Amy's Journey with...

Hypermobile Ehlers-Danlos syndrome (hEDS) ~ Postural Orthostatic Tachycardia Syndrome (POTS) ~ Focal Impaired Awareness (Complex Partial) Seizures ~ Fibromyalgia ~ Chronic Myofascial Pain (CMP) ~ Polycystic Ovarian Syndrome (PCOS) ~ TMJ Dysfunction ~ Bipolar Disorder Type I ~ Migraines ~ Obsessive Compulsive Disorder ~ Keratosis Pilaris (KP) ~ Complex-Post-Traumatic Stress Disorder (C-PTSD) ~ Panic Disorder ~ Generalized Anxiety Disorder (GAD) ~ Social Anxiety Disorder (SAD) ~ Non-suicidal Self-Injury (NSSI or SI) ~ Painful Piezogenic Pedal Papules ~ Hashimoto's Thyroiditis ~ Irritable Bowel Syndrome (IBS) ~ Seasonal Affective Disorder (SAD) ~ Multiple Phobias ~ Chronic Headaches

POTS & Mitral Valve Prolapse

Ehlers-Danlos & Dysautonomia (including POTS)


At this point I do not have a dysautonomia diagnosis, but since so many EDSers do have that diagnosis I've decided to do a page on it.  The best source I've been able to find on dysautonomia has been The Dysautonomia Information Network.  According to them:
Dysautonomia literally means dysregulation of the autonomic nervous system. The autonomic nervous system is the master regulator of organ function throughout the body. It is involved in the control of heart rate, blood pressure, temperature, respiration, digestion and other vital functions. Dysregulation of the autonomic nervous system can produce the apparent malfunction of the organs it regulates. For this reason, dysautonomia patients often present with numerous, seemingly unrelated maladies.
Though there are many different types of Dysautonomia, the type most often associated with EDS is Postural Orthostatic Tachycardia Syndrome or POTS.  Postural orthostatic refers to changes in position.  A person with POTS has problems when their body changes positions, especially upon standing.  Tachacardia means an abnormally large increase in heart rate.  People who have POTS always feel fatigued.  I read once that just standing in place for someone with POTS is like running in place for someone with POTS; it's that exhausting.

What are the symptoms of POTS anyway?  The symptoms can vary widely, but most often are:


The hallmark symptom of POTS is an increase in heart rate from the supine to upright position of more than 30 beats per minute or to a heart rate greater than 120 beats per minute within 12 minutes of head-up tilt.


This tachycardic response is sometimes accompanied by a decrease in blood pressure and a wide variety of symptoms associated with hypotension. Low blood pressure of any kind may promote the following:[3]
  • lightheadedness, sometimes called pre-syncope (pre-fainting) dizziness (but not vertigo, which is also called dizziness)[4]
  • exercise intolerance
  • extreme fatigue
  • syncope (fainting)
  • Excessive thirst (polydipsia) '


Chronic or acute hypoperfusion of tissues and organs in the upper parts of the body are thought to cause the following symptoms:
  • cold extremities
  • chest pain and discomfort
  • disorientation
  • tinnitus
  • dyspnea
  • headache
  • muscle weakness
  • tremulousness
  • visual disturbances
Autonomic dysfunction is thought to cause additional gastrointestinal symptoms:
  • abdominal pain or discomfort
  • bloating
  • constipation
  • diarrhea
  • nausea
  • vomiting
Cerebral hypoperfusion, when present, can cause cognitive and emotive difficulties. Symptoms that persist in the supine (recumbent) state are difficult to attribute to "cerebral hypoperfusion"
  • brain fog
  • burnout
  • decreased mental stamina
  • depression
  • difficulty finding the right word
  • impaired concentration
  • sleep disorders
Inappropriate levels of epinephrine and norepinephrine lead to anxiety-like symptoms:
  • chills
  • feelings of fear
  • flushing
  • overheating
  • nervousness
  • over-stimulation
This is what DINET has to say about the increased risk that people with Ehlers-Danlos Syndrome have for developing POTS:
Ehlers-Danlos Syndrome (EDS), a connective tissue disorder, is found in some POTS patients. Physicians propose that these syndromes occur together due to abnormal connective tissue in dependent blood vessels in those with EDS, which permits veins to distend excessively in response to ordinary hydrostatic pressures (Rowe, Barron, Calkins, Maumenee, Tong & Geraghty, 1999). Simply put, this connective tissue abnormality allows excessive amounts of blood to pool in these patients' lower limbs when they stand up.   
There are a variety of types of Ehlers-Danlos syndrome. Classical and type III EDS were originally reported in orthostatic intolerance patients (Rowe et al., 1999). Many POTS patients with EDS have type III (Grubb, 2002).
The classical form of Ehlers-Danlos syndrome (types I and II) is characterized by soft,hyperextensible skin; easy bruising; poor wound healing; thin, atrophic scars; hypermobile joints; varicose veins and prematurity of affected newborns (Wenstrup & Hoechstetter, 2001). Mutations in type V collagen are a major cause of the classical type of Ehlers-Danlos syndrome.
Clinical features associated with EDS III include soft skin and large and small joint hypermobility (Wenstrup & Hoechstetter, 2001). Skin may be stretchy but scarring tends to be normal. POTS patients with EDS III are often hypermoblie/hyperflexable, double jointed, pale, female and tend to have blond hair and blue eyes (Grubb, 2002). The genetic basis for EDS III is unknown.
A wide variety of medical complications may occur with the classical and hypermobile types of EDS. Mitral valve prolapse can occur in all types of EDS and delayed gastric emptying has been observed in type III (personal observations, Wenstrup & Hoechstetter, 2001). A significant number of individuals with both the classical form and the hypermobile form have dilation and/or rupture of the ascending aorta (Wenstrup & Hoechstetter, 2001). Hiatal hernia has been widely reported in adults with EDS (Steinman, Royce & Superti-Furga, 1993). As previously stated, premature rupture of the membranes in pregnancy (primarily classical type) and poor wound healing (particularly with severe classical type) may occur. Other complications include a mild to moderate increase in peripartum bleeding, joint dislocations, chronic pain (most common in the hypermobile type), surgical complications and intraoperative problems (more common in the classical than hypermobile type), diverticulitis, problems associated with fragile skin (particularly with the classical type) and motor delay (Wenstrup & Hoechstetter, 2001).
One study on patients with "joint hypermobility syndrome", a disorder similar if not identical to EDS III, showed that 78% had signs of dysautonomia, such as orthostatic hypotension, postural orthostatic tachycardia syndrome and uncategorized orthostatic intolerance (Gazit, Nahir, Grahame, & Jacob, 2003). These patients also had evidence of a-adrenergic and B-adrenergic hyperresponsiveness. 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".
Another study of one hundred and seventy women with joint hypermobility syndrome concluded that non-musculoskeletal symptoms are common in patients with joint hypermobility syndrome, and that individuals with these symptoms may express more fatigue, anxiety, migraine, flushing, night sweats, and poor sleep than their peers (Hakim & Grahame, 2004).
Remember my page about Chiari?  People with Chiari also have an increased risk for POTS.
Chiari malformation is a condition in which the cerebellar tonsils protrude down into the spinal cord. This can restrict the flow of cerebral spinal fluid. The symptoms of Chiari are similar to those of POTS. A number of Chiari patients have reported being diagnosed with POTS. Some of these patients proclaim a decrease or resolution in POTS symptoms after undergoing surgical correction of their Chiari malformation. Other POTS patients with Chiari malformation have not experienced any benefits from corrective surgery. Some physicians are convinced that Chiari malformation is a cause of POTS, others doubt the relationship.
Physicians from the NIH and The Chiari Institute believe their is a connection between Ehlers-Danlos syndrome, POTS and Chiari 1 malformation.
Dysautomonia  has a specific test that is usually used, called the Tilt Table Test (TTT).  I couldn't finished the TTT because I was in too much pain.  The TTT sounds weird and it kind of is.  You lay down on a table which you are strapped to and you get an IV.  They put a bunch of electrodes on you to measure heart rate, they test your oxygen levels, and they have a blood pressure cuff on each arm.  Then as the "table" tilts upwards until you are standing and they watch to see if your heart rate increases too fast or if you faint.  If you faint you won't fall because you are strapped to the table.  The IV is there as a precaution, because though it is very rare, some people have had their heart stop during the procedure.  The test can take quite awhile, but I don't know how long as mine had to be stopped.  I can't stand for very long.  The day I had the TTT done was a good pain day until I stood for 10 and was crying in pain.  I suppose in 10 minutes they would have caught any changes that were to happen.


The Tilt Table Test


Ehlers-Danlos & Mitral Valve Prolapse


***this section is in progress***


Mitral Valve Prolapse (MVP) is also common with people who have EDS.  
Mitral valve prolapse (MVP) occurs when the valve between your heart's left upper chamber (left atrium) and the left lower chamber (left ventricle) doesn't close properly. When the left ventricle contracts, the valve's flaps bulge (prolapse) upward or back into the atrium. Mitral (MIE-truhl) valve prolapse sometimes leads to blood leaking backward into the left atrium, a condition called mitral valve regurgitation.
In most people, mitral valve prolapse isn't life-threatening and doesn't require treatment or changes in lifestyle. Some people with mitral valve prolapse, however, require treatment.

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