Collagen is an extremely important connective tissue protein in our bodies, making up significant amounts of our skin, tendons, discs and ligaments. Collagen gives structure to these organs, allowing them to heal normally, to have elasticity, to be strong and to hold together well under the stresses of functional life. It also gives our major blood vessels and other organs the strength they need to remain working correctly. People vary very greatly in the suppleness of their joints and skin and show the whole range from very stiff jointed to very mobile or “double jointed”.
Ehlers-Danloss Syndrome (EDS) is an inheritable condition which affects the synthesis and viability of collagen as a structural protein. There are at least 10 forms of this condition but Ehlers-Danloss III (three) is also known as benign joint hypermobility syndrome, benign because there are no life-threatening symptoms as occur in some of the other types. These people have smooth, velvety and flexible skin and remarkably hypermobile joints, along with chronic joint or limb pains and recurrent joint dislocations, mostly of the shoulder and the patella. Slow or abnormal wound healing with large and relatively weak scars can also be a feature.
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, in the neck, low back or thoracic regions, is a common symptom which hypermobile patients complain of, and physiotherapists interpret this as a lack of stabilising muscle control and muscle balance. Physios do not manipulate these patients but mobilizations, core stability work, strengthening weak muscle groups and general exercise are typical approaches. Increasing the usually low muscle tone by gentle weight training or using resistive bands can help joint control in the mid positions and avoid stresses at end ranges. Hyperextension of the knee is a typical problem, leading to joint pain on weight bearing and later to osteoarthritis. Hamstring work to strengthen the muscle opposing the abnormal movement is useful, with patients typically working on the muscle balance of several body areas.
Hypermobility is a lifelong chronic condition so sufferers are faced with managing this daily in all their postures and activities. Dysfunctional muscle patterns are common when the joints are significantly stressed, forcing them into unsuitable positions where they suffer strains. Physiotherapists can help with retraining of muscle patterns and treatment of painful joints but the largest component is self management and therefore patient education.
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