Pain management in Ehlers-Danlos syndromes and hypermobility: a UK guide

The NHS describes Ehlers-Danlos syndromes as a group of rare inherited conditions that affect connective tissue, the support structure in skin, tendons, ligaments, blood vessels, internal organs and bones. Hypermobile EDS, the most common type, can bring joint hypermobility, loose and unstable joints that dislocate easily, joint pain and clicking joints, extreme tiredness, easy bruising and digestive problems. There are currently no tests to confirm hypermobile EDS; the diagnosis is made from history and examination.

Ehlers-Danlos Support UK explains that pain can come from joints moving too far, muscles working harder to stabilise the body and repeated strains or injuries, and that sleep, stress, illness and overdoing things influence it. It can be constant or fluctuating and can change over time. A pain clinician writing for the charity describes acute pain as a protective message to a particular injury, and chronic or persistent pain as lasting beyond the expected time of healing, usually beyond three to six months, when changes can occur in the brain and spinal cord so that the amount of pain no longer maps neatly onto the amount of damage.

The pain specialist guidance on the Ehlers-Danlos Support UK website says that it is often difficult to accept that no single treatment will completely relieve or cure the pain, but that approaching it from several angles can often achieve a noticeable reduction. It stresses a whole-person approach because people with EDS or HSD usually have a high symptom load from several body systems.

The NHS takes a similar view. It lists a physiotherapist, an occupational therapist, counselling and cognitive behavioural therapy (CBT) for people struggling to cope with long-term pain, and genetic counselling and hospital scans for some types, all arranged through a GP or consultant.

For joint hypermobility syndrome the NHS says the main treatment is improving muscle strength and fitness so the joints are better protected, and that a GP may refer to a physiotherapist, occupational therapist or podiatrist; these services can also be paid for privately. Physical therapy can help reduce pain and the risk of dislocations, improve muscle strength and fitness, and improve posture and balance.

Advice for people with EDS from the NHS is to be careful with activities that put a lot of strain on the joints, but not to be overprotective and avoid an otherwise normal life. Lower-risk activities such as swimming or pilates may be recommended, and people with fatigue can be taught to conserve energy and pace activities. For hypermobility, the NHS says to do gentle exercise such as low-impact swimming or cycling, maintain a healthy weight, wear supportive shoes and insoles if a podiatrist recommends them, avoid overextending joints just because you can and take regular breaks to avoid repetitive activity.

The charity guidance adds that specialist physiotherapy aimed at stabilising problem joints, dysfunctional movement and muscle spasm, with a small gentle daily routine as a baseline, can help prevent stiffness and deconditioning. It also describes TENS, massage, heat or cold, adapted tai chi, pilates and Alexander Technique, breathing techniques, and splints or braces used situationally.

The NHS says paracetamol and anti-inflammatory painkillers such as ibuprofen may ease joint pain in hypermobility, that a pharmacist can advise on the best painkiller, and that a GP may prescribe stronger painkillers or refer to a pain clinic if pain is severe.

Chronic pain guidance is more cautious. NICE guideline NG193 covers assessment of all chronic pain and management of chronic primary pain, meaning pain with no clear underlying cause or pain out of proportion to observable injury or disease. For chronic primary pain it recommends a supervised group exercise programme, psychological therapy and, with discussion of benefits and harms, an antidepressant such as amitriptyline, citalopram, duloxetine, fluoxetine, paroxetine or sertraline. It says not to start antiepileptic drugs including gabapentinoids (outside a trial), antipsychotics, benzodiazepines, ketamine, local anaesthetic or steroid trigger-point injections, NSAIDs, opioids or paracetamol for chronic primary pain.

It is important to apply this carefully. Pain in EDS often has an identifiable mechanical component, and NICE separates chronic secondary pain, where there is an underlying cause, from chronic primary pain. The Ehlers-Danlos Support UK guidance notes that for acute or nociceptive pain the usual medicines are paracetamol, NSAIDs and sometimes opioids, that for neuropathic pain UK guidance is to use one of amitriptyline, duloxetine, pregabalin or gabapentin, and that opioids become more problematic the longer and higher they are used and usually do not work well in primary pain. Which category applies to you is a question for your own clinician.

The NHS lists counselling and CBT as useful for coping with long-term pain in EDS. NICE recommends psychological therapy for chronic primary pain, and defines a pain management programme as an intervention with at least two components including a physical and a psychological component delivered by trained people. This does not imply that the pain is imagined; it reflects how pain, sleep, mood and activity interact.

Practical measures also matter: aids and equipment can be considered enabling rather than disabling, as the charity guidance puts it, because they can allow an activity that would otherwise cause too much pain. An occupational therapist can advise on this and on managing daily tasks.

See a GP if you have several troublesome symptoms of EDS, as the NHS advises, and ask about a referral to physiotherapy, occupational therapy or a pain service. A GP can refer to a rheumatologist for joint problems and to a local genetics service if one of the rarer types is possible. The NHS says further investigation can be through a specialist EDS diagnostic service in Sheffield or London.

Some pain needs urgent assessment rather than long-term management. Sudden severe chest, abdominal or head pain, especially in someone with a known or suspected vascular type, a joint that cannot be put back or has lost its circulation, or new weakness, numbness or loss of bladder or bowel control should lead to 999 or A&E. The NHS describes vascular EDS as the type most often considered to be the most serious, because vessels and organs can split open and cause life-threatening bleeding.

On the NHS, community physiotherapy, pain management programmes and specialist EDS services are accessed by GP or consultant referral and vary by area. The NHS notes that physiotherapy, occupational therapy and podiatry can also be paid for privately. If you do so, look for clinicians with experience of hypermobility, and tell your GP what you are doing so that the overall plan stays coherent.