'Lily died under voluntary assisted dying laws. Here's what I want you to know about one of her conditions.'

A little-known illness has been reported on more so than is usual; with 23-year-old South Australian woman Lily Thai sharing her brave decision to end her life on Wednesday of last week.

Lily did so under the state’s voluntary assisted dying laws, laws that have been in effect since the beginning of the year.

Before Lily Thai ends her life using voluntary assisted dying laws on Wednesday, the 23-year-old has made a final powerful statement on the joy of living. #theAdvertiser https://t.co/uZPPLQq59j pic.twitter.com/3Y64VcYxnB

— The Advertiser (@theTiser) June 19, 2023

Lily shared with The Advertiser that she lived with multiple chronic illnesses – Ehlers-Danlos Syndrome (EDS) being one of them. Lily was also living with the exceptionally rare autoimmune illness, auto-immune autonomic ganglionopathy (AAG) and suspected Motor Neurone Disease, as reported by The Advertiser. 

EDS can cause widespread symptoms including; chronic pain, autonomic nervous system dysfunction, gastrointestinal dysfunction, profound energy deficit, allergies and food intolerance. In AAG the immune system attacks the autonomic nervous system, which can lead to autonomic nervous system neuropathy, and can be life-threatening.

It's #DysautonomiaAwarenessMonth! Autoimmune autonomic ganglionopathy (AAG) is a rare form of dysautonomia in which the body’s immune system produces antibodies that bind to receptors in the autonomic nervous system. Learn more about AAG here: https://t.co/foDoJZy50U. pic.twitter.com/7vKeJJOCrz

— Dysautonomia Intl. (@Dysautonomia) October 25, 2021

The 23-year-old said when speaking with The Advertiser earlier this month about her decision;

"I’ll no longer have any pain, I will no longer suffer with any of these issues, and I’ll finally be free of all the suffering that I have endured for so many years."

Whilst Lily's individual case as described in The Advertiser explained the involvement of much more than EDS alone; a similar level of disability with profoundly significant impacts on daily living is a reality for many living with EDS.

As a person living with EDS, the heartache is real when reading stories of people not well supported, nor well engaged in the healthcare system.

Whilst I personally do have a fairly sizeable disease burden from my EDS and the complications of it, I am still doing "ok" comparatively speaking. 

I was fortunate to have obtained a fairly quick diagnosis when my lifelong symptoms went from relatively mild to more severe in 2018. I am also lucky to have a robust medical team who are supportive and have never questioned my diagnosis, or the complicating factors of it.

But my journey is not the norm, it is the exception. 

The first time I wore a neck brace, after an appointment with a neurosurgeon in Washington DC who prescribed it, in 2019. Image: Supplied. 

For many, the harm faced collectively and individually, if wrongly psychologised or told they have functional neurological or conversion disorder can be massive. These wrong diagnoses, impossible for many to ever fully shake.

Lily touched on this when she spoke to The Advertiser discussing how after undergoing an unsuccessful treatment for what was thought to be a spinal fluid leak in Sydney, she found herself back home in Adelaide where doctors "never believed her", telling The Advertiser, she was told she was "making it up and doing it for attention". 

So what exactly is EDS and why is it important to take this opportunity to talk about it?

EDS is a heritable connective tissue disorder, of which there are 13 subtypes, the hypermobile type (hEDS) being the most common. hEDS is now thought to affect many more people than historically reported; with current prevalence rates placing it at 1 in 500 people, this compares to a historical rate of around 1 in 3,000. 

The ongoing dogma that hEDS is a rare condition, means many within the community are not able to easily access a diagnosis, leading to management and care that is too often never optimised. 

The result is a reduction in quality of life along with a greater disease burden for individuals than possibly might have been experienced had their diagnostic journey not been so difficult – this is called the “diagnostic odyssey” and for good reason. 

For many with rare diseases or 'invisible illnesses', for want of a better term and one that is problematic in its own right, obtaining a correct diagnosis can take decades. 

This odyssey is felt by those living with other contested and complex chronic illnesses too. Illnesses such as postural orthostatic tachycardia syndrome, myalgic encephalomyelitis / chronic fatigue syndrome and now LongCOVID. These are the folks who land regularly and squarely in the grey zones of medicine. 

Notoriously EDS is thought of as a condition that just makes you bendy. A positive if you do yoga some say, but this couldn’t be further from the truth. Many clinicians still don’t understand the multi-systemic nature of the condition and it is this mindset, which sees it viewed as ‘just a musculoskeletal condition’ that leads to harm in many patients. Labelling EDS as a musculoskeletal condition only is a massive oversimplification, and it ignores just how debilitating and disabling life can be for many with the condition. 

Owing to the fact that connective tissue is quite literally the glue holding your body together and that in EDS the integrity of the connective tissue is impaired, it really is very simple to see how symptoms and disease presentation can go beyond the musculoskeletal system. 

This week the grief has felt heavy. The grief of not only the lives lost, but the years lost too – of lives impacted so dramatically by care that could have been better – if only these conditions were better recognised and understood.

The grief, huge and all-encompassing, and the 'if onlys' racking up is a sentiment echoed in research findings published early this year by the Indiana University School of Medicine;

"... participants underscored their belief that clinicians’ negative attitudes were fundamentally the consequence of their limited understanding of EDS…"

Grief for the identities that only ever existed in dreams; with individuals' entire life outlooks, plans and maps being turned on their heads, and sometimes in the blink of an eye, by a condition or disease that there is no cure for.  

As a patient myself, but also as an advocate and healthcare professional, finding ways to push ahead and to continue to advocate when faced with such continual adversity is a slimly resourced balancing act. 

An act of willpower, determination and grit, that seems to be two steps forward and one back and only ever at half steam ahead. The drive being to force systemic change.  

Sadly, though it takes the collective energy of the sickest of the sickest to maintain momentum and effect this change. 

Detail by detail they push hard every step of the way; to be seen, heard and have their conditions validated. To have treatments and management fully optimised, the latter rarely occurring. And if it does, it isn't without massive sacrifice to one's own health, time, relationships, financial security and more.

I’ve been thinking of Lily Thai all day. This should never have been the best option for her, but I’m glad she has it. I can’t imagine her strength or the extent of her suffering.

— KC Martin-Stone, 5x💉 (@KCMartinStone) June 21, 2023

These people working tirelessly to effect change are primarily women, as it is women who are predominantly most severely impacted by EDS and other complex chronic illnesses. We are the carers and the patients, trying to make a positive impact for those newly entering the all-consuming sphere that is, a life with chronic illness. 

Be it complex, contested, rare or otherwise. Chronicity is the trait we all share.

Donations to support Lily’s legacy were invited to be directed to support palliative research at The Hospital Research Foundation Group.

Janna is a 38-year-old mother of two residing in Sydney's Eastern Suburbs with her young family. She lives with the chronic illness and rare disease, Ehlers-Danlos Syndrome as well as ME/CFS and other comorbid conditions. Janna is a pharmacist, medical writer and chronic illness patient advocate. You can follow Janna on Insta @The.Rare.Writer and on Twitter @Jan_nahlinke.

Feature Image: Supplied.