The stones in your life

As all doctors will recall, the WHO definition of health is
Health is a state of complete physical, mental and social well-being and not merely the absence of disease or infirmity.
Like health, there is more to "healing" than just medicines or surgery or therapies. You can be deeply unhappy, and therefore unhealthy, even when you have good money, a good job, a good family and good physical health.

This is the image of a cairn, a pile of stones that stands balanced beautifully. Only, this is the cairn of health as described by Dr. Lissa Rankin, the person behind the "whole health" approach.

Yoga - a double edged sword in H-EDS/JHS

Yoga is one of the most effective system of fitness approaches to not just physical but also mental well-being. If you have H-EDS/JHS and suffer from chronic musculoskeletal pain, there is a likelihood of you gravitating to yoga at some point in your life to explore its curative claims. And if you are young as well, you will find yourself rather good at doing the difficult poses - fostering your belief that you are doing something good for your body and yoga will help your condition.

But a person with H-EDS/JHS is likely to do more damage than good to his body by yoga - not because yoga is bad for them but because they are more likely to be doing it wrong.

Indian Articles: Joint hypermobility in Indian children

"Joint hypermobility in Indian children." 

R. P. Hasija, R. P. Khubchandani, and S. Shenoi.
Clinical & Experimental Rheumatology 26.1 (2008): 146.

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Key Takeaway:
The most important finding of this article is the correlation between nutritional status and the incidence of hypermobility in children.

This is an article published in the journal Clinical and Experimental Rheumatology in 2008. The study used the currently accepted Beighton scoring system for assessing hypermobility of joints in 829 children in the age range 3-19 years from an urban low socio-economic strata in Mumbai. 734 children of these had moderate to severe malnutrition. 

It is one of the first two Indian studies that have assessed the prevalence of generalized joint hypermobility (gJHM) in Indian pediatric populations (the other one being this)/ More importantly, the present article explores the link between the present of gJHM and malnutrition. The study found a a prevalence of 58.7% joint hypermobility in the children and a positive correlation between the presence of malnutrition and joint hypermobility.

The article compares the results of other Indian and international studies on the prevalence of joint hypermobility in different population groups. The comparison results are divided into two groups, based on the hypermobility assessment method used (Beighton score and other methods).

The study showed that in addition to known demographic (e.g., sex), pathologic (e.g., parathyroid dysfunction) and lifestyle (e.g., training) factors that influence joint hypermobility, moderate or severe malnutrition can influence joint hypermobility.

The authors conclude:
"Our study reiterates the belief of other workers that ‘It is time to take hypermobility seriously’ [Ref]. There is a strong association with musculoskeletal symptoms. Children complaining of these musculoskeletal symptoms do not get the attention they deserve, largely because of scant physician awareness." 

The article has been cited many times worldwide in relation to this relation between nutritional status and JHM, and an Italian geneticist, Dr. Marco Castori, citing the article writes

The understanding of epigenetic effects of dietary supplementations in both health and disease in humans is in its infancy. Nevertheless, a couple of papers in the field of gJHM put the basis for possible future studies. In particular, Hasija and et al. [2008] demonstrate a direct relationship between degree of generalized gJHM and nutritional status in Indian children. This study envisages that a healthy and balanced diet may improve the range of joint motion also in the symptomatic pediatric patient, with or without a pre-existing diagnosis of EDS.



Ref: GRAHAME R: Time to take hypermobility seriously (in adults and children). Rheumatology (Oxford) 2001; 40: 485-7

Indian Articles: Brief Report on Joint Hypermobility in South Indian Children

"Joint hypermobility in south Indian children." 

Subramanyam, Vedavati, and K. V. Janaki. Indian pediatrics 33 (1996): 771-771.

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This article reports the first Indian study that studied the prevalence of joint hypermobility in pediatric population in India. One thousand children between the ages of 6 and 15 years in Madras were studied. There were equal number of boys and girls. Presence of grades III or IV malnutrition, Down's syndrome, Ehlers Danlos syndrome or any other musculoskeletal disorders were exclusion criteria. The researchers used the Carter-Wilkinson's scoring method for evaluating hypermobility of joints.

Results: The overall prevalence of benign hypermobility of the joints for both boys and girls was 17.2%.  In the 6-10 years group, 65% boys and 35% girls had hypermobility. In the age group 11 to 15 years, 41% and 59% girls had hypermobility.

~~~

The study assesses presence of joint hypermobility in children who had not been diagnosed with any connective tissue disorder. Different evaluation criteria, different age-groups of subjects, sample sizes, different ethnicity and other demographic factors lead to different results regarding the prevalence of joint hypermobility. It is generally accepted that joint hypermobility is common in children and it decreases with age.

It would be interesting to evaluate joint hypermobility in children and interviewing and assessing the parents of children who have hypermobile joints for the presence of H-EDS/JHS.

Joint hypermobility and Sports






Did you know that Sania Mirza has hypermobility of her wrist joints?

Not Joint Hypermobility Syndrome (JHS), but hypermobility of joints (JHM).
JHM is not a disease. It is often seen in athletes, dancers and yoga professionals. It can even provide an advantage to athletes. It can also lead to more injuries.

Most children who have JHM do not go on to be diagnosed with JHS. However, if their JHM is causing joint pain or injuries (see this paper*) the child should receive a specific management strategy. However, simply the incidental presence of JHM does not mean the child should stop sport activities. Good posture and gait training is always valuable. So are swimming and other core strengthening exercises. In fact a child should be encouraged to stay active to increase muscle flexibility (not joint, muscle flexibility!) and strength.

Advice the child with JHM not to show off their hypermobility by doing "tricks". They should also avoid obesity. They should be advised not to sit in W position.

If your child has been diagnosed with any kind or degree of hypermobility of joints, see this excellent article:
Joint hypermobility in children: what is it and how it affects fitness and motor skills 

Read more about management for hypermobile children here:
Hypermobile: Musculoskeletal conditions - The hypermobile child

In case of H-EDS/JHS, specific training program and advice required to prevent injuries and strengthen muscles. Contact sports may be avoided. See detailed information for a child with JHS here: http://hypermobility.org/help-advice/physio-and-occupational-therapy/flexible-kids/

If someone has Ehlers Danlos Syndrome Hypermobility type(H-EDS) /JHS, they require a more comprehensive approach for effective pain and health management, prevention of disability and maintenance of quality of life. Do not let anyone tell you that it is nothing and is only "benign".

However, JHM is not the same as JHS/H-EDS. A hypermobile joint can be prone to injury, and in case of widespread hypermobility, the child will have many other problems which need to be addressed by more than just pain medication. It is good to consult a rheumatologist for reassessment as advised. If your child has only JHM, it can be managed well enough to not cause any serious problems in the long run.

Let your child stay active, stay strong, and stay "smart" fit!
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
*Shanmugapriya, V., et al. "A ‘benign’condition masquerading as arthritis." BMJ case reports 2013 (2013): bcr2013010518.

Indian Articles: JHS in Indian Military Population


This is the first study on BJHS carried out in a Rheumatology Centre of the India Military Services.

Clinical profile of benign joint hypermobility syndrome from a tertiary care military hospital in India


Mullick, Gautam, et al. "Clinical profile of benign joint hypermobility syndrome from a tertiary care military hospital in India." International journal of rheumatic diseases 16.5 (2013): 590-594.

View PDF


Summary of the article: Eighty four army personnel diagnosed with BHJS were studied for demographic, clinical and other epidemiological details. The male to female ratio was 1.15 : 1, which is markedly different from the female preponderance observed in world over. The authors do not discuss this, but I think it is related to the preponderance of males in the armed forces. There was knee joint pain in all the patients, possibly because knee is a frequent site of injury in military training. The authors mention a "lateral head tilt" sign they observed in some patients, which could be attributable to a hypermobile cervical spine.


The authors conclude:
BJHS is often under-recognized in clinical practice and is usually missed because of a lack of awareness. A high index of clinical suspicion to diagnose this entity is essential due to its associated morbidities, especially among those exposed to strenuous physical activities

The case for renaming Hypermobility Syndrome

Hypermobility of joints (JHM) is not a disease. It is a feature of joints in which the range of movement at the joint is more than expected or normal. It can arise from many factors, genetic and acquired.

What is knows as the hypermobility syndrome has hypermobility of certain joints (defined by the Beighton score) as a component feature when the person is young. As the person grows older and joints become stiff, they may no longer have the "hypermobility of joints" as a clinical diagnostic indicator.

There is a growing group of experts that thinks that the names like BJHS, JHS, HMS, HS, etc should be replaced by the name Ehlers-Danlos Syndrome Hypermobility Type.






The hypermobility of the joints per se is not responsible for most of the musculoskeletal symptoms (only joint damage as a result of hyperextension is due to the hypermobility of the joints) and the myriad multisystemic manifestations. The defective connective tissue, arising from defect in the genetic code for collagens and possibly other proteins is responsible for the problems in the multiple bodily systems. The defect in the genetic coding for collagen in this condition is thought to result in an altered ratio of the different subtypes of collagen.




Many experts believe that there is a lack of clinical distinction between JHS and H-EDS and both are the variable expressions of the same disease [1].

The manifestations and severity of H-EDS/JHS can differ from patient to patient. There is no fixed set or severity of symptoms that can describe H-EDS/JHS. However, typically when there are few or mild symptoms, the person is diagnosed as Benign Joint Hypermobility Syndrome. When the same disease process leads to much severe course of disease with multisystem manifestations and recurrent dislocations of joints (partial or total), the person is likely to receive a diagnosis of Ehlers Danlos Syndrome Hypermobility Type (H-EDS or EDS-HT). There is a wide disparity between the accepted prevalence rates of H-EDS and JHM. It is likely that H-EDS patients are one of the extreme ends of this spectrum and there is a large pool of undiagnosed and untreated patients of this disease who have an intermediate range of severity.




Joint hypermobility syndrome immediately conjures up a prejudiced/ assumptive image of :


a "benign" disease (mentally related to JHM), 
consisting only of "flexible" joints, 
and
without "serious" complications or implications

This is a complete fallacy. 

Patients with H-EDS, in fact:


have considerable morbidity (proportion of sickness),
may not have hypermobility of joints,
and 
may have many serious complications. 

As Prof. Bravo writes in his article,
 Furthermore, and very important, when we speak of Joint Hypermobility Syndrome we do not give the subject the importance it deserves, since, at a glance, it implies a good condition to have, rather than a disease with frequent complications. This has brought us to conclude that the name that we have been using, diverts the awareness of this disease, and is the reason for the lack of interest by physicians and poor understanding of this disease by patients and public in general.

Often the H-EDS/JHS patient has a variety of seemingly unrelated multisystemic complaints and the clinical signs are not pronounced. There are no confirmatory laboratory or genetic tests that can diagnose H-EDS. Thus this condition remains sereiously underdiagnosed causing damaging impact on the patients' lives. The patient suffers from a variety of health problems and yet goes through life being dismissed by doctors. relatives and friends. The chronic pain, fatigue, and other neurological features lead to disability and yet the patient receives no long-term relief or management or support.

Therefore, to increase the awareness regarding H-EDS/JHS, there are two essential and interrelated steps:

1. Shift the attention of the clinical picture of just musculoskeletal and skin involvement to the actual chronology of symptom development, transition and evolution of this multisystemic disorder [2]. 


2. Rename Joint Hypermobility Syndrome to Ehlers Danlos Syndrome Hypermobility Type. 







References:

1. Tinkle, Brad T., et al. "The lack of clinical distinction between the hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome (aka hypermobility syndrome)." Am J Med Genet A 149.11 (2009): 2368-70.

PDF 

2. Castori, Marco, et al. "Re‐writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers–Danlos syndrome, hypermobility type." American Journal of Medical Genetics Part A 161.12 (2013): 2989-3004.

PDF 

3. Read the interesting story of how there came to be two different names for this disease and how the work on two developed divergently in the same city, creating this chasm, here:

Grahame, Rodney. "Joint hypermobility: emerging disease or illness behaviour?." Clinical Medicine 13.Suppl 6 (2013): s50-s52.

Is Ehlers-Danlos Syndrome rare – or just rarely diagnosed?

Is Ehlers-Danlos Syndrome rare – or just rarely diagnosed? 

Only 5% of Ehlers-Danlos Syndrome (EDS) sufferers are correctly diagnosed*.

I read this article by Jan Groh, EDS patient & support group leader,
An excerpt:
It’s been estimated that EDS affects more than 1 in 5,000 people worldwide. But, experts think that’s conservative. Each year in the US, 650,000* additional sufferers are un-diagnosed or misdiagnosed due to physician oversight or lack of knowledge about EDS. (*Estimate is according to a study in the UK by Dr. Rodney Grahame.) 
This led me to ask, what exactly is the number of people who might be affected with Ehlers Danlos Syndrome Hypermobility Type (H-EDS) in India?

Dr. Marco Castori writes [1]:
The epidemiology of JHS/EDS-HT has not been properly established. Literature defines EDS a rare disease with a cumulative frequency of 1/5,000 [Steinmann et al., 2002], with JHS/EDS-HT, EDS classic, and vascular types being the most common forms of EDS. Nevertheless, clinical practice suggests a much higher prevalence of JHS/EDS-HT with a proposed frequency of 0.75–2% in the general population [Hakim and Sahota, 2006]. Hence, the real prevalence of JHS/EDS-HT is unknown but likely ranges from ~ 1% to 1/5,000. 

With our population of over a billion, and Indians known to having a higher prevalence rate than caucasians, what do you think is the number of people affected in India?

According to the range proposed by Dr. Castori, the number of H-EDS/JHS patients in India is


1,00,00,000 to 2,00,000 (1 Crores to 2 Lakhs) patients in India


According to Dr. Derek Neilson, the incidence of EDS-HT or H-EDS is closer to 1/100 to 1/500 [2] which translates to

1,00,00,000 to 20,00,000 (1 Crores to 20 Lakhs) patients in India


This is not a rare disease.
~~~~~~~~~~~~~~~~~

Reference

[1] Castori, Marco, et al. "Re‐writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers–Danlos syndrome, hypermobility type." American Journal of Medical Genetics Part A 161.12 (2013): 2989-3004.

Indian Articles: How are JHS and POTS related?

"Somehow our devils are never quite what we expect when we meet them face to face". Nelson DeMille.
"The mind can cook up very subtle syndromes to throw at our bodies". Astrid Alauda.
The varying clinical presentations of not so well recognized diseases, which continue to haunt patients is well described in the above quotes.
Those are the opening lines of the article, "Joint Hypermobility Syndrome and Dysautonomia: Expanding Spectrum of Disease Presentation and Manifestation", by Dr. Shomu Bohora, Baroda Heart Institute and Research Centre, Vadodara, India. [Indian Pacing Electrophysiol J. 2010; 10(4): 158–161. Published online 2010 Apr 1.] 

The author also directs our attention to another article by a US group in the same issue of the journal, "Comparative Clinical Profile of Postural Orthostatic Tachycardia Patients With and Without Joint Hypermobility Syndrome" by Khalil Kanjwal et al. 

The author comments: 
Based on the present study by Kanjwal et al, it may be appropriate that all young patients who present with symptoms of migraine or syncope, who have symptoms and orthostatic stress suggesting POTS should also be screened for hypermobility of joints and for JHS.
 I found this interesting because I have migraine and I also have low blood pressure (usually 100-110/ 60-70) and occasional palpitations. When I was 20 years old, I consulted a physician for migraine and he gave me Propranolol which made my blood pressure drop to 90/ 56 mm Hg. I stopped the medicine after a few months.

I often wonder how would my life be if I was diagnosed when I was a teenager. Who would I be today? How would I be today? 


Note: I was elated to see the author has used the name JHS and not BJHS in his article.
 


Indian Articles: Benign Joint Hypermobility Syndrome – not so ‘Benign’

This is a recent and interesting Indian case report by rheumatologists at the P.D. Hinduja Hospital and Research Centre, Mumbai.

Indian Articles: Benign Joint Hypermobility Syndrome – not so ‘Benign’

Authors
Yathish GC, Canchi Balakrishnan, Mangat Gurmeet, Taral Parikh, Sagdeo Parikshit, Girish Kakade

Internet Journal of Rheumatology and Clinical Immunology 3.1 (2015)

PDF

The article reports the case of a 53-year-old lady whom the authors finally diagnosed with Joint Hypermobility Syndrome. The lady presented with a 16-year history of instability while walking, repeated knee injuries, and buckling and give away sensation at both knee joints. She had history of an elbow dislocation (traumatic) and had complaints of multiple joint and soft tissue pain while performing household activities. In extra-articular symptoms, she had been diagnosed with irritable bowel syndrome.

The corresponding author, Dr. Yatish, is a resident in Rheumatology. I had the opportunity to communicate with him and was enthused by his interest in hypermobility syndrome. Dr. Yatish wrote about how H-EDS/JHS was responsible for such a considerable morbidity in the patient and how underdiagnosed this condition is in India.

I hope that with more Indian rheumatologists getting interested in this condition, it is a matter of time when we will be able to develop customized treatment protocols and physical therapy regimes that are suited to Indian patients and the Indian health care infrastructure.

I also think that the millions of undiagnosed patients in India are important as a large pool of  unaddressed disease morbidity, the identification and treatment of which can benefit the community health as a whole. That should be of interest to the patients living in pain and to the health ministries and health departments of the nation.

What should be of great interest to medical researchers in the fields of Rheumatology, Orthopedics, Physical and Rehabilitation Medicine, Gastroenterology, Clinical Genetics and Public Health Medicine among others is that these millions of patients can lead to new insights, new studies (and scores of new articles if that is what matters in the end), and new knowledge.

The recent and revolutionary advances in medicine, physical therapy, genetics and technology enabling not just multidisciplinary but global information sharing and collaboration, have brought us to a momentous point in human history where we can identify and manage this disease that has hitherto been hiding in plain sight.

Chronic Pain in JHS

Ah, me! the Prison House of Pain!—what lessons
there are bought!—
Lessons of a sublimer strain
Than any elsewhere taught.

- Florence Earle Coates, Poems, Volume Two, The House of Pain



Chronic pain is a pain that lasts beyond three months and is an important feature of hypermobility syndrome contributing to the distress and disability caused by this disease. Prolonged pain adversely affects the person's body, mind and social life.

Chronic pain does not sound like much and yet is probably the most complex aspect of JHS and many other connective tissue disorders. It is as devastating to the quality of life of a person as it is difficult to treat.

The healers fail to heal

The person with undiagnosed or ill-managed JHS feels let down by the healthcare system and doctors. The only relief they are offered is painkillers which at the most only decrease the pain only fractionally. If the diagnosis of JHS is not considered, often it appears that there is absolutely no biological reason why the person should have so many myriad complaints - leading to the assumption, whether explicitly expressed or not, that the person really does not have these complaints.

Disruption of life

As the pain leads to further decrease in fitness, it makes difficult for the person to successfully or efficiently work - household chores, job and other responsibilities become challenging. The fatigue and sleep deprivation add to the pre-existing difficulties.

Further disruption of health

Chronic pain leads to a vicious cycle where a person avoids activity and movement due to fear of more pain and the inactivity in turn leads to more pain.



Neuro/Psychological Effects

There is a sense of isolation and loneliness because no one understands what you are going through. Relationships in life suffer. It is a common fact and also a medically accepted fact now that understanding support, availability of compassionate care and the opportunity to share one's feelings or problems with others combine to form a massive sustaining/healing/pro-health force. There is complete lack of these for the average JHS patient, because most people really don't believe they are sick, most people are unable to understand how and why they are sick, and most people find themselves unable to offer them the help they need.

The person with JHS may battle frustration and depressive moods. They may harbor several fears and anxieties. They may feel guilty for being unable to fulfil their roles. There is a sense of worthlessness, hopelessness and helplessness. There is sometimes chronic sleep deprivation and decrease of cognitive abilities. They may feel unable to bear it anymore during periods of heightened pain.

The psychological aspects and the actual perception of pain are intricately linked. Increased stress and depression actually lead to tense muscles, more pain and more severe distress as a result of the pain.  

The continued severe pain also has actual modifying effect on the brain neuronal pathways and leads the person to perceive any pain many times more severely than a normal person [1]. This does not mean it is a psychological or fake pain - the pain is really that severe for that person, although the trigger may not be severe enough to cause that much pain. Sometimes, I used to recognize this - "No way is this little bump supposed to pain like this - what is wrong with me?" or "What is this pain, it seems like I hurt myself - but I cannot remember when!"

Strategies for chronic pain management

In developed countries pain management has advanced to becoming a separate specialty. There are a number of separate and allied professional fields devoted to pain management. However, in India where pain is not effectively managed even for a dying cancer patient, perhaps it is too much to ask for a comprehensive pain management protocol for rheumatological diseases.

The strategies for pain management not only involve physical therapy or pharmacotherapy to actually manage the manifestations of the disease, they also involve many psychological based strategies that modify the way one finds the strength to bear the pain; they way one reacts and responds to the pain; and the way one learns to quieten the mind enough and relax the body enough to let the body and mind get some healing time.

I think there are two components to the pain I feel - a chronic physical pain and a chronic mental pain. There might be a variety of psychological terms for what I am labeling as mental pain, but in practical terms, in order to seek DIY holistic strategies, I found that these psychological terms, broadly speaking, constitute my mental pain.

In future posts, I will write on strategies that I have come across in relation to pain management. These not only involve cognitive behavior therapy but also many other books and methods that may be helpful to manage JHS pain, both physical and mental.

References
[1] Moseley, G. Lorimer, and Herta Flor. "Targeting cortical representations in the treatment of chronic pain a review." Neurorehabilitation and neural repair 26.6 (2012): 646-652.



Hands and arms: Little things that become challenges

If the joints of the elbows, wrists and fingers are injured or weak due to hypermobility syndrome, the person finds many day to day and mundane activities difficult or painful. They may be embarrassed to ask for help because these seems like absolutely easy things - especially for a seemingly healthy adult.

These are a few random things that I can think of right now which I find difficult to do. If you have such complaints, you will know that the list is bigger. But this enumeration is just to give an idea to people who may not be familiar with these challenges of a person with hypermobility syndrome.

Indian Articles: Postural deviance and pain in BJHS subjects : Vellore Study

Rheumatology International, December 2011, Volume 31, Issue 12, pp 1561-1565, Date: 20 May 2010

Evaluation of posture and pain in persons with benign joint hypermobility syndrome

Authors: Divya S. Booshanam, Binu Cherian, Charles Premkumar A. R. Joseph, John Mathew, Raji Thomas
Physiotherapy, Department of Physical Medicine & Rehabilitation, Department of Rheumatology, Christian Medical College, Vellore, India
The objective of the present study is to compare and quantify the postural differences and joint pain distribution between subjects with benign joint hypermobility syndrome (BJHS) and the normal population. This observational, non-randomized, and controlled study was conducted at Rheumatology and Physical Medicine and Rehabilitation Medicine Departments of a tertiary care teaching hospital. Subjects comprise 35 persons with diagnosis of BJHS, and the control group was matched for age and sex. Reedco’s Posture score (RPS) and visual analogue scale (VAS) were the outcome measures. The subjects were assessed for pain in ten major joints and rated on a VAS. A standard posture assessment was conducted using the Reedco’s Posture score. The same procedure was executed for an age- and sex-matched control group. Mean RPS for the BJHS group was 55.29 ± 8.15 and for the normal group it was 67 ± 11.94. The most common postural deviances in subjects with BJHS were identified in the following areas of head, hip (Sagittal plane), upper back, trunk, and lower back (Coronal plane). Intensity of pain was found to be more in BJHS persons than that of the normal persons, and the knee joints were the most affected. The present study compared and quantified the postural abnormalities and the pain in BJHS persons. The need for postural re-education and specific assessment and training for the most affected joints are discussed. There is a significant difference in posture between subjects with BJHS and the normal population. BJHS persons need special attention to their posture re-education during physiotherapy sessions to reduce long-term detrimental effects on the musculoskeletal system.

Study: Are Hypermobility Syndrome and Asthma Related?

Yes, conclude researchers:
We have demonstrated, for the first time, that individuals with HMS/BJHS and EDS have respiratory symptoms in association with various pulmonary physiological abnormalities. The increased prevalence of asthma may be due to linkage disequilibrium between the genes causing these conditions or a function of the connective tissue defect itself. In the non‐asthmatic population, changes in the mechanical properties of the bronchial airways and lung parenchyma may underlie the observed increased tendency of the airways to collapse.

 Details of the paper:

Asthma and airways collapse in two heritable disorders of connective tissue

A W Morgan, S B Pearson, S Davies, H C Gooi, and H A Bird

Ann Rheum Dis. 2007 Oct; 66(10): 1369–1373.
doi:  10.1136/ard.2006.062224

 

Indian Articles: The AIIMS Study on prevalence of BJHS in patients attending rheumatology clinic


Kumar, A., et al. "Benign joint hypermobility syndrome: a hospital-based study from northern India." Indian journal of Rheumatology 1.1 (2006): 8-12.

The conclusion is that BJHS is associated with musculoskeletal symptoms, leading the patients of BJHS to be referred to rheumatology clinics.Many of them are diagnosed incorrectly with other diseases like rheumatoid arthritis, rheumatic fever and ankylosing spondylitis in primary and secondary care settings. 
http://www.indianjrheumatol.com/article/S0973-3698%2810%2960515-8/abstract
Click to read

Hypermobility: An Indian Perspective


In response to the article, "Joint hypermobility syndrome", by J. Ross and R. Grahame ( BMJ 2011;342:c7167 , doi: http://dx.doi.org/10.1136/bmj.c7167 ) Dr. Arvind Chopra, Centre for Rheumatic Diseases, Pune, India, writes:

Undoubtedly hypermobility (HM) and hypermobility syndrome (HMS) is missed in clinical practice and many a patient made to suffer from needless investigations and therapy. Still worse, the patient may be wrongly diagnosed as having rheumatoid arthritis and treated with long term steroids and disease modifying and immunosuppressive agents.... a clinician ought to look out for HM in all cases with musculoskeletal and joint pains. Irrespective of any other rheumatic disorder that may coexist, it is very comforting for the patient to learn that the problem is largely a benign disorder called HMS and that drugs have a very little role if any.

I wish more clinicians would know this, and not just some scattered rheumatologists across India.

I just hope people would stop using the word "benign". I know that the morbidity in JHS is less than autoimmune inflammatory diseases of the joints, but it is sufficient and correct to say that JHS or HMS is not autoimmune and there is no place for steroids. Please do not tell me that the destruction of my life, health and career have been any less because my condition does not require steroids. 

I also agree that once correctly diagnosed, in an Indian setting, it would be reasonable to refer the patient with JHS back to an orthopedician and a physiotherapist and free up resources (the rheumatologist) for patients with serious autoimmune disorders of joints.

But where are the orthopedician and the physiotherapist who have heard of JHS and who know the correct management regime?