top of page

Managing Ehlers Danlos Syndrome and Hypermobility Spectrum Disorders

What are the Ehlers-Danlos Syndromes (EDS)?

The Ehlers-Danlos Syndromes (EDS) are a group of connective tissue disorders that cause poorly structured or 'weaker' collagen. In EDS there are genetic faults in the way that collagen &/or some collagen-related proteins are made. Collagen is everywhere in our bodies, so symptoms can be very widespread, affecting tissues, organs, bones, ligaments, tendons, lymphatic vessels etc. Depending on the genetic fault, different tissues and organs can be affected, which is why there are several subtypes. 

Subtypes of EDS

There are thirteen subtypes of EDS:

  1. Arthrochalasia EDS (aEDS)

  2. Brittle Cornea Syndrome (BCS)

  3. Cardiac-valvular EDS (cvEDS)

  4. Classical EDS (cEDS)

  5. Classical-like EDS (clEDS)

  6. Dermatosparaxis EDS (dEDS)

  7. Hypermobile EDS (hEDS)

  8. Muscularcontractural EDS (mcEDS)

  9. Myopathic EDS (mEDS)

  10. Peridontal EDS (pEDS)

  11. Kyphoscoliotic EDS (kEDS)

  12. Spondylodysplastic EDS (spEDS)

  13. Vascular EDS (vEDS)

What does the Zebra mean in EDS and HSD?

There are a few meanings: 

1) Medical students are usually taught this:

“When you hear hoofbeats behind you, don’t expect to see a zebra"

  • Essentially meaning to always look for the most common diagnosis 

  • However EDS and HSD ARE the unexpected, rarer diagnoses
    Therefore “Sometimes when you hear hoofbeats, it really is a zebra”

2) From afar all zebras look the same, BUT if you look closer their stripes aren't identical.

  • Just like no two people with EDS/HSD will have exactly the same types, symptoms and experiences 

3) A group of zebras is called a dazzle. 

  • With this supportive community and shared awareness, Together we dazzle! 

download.png

Prevalence of EDS

The overall prevalence of the Ehlers-Danlos syndromes is between 1 in 3,500 to 1 in 5,000 people. 

The hypermobile variant (hEDS) is by far the most common type and often may be missed or misdiagnosed as something else.  

Most Ehlers-Danlos Syndromes are rare (e.g. 1 in 40,000 – 200,000), and some are ultra-rare (e.g. less than 1 in a million people).

Ehlers-Danlos Syndromes are inherited and they can be passed down by either dominant or recessive autosomal patterns.

Symptoms of EDS

Symptoms will vary between each subtype of EDS, but also individuals with the same subtype often can experience very different and dissimilar symptoms. 

 

Whilst not an exhaustive list, here are some typical symptoms:

  • Hyperextensions of several joints

  • Frequent subluxations and dislocations of joints

  • Joint and muscle pain

  • Clicking joints

  • Variable mobility

  • Chronic fatigue

  • Easy bruising

  • Stretchy skin

  • Poor wound healing

  • Digestive issues

  • Issues with internal organs

  • Fragile blood vessels

  • Bladder issues

  • Curvature of the spine

  • Dysautonomia / dizziness upon standing

What are Hypermobility Spectrum Disorders? (HSD)

Hypermobility Spectrum Disorders (HSD) are a group of conditions related to joint hypermobility, which is the capability of joints to move beyond the normal range of motion.

Along the continuum fall these four types:

  1.  Generalised (joint) HSD (G-HSD) 

  2. Peripheral (joint) HSD (P-HSD)

  3.  Localised (joint) HSD (L-HSD)

  4. Historical (joint) HSD (H-HSD)

HSD are diagnosed after other possible conditions have been excluded, such as any of the Ehlers-Danlos syndromes (EDS) including hypermobile EDS (hEDS).

The difference between HSD and hEDS is merely by stricter diagnostic criteria, however both can be equal in severity. They require similar management strategies, validation, and care.

Prognosis of Ehlers-Danlos Syndromes (EDS) and HSD

Diagnosis of Ehlers-Danlos Syndromes are based on the 2017 diagnostic criteria which look at a patient's symptoms and align them to the according major or minor criteria to fit a certain subtype. 

For all EDS types apart from hEDS, testing exists to identify the responsible variant for the gene affected in each subtype. hEDS is therefore diagnosed as a 'clinical diagnosis' based on meeting pre-requisites 
in the criteria. 
Whilst there is no cure for Ehlers-Danlos Syndromes, there are management strategies which can improve quality of life. 

Next steps if you suspect them

Diagnosis of EDS and HSD is typically through a rheumatologist, however other specialists in fields where symptoms overlap may have knowledge and awareness and be able to diagnose or refer 
over to rheumatology.

You may suspect EDS/HSD and need to advocate for a referral from your primary care provider or General Practitioner (GP) for further investigations. You can find this GP toolkit here.
 

For many more resources and support: 
The Ehlers-Danlos Society

 

Ehlers-Danlos Support UK 

IG POSTS JAN 2022+ (6).png

Misconceptions about Ehlers-Danlos Syndromes (EDS) DEBUNKED

There are several misconceptions about Ehlers Danlos Syndromes and Hypermobility Spectrum Disorders, so across these image graphics you can learn more and educate yourself further.

How can EDS or HSD affect daily life?

Symptoms can vary and be very unpredictable. EDS and HSD can be disabling and create a huge myriad of issues with multiple triggers. Below you can find examples (whilst not an exhaustive list).

 

Cervical Instability

Triggers:

  • turning neck side-to-side 

  • bending forwards 

  • jumping

  • going up stairs or up hill

Shoulder, Wrist, Hand & Finger Instability

Triggers:

  • lifting anything  

  • opening heavy doors 

  • pressing buttons / typing

  • side sleeping

Pelvic Instability

Triggers:

  • sitting down 

  • twisting

  • crossing legs

  • any slight movement

Digestive Issues

Triggers:

  • many different foods 

  • co-morbidities include food allergies or sensitivities, MCAS, IBS and gastroparesis

Chronic Fatigue

Triggers:

  • any physical activity

  • specific foods

Dysautonomia (autonomic dysfunction)

Triggers:

  • standing up from sitting

  • changes in temperature

  • eating

  • exercise

Managing Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD)

Using several strategies together or ‘creating a flare up toolkit’

  • This takes trial and error and may be developed and added to over time

  • Each person will respond differently to any strategy or therapy so this combination will be unique 
         to each person

  • A guide to create your own is on this page

Physiotherapy and Exercise  

  • This is to enhance your positional sensing (proprioception) 

    • Physio, shoe inserts/orthotics, compressive clothing

  • Focusing on strengthening of muscles around the joints
    •  Gentle daily physiotherapy, repetitive strengthening movements, regular movement, low impact exercise e.g. walking / swimming, hydrotherapy, balneotherapy etc

  • Physiotherapy is a often a crucial management strategy for EDS and HSD.

    • Whilst lack of strength can increase the prevalence of subluxations/dislocations, de-conditioning can also cause further issues

  • You should find a specialist physio who’s experienced in managing EDS. They’ll evaluate your posture, range of movement, strength, gait and mobility, stability and proprioception - this will be unique for each person. Based on this they’ll recommend suitable exercises for you including helping to gain core strength, improving proprioception and strengthen those muscles around the joints. This is often low impact exercise like swimming or hydrotherapy, and gentle walking. They may suggest basic strengthening movements that can even be done lying down. Regular gentle movement may also be recommended which can include simply getting up to do a certain activity every so often and you may set timers or reminders to avoid you staying in that one position for too long. If stretching is advised, it should be very gentle with a good awareness of joint range of motion to avoid joint injury.

  • Remember everyone is very different in what helps them so your exercise should be always tailored by a qualified individual who's specialised in your condition.

Specific Medications or Devices

  • Oral medications for pain (with awareness of long term effects and use in conjunction with other strategies)

  • Acute medications and preventative medications for specific symptoms or co-morbidities
    crossing legs

  • Topical pain relief creams, balms, gels or patches

  • Heat or cold therapy

  • Mobility aids

  • Specific therapies or devices for certain symptoms

Specific joint supports, splints, tape or braces

  • Used as advised by a medical professional as prolonged use can weaken muscles and possibly further exacerbate symptoms

Use of cushions, pillows, supportive sofas and mattresses

  • Sofas that offer comfort and support for problem joints; chaise sofas, electric recliners, adaptable backrest sofas, ergonomic office chairs or makeshift support options

  • Coccyx cushions, lumbar supports, adjustable pillows wedge cushions, bolsters, U or V pillows, full body pillows, knee pillow, bean bag pillows etc.

    • A range of these can help to both prevent and alleviate pain and symptoms any physical activity


Nutrition Changes and Supplements 

  • These will be unique to each person and their co-morbidities 

  • May include avoiding trigger foods 

    • keep a food diary to establish these

  • Use of medications for alleviating symptoms with gastro flare ups or allergens

  • Vitamins or supplements that target mineral deficiencies or inflammation etc.

Having accommodations around the home to pre-empt a symptom 

  • Support and help from others

  • Specific gadgets or other useful tools or aids

  • Changing locations of items, adapting rooms and layouts or even moving homes

    • Support or evaluation from an occupational therapist could help with these implementations

Pacing of necessary weekly tasks or activities

  • Routine changes: prioritising only essential weekly tasks, spreading those tasks across the week into bitesize chunks

  • This may include workplace adjustments like working from home, flexible working, less hours working or stopping working to manage health

  • Balancing regular rest breaks with regular gentle movement wherever possible

    • A guide to implement pacing in more detail can be found on this page

 

Looking after Mental Health

  • Implementing mental health strategies that look at the cognitive perception of pain;

    • Cognitive Behavioural Therapy  (CBT) is frequently recommended by practitioners for pain management

    • Distraction techniques

    • Regulating the Autonomic Nervous System; Breathwork, Meditation, Mindfulness, Sophrology, EFT, other alternative therapies or strategies

  • Other types of therapy to manage co-morbidities such as anxiety and depression etc.

Support Networks in Place

  • Ensuring your friends and family are aware of how to best support you 

  • Connecting with other groups of people with similar conditions/experiences

  • Access to medical care teams for any changes in symptoms or co-morbidities

 

Learning to Advocate 

  • Preparing for medical appointments and tracking symptoms over time. You can find a guide here to help with preparation including a free template.

  • Educating others about your conditions - you may like to share this page with them for a general understanding of living with chronic illness

  • Knowing when to seek further medical care

  • Confidence to ask for help from others (especially in public) when needed [use of cards, badges or bracelets where required] - you can find templates to create your own here

Positivelychronictravels NEW Branding.png
bottom of page