David Napier who suffers with Stiff-Person Syndrome (SPS), shares his journey with LDA Research advocating the need for more medical research into this rare disorder, as well as disability and age care rights.
Stiff-Person Syndrome (SPS) is a rare, progressive neurological disorder for which there is currently no cure. It usually presents in people aged between 40 and 60 years old, with a prognosis ranging from 6-28 years.
The focus is on managing the symptoms with sedatives, muscle relaxants and steroids, immunotherapies such as intravenous Immunoglobulin and plasmapheresis may be prescribed, and slowing down the progression of the disorder.
David, aged 77, lives in Sydney with his wife, children and grandchildren. He said that he lived with his condition undiagnosed for more than 21 years. He is one in a million of people affected by it.
‘Much needs to be learned about how Stiff-Person Syndrome develops,’ he said. ‘It is believed to be an autoimmune disorder where the body’s immune system attacks healthy tissues. Similar to other autoimmune conditions, SPS affects more women than men.
‘The physical presentation of the disease makes people extremely uncomfortable.’ He described the seizures he experiences, ‘Your whole body is involved, it doesn’t last long, but you know it is coming. Then you are a passenger. In a grand mal seizure I am fully conscious and aware, and it is terrifying.’ He experiences ongoing speech difficulties, falls, his legs feel like they are turned to concrete, unable to initiate movement, balance issues, and chronic pain that never stops. ‘Standing or walking is agony, double vision now corrected by prisms in my glasses, my many mobility aids, and last but not least is the anxiety and deep depression.
‘But, in many ways the invisibility is what makes it impossible for the majority of us to be believed. If you don’t see something, is it for real? If you can’t tell when someone is in trouble or having a seizure, or has speech issues, or their legs and arms contort or are unable to initiate movement it can be hard to empathise. The pain is excruciating and put me in hospital for eight weeks, three weeks in acute care and five weeks in rehabilitation being taught to walk again, followed by complete loss of speech for almost three months and again being taught to speak all over again – it is very real.
‘And what happens when people do see the real you? We are responsible for how we manage our condition, but I share the distress this causes with the one I love, my wife and 24/7 carer. She sees when others are uncomfortable by how my condition presents. I take multiple medications and lately have spent three hours every month in hospital having an infusion IVIG.’
David’s independence has also been affected by the fact that he can no longer drive, but he has recently had his wheelchair upgraded although a second hand model. ‘This one has independent suspension on all wheels and my chin control is gone.’
Undaunted, David is a committed advocate, passionate about disability rights and Age Care. He is involved with research which he finds challenging but rewarding and he is fighting to involve more people in medical research.
‘I did a speech to a group of schoolchildren where I was a living book, and stopped the teacher in her tracks when I said that my disability was a blessing. She asked me to explain, and I spoke of the very close relationship that I’d had with my grandchildren from the day they were born and now all at or finishing university. How many grandparents would like that opportunity?
‘Also my advocacy in the disabled space and the amazing people I have met online, all climbing the same mountain as I describe our Stiff Person Syndrome journey. How our lived experience was helping them and doctors to better understand and treat people with rare diseases with professionalism, empathy and compassion. While passionate about equality in life, sadly in Australia this is definitely not the case, while those under 65 years of age with profound disabilities are catered for, but those over 65 are not funded to the same extent causing distress and in some cases premature death.
‘I hope on our journey climbing the same mountain together, while we may be on different parts of the climb, if we can remain roped together supporting each other, no one is left behind.’
For more information: The Stiff Person Syndrome Research Foundation
About LDA Research
LDA Research is an international qualitative research provider. Set up in 2011 by Lucy Doorbar, the LDA team specialises in providing global intelligence in the pharmaceutical industry and medical device sector. We have a network of international associates as well as dedicated healthcare panels in the UK and US.
