Managing your pain

Please note: The following text cannot and should not replace advice from the patient's healthcare professional(s). Any person who experiences symptoms or feels that something may be wrong should seek individual, professional help for evaluation and/or treatment. This information is for guidance only and is not intended to provide individual medical advice.

People who live with HSD or EDS, usually have a very high symptom load from the various affected systems in the body and the associated disorders. Pain is often very prominent, not just created by the underlying problems themselves but also by their consequences. To help manage this pain, a whole-person approach is necessary. It is often very difficult to accept that there is no single treatment that could totally alleviate or cure the pain but if we address it from multiple different angles, we can often achieve a noticeable reduction.

To start to have more control over your pain, it is helpful to understand a bit more about the types of pain and what you are experiencing. Acute pain is the pain experienced during an insult or injury to the body (for instance a bone breaking, a joint dislocating, burning yourself) and is a message to protect. Most often for acute pain, there is a cause that we could attempt to treat or remove. Chronic pain (or persistent pain) is when the pain persists past the expected time of healing, usually beyond three to six months. Chronic pain is complex, dependent on and influenced by a multitude of factors. Changes can occur in the brain and spinal cord, with ‘wind-up’ or sensitization taking place. There is not a simple relationship between how severe a condition is and the amount of pain we feel. It is closely linked with the areas of the brain where mood, appetite, sleep, threat and fear are also being processed and the same neuro-modulating chemical messengers play a role. Medications that act in these areas could be helpful but the person with pain can also modulate these pain pathways by engaging with various other strategies.

Pain can be further divided into nociceptive pain (more “direct” pain experienced in the affected area, for instance an inflamed joint) and neuropathic pain (generated by an issue within the nervous system itself and usually along the distribution of a nerve pathway; for instance a compressed nerve or small fibre neuropathy). New definitions also talk about ‘primary pain’: pain generated without any obvious cause, like fibromyalgia, or far exceeding what would have been expected for the underlying condition, and ‘’secondary pain’; where there is an underlying cause for the pain (whether treatable or not). These distinctions are helpful when deciding on medications to use and when judging their effects.

Medications

It is important to understand that every type of medication (including herbal) has to go through metabolism in the body to get it to act on the area where it was designed to act. This means it could interact with other medication or even food and drink that follow the same pathway or be affected by issues in the organ/s it has to be metabolised by in that person. It would have had safe doses established and doses where we know harm would occur. Any medications used need to clearly show benefit (a reduction in pain and an increase in function), no or negligible side-effects, and if used regularly/ long term, very low potential for harm.

It is important that medication trials or challenges (reviewing the current dose) take place in sequence and not all at the same time. This is to ensure clear observations on each drug’s benefits or side-effects.

• For acute pain/ nociceptive pain, the usual medications would be paracetamol, non-steroidal anti-inflammatory drugs (i.e. ibuprofen, diclofenac) and/or opioids (codeine, tramadol, morphine, oxycodone, buprenorphine and fentanyl). Opioids are a particular problem the longer you use them and the higher the daily dose, causing multiple metabolic problems, including suppression of the adrenal glands and the immune system. There is poor evidence for chronic use and they usually do not work well in primary pain. The best use, where appropriate, is on occasion with low doses.
• For neuropathic pain, UK guidance is to use one of the following four drugs: amitriptyline, duloxetine, pregabalin or gabapentin. Other drugs might be used by specialist centres.
• For primary pain, the new guidance is that any anti-depressant could be considered, depending on what suits the person.
• Pain medications applied through the skin, like capsaicin cream, menthol, anti-inflammatories or lidocaine (if you are responsive to it) might also be useful depending on the licensed indication.
• There might be drugs that are very particular to one of your conditions, for instance migraines (acute attack and preventative) or endometriosis (i.e. hormone suppression) or suppressing mast cell activation.
• In certain situations, other drugs may also need to be used in the short term only, for instance muscle relaxants or antispasmodics (anti-cramp).

Discuss with your GP or specialist whether referral for consideration of nerve block injections or botox injections (for instance in migraine or back pain) might be an option for you.

Surgery may need to be considered in particular situations, i.e. a very unstable joint.

Non-pharmacological modalities

• Specialist physiotherapy, especially around stabilising and strengthening problematic joints, dysfunctional movement and muscle spasm, and teaching general joint protection. It is useful to learn a small, gentle set of movements that could be done daily as a baseline to prevent stiffness and prevent deconditioning. This might even need to be a set that could be done when lying down, depending on your level of disability. You could then also be taught how to safely increase on this when doing better.
• Massage
• TENS (transcutaneous electrical nerve stimulation) especially one with four leads and different modes. You can get hypo-allergenic pads if you tend to have issues with glue. If hand dexterity is a problem, you can get wireless types (they usually are a flat butterfly-shaped stick-on device) that could still be helpful on larger flat areas for instance the lower back or lower abdomen.
• It could treat an acute episode well, or in the case of chronic pain, may reduce the level of pain enough to start working on another problem so that you are in a better place by the time the effect starts wearing off.
• Mind-body techniques. Tai-Chi (the adapted type might suit better) is excellent for breathing, proprioception, gentle joint mobility and relaxation. Pilates (especially when adapted by a practitioner with knowledge) and Alexander Technique are other techniques often found very helpful. Some people with EDS find yoga helpful but care needs to be taken with prolonged and excessive stretches, again adaptation of techniques may be required.
• Breathing techniques. For those with dysfunctional / shallow breathing (often those people with a lot of chest pain or rib spasms), this might need to be taught by a respiratory rehabilitation physiotherapist but there are also videos on YouTube. You could also look out for those techniques that calm the vagus nerve (important in gastric or cardiac autonomic dysfunction)
• Application of heat or cold, depending on which one provides you with relief.

Aids/equipment

Most people do not only have invisible disabilities but would have a so-called ‘dynamic disability’, in other words their difficulties are variable. This makes it very difficult to live with or for family and friends to understand. Consider any aids as enabling, rather than disabling, in other words at that moment allowing you to partake in an activity which you may not have been able to do otherwise, whether it reduces pain, improves stability or prevents fatigue.

• Splints and braces, including ring splints for fingers, dental braces to keep your jaw in neutral position at night, neck braces when a passenger in a car, stabilisation braces after dislocations, following surgery etc. Most of these would not be required all the time but are situational (the preferred method by physiotherapists and orthopaedic surgeons!)
• Walking sticks or crutches
• Strollers/rollators or wheelchairs
• Compression garments, but also clothes with soft fabrics and flat seams
• Custom insoles
• Various cushions to improve sitting, for instance wobble or coccyx types
• Body pillows and weighted blankets to aid sleep
• Light-filtering glasses/ sunglasses/ screen filters/ hats/ noise-cancelling headphones, all to reduce over-stimulation
• Ask to have a home assessment (via social services) or work assessment (via your line manager or occupational health department) done by an occupational therapist, regarding equipment that might make tasks easier for you for instance railings, seat raises, special chairs etc.
• Do you need a radar key for easy access to disability toilets?
• Would information cards regarding your condition or colour-coded arm bands to indicate your type of day to your family be helpful?

Lifestyle changes

Anything that reduces your overall symptom load, will also help with pain reduction. An example here would for instance be when irritable bowel syndrome is addressed, the painful bloating will reduce or stop.

• Pacing of your activities, to prevent ‘boom and bust’ cycles (overactivity when you feel stronger, resulting in total wipe-out following the activity)
• Sleep hygiene practices
• Dietary adaptions, including nutrition, supplements and hydration. Please note that trials of exclusion diets are best done with the help of a dietician, for instance trying a low FODMAP diet in IBS or a low hstamine diet
• Do you need to see your local bladder and bowel nursing team regarding evacuation techniques, bladder pain etc?
• It might be very helpful to keep a symptom diary for a few weeks (you could use an app to assist you) to determine which areas of your life might need the most attention.

Day-to-day living techniques are often taught on pain management programmes or by occupational therapists.

Looking after your mental health

Living with constant pain is draining and can lead to low mood and anxiety. Many people also live with a past life trauma, or even the trauma of not being believed. There might be the grief associated with the loss of a future you envisaged or difficulty coming to terms with your situation.

• Consider mindfulness meditation techniques or forms of mindful movement, if being still does not work for you—walking in nature, swimming, dance, Tai chi
• Distraction techniques: reading, listening to music or ASMR, visualisation.
• You can self-refer for counselling in most NHS trusts. The IAPT (improving access to psychological therapies) services also have practitioners with additional training in long term conditions, you could ask if such a clinician is available.
• You may need to be referred to your local mental health services, especially if your problem would require specialist knowledge or techniques.
• If you think you may have undiagnosed autism or ADHD, ask for a diagnostic referral. If you are known with these conditions, make sure to inform your healthcare practitioners about additional communication needs.
• Join a pain support group in your area.

In summary

In general, ‘pain management ‘comes down to ‘life management’. Every little bit of improved control provides overall reduction of the immense burden. These are just some ideas. Please bear in mind that everyone is different and what helps one person, will not necessarily help another. There are multiple heath-related smartphone apps out there, always try out their free part first before you commit to paying for something you might not use.  The golden rule is only try one new thing at a time. Also keep in mind that you cannot just keep adding activities, one thing may have to go to allow another one.

Ask your GP to refer you to a specialist pain clinic if you think you need more help. Alternatively, if you want help with a particular issue, it may work better to only discuss that problem in the time allocated with your GP, so that it gets its full attention. My advice for any referral is to write across the top in bold “for attention of a senior clinician with interest in connective tissue disorders”.

You may find the following website helpful:

https://my.livewellwithpain.co.uk/

A video recorded by Dr Theron earlier this year is available to EDS UK members on our website: https://www.ehlers-danlos.org/information/webinar-dr-johanna-theron-pain-management-in-eds-and-hsd/. You must be logged in to view it.