POTS is prevalent enough in the EDS community to warrant two separate talks.
Autonomic Dysfunction and EDS, by Dr. Satish Raj
**Note: POTS (postural orthostatic tachycardia syndrome) is a form of autonomic dysfunction or dysautonomia. It involves the hyperextensibility of blood vessels due to EDS, swelling, the heart/lungs, and the autonomic nervous system, so it can be found by any number of specialists. (Of course, most of us spend years struggling and end up in the hospital at least once before diagnosing ourselves on google, but in theory...)
My notes from his talk:
EDS can have surprising complications -- who is resistant to local anesthetics? (many people!)
dysautonomia isn't a diagnosis -- it's a cluster of diagnoses (POTS, vasovagal syncope, autonomic failure, congenital diseases...)
Symptoms of POTS -- rapid heartbeat, chest discomfort, shortness of breath, lightheadedness, brain fog, headaches, nausea, tremors (can have exercise intolerance, sleep problems, and fatigue)
orthostatic hypotension -- significant drop in BP when standing
POTS -- HR overcompensating for BP drop (increase of 30+bpm after standing)
Most people with POTS don't actually pass out during a tilt-table test. They feel terrible and keep saying "I'm going to pass out" but then never do -- it's extreme pre-syncope, but only sometimes full syncope
**good graphs showing high HR and erratic BP in POTS patients, as well as increased symptoms
Demographics/school experience:
~90% of POTS patients are female, as well as white
Can develop anytime in life, but usually around puberty
Interferes with school -- 89% of POTS kids missed school due to symptoms, 25% dropped out, 28% home schooled
**Even when present, makes it difficult to focus/learn
38% have to delay enrollment or completion of college
Often mistaken as psychiatric
Usually brushed off as extreme anxiety
Associated with higher rates of inattention -- memory problems, similar to ADHD
Not a higher rate of depression, anxiety, or panic disorders than general population, but often misdiagnosed (unable to get out of bed etc --> depression; high HR and difficulty breathing --> anxiety/panic)
Other research:
Similar quality of life to patients on dialysis
Many potential pathophysiologies and causes
“POTS isn’t a thing, it’s a collection of things”, can be different reasons and iterations
25% POTS patients self-describe as having EDS (do they just not know, or really don't have EDS?)
In Israeli study of Joint Hypermobility Syndrome, 78% had POTS symptoms
hEDS has more POTS symptoms than cEDS
Why does this connection exist?
No one knows why, but some theories...
Alpha- and beta-receptor sensitivities?
Laxity of blood vessels (vascular compliance and blood pooling)
Physical inactivity due to joint issues (deconditioning, fear of pain on standing)
Peripheral/small-fiber neuropathy
Other
Managing Life With Autonomic Symptoms
Dr. Rowe works at Johns Hopkins. I have a friend who has been seeing him since childhood. He has a lot of experience with EDS, POTS, and ME/CFS. I was impressed with not only his knowledge but his clear compassion and love for his patients.
My notes:
Normal response to standing (non-POTS) -- 10-20 BPM increase and vasoconstriction
Dyspnea means difficulty breathing; diaphoresis means excessive sweating (both symptoms of POTS)
Symptoms due to low blood flow to brain: lightheaded, syncope, low concentration, headache, blurred vision, fatigue, exercise intolerance
Symptoms due to hyperadrenergic response (too much adrenaline): dyspnea, chest discomfort, palpitations, tremulousness, anxiety, diaphoresis, nausea
Questions to ask patients (all of these really hit home for me!)
How do you feel in line/shopping? Standing at reception, in chorus, or at a service? After a hot bath/shower/sauna? After standing more than 5 mins?
Do you study in a reclined position, knees to chest, or feet under you?
Do you fidget or move around when standing?
People with POTS naturally prefer postures (or sitting) that promote higher BP -- a lot of kids retort with "See mom, I told ya!"
Tend to pace rapidly instead of standing (I always wondered why I had to pace while brushing my teeth!)
Neuromediated hypotension (NMH) /vasovagal syncope (doesn’t meet POTS numbers)
Dependent acrocyanosis -- change in color of limbs while standing (capillary refill test)
Possible reasons for POTS:
Not vasoconstricting (not sure why)
10-15% reduction in blood volume (not anemia OR concentrated hemoglobin)
Both cause increased sympatho-adrenal response
(epinephrine or norepinephrine)
Literature describes these as one or the other, but can actually be a spectrum and/or combined
Deconditioning is a contributing factor, but not the sole cause
"For every complex problem, there is a solution that is simple, neat, and wrong." H.L. Mencken
How to manage:
1. Non-pharma measures
wear compression gear
**mid-section spanx can help stop blood from pooling in the belly (or abdominal binder)
cooling garments (Neck wraps, cooling hats, towels, vests, etc **websites on the slide)
Drink at least 2L water (but too much water is nauseating); eat salt or take salt tablets
other salt options: Liquid IV, ceralyte, hydration multiplier, trioral
Avoid sleeping more than 12 hrs (dehydration)
don't just stop getting up! Avoid deconditioning. Exercise!!
**you lose 15% of blood volume in the first week or two of bed rest, but sl exercise stops this
avoid triggers
High carb meals can cause POTS immediately after eating
Raising the head of the bed is anti-diuretic effect to preserve blood volume (makes you pee less!)
--> Postural counter-measures:
Legs crossed, squatting, wearing heels (just a little), knee-chest, lean forward, foot rest, clench fists
***TRUST YOUR INSTINCTS
Driving with one food up or crossed under
The lower the seat, the better
2. Treat other conditions (EDS, Sjogren's, allergies, MCAS, ME/CFS, etc)
3. Medication -- often need to try many before finding the right fit
--> surprisingly Ritalin can treat POTS, so patients misdiagnosed with ADHD aren't too screwed!
(Search for Peter Rowe on YouTube for other webinars)