What is Ehlers-danlos syndrome? (EDS)

(see info brochure at bottom of page)

Ehlers-Danlos Syndrome is a genetic connective tissue disorder. It is a complex, painful, challenging, and debilitating systemic condition. EDS results in weakened connective tissues caused by weakened collagen - your body’s ‘glue.’ It also frequently co-occurrs with comorbidities. EDS presents with traits like hyper mobile (overly flexible and loose) joints, soft skin that is more sensitive to injury and is often stretchy like elastic (but can also be loose in some EDS types and/or with age), more visible veins and easily bruising, subtler features only geneticists might point out such little bumps on the back of the feet called piezogenic pupules (I have them!), facial features like deep set eyes, high cheekbones and overly crowded teeth, over bite and high, narrow palate and more fragile and sensitive dental enamel (and unfortunately with that, an increased risk of dental problems) and of course looking younger. This last trait can also bring about a superficial look of wellness which can then cause people (who don’t understand) to disbelieve that the EDS patient is unwell and suffering with pain, fatigue and much more. A big part of ableism stems from people thinking one needs to ‘look’ obviously sick and disabled to be sick and disabled - and EDS patients often take the brunt when it comes to this kind of ableism! (I call it ‘disbelief ableism.’)

Having EDS at this point and time in history is, for many if not most EDS patients, very difficult. People with EDS are plagued with several, various physical issues and this causes people who are ignorant to accuse us of being ‘hypochondriacs.’

However, the fact of the matter is, EDS - like the more-known Lupus - is a systemic condition. This means it affects many body systems such as; the musculoskeletal system (joints, bones, ligaments, spine), the vascular system (heart and circulation), the digestive system, the urinary system, the immune system, the autonomic nervous system, neurological health, dental health and more.

EDS causes comorbid conditions such as Dysautonomia (deregulation and disorder of the autonomic nervous system) especially a type of it called Post Orthostatic Tachycardia Syndrome or POTS, Mast Cell Activation Disorder or MCAS ; a frustrating disorder of the immune system causing overproduction and delayed breakdown of histamine thus causing various allergies, sensitivities and inflammation similar to that of which is present in autoimmune disorders like Lupus and RA. Some patients with MCAS can develop anaphylaxis too, which can be very dangerous. EDS also causes a very painful and difficult to treat spinal problem called CCI (craniocervical instability) this means there are unstable vertebrae and/or ligaments in the neck and/or cranial area. Instability is often due to damaged/worn ligaments - the ones that support the weight of the head.

Unfortunately, I have all three of these comorbid disorders!

I myself have EDS with POTS, MCAS and CCI. Day to day living is challenging. I am on several medications, as well as some supplements and special diet, to try and self-manage all of this - while partially mobility impaired, fatigued and in pain, and, as it stands it’s only partially helping. My main barrier is having to live with untreated CCI, and I am not alone unfortunately. Currently in Canada, there are no proper doctor specialties, surgeries, or nonsurgical treatments offered for people with CCI, or any other EDS-related spinal disease…and it’s presenting us with a pretty awful ethical dilemma. I’ve explained more about this issue (and how it affects me) in further detail on my own Go Fund Me - which is open to try and raise funds for me to get CCI treatment abroad.

Again though, EDS is - although brutal - mostly invisible… especially from the perspectives of people who don't know about it. Once again, since EDS includes that soft skin, so many people with EDS look young for their age and thus superficially ‘well.’ However, we are definitely not as well as we may look, and additionally EDS does cause things like severe dark under eye circles (something many of us, including myself, are self conscious about and use concealer to cover up!) But please remember, spite of EDS’ not being visibly obvious, EDS is a condition that can become very severe and debilitating, the more neglected it is, which can cause really poor quality of life and can occasionally be fatal. This is why people need to educate themselves, become aware and perhaps become an ally and/or advocate.

Currently, neglect of patients with EDS is a rampant problem and we need change. Since EDSers don’t often look as sick as they are, people and even doctors frequently discriminate against us and do not offer the support/treatment - or even compassion - needed.. that is until the disease complications are advanced to a point where it’s more obvious (and sometimes too late!) In addition to this (or maybe stemmed from it) public healthcare systems’ infrastructures' do not support access to EDS-specific treatment, likely because of lack of awareness and acceptance among both doctors and the public, as well as cost. EDSers very often have to pay privately, and sometimes even travel, to access the kind of treatment they need for more significant (beyond band-aid) improvement and better quality of life.

This is why we need Ehlers-Danlos Syndrome Awareness and change, so very badly.

I am profoundly affected by EDS today because of what I went through re: lack of being believed and accused of 'faking for attention' for several years before I was finally diagnosed and taken seriously. I have a port in my chest for weekly IV fluids and am currently stuck on a daily bubble pack of about 13 medications for various problems I’ve developed with POTS dysautonomia and my heart rate (tachycardia), neuropathic pain, antivirals and antihistamines for immune system problems like chronic infections, viral flares and MCAS, debilitating digestive problems like pancreatic insufficiency needing creon enzyme pills, sphincter of oddi disorder needing synthetic bile (ursodiol), and meds for IBS, and I also have improper insulin function as a result of MODY diabetes and am on medication for that. In addition to that, I rely on a large handful of supplements to help me feel at least reasonable enough to ‘get by.’ I also need to eat a tailored and specific diet, which I am in charge of preparing myself. Management of all this is, as one might imagine, is difficult, exhausting and relentless. (I sometimes share more about my personal day to day experience on my current blog/journal)

I believe things are as bad as they are because of the huge delays in terms of arriving at the proper diagnosis as well as identifying and managing complications. This began as far back as my childhood but got worse and more malignant after I tried to ask for help! Not only were things physically damaging but the years of discrimination, mistreatment and neglect have caused me to have post traumatic stress disorder, which is something I have been diagnosed with continue to battle with today. Unfortunately, I'm not the only EDS patient who has had this happen to them. I don’t want others to continue to go through what I have and I hope one day it stops.

The best and most effective treatments to improve EDS complications - and damaged ligaments (such as in my neck) - are currently only available via private pay and only in a few exclusive locations (forcing many of us to have to travel.. if we can afford that.) This is a huge gap of inequality in healthcare for those with EDS and other, similar less-recognized chronic illnesses. This is because public healthcare systems do not understand enough about EDS - nor are they willing to learn new information - in order to implement the best treatments. It shouldn't be this way, especially in a place like Canada where we claim to be so ‘non discriminatory’.. but sadly, when it comes to EDS care, this is the current reality. This even results in quality of life becoming so poor, and so unsustainable (when people are on disability and are sick with complex illness, it’s difficult and sometimes nearly impossible to pay for the right food and supplements needed to feel even humanely better enough.

This issue has gotten so bad that some chronically ill people on disability have felt cornered into utilizing Canada’s MAiD (medical assistance in dying) program. Recently Canada has further relaxed rules and regulations in order to qualify for MAiD, allowing people like me, who cannot financially sustain living humanely, to have themselves euthanized. This has caused a moral dilemma and ethical debate, and even protests with people holding up signs reading (apt) things like ‘assist us to live.’ Many people (accurately) feel that the government would sooner have people do this than pay to improve access to better healthcare and resources - because it’s cheaper. I myself am passionately against euthanasia being used when there are things that can be done to help the patient, but they cannot access them. At one point I myself felt so burnt out and helpless that I thought about MAiD, but I have come back from that deciding to keep fighting, realizing that this is ethically wrong for people like myself - who could still be helped by treatment and resources that they can’t access. The moral dilemma it creates to offer people like myself euthanasia stems from both ableism and classism in so many ways, and I realized I am not ready to go and need more time, and whatever time I have left here is time I need for a mission in terms of advocating for not only my life but all of this, and in my own way.

My dad calls EDS, as well as comorbids like POTS/Dysautonomia, MCAS and also things like ME/CFS ‘21st Century Diseases.’ They’re new concepts, more and more people are suffering from them - but there is not yet widespread proper treatment. It’s like where Lupus and MS were 20-30 years ago. It still needs to change and it’s no fun having to be a ‘pioneer’ but there is no choice and we must keep educating, and encouraging people - including you who may be reading this - to be open to learning. I know they say not to google ones’ symptoms but when it comes to EDS, google away please. It educates and helps stop ignorance.

 People need to stop assuming and accusing patients with EDS (and other invisible but serious illnesses) of whining, complaining, exaggerating and even lying about their pain, symptoms and disabilities. For example people with EDS may use a wheelchair on a ‘bad’ day or if they have to go far or be out for awhile, but can walk with a cane or stick, or even unaided, on another ‘better’ day or when not going to far or for as long. There is actually a name for this, it is called a dynamic disability. However, people often assume and accuse a person with a dynamic disability of faking and attention seeking, as opposed to educating themselves about the nature of the persons’ disability and illness, and how it actually works. Not only is this ignorant and disrespectful, It’s straight up abuse, and let’s call it what it is. 

For prevention of both physical and emotional damage, this kind of awful abuse needs to stop. Doctors and society alike need to be way, way more EDS aware and more compassionate. If more people only knew what many of us EDSers are faced with!

Not all illnesses and disabilities are obviously visible, in fact, so very many are not! In spite of this being a fact, people continue to discriminate against those with invisible illnesses and disabilities, EDS very much included…and I think we also need to explore deeper reasons for this behaviour such as projected phobias and belief in human worth. Society needs a paradigm shift when it comes to their thinking regarding chronic illness and disability. Don’t judge a book by it’s cover; Understand that, sometimes, pain and illness may not be obvious to the eyes!

EDS info Brochure Below

Credit for these images: edsawareness.com