Orthostatic intolerance

Orthostatic intolerance (OI) is a subcategory of Dysautonomia, a disorder of the autonomic nervous system. ^[1]

OI can also be defined as "the development of symptoms during upright standing relieved by recumbency," or by sitting back down again.^[2] Over 500,000 Americans have been diagnosed with OI. It affects more women than men (female-to-male ratio is at least 4:1), usually under the age of 35.^[3]

Orthostatic intolerance occurs in humans because standing upright is a fundamental stressor and requires rapid and effective circulatory and neurologic compensations to maintain blood pressure, cerebral blood flow, and consciousness. When a human stands, approximately 750 mL of thoracic blood is abruptly translocated downward. People who suffer from OI lack the basic mechanisms to compensate for this deficit.^[2] Changes in heart rate, blood pressure, and cerebral blood flow that produce OI "may be related to abnormalities in the interplay between blood volume control, the cardiovascular system, the autonomic nervous system and local circulatory mechanisms that regulate these basic physiological functions."^[4]

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Triggers

Symptoms of OI are triggered by the following:

• An upright posture for long periods of time (i.e., standing in line, standing in a shower, or even sitting at a desk.
  
• A warm environment (such as in hot summer weather, a hot crowded room, a hot shower or bath) after exercise.
  
• Emotionally stressful events (seeing blood or gory scenes, being scared or anxious).
  
• Inadequate fluid and salt intake.^[5]

Symptoms

Orthostatic intolerance is divided, roughly based on patient history, in two variants: acute and chronic.

Acute OI

Patients who suffer from acute OI usually manifest the disorder by a temporary loss of consciousness and posture, with rapid recovery (simple faints, or syncope), as well as remaining conscious during their loss of posture. This is different than a syncope caused by cardiac problems because there are known triggers for the fainting spell (standing, heat, emotion) and identifiable prodromal symptoms (nausea, blurred vision, headache). As Dr. Julian M. Stewart, an expert in OI from New York Medical College states, "Many syncopal patients have no intercurrent illness; between faints, they are well."^[2]

Symptoms:^[4]

• Lightheadedness
• Headache
• Fatigue
• Altered vision (blurred vision, "white outs," black outs)
• Weakness
• Hyperpnea or sensation of difficulty breathing or swallowing (see also hyperventilation syndrome
• Tremulousness
• Sweating
• Anxiety
• Heart palpitations, as the heart races to compensate for the falling blood pressure
• Exercise intolerance

A classic manifestation of acute OI is a soldier who faints after standing rigidly at attention for an extended period of time.

Chronic OI

Patients with chronic orthostatic intolerance have symptoms on most or all days. Their symptoms may include most of the symptoms of acute OI, plus:

• Sensitivity to heat
• Nausea
• Neurocognitive deficits, such as attention problems
• Sleep problems
• Pallor
• Other vasomotor symptoms.^[2]

Diagnosis

OI is "notoriously difficult to diagnose."^[6] As a result, many patients have gone undiagnosed or misdiagnosed and either untreated or treated for other disorders. Current tests for OI (tilt-testing, autonomic assessment, and vascular integrity) can also specify and simplify treatment.^[4] (See Dr. Julian Stewart's article, "Orthostatic Intolerance: An Overview" for a more detailed description of OI tests.)

Management and prognosis

Most patients experience in an improvement of their symptoms, but for some, OI can be gravely disabling and can be progressive in nature, particularly if it is caused by an underlying condition which is deteriorating. The ways in which symptoms present themselves vary greatly from patient to patient; as a result, individualized treatment plans are necessary.^[7]

OI is treated both pharmacologically and non-pharmacologically. Treatment does not cure OI; rather, it controls symptoms.

Physicians who specialize in treating OI agree that the single most important treatment is drinking more than two liters (eight cups) of fluids each day. A steady, large supply of water or other fluids reduces most, and for some patients all, of the major symptoms of this condition. Typically, patients fare best when they drink a glass of water no less frequently than every two hours during the day, instead of drinking a large quantity of water at a single point in the day.^[5]

For most severe cases and some milder cases, a combination of medications are used. Individual responses to different medications vary widely, and a drug which dramatically improves one patient's symptoms may make another patient's symptoms much worse. Medications focus on three main issues:^[5]

Medications that increase blood volume:

• Fludrocortisone (Florinef)
  
• Hormonal contraception
  
• Clonidine
  
• Erythropoietin
  

Medications that interfere with the release or action of epinephrine and norepinephrine:

• Beta-blockers (e.g., atenolol, propranolol)
  
• Disopyramide (Norpace)
  
• Angiotensin converting enzyme inhibitors
  

Medications that improve vasoconstriction:

• Stimulants: (e.g., Ritalin or Dexedrine)
  
• Midodrine (Proamatine)
  
• Ephedrine and pseudoephedrine (Sudafed)
  
• Theophylline (low-dose)
  
• Selective serotonin reuptake inhibitors (Prozac, Zoloft, and Paxil)

Behavioral changes that patients with OI can make are:

• avoiding triggers such as prolonged sitting, quiet standing, warm environments, or vasodilating medications;
• using postural maneuvers and pressure garments,
• treating co-existing medical conditions,
• increasing salt and fluid intake, and
• physical therapy and exercise^[5]

Famous patients

A notable sufferer of orthostatic intolerance is Greg Page, formerly of The Wiggles. It is due to this illness that Page chose to leave the group in 2006.^[6]

See also

• Orthostatic hypotension
• Postural orthostatic tachycardia syndrome

References

1. ^ What is dysautonomia?. National Dysautonomia Research Foundation (NDRF). Retrieved on 2007-08-20.
2. ^ ^{a b c d} Julian M. Stewart. Orthostatic Intolerance: An overview. WebMD. Retrieved on 2007-08-20.
3. ^ Vanderbilt autonomic dysfunction center. Vanderbilt Medical Center. Retrieved on 2007-08-20.
4. ^ ^{a b c} Julian M. Stewart. Orthostatic Intolerance. New York Medical College. Retrieved on 2007-08-20.
5. ^ ^{a b c d} Peter C. Rowe. General information brochure on Orthostatic Intolerance and its treatment. The Pediatric Network. Retrieved on 2007-08-21.
6. ^ ^{a b} Greg Page leaves the Wiggles. The Wiggles Home Page. Retrieved on 2007-08-21.
7. ^ National Dysautonomia Research Foundation. National Dysautonomia Research Foundation (NDRF). Retrieved on 2007-08-21.