I’ve long suffered from extreme temperature swings, always feeling too hot or too cold, whipping back and forth between them unless I remained very still in a constant temperature.
Searching for a hint of what causes this and suspecting it’s related to EDS, I searched for “EDS temperature sensitive unstable” and came across this article discussing several other symptoms too, like incontinence and digestive problems, which I thought were separate issues.
What ties them all together is that they are symptoms of autonomic dysregulation, a problem I didn’t think I had. But after reading this article, I can see how this additional issue contributes to my general physical instability.
The autonomic nervous system controls important functions like heart rate and blood pressure.
Typical signs and symptoms include
- fast heart rate,
- low blood pressure,
- digestive system problems,
- disturbed bladder function and
- sweating regulation
Autonomic dysfunction that impacts the heart and blood vessels (cardiovascular) may cause symptoms when you stand up, like lightheadedness and fainting.
The occurrence of these conditions aren’t well understood, but specialists suggest they are frequently seen in hEDS.
Many symptoms of autonomic dysfunction have been observed in hEDS, including heart and blood vessel (cardiovascular) issues, pupil, bladder, sweating dysfunction, and digestive system problems.
The causes of cardiovascular problems in hEDS are unclear.
Suggested mechanisms include:
- low blood pressure,
- increased blood pooling e.g. in the legs on standing or around the gut after eating,
- low circulating blood volume,
- medications with side effects that trigger or make problems worse,
- auto-immunity (immune responses to the patient’s own body) to things that regulate heart rate and blood pressure as well as other autonomic functions,
- excessive amounts of chemicals related to allergy (histamine), and
- rarely, brainstem or spinal cord restriction.
Research has found an excessive ability to stretch in major blood vessels in those with joint hypermobility syndrome. A link between problems with standing in hEDS could be due to abnormal connective tissue in blood vessels with veins distending excessively in response to ordinary pressures when standing, although this has not been confirmed by research.
This, in turn, could lead to increased pooling of blood in the lower half of the body, causing lightheadedness and sometimes fainting when standing up.
Certain heart valve problems can produce signs similar to autonomic dysfunction in hEDS. Heart valve conditions are not common in patients with hEDS, and in most cases is not harmful. Inactivity (deconditioning) and poor fitness are common findings in patients unwell with hEDS for a long time.
Symptoms when standing have been related to deconditioning. However, as to which is the cause and which is the consequence remains open to debate. Increased physical fitness may improve cardiovascular autonomic problems.
The extent to which physical deconditioning triggers problems, and the role of physical reconditioning in managing symptoms, warrants further research.
The more common symptoms of cardiovascular autonomic dysfunction are:
- fast heart rate (tachycardia),
- a temporary greying out of vision,
- limited concentration and poor memory (often described by patients as “brain fog”) with mental performance problems including word finding difficulties,
- chest pain,
- long-term tiredness,
- exercise intolerance and feeling worse after exercise,
- swelling and/or discoloration in the legs after standing for short periods of time,
- cold, dusky hands and feet,
- temperature dysregulation,
- sleep disturbance, and
- a sense of being about to blackout or actually fainting.
Formal evaluation of cardiovascular autonomic dysfunction should be made with a tilt table test usually done by an autonomic neurologist or electrophysiologist, but if this if not available, a simple clinic room ten-minute standing test can help assess whether a brief period of standing can provoke symptoms upon standing.
Several treatments used together are often needed. There is not yet evidence for specific treatments for hEDS subgroups.
- education on avoiding or reducing exposure to triggering factors,
- withdrawing medications that might worsen symptoms,
- maintaining good water intake and salt balance,
- raising legs when resting,
- compression garments,
- increasing exercise (adapted to hEDS needs).
When prescribing exercise, the program might be adapted:
- aerobic activities with a local resistive component,
- dynamic exercise (involving joint movement),
- exercising lying down,
- exercising in water (depending on temperature).
Training at a target heart rate of 75% of the estimated maximum heart rate (explain how to calculate this) for about 30 minutes per session, two to three times per week is advised, adapted according to level of disability.
Increase of fluid intake, with added sodium, and the use of medical compression stockings during and after exercise can be helpful
Meals should be avoided one hour prior to an exercise session. To prevent a sudden drop in blood pressure after training, a person should cool down with more gentle movements.
In those with more significant impairment of daily function, and poor response non-drug treatments, medication that may help include:
- beta blockers,
- hormonal contraceptives,
- dihydroxyphenylserine, and
This article is adapted from: Hakim A, O’Callaghan C, De Wandele I, Stiles L, Pocinki A, Rowe P. 2017. Cardiovascular autonomic dysfunction in Ehlers-Danlos syndrome—Hypermobile type. Am J Med Genet Part C Semin Med Genet 175C:168–174.
And here’s an article about the crazy temperature swings:
It’s worth checking it out to read all the patient comments.
Just when I thought no one could possibly have the weird combination of symptoms that I do, I see my strangeness echoed by so many others.