Ehlers-Danlos Syndromes are complex genetic disorders that affect connective tissue. Nutrition can play a part in managing its symptoms, as part of a multidisciplinary approach. By Hatty Willmoth.

Nutrition often plays a central part of the health management of people with chronic conditions.

But it can be a complicated task, often requiring a level of specialist expertise that may be hard to find, especially in the case of less common disorders.

Helen Spriggs, who specialises in nutrition for people with Ehlers-Danlos Syndromes (EDS), is all too familiar with the unique challenges it can bring.

What is Ehlers-Danlos?

EDS is a group of 13 inheritable connective tissue disorders, classed as ‘rare’ by the NHS.

Those with the conditions tend to have joint hypermobility (joints with an abnormally wide range of movement) and skin that’s stretchy, fragile, and easily bruised — but there are many other symptoms associated with each individual disorder.

The most common EDS subtype is hypermobile EDS (hEDS). People with hEDS tend to have loose and unstable joints that dislocate easily, digestive problems, fatigue, and dizziness and increased heart rate when standing up.

Spriggs says that hEDS is usually diagnosed through an assessment against various criteria by a rheumatologist; the other 12 types tend to be diagnosed through medical genetics (either family history or gene testing).

But hEDS is just one subtype of many. People with vascular EDS (vEDS), for example — a particularly uncommon syndrome — experience problems with their blood vessels and internal organs.

They may have thin skin with visible small blood vessels. Their blood vessels may bulge or tear, which can lead to internal bleeding. They may also have unusual facial features, hypermobile fingers and toes, and varicose veins.

“Like many conditions, there is a spectrum of symptom severity in EDS and its subtypes,” says Spriggs.

“Some subtypes of EDS can be more severe or life threatening, such as vEDS. However, less severe types like hEDS may be hugely impactful on quality of life.”

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If you're a practitioner wanting to learn more about advising clients with EDS, consider taking our CPD course on Hypermobility & The Ehlers-Danlos Syndromes, delivered by Helen Spriggs.

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Connective tissue in Ehlers-Danlos

The main characteristic of EDS, bringing these disparate symptoms under one umbrella, is the disruption of collagen and other connective tissue.

Spriggs says: “Connective tissues are found throughout the body, and collagen — the glue that holds the connective tissue together — is a key component of them.

“Collagen is the predominant protein that is impacted in EDS, yet it is not a lack of collagen in the body but a variation in the production ‘recipe’ that results in reduced collagen integrity.”

That means that cells connecting, binding, separating and supporting other tissues, in people with EDS, function abnormally and/or have an abnormal structure.

Ehlers-Danlos is not just hypermobility

Despite the many possible manifestations of EDS, it is often just thought of as hypermobility. But Spriggs is keen to emphasise that EDS and hypermobility are not one and the same.

She says that, even if an individual has “hypermobile joints, in one or several areas of the body”, this does not mean they have EDS.

And, she says, “individuals can fulfil hEDS criteria but score quite low on the Beighton Scale – a commonly used screening tool for hypermobility”, meaning some people with EDS are not even particularly hypermobile.

Zebras are the official symbol of the Ehlers-Danlos Syndromes. Confused? Find out more.

Complexities and comorbidities in Ehlers-Danlos

People with EDS often have symptoms and co-existing conditions.

Spriggs says: “The wider health implications make EDS a complex, multi system-involving condition.

“Several health conditions or manifestations are also recognised as existing alongside the musculoskeletal involvement [bones, muscles and connective tissues], and may include gastrointestinal [digestive system], cardiovascular [heart and blood] or neurological [brain and nerves], amongst others.”

Researchers are currently investigating the potential association of EDS with a host of other conditions, she says, including Postural Orthostatic Tachycardia Syndrome (POTs), mast cell disorders, ADHD and autism.

Managing Ehlers-Danlos: a multidisciplinary approach

There is no cure for EDS — they are genetic conditions — but management can make a big difference to quality of life.

Spriggs explains that this is often a team effort between professionals in all manner of fields, including nutrition.

Spriggs says that care differs for each individual, but often includes “a form of physical and/ or exercise therapy”, psychological support and pain management strategies, as a foundation.

Beyond this, people with EDS may benefit from the help of a range of specialists, such as those from gastroenterology, neurology, occupational health, podiatry, dentistry and nutrition.

“Multi-disciplinary teams or any healthcare professional involved in supporting EDS patients must understand the implications of EDS,” Spriggs says.

“This is for the patients’ outcomes and safety, and may be achieved by adapting care approaches or health goals.”

Nutrition for Ehlers-Danlos

Spriggs says nutrition can play a major role in supporting people with EDS.

“Nutrition is key for health,” she says. “Therefore, it is an important factor for an individual managing EDS or any other health condition.

“An aspect to highlight includes assessing nutrient status, as impacted digestion and absorption is often seen in EDS.

“However, fundamentally, nutrient intake can also be limited, as some individuals find themselves experiencing food reactions, leading to an ever-decreasing number of ‘safe’ foods and restricted dietary nutrient intake.

“Working with an experienced specialist clinician in EDS and the associated conditions, to understand if there are true allergies or intolerances, or if there are other factors at play, is one step to a clearer understanding and a path to increasing dietary and nutrient variety once again.

“Similarly, for some EDS patients, mobility limitations through pain and fatigue or anxiety may impact their access to sunlight, reducing the production of vitamin D.”

Spriggs also explains that other co-occurring conditions may add further challenges.

An individualised approach to Ehlers-Danlos

When tackling EDS — from a nutritional perspective, or otherwise — the personalisation of care is central to its effectiveness, Spriggs says.

This is because EDS is so variable and complex, and because every person is unique.

“There is no standard EDS ‘diet plan’, nor is there a standard EDS supplement protocol as a magic ‘fix all’,” she says.

“As individuals, with complex health concerns, and other factors such as geographic location, lifestyle, and stress levels, our nutrient requirements are likely to be very different.

“Medical history, current medical diagnoses, symptoms and medications, lifestyle, dietary intake, cultural and socioeconomic factors must first be factored in, alongside the context of an EDS diagnosis, before restrictive diets and large supplement ‘stacks’ are recommended, if at all.”

A lack of research, she says, can hold back the quality of interventions, but the situation is improving.

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To learn more about EDS, see the Ehlers-Danlos Society.

Access support via Ehlers-Danlos Support.

Contact Helen Spriggs here.

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