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LOTS OF INFORMATION ABOUT HYPERMOBILITY SYNDROME (HMS)
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WHAT IS HMS?
When the Hypermobility Syndrome was first put on the medical map in 1967, it was defined as the presence of musculoskeletal symptoms (predominantly pain) occurring in otherwise healthy individuals. Thirty years down the line we now think that there are probably two types of hypermobility.
The first is a milder type occurring in people whose joints are just like everyone else's but which have the capacity to move more than most people's joints. The other, a more marked form, has features that suggest that it may be part of an inherited connective tissue disorder similar to the hypermobile form of the Ehlers-Danlos Syndrome, formerly called EDS III. It probably is EDS. At the present time we simply do not know for certain. So it is called Hypermobility Syndrome.
There are many people with joint hypermobility in the community who experience no (or very little) pain. Most of them probably do not even know that they are hypermobile at all. Then something happens, and they start getting pains for the first time in their lives. Usually unexpected exercise or a change of job or lifestyle provokes the onset of pain. When ever symptoms commence, and irrespective of the cause of the hypermobility, the term 'Hypermobility Syndrome' is used to describe the condition. Hypermobile people without pain are just called hypermobile people.
The hypermobility syndrome is a fairly frequent finding in the young, especially female patient. Connective tissue proteins such as collagen give the body its intrinsic toughness. When they are differently formed, the results are mainly felt in the "moving parts" - the joints, muscles, tendons, ligaments - which are laxer and more fragile that is the case for most people. The result is joint laxity with hypermobility and with it comes vulnerability to the effects of injury.
HOW IS HYPERMOBILITY MEASURED?
Variations between one person and another make it difficult to measure hypermobility. The most popular system is the 'Carter and Wilkinson' score, (see figure below and the scoring chart). Give yourself one point for each of the five simple tests you can do. Do the tests on the arm and leg on both sides of your body, so the maximum score is nine. An average person might score 1 or even 2 of these. But only about 1 or 2 in a hundred healthy people score 5/9 or more. If you scored 5/9 or more, you have 'joint hypermobility' But still not neccessarily the syndrome.
 Scores:
1. Can you put your hands flat on the floor with your knees straight? (1 point)
2. Can you bend your elbow backwards? ( 1 point for each elbow = 2 points total)
3. Can you bend your knee backwards? (1 point for each knee = 2 points total)
4. Can you bend your thumb back on to the front of your forearm? (1 point for each thumb = 2 points total)
5. Can you bend your little finger up at 90° (right angles) to the back of your hand?
(1 point for each finger = 2 points in total)
MAXIMUM SCORE IS OUT OF 9.
WHAT IS THE DIAGNOSTIC CRITERIA FOR HYPERMOBILITY SYNDROME?
Major Diagnostic Criteria Includes:-
* Generalised joint hypermobility (floppy/double joints) The beighton scale is a test to be done to diagnose hypermobile joints. (please see above) You have to score 5/9 to have hypermobile joints. An average person might score 1 or even 2 of these
*(Pain) Arthralgia for longer than 3 months in 4 or more joints
Minor Diagnostic Criteria Includes:-
*Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.
*Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis.
*Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).
*Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactily [positive Steinberg/wrist signs].
*Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.
*Eye signs: drooping eyelids or myopia or antimongoloid slant.
*Varicose veins or hernia or uterine/rectal prolapse.
The BJHS is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.
BJHS is excluded by presence of Marfan or Ehlers-Danlos syndromes (other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1996 (8) and the Villefranche 1998 (9) criteria respectively). Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.
WHAT CAUSES HMS?
People are born hypermobile It is in their genes. It is the way they are made, but that doesn`t mean that they will develope the syndrome (the pain). In fact only a minor percentage of people develope the syndrome. So how is it that people with hypermobility can be literally fine for decades, only to be laid low from widespread pain, often out of the blue, which may make its unwelcome appearance during childhood, adolescence, or adult life? To explain these curious and seemingly inexplicable happenings, we postulate that the affected person, in this case the hypermobile person, had an inbuilt weakness of her (it is usually a 'her' but not always) strength-providing collagen or similar protein. This weakness renders body tissues less robust and hence less able to stand up to the physical strains of everyday life.
Current thinking suggests that there are four factors, which affect different people in different proportions. They are:
1. The shape of the ends of the bones (where they move at the joints)
2. Weak or stretched ligaments caused by problems with collagen and other proteins (the ligaments are bands which hold your joints in place)
3. The tone of your muscles (this affects whether joints are held loosely or more rigidly)
4. Your sense of joint movement (this is the sense that tells you exactly where your joint is positioned and whether it is over-stretched).
WHAT ARE THE SYMPTOMS OF HMS?
If you have hypermobility syndrome you may have the following symptoms.
The most frequent symptom is pain. This usually happens after hard physical work or exercise, where your muscles have to work much harder if the joints are supple than if they are stiff. As a result, what doctors call an 'over-use' develops in the muscles around the joint (though it may appear to come from the joint). Sometimes fluid collects inside the hypermobile joint, making it feel tense and stiff. This is probably because your body is trying to repair the small amounts of damage that are caused if the joint is over-stretched. Your pain will often get worse as the day goes on and improve at night with rest. Sometimes, however, pain also occurs at night. If your hypermobile joints are caused by altered collagen protein, then collagen may be weakened in other parts of your body. This can lead to hernias or varicose veins. You may also have a flat arch to your foot and this leads to foot ache, particularly after standing for a long period. Backache may affect you if the base of your spine is particularly supple, sometimes as a result of one vertebra slipping on another. This is called a spondylolisthesis. There is a whole host of painful injuries that can result, ranging from dislocations to fractures, disc prolapse, ligament sprains, muscle strains, pulled tendons (like tennis elbow or plantar fasciitis), etc.-conditions that can happen to anyone, but which occur more easily in those with the HMS/EDS.
You may experience acute pain, joint effusions, recurrent subluxations or dislocations, idiopathic scoliosis, carpal tunnel syndrome and other neuropathies, temporomandibular joint dysfunction, prolapse of the mitral valve, foot abnormalities and osteoarthritis. Patients with the HMS/EDS for some reason do not appear to experience the full anaesthetic affect of lignocaine injections when these are given for dental purposes, minor surgery or for epidural anaesthesia.
HOW IS HMS TREATED?
Hypermobility Syndorme is not well known enough, not even some doctors know what it is, therefore leaving treatments still to be investigated further. Sufferers will know that many conventional methods of treatment, of the kind offered for rheumatic complaints in general, are not particularly helpful in the HMS/EDS. Physiotherapy still carries the best prospect for pain relief, and it is encouraging that more physiotherapists are training in methods that are helpful in this condition. Although there are alot of physiotherapists that I've met that I`ve had to explain what hypermobility syndrome is never mind the treatment for it. Opinions do vary but some say gentle exercises to strengthen and condition the muscles around the joints that are particularly supple should be done. These exercises may be against weights or may just be the sort that anybody can do themselves at home, ask a physio or doctor who knows about hypermobility syndrome to advise you on what kinds of excersizes. The important thing is to do these strengthening exercises frequently and regularly and do not over-do them. Some people with joint hypermobility find it helpful to keep moving – you can wear a splint or a firm elasticated bandage over the supple joint to protect against dislocation. Occupational therapists can advise on these. Your doctor may prescribe painkillers (analgesics, but if the joint often swells up, an anti-inflammatory drug may be better. You can also apply either painkillers or anti-inflammatories onto the troublesome joint as a spray or cream. Another noteworthy development is the network of units throughout the country that are offering pain management programmes, an approach which has been shown to be beneficial in the HMS/EDS where intractable pain is present. Again the treatments are are all trial and error and self help trying to relieve a little and to cope.
WILL SURGERY HELP MY HYPERMOBILE JOINTS?
In general, you should avoid surgery if possible, not least because supple tissue does not always heal well and quickly. Also, some hypermobile people are prone to bruising easily and may require more blood transfusions if major surgery is carried out.
However, if you have the bad luck to rupture a tendon, which is more common if you have supple collagen, this should usually be repaired surgically. Otherwise, in general, you should avoid operations if you can. Operations may be suggested to you, such as removing a kneecap that dislocates frequently, or stabilising a very supple joint with a pin to fuse it. Avoid these if you can as they may eventually cause osteoarthritis. (Osteoarthritis is a type of arthritis which affects the cartilage within the joint.) Although surgery can bring on osteoarthritis, there is a link if your hypermobility is due to abnormal shape of the bone surfaces or to abnormal joint position sense, to developing osteoarthritis.
WHAT IS THE PROGNOSIS OF HMS & WILL THIS BE PASSED ON TO MY CHILDREN?
A sense called proprioception, which means knowing where parts of one's body are in space. If you close your eyes and somebody bends your finger for you or picks your arm up, you know immediately how far your finger is bent or where your arm now is. That is because you have good proprioception. Scientists have shown that people with the HMS/EDS are not quite as good as other people in knowing exactly where their fingers or arms etc are in space. This could lead to a further increase in the risk of injury. As decribed above people with hypermobility syndrome can develope flat-footedness, club-footedness, knock knees, swan neck along with other out of line joints. Hypermobile people seem to be prone to Osteoarthritis with an increased risk if developing hypermobility syndrome at a younger age. It doesn`t mean to say that everyone will develop this but it is a possibility.
Life expectancy is not proven to be reduced.
EDS is hereditary (that is, that the condition is passed on from one generation to the next) and as the kids inherit the gene from you or your partner and it is a dominant gene then the chances are 50percent for each of your children with having it. Unless it was a spontaneous mutation in your daughter/son making them the first in the family to show signs of it.
It is a 50 50 chance for each and every one of your children.
If your hypermobility comes from abnormal collagen proteins, there is often strong evidence of heredity. This means that, at worst, half of all children of a person with this type of hypermobility will be affected. though, for reasons we do not understand, the degree to which the child is affected is very variable. If your child does develop hypermobile joints it does not mean that they will automatically get the pain (syndrome/hms/eds) type just because you have it. There is no evidence to confirm or deny this. There is a combination of cases and about a quarter of all examples of hypermobility affect people who have no previous family history of it.
There is thought to be connections between FMS & HMS but they are still researching these at present. Although there is still much to learn, the understanding of HMS is advancing and the knowledge gained can help people deal with the various aspects.
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FIBROMYALGIA, HYPERMOBILITY SYNDROME, & CHRONIC FATIGUE SYNDORME / M.E
