HMS Research

Hi Everyone! Here is a list of current-ish research being conducted about HMS, which I copied from the Hypermobility Syndrome Association website. That page, www.hypermobility.org is a great resources, however, it is catered towards the UK rather than the US. Enjoy!

Latest Research

Prof Rodney Grahame & Dr Alan Hakim
Department of Rheumatology, University College Hospitals, London

1. Do you have HMS?

We have been working on a 5-part questionnaire to identify hypermobility. If you answer yes to at least 2 of the 5 questions then there is an 80-90% chance you are hypermobile. We hope it will become a simple aide memoire for doctors and allied health practitioners, encouraging them to think about hypermobility as the cause for musculoskeletal problems.

The questions are:

1. Can you now (or could you ever) place your hands flat on the floor without bending your knees?
2. Can you now (or could you ever) bend your thumb back to touch your forearm?
3. As a child did you amuse your friends by contorting your body into strange shapes or could you do the splits?
4. As a child or teenager did your shoulder or knee cap dislocate on more than one occassion?
5. Do you consider yourself to be double-jointed?

Ref: Hakim AJ, Graham R. A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain.
International Journal of Clinical Practice 2003; volume 57: p163-166

2. HMS and local anaesthesia

In our survey of questions for the 5-part questionnaire we also asked about local anaesthetics as there has been work published suggesting that patients with joint hypermobility syndromes may be more resistent to them. We found that people with Benign Joint Hypermobility Syndrome are three times more likely than non-hypermobile individuals to have noticed that either local anaesthetic did not work at all or that they needed to be given more than usual to gain any benefit. We do not know the mechanism behind this but clearly it is an important message to get accross to colleagues in both hospital and general practice as well as dentistry.

3. Vascular problems and HMS

There has been some interesting work done recently looking at the many other non-musculoskeletal symptoms experienced by those with benign joint hypermobility syndrome. In particular people often complain of vascular problems of feeling faint, actual fainting or palpitations. A lot of people also experience gastrointestinal symptoms such as heartburn and irregular bowel habit. In our clinic we found that up to 60% of our patients have these kinds of symptoms on top of the more classic ones of widespread pain, anxiety and fatigue.

Dr Gazit and colleagues from Israel have done some important work that suggests that these kinds of vascular symptoms may be the result of poor functioning of the autonomic nervous system. This is a part of the nervous system that we do not have specific control over. It is responsible, in part, for maintaining our blood pressure and pulse when we move from lying to standing, or increase our level of activity. For some people the autonomic nervous system does not work as well as it should and their blood pressure falls and their pulse rises when they stand up. This makes them feel dizzy and experience 'palpitations', possibly even chest tightness and shortness of breath. Prof Grahame and I, with colleagues from the National Hospital for Neurological Diseases, Queen Square, are looking at this in more detail at present.

Ref. Hakim AJ, Grahame R. Symptoms of autonomic nervous system dysfunction in the benign joint hypermobility syndrome. Rheumatology (Oxford) 2003; Volume 42 supplement: Abstract number 47.

4. Other projects that are underway or about to start are:

• A study looking at the association between hypermobility and complications of pregnancy, particularly rapid delivery and tissue damage in labour.
  
• A study exploring the benefits and complications of conservative and surgical management of orthopeadic problems in the shoulder and knee in patients with benign joint hypermobility syndrome.

5. Finally a book on the Hypermobility Syndrome...

...'Hypermobility Syndrome - Recognition and Management for Physiotherapists' by Prof Grahame and Rosemary Keer was published on 3 June 2003. Although it is written for physiotherapists, it would still be very accessible to the 'un-medical'.

(Editors note: 'Hypermobility Syndrome - Recognition and Management for Physiotherapists' by Prof Grahame and Rosemary Keer is available from Amazon for £32.39)

Latest Research in Scotland - Prof W Ferrell

Professor William Ferrell was awarded a grant from the Arthritis Research Campaign to compare the differences in muscle reflexes and the sensations originating from proprioceptors - sensory nerve endings which feed back information about muscles and tendons to the brain in individuals with HMS.

10 Quick HMS Facts

10 Quick HMS Diagnostic Questions

10 HMS Facts

10 Quick HMS Facts

1. Hypermobility Syndrome is a genetic collagen defect most people don’t even know they have, that weakens connective tissue around joints
2. HMS is a common cause of unexplained joint pain, but is diagnosed in less than 10% of sufferers who see their primary care physicians
3. HMS is up to 5.5 times more likely to occur in women than in men, and is also more common in people of Asian, African, and Middle Eastern decent
4. HMS symptoms can manifest at any age, from age 3 to 70, and its onset usually does not coincide with any direct accident or trauma
5. HMS symptoms can last anywhere from 15 days to 45 years
6. HMS can predispose sufferers, especially women, to fibromyalgia and osteoarthritis
7. HMS can cause serious pregnancy complications, including premature birth, rapid labor, and uterine prolapse
8. People with HMS have diminished proprioception, or sense of their bodies in space, which increases the likelihood of joint injury
9. HMS patients do not experience the full anaesthetic affect of lignocaine, a drug used in dental work, minor surgery, and epidurals
10. There is currently no consensus as to how HMS should be diagnosed, and no proven treatment plan

10 Diagnostic Questions

10 Quick Diagnostic HMS Questions

1. Does one or more of your joints have a greater range of motion than most people’s

2. As a kid, did you ever entertain your friends by contorting your body in weird ways or by doing the splits?

3. Do you consider yourself ‘double-jointed,’ and/or does your family have a history of ‘double-jointedness?’

4. Is their a history of rectal, mitral valve, or uterine prolapse or hernias in your family?

5. Have you ever suffered unexplained joint pain, or joint pain that started out of the blue, aka was not due to any direct injury or trauma?

6. Have you ever failed to receive relief from inflammation related treatments, such as nonsteroidal anti-inflammatory drugs (NSAIDs) or ice?

7. Does stability, such as stabilizing exercises or stabilizing tape or braces, help your joint pain?

8. Do you or your family have a history of thin, fragile, or silky smooth skin, easy bruising, or varicose veins?

9. Have you ever had to ask your dentist or doctor for more lignociane during dental work or minor surgery, because you were not feeling the full anaesthetic effect?

10. Have you or any of the women in your family experienced a flair up of joint pain during or immediately after pregnancy?

- If you answered ‘yes’ to three or more of the above questions, you may want to talk to your doctor about Hypermobility Syndrome

What You Need to Know - Scholarly Articles, Batch #2

"Hypermobility Syndrome" L. Russek (Physical Therapy Journal) 1999
• “HMS has been given relatively little attention in the literature. Most reports are in the rheumatology literature, with virtually none in the orthopedic or physical therapy literatures.”
• HMS patients, however, usually see orthopedists or physical therapists for acute joint pain, and are treated without the underlying problem, aka their HMS, ever being acknowledged.
• “Because HMS lacks a definitive pharmacological or surgical treatment, physicians may have perceived little benefit in its diagnosis.”
• “Individuals may be improperly identified as having hypochondria, as malingering, or as having nonspecific chronic pain, without further investigation into the source of their complaints … Failure to recognize the underlying HMS may lead to unnecessary or inappropriate diagnostic studies, surgical procedures, and patient management.”
• “HMS may be from 1.1 times to 5.5 times more prevalent among females than among men. Hypermobility Syndrome is also more prevalent among Asians than among Africans, ant it is more prevalent in Africans than among Caucasians.”
• “There are neither agreed on criteria nor agreed on scores for the diagnosis of HMS.”
• “Easy bruising and poor wound healing may be seen in patients with HMS as well as patients with EDS or Marfan syndrome.”
• “People with HMS have complaints that have lasted from 15 days to 45 years, and onset of symptoms may occur at any age from 3 to 70 years.”
• HMS patients present “a wide range of traumatic or nontraumatic pain complaints … They typically lack the positive laboratory findings found in rheumatologic disorders and, in the absence of acute trauma, lack the radiological changes, inflammation, swelling, and decreased mobility typical of orthopedic pathology.”
• “People with HMS are more likely (69%) to have anxiety disorders than are comparison groups with rheumatological conditions (22%) … Anxiety may also be due to the perception of joint instability and frequent pain and injury without understandable antecedent.”
• “The high incidence of psychological disorders in people with HMS is similar to that seen with fibromyalgia. Ninety percent of individuals with HMS and fibromyalgia reported sleep disturbances. Because HMS and fibromyalgia appear to be related, the causes of psychological disorders in people with HMS may be similar to those proposed for fibromyalgia: abnormalities in serotonin metabolism, stage IV sleep patterns, and levels of substance P, or perceptual hypervigilance.”
• HMS patients have also reported increased cases of the following; uterine prolapse, rectal prolapse, varicose veins, increased bruising, increased skin elasticity, and decreased skin thickness.
• HMS appears to be a gender-influences dominant trait that causes an abnormality in type I collagen, which is abundant in connective tissues. “In patients with HMS, the ratio of type III collagen to type III + type I collagen is increased. Normally, this ratio is 18%:21%, whereas in patients with HMS, it is 28%:46%.”
• “Nerve tissue also appears to be affected in patients with HMS … Increased mobility and decreased joint position sense could make the joints of people with HMS more vulnerable to damage.”
• “Education is probably the most important treatment that physical therapists can provide to individuals with HMS … education regarding joint protection in HMS may similarly decrease pain, traumatic injury, and subsequent degenerative disease in vulnerable joints. It seems appropriate to advise individuals with HMS in the selection of jobs, sports, or recreational activities that will not exacerbate their condition.”
• “Although exercise will not increase stiffness of the lax ligaments seen in patients with HMS, strengthening and proprioceptive exercises are recommended for musculature surrounding affected joints … Indiscriminant exercise, however, could be harmful … [One should] advise individuals with HMS to use stretching exercises cautiously, distinguishing between stretching muscles and stretching joints.”

"Examination and Treatment of a Patient with HMS" L. Russek (2000)

• “The signs and symptoms of HMS are common among patients seen in orthopedic physical therapy clinics; however, the HMS may be overlooked while treating individual joints or tissues causing pain … Recognition of HMS underlying common orthopedic problems may facilitate appropriate patient education and management.”
• “Although the pathophysiology in HMS is not yet understood, the disorder appears to be a systemic collagen abnormality. The ratio of type I to type III collagen is decreased in skin. Abnormaility in collagen ratios is associated with joint hypermobility and laxity of other tissues … HMS also affects cardiac tissue and smooth muscle in the gastrointestinal system and in the female genital system.”
• What the patient reported
• “Pain usually developed without known cause, persisted for several weeks to several months, and then subsided.”
• Easy bruising, frequent skin cuts that healed slowly, problems sleeping, chronic low-level gastrointestinal irritability, chronic fatigue
• “After several episodes of seeing physicians without receiving diagnoses or recommended treatments, she discontinued seeking medical attention.”
• “Mother had diffuse chronic joint and muscle pain … [and] had been various diagnoses, including osteoarthritis of the knee, sacroiliac dysfunction, and lumbar disk herniation, for her lower-extremity pain.”
• “It is important to identify and address the underlying hypermobility rather than treat the individual symptomatic joints. This is because I believed her symptoms were caused by stresses that exceeded the hypermobile tissues’ ability to resist.”
• “On the one hand, there is no cure for the disorder. The goal for treatment, therefore, is not to return to ‘normal’ (ie, not hypermobile) joint mobility but restoration of relatively pain-free function. That is, treatment does not eliminate the underlying impairment of excessive mobility.”
• “Patients with HMS can function and their quality of life often can be improved with treatment but they will usually have chronic or recurrent problems.”
• “The primary emphasis of intervention with this patient was education about the syndrome, about body mechanics and joint protection, and about lifestyle modification.”
• “Although research on joint protection has not been done on HMS, the joint instability in HMS is similar to that seen in the active phase of rheumatoid arthritis.”
• “[The patient] reported that activity modification protecting one joint sometimes transferred stress to other joints, which subsequently became symptomatic. Therefore, she was unable to avoid stress to all joints all of the time.”
• “She reported having had continued difficulty sleeping … [and] was prescribed low doses of notriptyline (a tricyclic antidepressant that acts as a serotonin reuptake inhibitor) … Sleep disturbance, although seldom studied in patients with HMS, may be common: 90% of individuals with both HMS and fibromyalgia reported sleep disturbances … HMS might share some of the physiological abnormalities, such as the decreased cerebrospinal fluid serotonin levels, seen in fibromyalgia.”
• “Because she knew the limitations of what the medical community could do for her disorder, she decreased use of health care services that she knew would be of no benefit.”

"BJHS: Evaluation, Diagnosis, and Management" M. Simpson (US Army Health Clinic) 2005

• “Although BHJS has been well recognized in the rheumatology and orthopedic literature, it has not been discussed in the family medicine literature.”
• “When patients with this syndrome are first seen by a physician, their chief complaint is joint pain, so BJHS can be easily overlooked and not considered in the differential diagnosis.”
• “In general, women have greater joint laxity than men, and up to 5% of healthy women have symptomatic joint hypermobility compared with 0.6% of men. People of African, Asian, and Middle Eastern descent also have increased joint laxity.”
• “First-degree relatives with the disorder can be identified in as many as 50% of cases.”
• Patients with BJHS present with joint pain because “excessive joint laxity leads to wear and tear on joint surfaces and strains or fatigues the soft tissue surrounding these joints … [proprioception] impairment can also lead to excessive joint trauma due to impaired feedback from the joint affected.”
• “The onset of symptoms can occur at any age … Physical activity or repetitive use of the affected joint often exacerbates the pain. Consequently, pain usually occurs later in the day and morning stiffness is uncommon.”
• “Patients with BJHS often say that they are ‘double-jointed’ … or have a family history of ‘double-jointedness.’ Other presentations include easy bruising, ligament or tendon rupture … [and] thin skin.”
• “Diagnoses of BJHS is one of exclusion.”
• “BJHS is thought to be a mild variation of EDS and most closely resembles EDS type III … researchers have suggested that BJHS lies on a continuum with EDS and may be its mildest form because of their overlapping features.”
• Effective treatment may include lifestyle modification, joint protection and stabilization exercises, proper body mechanics, and improving proprioception
• “Hypermobility is a common cause of unexplained joint pain, yet is often misdiagnosed in primary care. According to one source, primary care providers recognized generalized hypermobility in less than 10% of patients with generalized hypermobility who were referred to rheumatologists.”
• “The potential complications of BJHS underscore the importance of making an early diagnosis and educating the patient … a prompt diagnosis improves pain control and decreases disruptions in these patients’ physical activities, school, work, and quality of life.”

"Genetic basis of joint hypermobility syndromes" F. Malfait, A. Hakim, A. De Paepe, R. Grahame – (2005)

• “Rheumatologists have long considered that joint hypermobility is inherited … Later it became apparent that connective tissues other than joints, such as skin, bone and eye, participated in the connective tissue fragility seen in JHS.”
• “It became evident that JHS is itself an under-recognized form of an heritable disorder of connective tissue (HDCT). Patients with JHS can present mild expression of marfanoid habitus, osteoporosis, blue sclerae, skin hyperextensibility, atrophic scarring or easy bruising. Since these features can also be present in the most common form of EDS, the hypermobility type (EDS III), it seems increasingly likely that JHS is, if not identical, indistinguishable from the hypermobility type of EDS.”
• “To date, the genetic basis of joint hypermobility remains largely unknown … Reports of genetic studies in patients and/or families with EDS, hypermobility type, or with JHS are scarce.”
• “Classic EDS is characterized by soft and velvety skin, abnormal scar formation and marked joint hypermobility. The joint hypermobility is usually generalized, affecting both large and small joints, and it can range in severity from mild to severe, with major articulate complications such as habitual subluxation and dislocation of the joints.”

"Time to take Hypermobility Seriously" R. Grahame (Brit Soc of Rheum) 2001

• “From the outset it was perceived more as a curiosity than as an entity that could have significant (let alone serious) import”
• “There is now abundant evidence … that hypermobility can have a serious impact on people’s lives”
• “Patients with BJHS have diminished joint proprioceptive acuity and are less responsive than normal to the local anaesthetic effects of lignocaine”
• “Failure to receive effective intervention not infrequently results in a complicating chronic pain syndrome, fibromyalgia, and depression”
• There appears to be a total lack of consensus on what criteria to use in diagnosis … a distinct lack or familiarity with recently published literature, in particular on the question of the impact on patient’s lives in terms of the effects of chronic pain and the psychosocial sequelae”
• “BJHS is not an easy condition to treat. Physiotherapy forms the mainstay of treatment but has to be tailored to the needs of intrinsically vulnerable tissues, otherwise it may aggravate rather than relieve. Analgesics and non-steroidal anti-inflammatory drugs are generally ineffective, and surgery can be counterproductive”

"British Consultant Rheumatologists' Perceptions of the Hypermobility Syndrome: A National Survey" R. Grahame, H. Bird (UCL) 2001

• Joint hypermobility is a condition considered controversial by rheumatologists
• 92% believed in HMS as a “distinct clinical entity” but only 39% accepted it as a “distinct pathological entity.”
• Only 42% would comment on whether HMS and EDS III (hypermobility type) are one in the same, and only 14% believed it was true
• 50% were skeptical that HMS has “a significant impact” on people’s lives
• “The only logical explanation for these perceived differences [in opinion] is the failure on the part of many consultants to recognize the presence of hypermobility in their patients.”
• “Joint hypermobility is easy to recognize if our looks for it, but equally easy to overlook if one does not.”
• “In terms of the clinical consequences of hypermobility – pain, instability, dislocation, etc. – which may occur in a single lax joint, it matters not how many other joints may or may not be affected. It is clearly illogical to deny a patient a (correct) diagnosis of BJHS” simply because they do not show sufficient hypermobility in the rest of their joints
• “A striking finding was that 61% [of rheumatologists] required a negative laboratory screen before they would be prepared to summon up significant courage to make a diagnosis of BJHS. This smacks of defensive medicine. It is due to a lack of confidence in one’s clinical findings, a need to make such a diagnosis by exclusion rather than by inclusion, or does it signify a lack of conviction in the very existence of the entity itself?”
• “The low perception of the impact of the syndrome on individuals’ lives … is challenged by the patients themselves, and is contrary to published findings.”
• “This survey confirms previous suspicions that the BJHS is a condition that is under-recognized and under-estimated by rheumatologists. There was little sign of awareness of the findings of recent published studies.”

"Living with the Hypermobility Syndrome" S. Gurley-Green (HMSA) 2001

• A majority of HMS patients feel that their doctors don’t understand the impact HMA has on their lives, nor is the medical community able to provide effective treatment
• There are 2 types of HMS patients 1) Those who suffer periods of pain (for a few weeks) but can be symptom free for some time 2) Those for whom every day is a struggle against pain
• Literature suggests symptoms decrease with age, but the stiffening with age does not always mean less pain, and many patients feel their condition deteriorates over time, especially after pregnancy
• There is currently no effective treatment for the majority of patients, for whom pain is sometimes unbearable
• HMS makes performing in the workplace and caring for a family extremely difficult; for some the daily tasks of living, like grocery shopping and laundry, are too much
• Parents are often afraid of passing HMS to their children, and many women are worried that they will not be able to meet the demands of motherhood
• Many patients are accused of having psychosomatic pain because there is often no clear accident or trauma that coincides with the start of the pain, so doctors have a hard time taking the patient’s complaints seriously
• The problem of chronic pain is exacerbated because there is often no link between actions and the onset of pain, so it is difficult to lower pain through behavioral modification
• Many patients see multiple doctors, and suffer from the lack of continuity in treatment and conflicting advice

Scholarly Articles - Batch #2

Below are links to some great articles concerning HMS.

1) “Hypermobility Syndrome” L Russek

2)
“Examination and Treatment of a Patient with Hypermobility Syndrome” L Russek

3) “Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management” M. Simpson

4) “The Genetic Basis of the joint hypermobility syndromes” F. Malfait, A. Hakim, A. De Paepe, R. Grahame

5) “Time to Take Hypermobility Seriously” R. Grahame

6) “British Consultant Rheumatologists’ Perceptions about the Hypermobility Syndrome: A National Survey” R. Grahame, H. Bird

7) “Living with the Hypermobility Syndrome” S. Gurley-Green

8) “Is the Benign Joint Hypermobility Syndrome Benign? H. El-Shahaly, A. El-Sherif

What You Need to Know - Scholarly Articles, Batch #1

R. Grahame (UCL Hospitals) HMS and Pregnancy
• Symptoms of HMS during pregnancy include,
o Joint and spinal pains increase
o Tendency to premature labour and delivery
o Tendency to rapid labour
o Resistance to effects of local anaesthetics can cause problems during the epidural
o Healing of tear or episiotomy may be impaired and/or prolonged
o Pelvic floor problems (especially uterine prolapse), therefore post-natal exercise is very important
o 50% chance child will have HMS


Larson, Baum, Mudholkar 1981
• “Hypermobility was a predominantly female characteristic”
• Joint laxity declines naturally with age, but “not to a statistically significant degree”
• In men, joint laxity started to decline in mid-twenties, in women it continued through mid-forties


Bridges, Smith, Reid
• Most patients with hypermobility had common musculoskeletal problems as the main reason for their referral (usual to a rheumatologist)
• “Results show that joint hypermobility is common, familial, found in association with common rheumatic disorders, and statistically associated with osteoarthritis”
• “The findings support the hypothesis that joint hypermobility predisposes to musculoskeletal disorders, especially osteoarthritis”

R Graham (UCL Hospitals) Pain and Hypermobility Syndrome 2009
• “At the present time we simply do not know for certain whether or not HMS is a less severe type of Ehlers-Danlos Syndrome III.”
• “Usually unexpected exercise or a change of job or lifestyle provokes the onset of pain. Whenever symptoms commence … the term ‘Hypermobility Syndrome’ is used … hypermobile people without pain are just called hypermobile people.”
• “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?”
o A hypermobile person has an inbuilt weakness if their “strength-providing” collagen. Injuries occur “whenever there is a mismatch between the physical demands on one hand, and the strength of the parts being asked to perform on the other… If one only knew one’s strength (or lack of it), one should be able, in theory at least, to stay within the safety margin and thereby protect oneself from injury.”
• “Neither susceptibility to injury or overuse … explains all the pain that is felt in HMS.”
o Pain is a subjective experience, and “the severity of the pain we feel is greatly influenced by our state of mind.”
o “Lack of understanding of the condition is widespread, and this, coupled with failure to receive adequate treatment for relief of symptoms, leads to frustration, resentment, anger, and ultimately depression.”
o HMS and fibromyalgia occur together quite often, and “fibromyalgia should be regarded as a signal emanating from a distressed HMS person.”
• There may be two clues to explaining the burden of pain bourn by HMS patients
o First, people with HMS have diminished proprioception, that is to say, “people with HMS 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.”
o Secondly, “patients with HMS 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.”
o The above “suggest that people with HMS … may also have a fault in the way their pain signals are picked up for onward transmission to the brain, where they reach consciousness.”

Howes, Isdale (New Zealand)
• They studies 102 cases of backache – 59 men, 43 women. Out of the 59 men, none had hypermobility, but rather had disc lesions, spondyiolysis, etc. One half of the 43 women suffered from those same ailments, while the majority of the remainder suffered from HMS
• “It is suggested that this is an important differential diagnosis of backache in women”

Scholarly Articles - Batch #1

Pregnancy and JHS/EDSHM

RODNEY GRAHAME CBE, MD, FRCP, FACP
Emeritus Professor of Rheumatology, University College Hospital, LONDON.

Unlike the vascular form of the Ehlers-Danlos Syndrome ( EDS ), formerly EDS Type IV, the Joint Hypermobility Syndrome (JHS), which is equivalent to EDS–hypermobility type, formerly EDS III ) is not associated with heart disease or major hazards during pregnancy and labour. However there are a number of considerations that should be borne in mind, which are listed here:

• Joint and spinal pains may increase during the course of the pregnancy.
• There is a tendency to premature rupture of the membranes and thus of premature labour and delivery.
• There is a tendency to rapid labour.
• There is an apparent resistance to the effects of local anaesthetics is seen in about two thirds of patients and can cause problems for the unwary during epidural anaesthesia or infiltration for repair of a tear of episiotomy.
• Healing of tear or episiotomy may be impaired and/or prolonged and surgical technique may need to be modified accordingly.
• She may find lactation and care of the newborn baby more taxing than most.
• Pelvic floor problems (uterine prolapse etc) may occur in later life so that the practice of post-natal exercises is particularly important.
• Since JHS follows a dominant pattern of inheritance there is a 50% chance that any offspring will carry the gene, although this does not mean that he/she will necessary develop symptoms of tissue laxity subsequently.

Hypermobility: features and differential incidence between the sexes.
Larsson LG, Baum J, Mudholkar GS.

Department of Medicine, Monroe Community Hospital, Rochester, NY 14603.

Six hundred sixty individuals from a music school were studied to determine the frequency of incidence and the nature of their hypermobility. They were interviewed and examined for the 5 recognized features of hypermobility (laxity of the thumbs, fingers, elbows, spine, and knees). The incidence of hypermobility, according to the number of joints involved, followed an empiric geometric law. Approximately 27% of the individuals had 1 lax joint, whereas only 3% possessed all 5 features. Specific features were present at different frequencies in the 2 sexes. The ratio of the occurrence of 2 features in women compared with the occurrence in men was 2:1. Ratios for the occurrence of 3, 4, and 5 features were 4:1, 8:1, and 3:1, respectively. Thus, hypermobility was a predominantly female characteristic. Joint laxity declined with age, although not to a statistically significant degree. In men, the decline started when they were in their mid-twenties; however, in women, joint laxity continued through the mid-forties.

PMID: 3435571 [PubMed - indexed for MEDLINE]

Annals of the Rheumatic Diseases 1981;40:541-546; doi:10.1136/ard.40.6.541

Copyright © 1981 BMJ Publishing Group Ltd & European League Against Rheumatism.

A clinical and echocardiographic study of patients with the hypermobility syndrome.

R Grahame, J C Edwards, D Pitcher, A Gabell and W Harvey

Three age- and symptom-matched groups of patients with a hypermobility score of 5-9, 3-4, and 0-2 (controls), respectively, were examined for clinical and echocardiographic evidence of mitral valve prolapse and other stigmata of a collagen disorder. Mitral valve prolapse, a reduced upper segment/lower segment mitral ratio, reduced skin thickness, spinal anomalies, and a history of fracture were found to be significantly commoner among the hypermobile patients than the controls. The data suggest that the so-called hypermobility syndrome, far from being a benign locomotor disorder of healthy persons, may be a forme fruste of a hereditary disorder of connective tissue.

Annals of the Rheumatic Diseases 1992;51:793-796; doi:10.1136/ard.51.6.793

Copyright © 1992 BMJ Publishing Group Ltd & European League Against Rheumatism.

Joint hypermobility in adults referred to rheumatology clinics.

A J Bridges, E Smith and J Reid

Department of Medicine, University of Missouri School of Medicine, Columbia.

Joint hypermobility is a rarely recognised aetiology for focal or diffuse musculoskeletal symptoms. To assess the occurrence and importance of joint hypermobility in adult patients referred to a rheumatologist, we prospectively evaluated 130 consecutive new patients for joint hypermobility. Twenty women (15%) had joint hypermobility at three or more locations (greater than or equal to 5 points on a 9 point scale). Most patients with joint hypermobility had common musculoskeletal problems as the reason for referral. Two patients referred with a diagnosis of rheumatoid arthritis were correctly reassigned a diagnosis of hypermobility syndrome. Three patients with systemic lupus erythematosus had diffuse joint hypermobility. There was a statistically significant association between diffuse joint hypermobility and osteoarthritis. Most patients (65%) had first degree family members with a history of joint hypermobility. These results show that joint hypermobility is common, familial, found in association with common rheumatic disorders, and statistically associated with osteoarthritis. The findings support the hypothesis that joint hypermobility predisposes to musculoskeletal disorders, especially osteoarthritis.

Pain & the Hypermobility Syndrome

RODNEY GRAHAME CBE, MD, FRCP, FACP
Emeritus Professor of Rheumatology, University College Hospital, LONDON.

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 whether or not HMS is merely a less severe type of EDS III. Pain can occur in other forms. For the rest of this article, in order to avoid confusion, I will refer to it as one condition, the HMS/EDS. 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.

A little known fact is that hypermobility occurs in many individuals in a few joints only. It does not necessarily have to affect all one's joints. Even hypermobility in a single joint can cause pain and/or instability in that joint; the diagnosis is still Hypermobility Syndrome (HMS/EDS).

People are born hypermobile. It is in their genes. It is the way they are made. 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. We can explain a lot of the pain that arises on the basis of a series of (often quite minor) injuries that occur whenever there is a mismatch between the physical demands on one hand, and the strength of the parts being asked to perform them on the other. 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. If one only knew one's strength (or lack of it), one should be able, in theory at least, to stay within the safety margin and thereby protect oneself from injury. Up to a point this is so. Many people with the HMS/EDS are able to modify their lifestyle and do just that. Others find it more difficult.

A joint that is beginning to wear starts to lose its cartilage or gristle, which is essential for the smooth movement between the adjoining bones. This wear heralds the onset of osteoarthritis, a condition painful in its own right. It is a very common form of arthritis in middle and old age, and one to which hypermobile people seem to be prone, in many cases at an early age. It is important to establish as far as possible to what extent the pain is due to trauma/overuse or to early onset osteoarthritis, as the treatment is very different. The emphasis with the former is on prevention, while with the latter it is on treatment by one form or another.

However, neither susceptibility to injury or overuse (repetitive injury), nor osteoarthritis, explains all the pain that is felt in the HMS/EDS. There is more to it than that.

"Emotional influences can amplify pain, but they do not cause it."

Let us try to piece the jigsaw together. Pain is something we feel. Even if it has a physical cause, as it undoubtedly has in the HMS/EDS, it is still a subjective experience. It is often accompanied by an intense sense of exhaustion. the severity of the pain we feel is greatly influenced by our state of mind. If we are upset or agitated it tends to increase. If we are content, relaxed or just happy it tends to diminish. The HMS/EDS people are often in the former category, and for good reason! Lack of understanding of the condition is widespread, and this, coupled with failure to receive adequate treatment for relief of symptoms, leads to frustration, resentment, anger (and lots more emotions which I could list but readers know them all only too well!) and, ultimately depression. These emotional influences can amplify pain, but they do not cause it.

Another piece of the jigsaw is a mysterious condition called fibromyalgia. This condition causes widespread chronic pain in muscles and is identified by the finding of multiple 'tender points' at specific sites in the body. For years the debate has raged as to whether it is a physical disease affecting the muscles, or whether it is an emotional disorder. The prevailing view is that it is probably some form of distress signal that can arise in people with a number of different and unrelated conditions. How this relates to hypermobility is that it has been shown that hypermobility and fibromyalgia occur together in the same person more often than one would expect on the basis of a chance happening. It does not mean that they are part of the same condition. It would, indeed be very unlikely that what is clearly an acquired disorder (fibromyalgia) could be an integral part of what is clearly a genetic one (HMS/EDS). More likely, the fibromyalgia should be regarded as a signal emanating from a distressed HMS/EDS person. But fibromyalgia occurs only uncommonly in the HMS/EDS. We must look further afield.

It seems quite likely that there may be a totally different explanation for the burden of pain borne by people with the HMS/EDS. Here are two clues:

The first clue relates to the 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.

The second clue is the discovery that 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 (I wonder how many readers are nodding their heads as they read this!).

At the present time we do not know quite what either of these clues means or whether they relate to one another. But they do suggest that people with the HMS/EDS, in addition to their proneness to injury, dislocation and osteoarthritis, may also have a fault in the way their pain signals are picked up for onward transmission to the brain, where they reach conciousness. Research work is in progress to try to sort out this enigma. Much more needs to be done. Watch this space!

Readers 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. 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.

Revised 15 March 2009

THE LOOSE BACK: AN UNRECOGNIZED SYNDROME

R. G. HOWES and I. C. ISDALE

From the Queen Elizabeth Hospital Rotorua, New Zealand

A prospective study of 102 cases of backache with particular regard to the presence of ligamentous laxity is reported. Among the 59 men there was no excess of cases showing unusual spinal or peripheral joint mobility; nearly all had features of disc lesions, spondylolysis, spondylosis, or spondylitis. In contrast, only half of the 43 women could be diagnosed as suffering from these conditions, while the majority of the remainder showed central and peripheral hypermobility. The clinical features of the "loose back syndrome" are outlined. It is suggested that this is an important differential diagnosis of backache in women.

Welcome to Hypermobility Hope!

Hi everyone and welcome to Hypermobility Hope!
The purpose of this site to to raise awareness about Hypermobility Syndrome, and to give those who suffer from HMS, or know someone who has HMS, or just think they might have HMS, the information they need to make sure they receive proper care. As a HMS patient myself, I know how frustrating it is to find doctors who will listen to you, who understand how difficult and destructive HMS can be, and who genunuinly respect the implications and complications HMS can have on daily life.
I have tried to create the kind of site that I wish I had been able to find - a compassionate and welcoming environment where one can easily find explinations, information, advice and comradery. I have done extensive research into HMS, and I have provided links to as much information as I could find. Along with the links to scholarly journals I have provided cheatsheets that will tell you what is important and what you need to know from that particular article. I also have attached a fact sheet and diagnostic question list that will help you learn what you need to know about HMS in the most painless way possible. I also hope to upload video interviews with various doctors and other HMS patients.
In addition to general HMS information, I will be sharing my own personal journey with HMS in the hopes that others out there with HMS will be able to relate to my pain, anger, and lonliness, and will be able to glean some hope knowing that they are not alone in this fight.
The medical community has ignored Hypermobility Syndrome since its appearence in medical journals in 1967, and most doctors view HMS more as a curiosity than a serious medical condition. It is my mission to make them see that HMS is a real condition with disasterously negative affects on patients, and that it is time to do something about it!
Please feel free to post comments or suggestions, it is up to us to raise awareness, our fate is in our own hands.
Remember, Awareness = Power + Hope!
Always,
Chelsea