Managing dislocations and subluxations in hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders
Jason Parry, Extended Scope Physiotherapist / Clinical Specialist Physiotherapist, University College London Hospital and The Hypermobility Unit, Hospital of St John and St Elizabeth, London
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.
Joint dislocations and subluxations can commonly occur in people with hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD). Such events can obviously prove very distressing so it can be valuable to learn how to manage and cope when such situations arise. A good place to begin is to understand the difference between a dislocation and a subluxation:
A ‘dislocation’ is defined as “displacement of a bone from its natural position in the joint”. This is where the two bones that form a joint fully separate from each other. If we consider a shoulder for example, which is a ball and socket joint, this can be illustrated whereby the ball (which forms the top of the arm bone) slips entirely out of the socket it should sit in within the shoulder. This can happen in any direction, but the main point is that the two bones completely separate. Very painful!
A ‘subluxation’ is basically defined as “a partial dislocation”. It can be no less painful than a full dislocation but the two bones that form the joint are still partially in contact with each other. So once again if we consider the shoulder joint as an example, the ball which completely came out of the socket in the dislocation example above would still be partially sitting in the socket if it was a subluxation.
These events can happen in almost any joint but some are more common than others. Shoulders, knees, thumbs and ankles seem to be some of the most prevalent. Both dislocations and subluxations can be painful, irritating, infuriating and occasionally debilitating problems. However, they don’t need to ruin your life and can with patience, effort, trust or time be managed. Not necessarily completely eliminated, but managed.
Why does this happen?
The main reason is abnormal collagen composition. Collagen – primarily Type I collagen – is the main structural protein of the various connective tissues in the body. It is found in ligaments, tendons and joint capsules and makes ligaments and tendons strong like little ‘guy ropes’. What do ligaments and tendons do? Ligaments connect bone to bone, tendons connect muscles to bone, and joint capsules are like envelopes of tissue that surround a moveable (synovial) joint. So we can see that these ligaments, tendons and joint capsules play an important role in giving a joint stability.
So let us now consider hypermobile EDS (hEDS). This is a heritable disorder of connective tissue (HDCT) caused by a defect in the structure, production or processing of collagen, which makes the collagen in ligaments and tendons stretchier (more lax). This means that joints are potentially less stable – hence greater propensity for subluxations and dislocations.
There are other reasons for dislocations and subluxations:
- Altered muscle tone – this can often account for dislocations. Inappropriate muscle patterning, whereby certain muscles around a joint switch on when they shouldn’t necessarily and then inappropriately work way too hard, can often ‘pull’ a joint out of place. The joint then also becomes easier to slip out, of course, if it is more lax in the first place. Muscle fatigue, spasms and stress can all play a part in this too.
- Impaired proprioception – proprioception is the body’s ability to sense position and movement within joints and enables us to know where our limbs are ‘in space’ without us looking. It relates to coordination. Impaired joint position sense can cause joints to slip out of place.
- Repeated overstretching – otherwise known as too many ‘party tricks’. Some hypermobile patients have the capacity to ‘amaze’ people with their ability to wrap themselves into weird and wonderful positions, putting their arms and legs into positions that the rest of us gawp at, such as choosing to pop shoulders in and out of joint or folding legs over the head. As ‘amazing’ as this may be, it’s time to stop! Repeated overstretching to that degree will only exacerbate the laxity and the chances of the joints slipping out of place. It is perfectly acceptable for hypermobile people to undertake regular stretches within ‘normal’ joint ranges, but don’t stretch your joints way beyond this ‘normal’ range and give up the party tricks. Just because the joint ‘goes there’ doesn’t mean you should take it there.
- The shape of your joint surfaces – some of you may be born with shallow shaped joint sockets or other bony shaped ‘anomalies’ that predispose a joint to possibly slipping out of position more easily. Unfortunately, that just may happen to be the shape of your skeleton.
- Traumatic incident – one of the other reasons for a joint to come out of place (and the most common one for those without EDS). Traumatic incidents can happen to anyone, but extra joint laxity may actually work a little in your favour with this one, whereby it may prevent you damaging some of your ligaments/tissues in the way that a non-hypermobile person who suffered a traumatic dislocation probably would.
How frequent are these events?
The answer to that is different for different people. Some people can get them maybe just once or twice a year, others once a month. Some people have them once a week and others once a day. Some people get them happening repeatedly several times throughout the day and in certain people they never seem to stop. In some, when it happens the joint just finds its own way back in to place, which is a relief, but even then we need to try to reduce the frequency if we can. But in others, once the joint slips out it won’t go back in again, and the natural reaction then is to panic! This is perfectly understandable. The joint is out of place, the pain kicks in (often in a big way) and the panic starts. At this point, some people understandably pick up the phone, call for an ambulance and head off to A&E.
DON’T PANIC! STOP FOR A SECOND, BREATHE NORMALLY AND STAY CALM
Panic causes more stress. Panic causes more muscle spasms. Stress and muscle spasms cause more pain and then there is less chance of resolving the dislocation. In order to start managing this situation and taking control you are going to have to start learning not panic. Because what happens at A&E? They will often give you pain relief of some sort (perhaps Entonox) or they may go the whole hog and give you a general anaesthetic. Then they’ll yank your joint back into place. However frequently, and we could be talking within minutes, the joint will often pull itself back out of place again because of the muscles still spasming around the joint, and you’re back to where you started.
In these situations, some A&E doctors might then stick you in a plaster cast to ‘hold’ the joint in place. Imagine, then, the battle going on under that cast, with your joint often trying to pull itself back out of position again while being forcibly held in place by the cast. It’s often very painful and then when do you take the cast off? This is not a viable management solution or a good way of life for you.
So what should you do if your joint comes out?
Six key principles of management
Here are the six key principles that I suggest you need to start utilising in order to begin to get a grip on managing this situation as opposed to this situation managing you. The main aims are to stay calm, keep on top of the pain and allow the muscles to relax. It takes lots of practice and patience, but it can be done.
Use slow deep, relaxed breaths. Try using some relaxation techniques, there are lots of different ones out there. As painful as it is, and as difficult as it may sound, you need to start to try to take control of this situation. So start to learn how to breathe through it.
Take some appropriate painkillers (analgesia) if you have some. However, note the word “appropriate”. You should only ever take analgesia according to the dosage indicated by your prescriber. Never take more than the suggested dose. You might feel like it may not be enough at the time, but if it can take some of the edge off, then that’s a great start. Please don’t ever overdose. What about Entonox (commonly known as gas and air) as pain relief? Some people have access to Entonox at home, or use it at A&E. There can be a role for it, but this must be used with caution. Prolonged use can lead to vitamin B12 deficiency and can interfere with DNA synthesis, not to mention cultivating a dependency, all of which are big issues.
Support the joint
You need to try to make yourself as comfortable as possible (I know it’s not easy). Use pillows or a sling if you have one. Find a comfortable resting position as much as possible. This allows the muscles to relax and stop spasming.
Hot water bottles, wheat bags and a warm bath can all help to relax spasming and overactive muscles.
Try to take your focus away from the pain and the situation. Listen to music, watch a film if you can, talk to friends/family, try a relaxation CD/MP3. This can be helpful as a short-term pain relieving strategy. Again it can help muscles relax.
Sometimes gentle massage around the joint can help relax the muscles enough to be able to gently relocate the joint or for the joint to just slip back into place by itself.
What if it doesn’t go back?
Don’t expect the joint to go straight back in. It is often not unusual for joints to remain out of place for hours or even days. But once it’s out, it’s out. It’s not going out even more, so try not to panic.
Is it dangerous? Am I damaging my joint if it comes out?
It is highly unlikely. Your joint laxity allows for your ligaments and capsules to stretch. It is mostly just distressing as opposed to damaging.
When should you go to hospital or get help?
- If the limb starts to change colour due to a lack of blood supply.
- If your limb goes completely numb.
- If you have tried strategies 1-6 above, have waited a reasonable amount of time and are still desperately struggling.
But as mentioned earlier, it is not unusual for A&E to relocate your joint only for it to pop straight out again or when the anaesthetic wears off. Therefore you need to learn to stay calm and to start to self-manage.
Reflect and learn lessons
One of the most valuable things you can do after a subluxation/dislocation is to reflect on the event (once you have had a chance to calm down). Were you moving in a way that normally causes the joint to dislocate? Did you move without thinking? What was your posture like? Were you tired or overdoing it? Were you stressed about something? It is so valuable to reflect on these types of questions to look for triggers as to why the event may have happened. It may have been none of these reasons, but if it was then you can hopefully learn to avoid repeating them in the future.
Finally, prevention is better than cure. It is obviously better if we can prevent these situations occurring in the first place as opposed to having to deal with them. So to that end, the following can hopefully help to reduce the frequency of such occurrences:
- Physiotherapy to learn to control the muscles around joints and to use the right ones.
- Rehabilitation to improve proprioception.
- Possible use of supports/braces if required.
- Try to manage stress and anxieties.
But ultimately, stay calm! The more you stay calm when these events happen and manage it yourself, the easier it should get each time.
Peer reviewed by: Dr Helen Cohen, Consultant in Rheumatology and Pain Management, Royal National Orthopaedic Hospital, Stanmore
Date of last review: 01/11/2017
Date of next review: 01/11/2020