Like a lot of other patients I spend most of my life explaining Dysautonomia and correcting inaccuracies. It seems like an endless chore. From how to spell it, to what the hell is it, I have explained it more times than I can count. After a particularly whingey, "why don't people understand?" type of day, Mr Grumpy pointed out that I should simply do a Dysautonomia for Dummies post that clarifies some of the major points in less that five minutes. So here goes:
What is Dysautonomia?
Dysautonomia is an umbrella term for a collection of disorders that affect the Autonomic Nervous System (ANS). The term 'Dysautonomia' is akin to the word 'Cancer'. There are many forms of cancer eg breast, brain, ovarian. Similarly there are a many forms of Dysautonomia, for example:
Neurocardiogenic Syncope/Neurally Mediated Hypotension (NCS/NMH)
Postural Orthostatic Tachycardia Syndrome (POTS)
Orthostatic Intolerance (OI)
Orthostatic Hypotension (OH)
Pure Autonomic Failure (PAF)
Multiple Systems Atrophy (MSA)
Baroreflex Failure (BF)
Then there are those like myself with clear autonomic dysfunction who don't quite fit into any single subgroup.
Terms and diagnostic criteria change as knowledge evolves. The latest criteria for POTS, IST and VVS (2015 Expert Consensus Statement by the Heart Rhythm Society) can be downloaded from here.
Familial Dysautonomia (FD): this is the only form of Dysautonomia with a clear genetic origin and is almost exclusively seen within the Ashkinazi Jewish populations.
There are also many other disorders that may have autonomic involvement eg Parkinson's disease.
Dysautonomia can also develop following traumatic brain injury.
The two words that may best describe Dysautonomia would be variable and unpredictable.
What is the Autonomic Nervous System (ANS)?
The ANS is the body's autopilot and is responsible for maintaining homeostasis, or simply put, keeping our body stable. It controls all the unconscious bodily functions such as, heart rate, blood pressure, breathing, digestion, temperature regulation and even how our pupils react to light. When this system malfunctions or fails you develop Dysautonomia. It is further separated into the Sympathetic (excitory) and Parasympathetic (inhibitory) Nervous Systems, which at their simplest work in opposition to each other to maintain homeostasis, although there are times that they work together. The Enteric (gastric) Nervous System is also sometimes included as part of the ANS. For a more in-depth explanation of the ANS, click here.
No two patients experience Dysautonomia the same.
Even when two patients have the same diagnosis eg POTS, their experiences may differ greatly. Some patients have very mild symptoms and can continue their daily life as normal. Yet others may be totally housebound and reliant on others for aide with daily tasks eg showering.
The combination of symptoms a patient experiences can also differ. The ANS controls a wide variety of bodily functions. As a result one patient may have issues with heart rate and temperature regulation, another may have additional issues with blood pressure and digestion. Depending on what areas are affected and to what degree, this will influence the symptoms that a patient presents with.
What medications work well for one patient may do nothing, or cause horrendous side-effects for another. There are a core group of medications that are commonly used in Dysautonomia patients, however it is often a case of trial and error to see what works for each patient. Not only with type of medication, but also dosage.
What causes Dysatuonomia?
Causes are varied and include: viral, mitochodrial diseases, connective tissue disorders, neuropathies, autoimmune disorders, genetic disorders, neurotransmitter dysfunction. It is important for patients to attempt to uncover their underlying disorder as if treated they may reverse, slow, or control the autonomic symptoms. In many cases an underlying aetiology may remain elusive despite extensive testing.
Many patients also have a variety of comorbid medical conditions eg diabetes or thyroid issues which complicate both diagnosis and treatment. These may be pre-existing or develop over time.
There is no single treatment that will work for every patient. In general, treatment involves an individually tailored program which may include variations of medications, lifestyle changes and diet, and a graduated exercise program.
This program will generally be dictated by the symptoms a patient experiences. For example, if a patient has tachycardia (fast heart rate) they may be prescribed a beta-blocker, which slows down the heart rate. If a patient has low blood pressure they may be prescribed fludrocortisone, which increases blood volume.
In general, patients require an increase in both their salt and water intake. A change to eating patterns in the form of small, frequent meals, are also recommended to prevent blood pooling in the gut. Patient's may also require aides such as medical compression stockings to prevent blood pooling in their legs, and items such as shower chairs to help prevent fainting in the shower. Graduated exercise, particularly reclining exercises and lower limb strengthening, are also necessary as deconditioning can occur rapidly in most patients.
Like symptoms and causes, long term outcomes vary. Some patients will recover, especially if younger and if their symptoms began after a virus. Some patients, will have a stable but chronic pattern. Some will have a relapsing remitting profile similar to MS. And yet others will progress over time.
There is currently no cure for Dysautonomia, although in some cases if the underlying cause is discovered, treatment of that condition may alleviate the autonomic symptoms.
It is important for family members and friends to understand that a patient's symptoms may vary day-to-day and hour-to-hour. As such, what they may be able to do one day, is impossible the next. For example, going to a cafe with friends may be possible for a couple of hours one day, but the patient may be unable to do it another, or have to cancel at the last minute. Additionally, that simple outing may place some patients in bed for the next week.
Patients aren't crazy or lazy (just two of the terms that are frequently thrown at patients), they are just dealing with a complex, often scary, and frequently unpredictable illness that most people, both in the medical field and wider community, have little to no knowledge.
What's it like to live with day-to-day?
This blog is a history of my experience with Dysautonomia over the past ten years. From diagnosis to treatment, coping with change and the medical system, you can get an idea of the day-to-day by clicking any of the tabs above. But for a short glimpse into life with this illness you can check out
Dysautonomia. Invisible Illness My Arse!
Links for more information.
DINET (US site)
Vanderbilt Autonomic Dysfunction Centre (US Site)
Syncope Trust And Reflex Anoxic Seizures (STARS: UK site)
Dysautonomia International (US site)
Dysautonomia Information Network (DINET: US site)
Dysautonomia Advocacy Foundation (US site)
The Dysautonomia Project (US site)
General Information on Orthostatic Intolerance
You'll find most patients develop a rather warped sense of humour as it's far better to laugh than dwell on the difficulties we face. In that vein, I give you my life motto as sung by Monty Python.