A Life Worth Living: Pushing the Limits of Duchenne
This was the official website for promoting the documentary film, A Life Worth Living: Pushing the Limits of Duchenne.
The content below is from the site's 2011-2012 archived pages and other outside sources.
Jon Hastie has Duchenne - a severe form of Muscular Dystrophy. Prognosis - paralysis, 24 hour ventilation and an early death. Time is precious. Time is not for wasting. Jon decides to travel around the UK and Holland visiting a number of amazing Duchenners who have carved out remarkable lives for themselves, despite this most crippling of disabilities. A Life Worth Living is no ordinary road movie. It's a fun, inspiring, poignant and surprising rock 'n roll journey that takes us into the lives of guys who, against all the odds, show us how valuable life is. The film bears witness to the fact that life is to be treasured and lived to the full.
A Life Worth Living: Pushing the Limits of Duchenne
2 MINUTE TRAILER
Currently preparing for festival entries prior to full release in 2013
“Strength does not come from physical capacity. It comes from an indomitable will.”
Imagine you are three years old and your parents have just been informed that you have a gradual, increasingly debilitating disease and therefore a probable lifespan of 20 years… maybe. You have been given a death sentence. Mum and Dad are devastated, older brother is confused but you still feel like any other three year old except for frequent tumbles and a strange way of bending down.
Now fast-forward to February 2011, you and your family are celebrating your thirtieth birthday – nothing special in the big scheme of life but an extraordinary milestone for you and those around you. In the passing years you have progressed from falling over to walking with calipers, to sitting in a wheelchair, to being helped with bathing and going to the toilet, to being hoisted into bed every evening, to living in a specially adapted wheelchair and now being on a ventilator at night.
You can’t scratch your nose and now need 24/7 care from a variety of professionals. You aren’t able to hug or pat your best friend Jack and he’s probably the only soul who truly understands and accepts you as you are. You never get a good night’s rest, as you need to be turned over numerous times during your sleep. You’ve lost almost all use of your body from the neck down but you can still think, breathe, talk, laugh and enjoy a good glass of wine – albeit through a straw.
Soon you will be on a ventilator all day, not just at night. In the near future your other organs will cease to function properly. Eventually your heart will just stop. You have DMD (Duchenne Muscular Dystrophy) – a severe recessive form of muscular dystrophy typified by rapid progression of muscle degeneration, eventually leading to paralysis and early death.
During your birthday celebrations, you suddenly get a crazy idea. You are going to make a film: a film about some remarkable Duchenners who are all living productive, creative and fulfilling lives. You want to inspire a younger Duchenne generation and their parents by showing them just what can be achieved even under this most crippling of disabilities.
- Stuart is 28 years old, lives in Solihull and has written a World War II thriller.
- Carl is 23 years old, lives in Manchester and was voted ‘Mancunian Of The Year’ in 2010.
- Ian is 26 years old, lives in Wales and is a prolific artist
- Mark is 40 years old, lives in Edinburgh in his own flat and is a designer.
- Mahesh is 28 years, lives in London and is an avid fundraiser and campaigner.
- John is 42 years, lives in Holland and organizes pop concerts.
You want to go on a road trip to meet these amazing guys. This will be an extraordinary, demanding undertaking in itself considering the stage of your illness and your needs. But you want them to tell the world, especially the Duchenne world, not to give up hope and live life to the full. You had mentors and shining personalities who inspired you when you were younger, now you wish to encourage others by leaving a supportive, heartfelt message behind – Never give in. Never give up.
Tomorrow is the start of the journey – (raising the funds to finance the project). You’re nervous. You’re excited. You’re hesitant. You’re determined and eager to go. You are Jon Hastie, 30 years old, living in Worthing and you will make this film, this year.
Jon is 32 years old and has Duchenne Muscular Dystrophy. He is from the UK and lives in his own flat in Shoreham, West Sussex, having moved out from the family home shortly after the film was made. He is supported by a team of carers who provide his personal care. Jon has very little mobility throughout his body, requires non-invasive ventilation at night and increasingly during the day and can eat only soft or puréed foods.
Despite his complex care needs, he works part-time at a local disability organisation and is an active campaigner for the rights of disabled people. He also enjoys a good glass of wine or two with friends down the pub, and enjoys cinema, theatre and comedy shows.
Between 1999-2007 Jon lived independently at the University of Essex in Colchester. Over a period of eight years, he gained a Bachelor’s Degree in Politics and International Relations, a Masters degree in Environmental Studies and finally a Ph.D. in Government. His early university years were fairly typical of most students, characterised by clubbing, drinking, practical jokes and the odd bit of studying.
What Jon says about the film:
“As I reach my 32nd year, my deteriorating health makes me realise that there are more years behind me than ahead. But I’m not dead yet, and I’m determined to document just what can be achieved by people living with Duchenne Muscular Dystrophy. There are so many remarkable, spirited Duchenners out there who have so much to offer and can inspire a younger generation… it’s time that story was told.”
A Life Worth Living Day Twelve
The Cameraman Cometh
14/07/2011 BY JON
So here’s what the main cameraman had to say about his journey filming A Life Worth Living:
Jon’s Post-shoot thoughts
07/07/2011 BY JON
A few days after the shoot, here’s a roundup of my thoughts:
Netherlands Shoot Day Three
03/07/2011 BY JON
Greetings from Amsterdam…not a pillow in sight!
Netherlands Shoot Day Two
02/07/2011 BY JON
My thoughts on Netherlands Day 2:
07/10/2012 BY JON
We have made a decision about A Life Worth Living, this weekend. Following a few unsuccessful festival submissions, we have decided to focus on festivals without a requirement that the film be a premiere. This means that while continuing apply to festivals, we can also move forward with selling the DVD, which should be on […]
Back on track
03/08/2012 BY JON
It has been too long since the last blog… and there is so much to catch up on. We have had several screenings of A Life Worth Living, and have now finalised the DVD copy for release, once the film festival season completes. One thing which I don’t do well is waiting. Perhaps this is […]
Channel 4 News
05/04/2012 BY JON
This week saw A Life Worth Living go national, with a fantastic piece on the film on UK Channel 4 News. Katie Razzall does a fantastic and very complimentary job highlighting my life and the film. I’m greatly honoured! Check out the piece here:
18/03/2012 BY JON
Well things are really starting to heat up with A Life Worth Living, as we enter the promotion and distribution phase for the project. Another steep learning curve as we attempt to get our heads around the intricacies of film Festival submissions, DVD production & sales, TV commissioning processes – it’s enough to send your […]
The film A life worth living: pushing the limits of Duchenne is the story of academic, activist and now film maker, Dr Jon Hastie’s journey to meet other adults with Duchenne muscular dystrophy who are living productive, creative and fulfilling lives.
Living with Duchenne muscular dystrophy means living with a progressive, long-term health condition that presents you with a future of ongoing wasting of muscle until you are unable to move or even breathe without help. Indeed, for many parents of children recently diagnosed, it is a crippling blow from which some never fully recover.
With this in mind, one might think that a movie which documents the lives of adults living with this condition would be depressing, sad and bleak. But, in reality, the lives presented in A Life Worth Living are far more positive. These men show immense personal strength, adaptability and a determination to live their lives to the fullest extent possible, despite the day-to-day challenges they face. This story is about overcoming adversity, and creating a place for yourself in a world that is often disabling.
The idea for this movie came to me while sailing back from New York to Southampton on the Queen Mary 2, at the end of a month long trip. This represented a turning point in my own life, the culmination of a year of extensive planning for the most adventurous (and expensive!) journey I will ever take. It was for me the fulfillment of an ambition, and a reward for finishing 4 years of hard slog to complete a PhD in Environmental Politics.In the closing stages of this journey I was presented with 2 options: either treat this as the pinnacle of my life’s achievements and settle in for the forthcoming decline in my own ability in as much comfort as I could find or I could move on to the next project, and dream bigger! My own personality meant that wasn’t really very much of a choice. In many ways, I enjoy the planning and hard graft of bringing a project to fruition as much as the final result of the project itself. In contrast, sitting around waiting
I already knew, from planning my New York trip, that there were a number of people out there who, like me, have tried not to let Duchenne define their lives and achievements. Plus, I knew how comforting it had been for me to find out just a year previously that some people with Duchenne were still alive into their early 40s. Knowing all this made me intensely curious as to how other adults with Duchenne made this all work, but more importantly, made it clear to me that this was a story that others would want to hear too.
Drawing on recent disability road trip movies (including award-winners Darius goes west and 39 pounds of love), I decided to make a road trip movie following my journey to meet other adults with Duchenne. I knew the journey in itself was not going to be easy, requiring extensive planning to deal with the logistical challenges of transporting me, my carers and my equipment, plus the film crew and their equipment too.
Over the following year I started looking for people with Duchenne who were noted for various achievements beyond simply having the condition. The final cast totalled 6 people, including a digital artist, novelist and music promoter, all aged between 24 and 46.
On the advice of a friend in the film business, I then posted an advert for what I hoped to achieve on the film-making website Shooting People. This resulted in an overwhelming response and, after some research, I identified a director who shared a real passion for documentary and, having never encountered Duchenne before, was able to bring a fresh angle to the film that might appeal to the wider population.
Lacking substantial investment, I then began a two-pronged fundraising campaign to fund the costs of film production. On the one hand was an appeal via a Facebook page and dedicated website with regular video blogs for family, friends and the wider muscular dystrophy community to donate whatever they could to make the film a reality.
Alongside this was an appeal to charity and corporate sponsors to invest in a film, which could ultimately yield a financial return through embedded product placement and sales. This resulted in thousands of pounds of donations from some incredibly generous individuals inspired by the project, and investment from companies such as Etac and Brotherwood, who manufactured my wheelchair and adapted the car I use in the film. It also included funding from the charities Action Duchenne and the Muscular Dystrophy Campaign’s Trailblazers, who were given sales rights on the first batch of DVDs.
With the cast, crew and funding secured, filming began in June 2011 and lasted 2 and a half weeks. In that time, we travelled 2,200 miles, interviewed 6 adults with Duchenne, a young family whose son had been recently diagnosed and a renowned professor actively involved in providing medical care and advancing research for a cure for the condition.
The film we produced provides a revealing, honest and poignant glimpse into the lives of these remarkable men. No topic is off limits, we talk of the negative aspects of disability – dealing with anger and frustration, isolation and bullying at school – as well as more complex issues of family relationships, living independently, cultural views of disability, sex and relationships with partners, and dealing with the shadow of a shortened life expectancy. But, throughout, the positive attitudes and determination of these men shine through.
We see that, whatever situations these men are presented with, they are able to carve out a niche for themselves where they transcend the limitations of their condition. For those who receive personalised care and support to live independently, they are truly enabled to thrive, and establish for themselves a real life worth living.
By Jon Hasties
You can buy A life worth living from the Muscular Dystrophy Campaign
An aside: Duchenne muscular dystrophy was first described by the French neurologist Guillaume Benjamin Amand Duchenne in the 1860s, but until the 1980s, little was known about the cause of any kind of muscular dystrophy.
For people who do not know what Muscular dystrophy is:
Muscular dystrophy is a group of diseases that cause progressive weakness and loss of muscle mass. In muscular dystrophy, abnormal genes (mutations) interfere with the production of proteins needed to form healthy muscle.There are many different kinds of muscular dystrophy. Symptoms of the most common variety begin in childhood, mostly in boys. Other types don't surface until adulthood. There's no cure for muscular dystrophy. But medications and therapy can help manage symptoms and slow the course of the disease.
Duchenne type muscular dystrophy
This is the most common form of muscular dystrophy. Although girls can be carriers and mildly affected, it's much more common in boys.
About one-third of boys with Duchenne muscular dystrophy (DMD) don't have a family history of the disease, possibly because the gene involved may be subject to sudden abnormal change (spontaneous mutation).
Signs and symptoms typically appear in early childhood
People with Duchenne muscular dystrophy typically require a wheelchair before their teenage years. The life expectancy for those with this disease is late teens or 20s.
Certain genes are involved in making proteins that protect muscle fibers from damage. Muscular dystrophy occurs when one of these genes is defective.
Each form of muscular dystrophy is caused by a genetic mutation particular to that type of the disease. Many of these mutations are inherited. But some occur spontaneously in the mother's egg or the developing embryo and can be passed on to the next generation
In the late 1980's my cousin was seven he was diagnosed with Duchenne. MDA-supported researchers had just recently identified a gene on the X chromosome that, when mutated, causes Duchenne and other forms of muscular dystrophies. His symptoms worsened rather quickly and by the time he was around eleven he was confined to a wheelchair. My uncle was devastated and started drinking heavily. By the time my cousin died my uncle was a full blown alcoholic. Unlike today where there are a number of options to help someone quit drinking or control the impulse to binge drink, there was only going into rehab or joining AA, both requiring total abstinence. The National Institute on Alcohol Abuse and Alcoholism (NIAAA) still defines an Alcohol Use Disorder as a “relapsing brain disease”, but I think my uncle's drinking was caused by the anguish he felt over my cousin's diagnosis and it's bleak prognosis, not because he had a relapsing brain disease. I just finished working writing content for a website called LifeBac that is not a rehab or treatment clinic, but a collection of modern, science-based tools to empower people to take control over their excessive drinking. The program combines pharmacotherapy and psychotherapy. Interestingly the drug, baclofen, that is used in the LifeBac program is normally used to treat muscle spasms, stiffness, and other back conditions often associated with multiple sclerosis and spinal cord injuries. Researchers are exploring the possible predictors of response to baclofen when used for treatment of alcohol and drug use disorders. And although it has not yet received approval by the U.S. Food & Drug Administration (FDA) as a safe and effective treatment for alcohol or drug use disorders, doctors in Europe have been prescribing it as the primary treatment for people who drink excessively. I think the approach advocated by LifeBac would have probably helped my uncle.
This documentary was very inspiring from its initial concept and inspiration to actually seeing how people with Duchenne live their lives to their fullest. I am humbled by these remarkable young men.