Ehlers-Danlos syndrome (EDS) is a connective tissue disorder. It can affect blood vessel function and blood flow regulation. Sometimes, this leads to symptoms of postural orthostatic tachycardia syndrome (POTS).

EDS and POTS are two distinct yet often related chronic conditions.

EDS is a collection of inherited disorders that affect the connective tissues of the skin, bones, blood vessels, and other organs. Symptoms of EDS include joint hypermobility, very stretchy skin, and fragile tissue.

POTS is a distinct condition characterized by an excessive increase in heart rate when a person stands up. This leads to symptoms such as dizziness, lightheadedness, and fainting.

The overlap between these conditions is significant, and many individuals with EDS also experience POTS.

Keep reading to learn about the link between EDS and POTS, as well as the diagnosis and treatment of these conditions.

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EDS is a group of genetic connective tissue disorders that a person can inherit from their parents. It occurs due to differences in the genes responsible for collagen synthesis, processing, or both. Collagen is a protein that makes up connective tissue.

There are several types of EDS. The most common forms are hypermobile EDS (hEDS), which affects between 1 in 10,000 and 1 in 15,000 people, and EDS classical type, which affects 1 per 10,000 to 1 per 20,000 people. Other forms of EDS are rare.

The symptoms of EDS can include:

  • joints that move beyond the typical range of motion (hypermobility)
  • skin that stretches easily (hyperextensibility)
  • tissue fragility
  • chronic pain
  • easy bruising

In contrast, POTS is a condition affecting the autonomic nervous system, leading to an unusual increase in heart rate when a person stands up. The symptoms include:

  • rapid heartbeat upon standing
  • dizziness and lightheadedness
  • fainting
  • fatigue
  • nausea
  • brain fog

Around 80% of people with EDS also have POTS. Conversely, research suggests that around 31% of people with POTS have hEDS.

It is important to note that not everyone with joint hypermobility has EDS. Some individuals may have hypermobility spectrum disorder (HSD). HSD encompasses various conditions related to hypermobility that do not meet the full criteria for EDS.

Some individuals with hypermobile joints do not experience negative symptoms. They might have benign joint hypermobility, which can be advantageous in activities that require a greater range of motion, such as gymnastics or dance. Benign hypermobility does not typically lead to chronic pain or other issues.

There are several reasons why POTS may be so common in individuals with EDS.

EDS may affect blood vessels, leading to issues with blood flow. This, in turn, may contribute to the symptoms of POTS.

Another possibility is that the autonomic nervous system dysfunction in POTS may exacerbate the symptoms of EDS, creating a cycle of symptom exacerbation.

Doctors can find it challenging to diagnose EDS and POTS because these conditions share symptoms with many other disorders.

Additionally, both EDS and POTS have a broad spectrum of symptoms that can vary significantly between individuals.

For EDS, diagnosis often involves a combination of clinical evaluation, family history, and genetic testing. Healthcare professionals assess specific criteria related to joint hypermobility, skin texture, and other connective tissue manifestations.

For POTS, diagnosis typically includes:

  • heart rate monitoring
  • blood tests to rule out other conditions that might cause similar symptoms, such as anemia or thyroid disorders
  • tests to evaluate the autonomic nervous system function to identify abnormalities

Doctors may use a tilt table test to assess a person in more detail. This involves the person lying on an adjustable table that can tilt upright, allowing doctors to measure the effect of positional changes on heart rate and blood pressure.

Doctors diagnose POTS when:

  • symptoms worsen upon standing
  • heart rate increases above 30 beats per minute when moving from lying down to standing or 40 beats per minute in people ages 12 to 19 years
  • there is no significant drop in blood pressure upon standing

Treatment for EDS focuses on managing symptoms and preventing complications. A management plan may include:

  • physical therapy
  • pain management
  • surgical interventions for severe joint issues

POTS treatment often involves lifestyle modifications such as:

  • increased salt and fluid intake
  • compression garments
  • medications to manage heart rate and blood pressure

Both conditions benefit from a personalized treatment plan tailored to the individual’s specific needs.

Living with the complex and overlapping symptoms of EDS and POTS can be challenging. Many individuals require care from a multidisciplinary team, which may include:

  • rheumatologists
  • cardiologists
  • neurologists
  • physical therapists

A comprehensive care plan may address both the physical and psychological aspects of these conditions.

In addition to medications and daily management, people should also attend regular heart screenings to check for structural problems. Echocardiograms can detect these problems early, potentially preventing complications such as heart failure.

Things to avoid

People with EDS may need to avoid potentially harmful activities that could overextend the joints and cause dislocations. These include heavy weightlifting and high impact sports, such as running.

Those with POTS may also need to be mindful of safety when exercising to prevent fainting. Some may find they can only exercise lying down, such as by using a recumbent exercise bike.

With both conditions, though, exercise can still be beneficial. People may need to start very slowly to determine the type and duration of exercise they can tolerate.

EDS, POTS, and pregnancy

Anyone with EDS or POTS who is considering becoming pregnant should talk with an obstetrician trained in high risk pregnancies.

These specialists are well-versed in the unique challenges of EDS during pregnancy, such as increased risks of joint dislocations, heart issues, and complications during labor and delivery.

Below are some answers to frequently asked questions about POTS and EDS.

What is the EDS POTS triad?

The EDS POTS triad refers to the common coexistence of EDS, POTS, and mast cell activation syndrome (MCAS). MCAS involves atypical mast cell activity, which can cause symptoms such as allergic reactions, gastrointestinal issues, and heart problems.

What is the life expectancy of someone with EDS and POTS?

Overall, hypermobile and classic subtypes of EDS do not affect life expectancy, and there have been no deaths attributed directly to POTS.

Many individuals with these conditions can live relatively average life spans with appropriate management and care.

However, these conditions may raise the risk of other complications, which could affect lift expectancy.

Ehlers-Danlos syndrome (EDS) and postural orthostatic tachycardia syndrome (POTS) are distinct conditions that often coexist.

EDS affects connective tissues, leading to symptoms such as joint hypermobility and easily bruised skin. In contrast, POTS impacts the autonomic nervous system, causing dizziness and rapid heart rate upon standing.

The relationship between these conditions can cause diagnostic and treatment challenges. People living with EDS and POTS require a comprehensive management plan and care from a team of specialists in cardiology, neurology, and physical therapy.