Friday, June 29, 2012

I Have a Diagnosis of Dysautonomia. Now What?

So you or someone you know has a diagnosis of dysautonomia.  What is "dys-auto-no-mia" anyway?   It is "dys"function (abnormal function) of the autonomic nervous system, or ANS.  What is the ANS?  The ANS is in charge of all the things you don't have to think about to live in a human body.  The ANS "runs" the balance between your heart rate and blood pressure, keeps you from fainting when you stand up, digests your food, tells you when you need to urinate, and performs a variety of other functions (Figure 1).

Fig. 1. The Human Nervous Systems.  The Central and Peripheral Nervous Systems are most commonly known for their effects on brain injury, spinal cord injury, and paralysis.  A completely separate system, the Autonomic Nervous System (ANS) is divided into the Parasympathetic and Sympathetic Nervous Systems.

While the following information may not apply to every single person with dysautonomia, we hope this is helpful. Please ask your doctor for specific guidelines for your case. No one but your doctor can take the place of giving you medical advice. The following are general guidelines that are routinely followed, and are not meant to be prescribed as medical advice for you. Consult with your doctor about which of the following apply to you.

Generally speaking, it is preferred that the medical management of any condition should first seek to optimize nutrition, hydration, sleep, and physical activity which includes rehabilitation and physical therapy. After the patient is thus optimized, drug therapy may then be considered. Drug therapies are discussed at the end of each categorization. For the treatment of hypotension, tachycardia, and anxiety, a new blog title follows.

Treat Underlying Condition.  Dysautonomia frequently exists in parallel with another diagnosis like chronic fatigue syndrome, Crohn's Disease, traumatic brain injury, viral syndrome, diabetes, and joint hypermobility. An extensive list follows this article. If you have one of these diagnoses, your doctor will provide you with instructions on how to manage it.  If you get low blood pressure and high heart rate on standing, your doctor may diagnose you with dysautonomia proper.  If your blood pressure does not change when you stand up, your doctor may diagnose you with Postural Orthostatic Tachycardia Syndrome (POTS).

Hypovolemia and Failure of Normal Compensations: Compression Aids.  A relatively benign intervention is the use of compression stockings and abdominal binders. Both leg and abdominal compression devices squeeze blood to return it to the right atrium of the heart. In this way, filling occurs and brain perfusion is maintained. This is particularly useful for times when a patient will be standing or going up and down, as cerebral perfusion may be optimized with these compression aids.
       Compression stockings for the legs may best be used in the form of "thigh high" stockings that stay up by themselves, perhaps at 30 - 40 mmHg compression (tighter) or less tight, at 20-30 mmHg (it requires a prescription to specify the pressure) strength. Before putting stockings on, it is advisable to first lift the legs into the air for 3-5 min, while laying in the supine position. Abdominal binders may include the traditional white velcroe-strapped abdominal wraps.

Dehydration and Hypovolemia: Avoidance of Heat and Sweating.  Easier said than done, but a special note is needed here. This is one area where the patient needs special consideration and attention to ensure a cool environment is maintained. Since patients require understanding from those around them, support people like significant others, room mates, family and friends need to optimize a cool environment whenever possible. Heat exhaustion affects patients with dysautonomia by causing dehydration. Symptoms the patient may have that are due to heat intolerance include being slurry speech, lightheadedness, fainting or syncope, pre-syncope, and exacerbation of dysautonomia such that upon standing, tachycardia ensues. Treatment is with cool air, fluids, and salt. For extreme cases, an ambulance can be called to take the patient to the emergency room.

Water and Salt Replenishing: High Salt Diet and Increased Hydration.  These are the mainstays of treatment. One goal is to consume 200 mEq/day sodium (Na) salt. Some doctors prescribe Na tablets so that 1 gm is given three times per day, with meals. Drinking 8-10 cups of water/day is needed. Some patients require continuous infusion of fluid for a period of time such that a peripherally-inserted central catheter (PICC) line or Hickman Catheter is placed semi-permanently. For those that need either emergency iv hydration, or are living on an intravenous infusion, both 5% Dextrose Normal Saline (D5NS) and 0.9% Normal Saline (NS) are used. Emergent therapy at 1 L/1 hour NS may be provided to improve tachycardia, hypotension, altered mental status, general malaise, nausea, vomiting, and/or fatigue.

Urinary Tract Infections: Supplemental Cranberry.  Patients with dysautonomia may have a neurogenic bladder whereby the urge to urinate is absent or late because the sensation is gone. Urinary frequency, urinary tract infections, and cystitis may occur, such that prevention of spills is a consideration. Treatment can incorporate bladder training every 2 -3 hours even if no urge to urinate is present, in order to keep the bladder from distending.
     For patients with a neurogenic urinary bladder and frequent urinary tract infections (UTI's), cranberry supplementation may take the form of drinking cranberry juice, ingesting cranberry concentrate, or using cranberry pills (obviates the need for all the calories that are in the drink). Prompt treatment of UTIs must occur to prevent dehydration. Treatment usually includes taking two pill prescriptions: one is an antibiotic and the other is a pain medication.  Pyridium is usually the pain medication of choice; it turns the urine bright yellow-orange and this is normal as it transits the urinary tract to anesthetize it.

Gastroparesis: Small, frequent meals.  Dysautonomia may include symptoms of gastroparesis, or slow emptying of the stomach and/or intestines. Patients may be unable to consume a normal full meal. The first treatment is to avoid large meals and instead eat frequent, small meals. For some patients whose gastric mobility adversely affects nutritional status, the need for a feeding tube may occur. This may occur either through a gastric tube (G-tube) placed in the stomach, of a J-tube placed in the jejunum (small intestine). Drugs that increase gastric motility include metoclopramide and erythromycin.

All these empiric therapies can have a major role in the treatment of dysautonomia. If hydration and salt intake remain ongoing and regular during the day, this helps optimize blood volume and minimize orthostatic hypotension. Avoidance of activities that lead to heat exhaustion and sweating are also important in the treatment of dysautonomia. The use of compression stockings and abdominal binders can not be underestimated as a means to potentially allow a bed-ridden patient to get out of bed. Drug therapy for the treatment of hypotension and/or tachycardia is a separate discussion, to be entered into only after the above-mentioned therapies are optimized. Otherwise, issues like blood volume changes and salt consumption can be too variable, thwarting efforts to institute cardiac or other drug therapy.

See also:

Beta-Blockers: What do They Block Anyway? 
The Anatomy and Physiology of Dysautonomia (Image to the Right)
The History of Dysautonomia: 1869 to 1999
Disorders of the ANS
Research Updates on POTS and MSA
Beta-Blockers and POTS/Dysautonomia and the History/Physical Exam
Dysautonomia and POTS Syndrome and Social Media

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