyme disease
  • Gastroparesis

Known Triggers and Symptoms of Dysautonomia

Dysautonomia can affect a variety of body systems, which means symptoms of dysautonomia can vary widely.

  • Balance/Focus
    • Balance problems
    • Dizziness, vertigo, lightheadedness
    • Forgetfulness
  • Mood swings or anxiety
  • Migraines or frequent headaches
  • Breathing
    • Shortness of breath
  • Heart/cardiovascular
    • Chest pain/discomfort
    • Big swings in heart and blood pressure
    • Heart palpitations
  • Blurred vision
  • Digestive system
    • Difficulty swallowing
    • Nausea and vomiting
    • Low blood sugar
  • Incontinence
  • Erectile dysfunction
  • Weakness

The various combinations of these symptoms are part of why dysautonomia is difficult to diagnose. One of our readers, Kathleen, said it took her over 10 visits and several doctors to finally get diagnosed.

Known triggers for this condition include hot weather, dehydration, tight clothing, alcohol, and stress. Dr. Peter Rowe of Johns Hopkins University has developed a patient information brochure on distress associated with standing up and how it can be treated.

Diagnostic Testing

Breathing test, blood work, sweating tests, and electrocardiography (heart function) are commonly used to diagnose dysautonomia. A tilt-table-test may also be conducted, where a machine monitors the body’s ability to normalize blood pressure and heart rate in a person lying down on a table that can tilt at various angles.

Are There Any Effective Treatments?

Dysautonomia cannot be cured. If dysautonomia emerges as a result of another condition, treatment of that condition could improve symptoms of dysautonomia.

The best people to answer whether or not treatments are effective are the people living with dysautonomia. We are going to do that and follow up to this article.

Maintenance Therapy & Care

Symptoms associated with dysautonomia can be kept under control by supportive care such as elevating the head of the bed, giving the patient a large infusion of water through their vein, drinking plenty of water, and treatment with certain medications.

Patients with certain forms of dysautonomia are also advised a high-salt diet. Please check with your physician before integrating this or any other dietary / lifestyle change.


Glossary of terms

Atrophy: a wasting away of body tissues (like muscle) or organs

Autonomic nervous system: the part of the nervous system that regulates organ function without any conscious recognition or effort

Sympathetic nervous system: connects all our organs to the brain via spinal nerves. This system controls our fight-or-flight response.

Parasympathetic nervous system: this system controls visceral organs (those located in the chest, abdomen, and pelvis) and regulates the body’s resting and feeding response.

Neuropathy: also known as peripheral neuropathy, it is the feeling of tingling, numbness, or muscle weakness resulting from damaged nerves of the peripheral nervous system. These are nerves that are outside of the brain and spinal cord.

Neurocardiogenic: a stress-related condition stimulated by nerves

Orthostatic: related to an upright posture

Syncope: temporary loss of consciousness caused by a fall in blood pressure

Tachycardia: fast heart rate


Patient Input

We asked our Facebook community if they had anything to add to this conversation. Here’s what they said:

Kathleen says

– “Dysautonomia affects each person individually which makes it so hard to diagnose. (It) may affect the functioning of the heart, bladder, intestines, sweat glands, pupils, and blood vessels. I have Dsyautonomia after 25 years of Gastroparesis. My symptoms include a ridiculously low blood pressure, dizziness, fatigue, and POTS because my pulse elevates more than 25 points when I stand up.

Dysautonomia has many causes, not all of which may be classified as neuropathic. When trying to find a Specialist it’s best to begin with a physician who specializes in Dysautonomia, not all do.”

According to the Dysautonomia Youth Network of America, a physician specializing in dysautonomia might be a cardiologist, neurologist, even a gastroenterologist.


RoseAnn asked

– “What causes this condition and is there a treatment for it?”

Great question. We talk about Cause in the article above and I want to show you where we got the information from. It’s from this page by the Cleveland Clinic. When we need to learn about a disease, we always go to the experts… Cleveland Clinic, Mayo Clinic, NIH, etc.

As for Treatments, that’s more complicated because dysautonomia can manifest in so many different ways. Some of the symptoms patients experience have treatments (not to be confused with a cure) but many do not.


Elaine asked

– “Since it’s so hard to get an accurate diagnosis, do the treatments improve quality of life enough to go that extra mile to get the diagnosis and treatment? Especially if the clinics that specialize in treating it are far away and/or severely backlogged?

For instance, our pediatrician and allergist agree that we have some mast cell and POTS type issues going on, but the treatments that they say are available make it seem like it’s not worth the many, many hoops I’d have to jump through to get specialized help.”

As patients, we ask that question of ourselves a lot – Is everything that goes into engaging with healthcare going to be worth it in the end?

Bari responded to Elaine‘s question, saying

– “Huge return of investment. Different disease, but had to travel to the best and it saved my life!”

The bottom line Elaine, is that we all have to make that decision for ourselves. One idea is to have a telehealth visit with this specialist and ask frank questions. Another idea is to join a patient support organization like the Dysautonomia Support Network and ask others who have had to make that same choice.


Additional Resources

  1. Information on clinical trials for dysautonomia: https://clinicaltrials.gov/search/term=Dysautonomia.
  2. Dysautonomia International: http://www.dysautonomiainternational.org/index.php.
  3. Dysautonomia support groups: http://www.dysautonomiainternational.org/page.php?ID=24.

Featured image: Josie Steinauer, used with permission

Surabhi Dangi-Garimella, Ph.D., Principal, SDG AdvoHealth, LLC, researched and summarized this article. Improving patient access is our mission and we are happy to utilize a variety of experts to carry that out.


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