Symptoms began with slurred speech and occasional falls. Then my mom was told she had about six years to live.
Two years have passed since then, and things like walking down the street, getting dressed, or making a bed are things of the past for her.
My mom, Rhonda Boultier, was diagnosed with PSP, Progressive Supranuclear Palsy.
This brain disorder moves quickly and affects certain parts of the brain that control thinking and movement.
It took a lot of effort to convince my mom to use a walker when she was first diagnosed two years ago; she now uses a wheelchair.
A rare diagnosis
Around 180 Australians will develop the first signs of PSP each year, and around 1300 are currently living with it, according to the information group Fight Parkinson’s disease.
There is no cure, and researchers still don’t know what causes it.
Rhonda, a former elementary school teacher, was expecting the wrong diagnosis.
The neurologist told her, “The maximum time you have left to live is about six years; that’s about half the time of Parkinson’s disease.”
“But he said it changes for everyone,” she recalls.
My mother is 65 and most people diagnosed with the condition are around her age.
Dad, Chris Boultier, says it was frustrating to hear that progress was coming so quickly, but he’s learned to focus on what he can do to help.
“I’m going into ‘fight mode,'” says the retired cop.
Impulsive behavior and clumsy movements
Sam Balito, a neurologist at St Vincent’s Hospital, says life expectancy is a wide spectrum, and he sees patients with PSP who “live well beyond six years and still have a good quality of life”.
Mom’s PSP symptoms include focusing on small things that can consume her thoughts for days, and her social filter has become less tuned.
Changes in thought process and impulsivity are some of the keys used to diagnose this type of condition, says Dr. Balito.
Other clues include problems with balance and walking; stiffness; inability to track left and right or up and down; apathy; bladder control; problems with swallowing; and slurred or slurred speech.
Mom meets the requirements.
Her falls became more frequent and would have been more if her dad wasn’t there to catch her.
“When I bend over, I feel like I’m going to fall now, and I’ve lost faith in myself,” Mom says.
My sister Rebecca explains that she feels “on edge when mom walks” – and I feel the same way.
Especially when she’s not wearing a cochlear implant and can’t hear what’s going on around her.
“Watches every fall; every medical alert call I get gives me heart palpitations, says Rebecca.
More Australians could have PSP – and not know it
Fight Parkinson’s says only around 22 per cent of people in Australia with PSP receive the correct initial diagnosis.
Dr. Balito says this is because PSP has similarities to Parkinson’s disease.
“Classically, Parkinson’s disease starts in one part of the brain… the part that makes dopamine,” he explains.
Patients with early-stage Parkinson’s disease are treated with dopamine replacement drugs and improve.
But PSP doesn’t respond well to these drugs because it has a broader effect on the brain, Dr. Balito says.
The NDIS provides support, but it is a challenge
In Adelaide, Dad is putting strong tiles in their house for the equipment Mum will soon need and widening the doors so Mum can drive through with her wheelchair.
He can no longer leave his mother alone in their house.
Through the National Disability Insurance Scheme (NDIS), mum receives support for personal hygiene, speech therapy and physical activity.
Some of Dr. Balito’s patients were not so lucky.
“Many people with degenerative problems like PSP are initially turned down for the NDIS,” says Dr Balito.
But dad tells me he’s frustrated with the system not moving fast enough as my mother’s condition worsens.
“I’d like to get everything in place before any further progress actually happens, because if it does, we’re going to need it right there,” he says.
“You can’t change a situation, but you can make it better”
Mom advises everyone with such a disease – or a similar one – to talk about their experience.
“It’s important for the family to know what you’re going through because you can’t change the situation, but you can make it better,” she says.
The other day my mom said to my sister, “Imagine what Lyla (Rebecca’s four-year-old daughter) will be like when she’s 14, I probably won’t be around.”
My sister reacted by telling my mom, “Don’t say things like that!” But she knows it could be a reality.
Mom’s diary became the Bible in their home, which is now filled with notes on medical and therapy appointments.
But the most important thing for her is meetings with friends and family.