Chronic Pain India - A new initiative

There is a new initiative by chronic pain warriors in India which is an ambitious project to build a community of support and a platform for awareness and advocacy for people suffering from chronic pain  and invisible illnesses in India. 

I quote from the about page:

"At Chronic Pain India, We plan to build a strong community for every chronic pain sufferer in India, suffering from any illness. We tend to unite every chronic pain sufferer as one and plan to make their voices be heard through the medium of writing. It will be used as a mean to raise awareness about various chronic pain conditions and chronic illness which are usually invisible through naked eyes. We plan to share the pain experiences of sufferers so that the general population can have an insight to it and they could have a better understanding. This will be like a safe house for every chronic pain sufferer in India to shed down their problems and we will slowly try to work on them & try to bring a change. It can also be used by medical practitioners to understand the perspective of the pain sufferer and they can have a better understanding of the pain patients and can provide them with a holistic approach towards their treatment."

Wishing Chronic Pain India the best! Here is the link to the site: 


Where do missing zebras go?




After I got my diagnosis, I researched online about hypermobility disorders and a good deal of useful information I found was on the blogs of fellow zebras. As you read someone's blog, sometimes from years ago, and follow their story - how they were diagnosed and their journey afterwards, you get entwined with the journey at some level. You hope the best for them, you root for them. You cry for the pain they faced and find support in their trials and tribulations. You understand their views and value their insights. And then the blog ends suddenly. The last post was some years or some months ago. 

Some blogs were a few posts long, even one post long. Some wrote for a few years apparently and then stopped. I wondered about them, and sometimes enough to try to figure out from various clues on the blog who might they be, and thus be able to try to find where they might be now, and then figure out if they are alright. It does not really work, because anonymous or pseudonymous blogs, that too by a person of a different country and culture, don't offer too many clues. 

I always feel the world is moving at a speed that I cannot keep up with and I am frequently late for everything under the sun. But a comrade-in-chronic-illness - a हमराही of sorts - is like someone you get used to meeting at the same time under a certain set of circumstances - online, or at a clinic, or a restaurant. And one day that person stops coming. 

image courtesy pixabay.com

When I started my blog, my ideas were very clear as to what was my aim. I knew that my blog would be information oriented and would hardly have any personal stories of my own life. I knew that sooner or later it would show up on the first page of google if people searched for hypermobility + syndrome + India, and that I hoped would lead me to discover other Indian zebras and we could swap notes and in future probably create a support platform. 


However, things did not work out as planned or as soon or as easily. Things happened in my life that changed my situation drastically. For the worse in some aspects. I developed nerve problems in my hand adding to my woes. My computer crashed. Bad things happened to people I love. My family became uprooted. And then my blog too became one of the lost bloggers. I found myself unable to even answer queries from readers, which has always been a priority (snails can have priorities too). 

There are many prisons I fight, I feel. At a personal level, I fight all the lack of liberty that comes with being a woman. At a societal level, I fight all the confining unreasonable judgmental norms that come with my culture. At an internal level, I fight myself. My brain feels caged by my body. My body feels imprisoned by my mind. Ultimately, to be free, perhaps not as free as a healthy person, not free to sprint to catch a bus (that is one of the personal goals I laid down in the assessment interviews with physiotherapists), but to be "freer", my mind must quit the job of a prison warden. I have been concentrating on that for many years, with varying degrees of success. But varying degrees of success, and not of failure. I am still alive, ain't I? 

However, an email from a chronic pain warrior suddenly brought a wisp of light into my prison. And I pulled together time, energy and hardware (all scarce resources) to write this post. And the next one that I am going to write. I truly hope my blog and my writing are only dormant and not dead. And I hope this post will be helpful to anyone who too fights chronic illness and wondered what happened to this blogger. I am alright and still fighting. In spite of my situation which is one of the harder ones I have faced in life, I am not really at the worst place of my life. I hope the Chronic Pain India site will be really helpful to people, I wish everyone associated with it the best. And I hope I will contribute to it as well. 

March, 2018
Image courtesy GDJ, pixabay.com


New Updates on HSD, JHS, and h-EDS (2017)

The new definitions, terminology and criteria for a better understanding of joint hypermobility disorders are here (2017). All the relevant links to articles in the Ehlers-Danlos Society website can be seen in one place: click here

What is HSD?

The Unending Moment of Torment


Chronic pain at its peak was like an unrelenting hollowness, a continuous  deafening hum, an endless torture session where every moment felt like dying but death never came. Though these lines by Gulzar allude to a different context, I often found them ringing in my head when I was submerged and drowning in pain. 


दिल दर्द का टुकडा है पत्थर की डली सी है
एक अंधा कुंवां है या एक बंद गली सी है
एक छोटा सा लम्हा है जो ख़त्म नहीं होता
मैं लाख जलाता हूं ये भस्म नहीं होता
ये भस्म नहीं होता 

Exercising in hEDS

An excellent talk I highly recommend - I found it very helpful. Watch it full-screen, I am sure if you are new to this new to this condition and are watching it for the first time, you will want to take notes! 

Intelligent Exercise-How You Can Take Control with EDS
Webinar by Kathryn Lister,  October 28, 2015.





-----------------------------------------------------------------------------------------------------------------------------------------------

As you live with this disease after getting a diagnosis, you know more and more about it and manage yourself better and better with time. It is different from intuitively figuring out a few things that make you better or worse, without diagnosis. In the latter, you often don't have the necessary evidence or the scientific causality as to the WHY something aggravates your condition. So again, I think a diagnosis, even a partial but correct diagnosis is important. 

I am continuously learning, and as I find some things worth sharing I try to write here or on FB. Some ideas change or get refined too with time as I find more information or understand things better.  

I am writing below some key points I have learned over time about exercising, they are relevant to me and may not be all useful for another person. Every body and each case of hEDS/HSD is different and this is just a list of things that I found useful. These points are not just about exercises, they extend to other topics too, because often everything is interrelated. 


Some old talks on EDS/hEDS

New changes are happening in the diagnostic criteria of EDS, hEDS and hypermobility spectrum disorders. I would like to share some old talks again that are extremely informative and must-watch for anyone wanting to know about these  conditions. 

Important Information

Feb 2017

Many changes are taking place that are going to lead to changes in the terminology associated with hypermobility related diseases. The future posts in this blog will reflect that, and where I can, I will edit/delete past posts to reflect that updates for the reader. However, I am waiting to fully understand these changes, and the updated diagnostic criteria on EDS that will be published in March 2017. I am also inclined to keep those pages (with an update line on top)  as a reminder of how confusing the things were - that is hoping things will be less confusing in the future.

You can find some information here: http://ehlers-danlos.com/wp-content/uploads/QandA-2.pdf


Those looking for more information on hypermobility type EDS and hypermobility spectrum disorders, will still find many useful links on this blog. I also highly recommend my favorite blogger Jan Groh's site: http://ohtwist.com (check out all the "about" pages) for comprehensive info and links.  


As far as JHS, BJHS, HMS and other such terms, they are all being brought under the umbrella diagnosis of Hypermobility spectrum disorders (HSD) where the new strict criteria for hEDS is not fulfilled but there is joint hypermobility (generalized, or localized or peripheral) leading to pain symptoms and other systemic symptoms. Overall, I am optimistic that in India, this will bring about a new era where people with multiple seemingly unrelated systemic problems with coexisting joint hypermobility will be re-evaluated for HSD instead of getting dismissed as overthinking hypochondriacs, or worse, as malingerers. Often the person with HSD is being treated for musculoskeletal symptoms with miscellaneous semicorrect or inadequate diagnoses by an orthoped (who has no idea about all the other problems she/he faces) while all other systemic problems are being treated by various specialists who also have no clue about the possibility of a connective tissue disease in her/him. This is ineffectual for long term management as well as frustrating for both the patient and doctors. HSD is not rare, and HSD being a spectrum, an extremely variegated presentation profile of patients is seen. Recognition of HSD as a newly defined disease will lead to the diagnosis and holistic treatment of millions of people. That is what I hope. 

Latest Updates: Feb 2017: A Framework for the Classification of Joint Hypermobility and Related Conditions

A Framework for the Classification of Joint Hypermobility and Related Conditions

Authors: MARCO CASTORI,* BRAD TINKLE, HOWARD LEVY, RODNEY GRAHAME,
FRANSISKA MALFAIT, AND ALAN HAKIM

Am J Med Genet Part C Semin Med Genet 9999C:1–10.

-A new classification system for Ehlers–Danlos syndrome (EDS) identifies more than 20 types of EDS which has led to patients who do not fulfil the criteria of "full-blown" EDS-HT but have many features of hEDS and Joint Hypermobility, to be left without a diagnosis.

- Highlights the need for a single set of criteria that can substitute the previous criteria for the overlapping conditions of JHS and EDS-HT

- Introduces a concept of a spectrum of pathogenetically related manifestations of joint hypermobility intersecting with syndromes that manifest as multiple conditions including joint hypermobility resulting from a single gene (pleiotropic syndromes)  - as distinctly apart from other syndromes with joint hypermobility

Proposed classification:




Latest Updates: Feb 2017: Hypermobile Ehlers–Danlos Syndrome:Clinical Description and Natural History

Top experts on HEDS come together in this review article to describe what is known and understood so far about the cause, presentation and management of HEDS.

Hypermobile Ehlers–Danlos Syndrome (a.k.a. Ehlers–Danlos Syndrome Type III and Ehlers–Danlos Syndrome Hypermobility Type): Clinical Description and Natural History

Authors: BRAD TINKLE, MARCO CASTORI, BRITTA BERGLUND, HELEN COHEN, RODNEY GRAHAME, HANADI KAZKAZ, AND HOWARD LEVY

Am J Med Genet Part C Semin Med Genet 9999C:1–22.


Quick Info (2016)

The content in this post is outdated. There are new updates to the current understanding of joint hypermobility disorders. Please read this post

I am keeping this post and the content as a historic record of our understanding of the disease until 2016. 

Not just the joints: A review article on the comorbidities of JHS/HEDS

Ehlers–Danlos Syndrome - Hypermobility Type: A Much Neglected Multisystemic Disorder

Yael Gazit, Giris Jacob, and Rodney Grahame.

Excerpt from abstract:
Throughout the years, extra-articular manifestations have been described, including cardiovascular, autonomic nervous system, gastrointestinal, hematologic, ocular, gynecologic, neurologic, and psychiatric manifestations, emphasizing the multisystemic nature of EDS-HT. Unfortunately , EDS-HT is underrecognized and inadequately managed, leading to neglect of these patients, which may lead to severe disability that almost certainly could have been avoided. In this review article we will describe the known manifestations of the extra-articular systems. 

Review Article: Hypermobility syndromes and chronic or recurrent MSK pain in children (Oct 2015)

By Marco Cattalini, Raju Khubchandani, and Rolando Cimaz
(Dr. Raju Khubchandani is a Pediatric Rheumatologist at the Department of Paediatrics, Jaslok Hospital and Research Centre, Mumbai, India)

Abstract

Chronic or recurrent musculoskeletal pain is a common complaint in children. Among the most common causes for this problem are different conditions associated with hypermobility. Pediatricians and allied professionals should be well aware of the characteristics of the different syndromes associated with hypermobility and facilitate early recognition and appropriate management. In this review we provide information on Benign Joint Hypermobility Syndrome, Ehlers-Danlos Syndrome, Marfan Syndrome, Loeys-Dietz syndrome and Stickler syndrome, and discuss their characteristics and clinical management.

Pediatr Rheumatol Online J. 2015; 13: 40.
Published online 2015 Oct 6. 
PMCID: PMC4596461
===================

Excerpts relevant to HEDS:

Benign joint hypermobility syndrome (BJHS)
Children with hypermobile joints by definition display a range of movement that is considered excessive, taking into consideration the age, gender and ethnic background of the individual. It is estimated that at least 10–15 % of normal children have hypermobile joints and the term joint hypermobility syndrome (JHS) is reserved to the cases of joint hypermobility associated with symptoms with no other causes found for them []. JHS can be associated with hereditary connective tissue disorders, and the term “Benign” is used in contrast to more serious and potentially complicated or life-threatening musculoskeletal syndromes such as some forms of Ehlers-Danlos syndrome (EDS), Marfan syndrome, and Loeys-Dietz syndrome. The prevalence of JHS is not known with precision, given the lack of studies of large cohorts. Sperotto et al., conducted a cross sectional study in a cohort of healthy schoolchildren, aged 8–13 years from the province of Padua, Italy, and found that BJHS occurred in the 13,2 % of the 289 children evaluated []. 
Even if BJHS is very common, this condition is largely under-recognized by primary care physicians and often poorly managed. Symptoms frequently start in childhood and continue into adult life. The pathophysiology of benign joint hypermobility is unclear. Hypermobility is more common in childhood and adolescence, in females, and in some ethnicities, and it tends to lessen during adulthood. Still, polyarticular hypermobility may be present in up to 30 % of males and 40 % of females during early adulthood []. For the majority of individuals joint hypermobility may be of no consequence, and what brings a proportion of subjects to develop BJHS is not fully understood. BJHS seems to be transmitted by an autosomal pattern, and first-degree relatives with the disorders can be identified in many cases. Variable penetrance is generally observed []. With the exception of a minority of patients, who show a deficiency of tenascin X, no abnormality in collagen or related proteins has been identified as a cause for BJHS []. Joint pain is thought to be caused by excessive movement, increasing stress on joint surfaces, ligaments and adjacent structures. Other factors may contribute to the development of the syndrome, such as poor proprioception, autonomic dysfunctions and fatigue secondary to poor sleep []. 
The predominant presenting complaint is pain, which may be widespread and debilitating. The pain typically starts during or after activity. The most common affected sites are the lower limbs after walking (for example walking to and from school). Children usually report excess fatigue, handwriting difficulties or ‘clicking or cracking’ joints. Occasionally episodes of joint swelling lasting hours to days, joint dislocations, or more commonly subluxations with spontaneous reduction are reported. Back-pain is also a common complaint because the lumbar spine is one of the most mobile sections of the vertebral column and the excessive movements may lead to pain in hypermobile subjects. Heavy school bags are often an aggravating feature. Chronic pain results in a reduced exercise tolerance and can negatively impact patients’ life. 
A significant proportion of subjects progressively quit sports and other physical activities. In addition, pain amplification is a common feature in these cases []. BJHS has been considered to cause only musculoskeletal symptoms for many years, but there is now mounting evidence that many other extra-skeletal manifestations may occur. This symptoms arise usually after the third decade of life, but have been described in adolescents, and may be due to connective tissue abnormalities, linking BJHS and other hereditary disorders of connective tissues, namely Ehlers-Danlos syndrome type III. These include functional and anatomic gastrointestinal tract abnormalities (constipation, bloating, diarrhea, hiatal hernias), autonomic dysfunctions (postural tachycardia syndrome, palpitations, orthostatic intolerance, headache, fatigue) and skin abnormalities (easy bruising, striae) []. Some of these symptoms are overlapping with those observed in Juvenile Fibromyalgia (JFM), and indeed there are few reports describing high incidence of BJHS in children with JFM. Furthermore, children who have both JFM and BJHS may exhibit lower tender-points thresholds and a greater number of tender-points compared to children with JFM but no benign joint hypermobility []. 
The “Beighton score” (derived from the original one by Carter and Wilkinson) is commonly used to diagnose hypermobility. Hypermobility is present if 4 out of 9 points are scored. When this score is applied to normal children, a large proportion of the population is hypermobile (Table 1, Figs. 1,22,22 and and3).3and3).3). This reflects the fact that, as already discussed, connective tissue structures may be looser and joints hypermobile in childhood, especially compared to adults. For these reasons it may be better to consider a Beighton score of 5 or more positive []. The Beighton score has been incorporated into a more comprehensive set of criteria called the Brighton Criteria (Table 2), which take into account the possible multisystemic nature of this condition. Although these criteria have not been formally validated in a pediatric population, they have been used in some studies on children with hypermobility []. 
The management of individuals with BJHS can be very challenging and there are no evidence-based management strategies currently available. Acute pain episodes are commonly managed using taping, bracing or splinting or with non-steroidal anti-inflammatory drugs as needed. However reassurance and a multi-disciplinary training program are the mainstays of long term management. Physical therapy is of the outmost importance, and encouraging an active lifestyle may improve function and enhance quality of life []. As general principles, strengthening exercises focused on muscles around hypermobile joints may help to enhance joint support throughout movement and reduce pain; closed chain exercises may enhance proprioceptive feedback and optimize muscle action. Proprioception may be improved also by coordination and balance exercises. Physical therapy should also encompass a generalized exercise programme, addressing cardio-respiratory, musculoskeletal and neurological aspects of movement with the aim to reduce deconditioning []. As already mentioned, the Health Related Quality of Life in children with hypermobility and their parents may be worse than that of healthy controls and this may well be secondary to the presence of chronic pain and fatigue, that act as stressors in everyday life. This is an important aspect to consider for physicians approaching a child with BJHS, that should not underestimate the burden of this condition, often interpreted as benign and not worth any intervention [].

....

In "Ehlers-Danlos Syndromes (EDSs)", the authors write:

For pediatric rheumatologists, a real diagnostic challenge is represented by the hypermobility subtype of EDS (EDS-HT), which is by far the most common subtype. The genetic basis of EDS-Hybermobile is largely unknown and a reliable diagnostic test for this EDS subtype is lacking []. According to the Villefranche classification, the major diagnostic criteria are generalized joint hypermobility and presence of typical skin manifestations. However, these features are usually more subtle than in the classic type of EDS but these criteria are nevertheless helpful to differentiate this form of EDS from the more common “Benign joint hypermobility syndrome (BJHS)” []. It is still a matter of debate if EDS-HT and BJHS really represents two different diseases or if they should be reviewed as a spectrum of a single entity, sharing common genetic bases and showing considerable variability in clinical presentation, between as well as within families. 
Joint hypermobility is typically limited to the small joints of the hands in the vascular subtype. This subtype has the worst prognosis because of a high rate of spontaneous arterial rupture usually in the third or the fourth decade of life. Unlike other EDS types, the skin is not hyper-extensible, but rather thin and translucent, showing a visible venous pattern over the chest, abdomen and extremities. Excessive bruising is the most common sign and is often the presenting complaint, especially in children. Other early manifestations include premature rupture of the membranes, congenital clubfoot or congenital hip dislocation, inguinal hernia, and severe varicosities. The facial and cutaneous features may be very subtle or even absent []. If there is a strong clinical suspicion of vascular EDS, direct DNA analysis is mandatory, even in the absence of an abnormal biochemical abnormality. 
The management of children with Ehlers-Danlos syndromes requires a multidisciplinary approach. Children with pronounced skin fragility should be advised to avoid contact sports and to wear protective pads or bandages in order to prevent bruises and hematomas. Cutaneous stitches should be left in place twice as long as usual, and additional fixation of adjacent skin with adhesive tape can help to prevent stretching of the scar. In children physio-therapeutic support is important. Acetaminophen should be preferred over NSAIDs for joint pain and thus minimizing the risk of easy bruising due to platelet disfunction. For the same reason COX-2 inhibitors may be an option, although no studies have been published on their use in EDS. Patients with mitral valve prolapse and regurgitation require antibiotic prophylaxis for bacterial endocarditis. A baseline echocardiogram with aortic diameters measurement is recommended before 10 years of age, with follow-up studies timed according to whether an abnormal measurement is found. A useful resource for these measurements is parameterz.blogspot.in. For the vascular and vascular-like types of EDS, some prophylactic measures are of particular importance. Invasive vascular procedures such as arteriography and catheterization should also be avoided because of the risk for life-threatening vascular rupture. Surgical interventions are generally discouraged because of increased vascular fragility, and conservative therapy is recommended [,].

A Parable for HEDS


#EDS   #Hypermobility Syndrome   #HMS   #JHS   #EDS-HT  

Information booklet for JHS/HEDS in kids, part - 2

Last month, I made a brochure (Part 1) aiming to provide information to school teachers about what is JHS/HEDS and how it affects children.

The brochure in this post is the second in the series, and offers tips to teachers on how they can support a child with EDS.

These are printable on A4 sheets.




The pdf file can be downloaded here.