Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

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all patients

1st line – general recommendations

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all patients
1st line – 

general recommendations

The evidence base underpinning various treatment options is patchy. Many recommendations are based on expert opinion. Therapies are usually tailored to individual needs. Multidisciplinary input may be necessary.

Asymptomatic patients do not require any specific treatment. However, all patients should be advised to avoid contact sports because of the risk of injury to soft tissue and bone. Fitness should be encouraged to minimise risk of injury. Certain occupations are physically demanding and are best avoided such as nursing, professional dancing, and heavy manual labour.

Plus – genetic counselling/education

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all patients
Plus – 

genetic counselling/education

Treatment recommended for ALL patients in selected patient group

All patients (and/or parents) should be provided with detailed information regarding the disorder. Inheritance patterns should be explained and at-risk family members identified. The outcome and natural history of the particular EDS subtype should be discussed. It is not necessary to advise patients with hypermobile EDS (hEDS) to avoid having children, but parents should be encouraged to be alert to childhood and adolescent symptoms. Patients and their families should be provided with information on available support groups and other such resources. Ehlers-Danlos Support Group (UK) Opens in new window Ehlers-Danlos Society Opens in new window

Plus – pain management

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all patients
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with musculoskeletal pain or joint instability
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pain management

Treatment recommended for ALL patients in selected patient group

A standard approach to pain management applies. There have been no randomised controlled trials of pain management in EDS, and clinicians tend to follow guidance from the management of fibromyalgia. The causes of pain in EDS are multifactorial and hence treatment options vary.[36]

Non-steroidal anti-inflammatory drugs (NSAIDs), skeletal muscle relaxants, and opioid analgesics play a role in treating joint, muscle, and/or spine pain.

Other options include drugs used for neuropathic pain, serotonin-noradrenaline (serotonin-norepinephrine) reuptake inhibitors, and tricyclic antidepressants.

Corticosteroid injections and/or local anaesthetic agents may be considered when oral analgesics do not adequately relieve pain (e.g., trigger-point injections, bursa injections, intra-articular injections).[18] Local anaesthetic agents, in a higher than average dose, provide relief despite apparent resistance.

Nerve-root blocks and spinal-cord stimulators may be useful adjuncts in select cases of intractable chronic pain.

Plus – physiotherapy and occupational therapy

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all patients
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with musculoskeletal pain or joint instability
Plus – 

physiotherapy and occupational therapy

Treatment recommended for ALL patients in selected patient group

The principles of therapy include: 1) restoring normal range of motion for that particular patient, even if it is hypermobile, 2) restoring efficient and effective movement patterns throughout the full range of motion, including the hypermobile range (this involves correcting and preventing movement dysfunction and regaining joint stability), 3) educating, reassuring, advising, and problem-solving, 4) improving general fitness to avoid de-conditioning (usually achieved with muscle toning to help stabilise loose joints, and avoiding high-resistance strengthening-type exercises).

Each patient requires a detailed assessment and individualised programme. Techniques may include neural biofeedback, and therapies often involve the principles of Pilates, the Alexander technique, and tai chi.[39] Myofascial release (a form of soft-tissue therapy) may be performed to reduce muscle spasm.

Consider – CBT ± antidepressant and/or anxiolytic

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all patients
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with musculoskeletal pain or joint instability
Consider – 

CBT ± antidepressant and/or anxiolytic

Additional treatment recommended for SOME patients in selected patient group

Chronic and unremitting pain with profound physical de-conditioning may require a multidisciplinary pain management programme involving cognitive behavioural therapy (CBT).

Furthermore, with chronic pain may come reactive depression, anxiety, and/or phobic states requiring short-term antidepressant and/or anxiolytic therapy to support or facilitate other therapeutic interventions.

Combined physiotherapy and cognitive therapy have been shown to be effective in reducing pain in individuals with joint hypermobility.[37]

Consider – splints and/or orthotics

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all patients
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with musculoskeletal pain or joint instability
Consider – 

splints and/or orthotics

Additional treatment recommended for SOME patients in selected patient group

While physiotherapy and occupational therapy encourage strengthening and endurance with a goal of avoiding supports and splints (the best splint is the patient's own musculature), supports may inevitably be required to stabilise non-responding joints or to support one region in order that joints above and below may be strengthened without undue stress (e.g., strapping the knee to focus on re-aligning and stabilising the hip and ankle). In addition, orthotics are valuable in re-aligning the foot and reducing pain.

Plus – joint reduction and immobilisation

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all patients
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with joint dislocation
Plus – 

joint reduction and immobilisation

Treatment recommended for ALL patients in selected patient group

Often involves closed reduction as soon as possible to decrease potential complications, including soft-tissue injury, articular surface injury, and neurovascular compromise. Reduction usually requires sedation and analgesia.

A period of immobilisation should be followed by active motion exercises and isometric strengthening exercises.

Consider – specialist referral

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all patients
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with autonomic dysfunction and/or weakness of supporting structures
Consider – 

specialist referral

Additional treatment recommended for SOME patients in selected patient group

For patients with autonomic dysfunction and weakness of supporting structures (e.g., uterine or rectal prolapse; myopia; abdominal wall, inguinal, or para-umbilical hernia; mitral valve prolapse) referral to specialist cardiac, autonomic neurology, gastroenterology, ophthalmological, gynaecological, and/or surgical services should be made if there are specific concerns.

Non-pharmacological measures for orthostatic hypotension include increased dietary salt intake, use of compression stockings, graded exercise therapy, and avoidance of meals 1 hour prior to exercise.

Consider – GI medication and/or dietary change

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all patients
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with autonomic dysfunction and/or weakness of supporting structures
Consider – 

GI medication and/or dietary change

Additional treatment recommended for SOME patients in selected patient group

Gastrointestinal (GI) disorders are treated as for non-EDS patients. For example, antacids for gastritis or promotility agents for gastroparesis; fibre, avoiding certain food products, and smooth muscle relaxants for irritable bowel syndrome.

For abnormal gut flora, antibiotic therapy may be considered. Consult local guidance.

For constipation, docusate sodium or glycerin suppositories are an option.

For slow transit, prokinetic agents (such as erythromycin and domperidone) enhance GI motility, especially for those patients with reflux/dysphagia. Due to a review that found domperidone was associated with a small increased risk of potentially life-threatening cardiac effects, the risks and benefits should be weighed carefully before using the drug for this off-label indication.[43] Domperidone should be used at the lowest effective dose for the shortest possible duration and the maximum treatment duration should not usually exceed 1 week. A probiotic (such as VSL3) may also be considered for slow-transit issues.

A low-FODMAP diet may be considered for those suffering from bloating. This refers to a diet low in fermentable oligo-, di-, and mono-saccharides, and polyols. These are short-chain carbohydrates that are osmotically active, causing diarrhoea after ingestion by attracting water from the intestinal vessels into the intestinal lumen. Fermentation by intestinal bacteria then yields large volumes of gases (hydrogen or carbon dioxide) leading to bloating.

Medical management and nutritional support should be offered in the context of a multidisciplinary care model.

Primary options

constipation

docusate sodium: 100 mg orally once or twice daily

or

glycerol rectal: 1 suppository inserted into rectum once or twice daily when required

OR

slow transit

erythromycin base: 125-250 mg orally three to four times daily

or

domperidone: 10 mg orally three times daily for a maximum of 7 days, maximum 30 mg/day

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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