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Autonomic Dysfunction (Dysautonomia), Postural Orthostatic Tachycardia Syndrome (PoT's) and EDS-H / JHS

Dysautonomia means Disorder of the Autonomic Nervous System.  There are many types of dysautonomia. Here we will discuss the type of dysautonomia that is most often associated with EDS-H / JHS;  Postural Orthostatic Tachycardia syndrome (PoTS).

 

The autonomic nervous system is the body’s involuntary nervous system. This system plays an important role in the body because it deals with the vital systems of the body i.e. heart rate, breathing rate, blood pressure, body temperature, digestion and the function of the bowel and bladder. A person does not have to consciously think about these systems in order for them to work, instead, the autonomic nervous system provides the connection between your brain and your internal organs. For instance, it connects to the liver, the heart, the sweat glands, and even the inner muscles of the eye.


'Besides minor structural heart anomalies, dysautonomia is, by far, the most clinically relevant cardiovascular feature in JHS/EDS-HT...  Postural tachycardia syndrome has been defined as the most specific form of cardiovascular autonomic dysfunction in JHS/EDS-HT.' 

(Quote: Rowe et al)

 

'Dysautonomia is a very frequent, yet usually undiagnosed, problem that we see and treat daily, with good results. In our study of 1226 Joint Hypermobility Syndrome patients, Dysautonomia was seen, in patients younger than 30 years, in 72% of females and 40% of males.  ...Patients present with chronic fatigue, sleepiness, with no energy to attend social events. Usually they are labeled as lazy, depressed or unsociable, because they have no energy to interact with other people.' Frequently they have cold intolerance and low blood pressure (unfortunately, most physicians do not know that low blood pressure is not normal). (Quote:  Jaime F. Bravo, MD Rheumatology and Professor of Medicine).

 

Sometimes Dysautonimia will manifest itself as low blood pressure; often this is unknown to the patient. At times however, instead of low blood pressure, Dysautonomia will manifest itself as tachycardia (this is called Postural Orthostatic Tachycardia).  The body tries to compensate the decrease of oxygen to the brain with tachycardia (rapid pulse).  Othostatic tachycardia may be accompanied by syptoms of cerabral hypoperfusion (reduced blood flow to the brain) and autonomic overactivity (palpitations,tremulousness,shortness of breath, chest discomfort and/or pain).​

 

POSTURAL ORTHOSTATIC TACHYCARDIA SYNDROME (POTS) is the most common form of Autonomic Dysfunction in people with EDS-H / JHS.

 

It is not a new illness, but POTS was only recently clearly described.  There is now increasing recognition and interest in POTS and research is being undertaken to help to understand and treat this condition.

 

Studies show a large percentage of people with EDS-H / JHS also display signs of dysautonomia, such as postural orthostatic tachycardia syndrome and uncategorized orthostatic intolerance. One such study found  this percentage to be as high ast 63-78% (Gazit, Nahir, Grahame, & Jacob, 2003).  The authors of this study note that patients with the joint hypermobility syndrome have apparently intact vagal control of heart rate with disturbed sympathetic function. They further state that "the sympathetic dysregulation associated with joint hypermobility syndrome may have several explanations, such as peripheral neuropathy, blood pooling in the lower limbs, impaired central sympathetic control, or deconditioning due to muscle disuse through pain or fear of pain". The body's autonomic nervous system does not control  their heart rate and blood pressure properly, possibly due to over-stretchy blood vessels caused by the defective collagen found in people with EDS-H / JHS. 

 

When someone stands up, blood tends to drop into their abdominal cavity, their arms and their legs. The normal response would be for blood vessels to narrow and the heart rate to increase a little to maintain blood flow to the head and heart. In EDS-H / JHS, blood vessels may be unable to narrow efficiently and blood tends to pool in the limbs and abdomen. As a result, less blood is returned to the heart and pumped up to the brain. When blood does return to the heart, it ‘races’ to maintain blood pressure; excess adrenaline may be produced and this can result in some of the following symptoms:

 

  • Fatigue

  • Dizziness

  • In some, near-fainting or fainting

  • Palpitations

  • Headache

  • Neck/shoulder pain

  • Nausea

  • Shaking

  • Purple, puffy hands/feet

  • Sweats

  • Visual disturbances

  • Breathlessness

  • Bowel problems

 

 

A more in depth description of some of the characteristics seen in many of those with Dysautonomia is given below:

 

  • Chronic fatigue and somnolence (a strong desire for sleep). More than tiredness, it is a lack of energy, like “running out of batteries”. It is interesting to note that this fatigue, somnolence and lack of energy, often does not happen when the person is working or playing a sport, but after finishing it. Usually Dysautonomia is mistaken as chronic fatigue due to something like anemia or other chronic diseases, hypoglycemia (low sugar), hypothyroidism or depression.

 

  • Dizziness. The patient gets dizzy when standing up suddenly or getting out of bed in a hurry. True syncope (passing out / fainting) is rare, but it is frequent that these patients have pre-syncopal episodes.

 

  • Temperature intolerance. This symptom is usually quite pronounced. Even though these patients usually prefer warmer weather, the heat occasionally causes them to feel suffocated, they describe intolerance to prolonged hot baths or saunas what we call “broken thermostat”.  These patients usually complain of cold hands and feet and that if they have a nap without covering themselves with a blanket, they get a cold.

 

  • Raynauds’s Type Symptoms of the hands (see 'Symptoms of EDS-H / JHS' in the 'Understanding EDS-H / JHS' section of the main menu). This phenomenon happens not only with cold weather, but also with inactivity of the hands  (due to poor circulation). 

POTS is not thought to reduce life expectancy, but for some it can cause significant levels of disability.  In the unlucky few, the level of disablility can be equivalent to conditions like heart failure and chronic obstructive pulmonary disease (COPD).

 

Misdiagnosis:

 

The diagnosis of POTS is often missed. This may be due to a number of problems. The primary problem is that usually patients are sitting down when their pulse and blood pressure are measured and the recordings may therefore be normal, even if they have POTS. Measurements of the sustained heart rate increases that form part of the diagnosis for POTs need to be taken, not only in the sitting position, but also within 10 minutes of standing up or head up tilt (see the 'current diagnostic criteria' listed below.   Many doctors and nurses have not heard of POTS and patients may not realise the significance of their own symptoms. POTS may be mistaken for other conditions with similar symptoms, such as anxiety, panic disorder, chronic fatigue syndrome, vasovagal syncope, other causes of low blood pressure, inappropriate tachycardia syndrome and phaeochromocytoma (adrenaline-producing tumour).

 

Investigation:

 

'Many individuals with EDS, Hypermobility (and Classical) Type report atypical chest pain, palpitations at rest or on exertion, and/or orthostatic intolerance with syncope (fainting) or near syncope [Rowe et al 1999, Gazit et al 2003, Mathias et al 2012]. Holter monitoring usually shows normal sinus rhythm, but sometimes reveals premature atrial complexes or paroxysmal supraventricular tachycardia. Tilt table testing may reveal neurally mediated hypotension (NMH) and/or postural orthostatic tachycardia syndrome (POTS).'  (Quote: Howard P Levy, MD, PhD)

 

Current Diagnostic Criteria:

 

  • Sustained heart rate increase of 30 beats per minute or more within 10 minutes of standing up or head up tilt.  This changes slightly for adolecents, with a heart rate increase of 40 beats required in the absence of orthostatic hypotension. (In a person without POTs you would expect the heart rate to increase by 10 - 20 beats)

  • The patient's standing heart rate is usually 120 beats per minute or more within 10 minutes of standing up or head up tilt.

  • Othostatic tachycardia may be accompanied by syptoms of cerabral hypoperfusion (reduced blood flow to the brain) and autonomic overactivity (palpitations,tremulousness,shortness of breath, chest discomfort and/or pain)

  • Criteria not applicable for low resting heart rate

Diagnosis of POTS  may sometimes be made by a patient undergoing a TILT TABLE TEST.

The patient lies flat on a special bed, which is then tilted upright to around 60 degrees. It may provoke the above symptoms and some patients may briefly faint. During investigations, patients may have other cardiology and neurology tests

 

How is postural orthostatic tachycardia syndrome treated?

 

The main aim of treatment is to maintain blood supply to the heart and brain.

 

  • Consult you doctor about changing any current medication that may worsen symptoms  (e.g. drugs that lower blood pressure). 

  • Strong calf muscles, general fitness and support tights all help to pump blood around the body.

  • Raising the head end of the bed will increase blood volume in the morning.  

  • Dehydration reduces the volume of circulating blood. Heat and alcohol dilate blood vessels. Heavy meals divert blood away from the brain to the gut and can worsen symptoms of POTS.

  • Have a high fluid intake (over two litres per day or until urine is clear-coloured).

  • Some patients are advised to increase dietary salt by 2-4 g per day (only after advice from a doctor - not in hyperadrenergic POTS).

  • Regular exercise - swimming is ideal.

  • Sleep with the head end of the bed elevated (a brick may be used).

  • Wear strong support tights (class 3, waist high).

  • Have small frequent meals, low in refined carbohydrate - ie avoid sugars, white flour.

  • Avoid excessive standing and sitting, heat and alcohol.

  • Do postural manoeuvres to avoid fainting/dizziness - eg lie down and elevate the legs if possible; otherwise, cross the legs and squeeze the thighs together, and tightly clench the buttocks and fists.

  • Drink water rapidly - two glasses of water drunk quickly has been shown to reduce heart rate in POTS.

 

Medication for POTS

 

Patients may be asked to try self-help treatments for 3-6 months. If unsuccessful, medication may be used, although few scientific studies have been performed to evaluate which treatment is best for which patient. Medicines for POTS are mostly unlicensed which means that your GP is not obliged to prescribe them. GPs who agree to prescribe will probably need recommendation from a hospital specialist.

 

If you have EDS-H / JHS and feel you may be suffering from symptoms of Dysautonomia you may wish to visit your GP.  Many doctors have little knowledge of autonomic problems, so it may be useful to take supporting information with you, to help explain your concerns.

 

Referral should be to a consultant with special interest in autonomic problems and an understanding of Ehlers-Danlos Syndrome / Hereditary Defects of the Connective Tissue.

 

  by edhs.info

 

References:  For references and information sources used within this site, please see 'References' page, under 'Resources and Links' on the main menu.

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