NS Disease 3 Dysautonomia

Dysautonomia

What is Dysautonomia?

Dysautonomia is an umbrella term used to describe several different medical conditions that cause a malfunction of the Autonomic Nervous System. 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. People living with various forms of dysautonomia have trouble regulating these systems, which can result in lightheadedness, fainting, unstable blood pressure, abnormal heart rates, malnutrition, and in severe cases, death.

Dysautonomia is not rare. Over 70 million people worldwide live with various forms of dysautonomia. People of any age, gender or race can be impacted. There is no cure for any form of dysautonomia at this time, but Dysautonomia International is funding research to develop better treatments, and hopefully someday a cure for each form of dysautonomia. Despite the high prevalence of dysautonomia, most patients take years to get diagnosed due to a lack of awareness amongst the public and within the medical profession. 

What are the symptoms of Dysautonomia?

The symptoms of dysautonomia conditions are usually “invisible” to the untrained eye. To the casual observer, the dysautonomia patient can appear to be healthy. The manifestations of the conditions are occurring internally, and although the symptoms are quantifiable and verifiable medically they are not visible on the outside (people cannot see fast heart rates, blood pressure changes, dizziness, migraines, stomach pain etc.). Symptoms of dysautonomia can be unpredictable, may come and go, appear in any combination, and usually vary in severity (wax and wane). Some symptoms seem to occur in intense phases. Patients often become more symptomatic after a stressor or physical activity (which can lead to chronic avoidance of physical activity contributing to additional decline). Symptoms can occasionally be severe enough that patients may require consideration for placement in full or part time home bound teaching programs for health impaired students; others will be able to attend school, often with modifications in their educational plans. The social isolation experienced by not being able to attend school or community activities is one of the hardest things for these children to deal with.  It is essential that school systems make every possible effort to help these students remain in school and to keep home bound students connected to their peers. Since patients afflicted with dysautonomia are usually normal in appearance, it can be a hard condition for laypeople to understand. Even the general physician sometimes misses the clues leading to a proper diagnosis. The symptoms are often difficult for the very young patient to verbalize and the conditions are not always promptly recognized. Traditionally, cardiologists and cardiac electrophysiologists can efficiently diagnose and treat dysautonomia patients. Sometimes neurologists and other specialists get involved as well. Families often find themselves desperately traveling great distances to the few pediatric dysautonomia specialists throughout the country for a proper diagnosis and innovative treatment.

Different types of Dysautonomia

Baroreflex Failure 
The baroreceptor reflex, or baroreflex, is one of the body's homeostatic mechanisms for maintaining blood pressure. If the baroreceptor itself or part of its messaging system fails, this is referred to as Baroreflex Failure. Patients often deal with normal or low resting blood pressure and very high or volatile blood pressure during periods of stress. 

Cerebral Salt Wasting Syndrome 

Cerebral salt-wasting syndrome (CSWS) is a rare condition featuring hyponatremia and dehydration in response to a physical injury or the presence of tumors in or surrounding the brain. The hyponatraemia is due to excessive sodium excretion from the kidney resulting from a centrally mediated process. 

Diabetic Autonomic Neuropathy 
Diabetic Autonomic Neuropathy is a secondary form of autonomic dysfunction, but it is likely the most common form of autonomic dysfunction in the world. An estimated 20% of all diabetics suffer from Diabetic Autonomic Neuropathy, which equates to approximately 69 million people worldwide. Diabetic Autonomic Neuropathy is a serious complication of diabetes. It is associated with an increased risk of cardiovascular mortality. 

When you should see a doctor

• Blood pressure fluctuations
• Difficulty with breathing or swallowing
• Shortness of breath with activity or exercise
• Distension of the abdomen

Why do people contract dysautonomia?

Dysautonomia may be due to inherited or degenerative neurologic diseases (primary dysautonomia) or it may occur due to injury of the autonomic nervous system from an acquired disorder (secondary dysautonomia). Side effects of drugs can cause abnormalities in the function of the autonomic nervous system, producing an iatrogenic form of dysautonomia.