Our skin, discs, tendons, ligaments and many other tissues all rely on the protein collagen for their structural integrity, ensuring normal flexibility, healing capacity, strength and cohesiveness. This allows us to put the considerable daily stresses on these tissues without problems and collagen is also very involved in the strength and elasticity of internal organs such as our arteries. Some people are called “double jointed” because they are so bendy whilst others have very stiff and tight joints. Collagen is a very important protein in our connective tissues, at least partly dictating what activities we can perform and how we recover from injury.
Ehlers-Danloss syndrome is caused by an abnormality in the way collagen is produced and acted upon in the body, causing an inheritable deficiency in the strength of the substance. 10 forms of EDS are known to exist, with much overlap, and EDS Three is considered the same as benign joint hypermobility syndrome, called benign because the serious changes such as in the arteries are not present in this form. Very hypermobile joints are the most obvious sign of this syndrome, with a smooth, flexible skin which tends to heal slowly and scar poorly in terms of wide and thin scars.
The typical signs and symptoms of joint hypermobility syndrome are a hyperextensible skin, hypermobile joints, tendency to dislocations, fragile tissues, poor wound healing and a tendency to bruise easily. Chronic joint and limb pain is also common and many sufferers live with constant and multiple pain complaints. Patients show many abnormal muscle balances and have difficulty stabilising their joints appropriately, causing incorrect muscle uses which can lead to pain problems. Functional limitation is common in this group, who cannot participate in contact or vigorous sports without significant joint injuries and pain.
Self management in hypermobility syndrome is the main aim of intervention, with patient education taking a strong role to equip the patient to manage their lifelong condition. Due to the abnormally large ranges of joint movement they are vulnerable to ligament or joint strain if they are held posturally at end range or moved with momentum. Hypermobile patients should practice joint protection like arthritic patients, avoiding party pieces like showing off with extreme movements or joint dislocations. Yoga or high momentum activities such as contact sports are particularly unsuitable for these patients.
As it takes far less trauma to damage a hypermobile joint than a normal one the incidence of acute injuries is higher in these patients as they go about their daily activities. The joint injuries and general painful problems which occur are managed by physiotherapy intervention. The shoulder is a highly mobile but unstable joint in the best circumstances and in hypermobile patients it presents particular problems of stability. The socket is small and the shoulder girdle muscle control must keep the humeral head aligned against it, difficult if the pattern of muscle action is abnormal. Repeated subluxation or dislocation with consequent pain is common and difficult to treat.
Spinal pain is common in hypermobility perhaps secondary to the difficulty in keeping good stability of the joints as forces act upon them. Gentle mobilizations can be performed for local problems but manipulation is likely to be unwise. General exercises to keep the joint moving can be helpful but end range and stretching exercises should be avoided. Gentle weight training and core stability work may help stabilise the spine and other joints, increasing the muscle tone to hold joints in their mid ranges and prevent excessive play. The knees may bend back excessively and suffer arthritic change in time, so work on the hamstrings to counteract this in standing is an option. Often patients need to work on several areas, maintaining muscle strength and control.
All postures and activities are a challenge to a patient with hypermobility as unsuitable stresses are very easy to apply, causing pain. The patterns of muscle activity are abnormal when the joints are under load, pushing them into end range positions where the ligaments and capsules suffer from strains. Physiotherapy retraining of poor muscle balance can be helpful but patients need to be constantly vigilant and work at their weaknesses persistently. The most important factor overall is patient education as the condition is a long term one and all physical activities challenge the joints.