Due to his muscle disease, Alex lost his partner and his business — but not his positivity: “I give my life a 9.”

Bron: RTL   Auteur: Roxanne Vis

HSP? If you've heard of it before, it's probably as an abbreviation for "highly sensitive person," the way some people describe themselves. But HSP is also a rare, hereditary, incurable disease that affects around a thousand people in the Netherlands. Alex Sijm (49) is one of them. The disease has drastically changed his life. "Friends and family can't help me accept the decline. That’s something I have to do on my own."

As a child, he already walked "differently." Alex had a noticeable gait. When he was about 6 or 7 years old, his parents took him from one doctor to another. But no clear cause was found. "They said the spinal cord was probably damaged around birth."

It seemed manageable; despite the unusual walk, he could do everything others could—and more. Alex dreamed of a career as a circus director, and he became one, even though everyone said he’d never make it. “At one point, I was running four circuses,” he says. “Not traditional circuses that traveled from A to B, but things like Circus in de Zorg (‘Circus in Healthcare’), where I visited care institutions. And Gay Circus Amsterdam, in collaboration with the AIDS Fund. I worked day and night and was always on the road.”

That all went fine—until, in 2022, he suddenly began experiencing all kinds of symptoms. At first, he blamed COVID. But one day, something happened he couldn’t ignore. “I was driving home on the A2 when I suddenly felt myself peeing my pants. I couldn’t stop it. Of course, I was shocked. I thought: Holy shit, what is going on here? There I was, sitting with soaking wet pants and a drenched car seat.” A serious wake-up call. “Now something was really wrong.”

Spastic legs

He was soon able to go to the Radboud university medical center in Nijmegen, to the expert center for movement disorders. There, on July 11, 2022, he received the diagnosis that derailed the life he had so carefully built: he has HSP, hereditary spastic paraplegia.

This hereditary disease of the spinal cord always involves spasticity in the legs but presents in many different forms. For some, it’s limited to spastic legs and incontinence, while others also experience neurological problems such as coordination issues, memory loss, and learning difficulties. In some cases, it can lead to severe disability early on.

In Alex’s case, it appears to be the so-called "pure" form, meaning only his lower body is affected. From that alarming moment in the car, things went downhill quickly. “Walking became more and more difficult, which caused severe back pain. The disease had always been stable in my case, but suddenly it started progressing.”

Three years later, he is now in a wheelchair. Physically, he’s lost a great deal, but it has taken its toll mentally too. “I could no longer continue my work, so I had to sell my business. I also lost my partner; five months after the diagnosis—on Boxing Day—he told me he couldn’t handle it. It felt like a bomb had gone off in my life that year.”

There’s also a 50 percent chance his daughter carries the gene. “As a parent, that’s the last thing you want.” And next week, his house is going up for sale, because he can no longer live there. “I can’t manage the stairs anymore and need an adapted bathroom and kitchen. You can imagine: if all of that happens within three years, it has a major impact.”

Accepting the decline

What he finds hardest is accepting the decline. “You constantly have to reinvent the wheel. I call it ‘living loss.’ Sometimes I feel like a lonely cyclist on a barren country road, riding a broken bike without electric support, pedaling into the wind. Deeply alone. Because even though I have the most loving friends and family around me who truly support me—they can’t help me accept the decline. That part, you have to do alone.”

But giving up is not in his nature. The title of the book he wrote about his experience says it all: Staying Down Is Not an Option,which he also turned into a theater show earlier this year. “Being on stage, inspiring people—that gives me strength and energy.”

Incredible perseverance

The disease is incurable, but symptoms can be relieved. Every three months, Alex receives twelve botulinum toxin injections to relax his muscles and stop the spasms. And he exercises every day, because it's now clear that physical activity is crucial in managing the disease.

“It takes an enormous amount of perseverance, because you get tired quickly. Someone once described it perfectly: it’s like driving down the highway pulling a caravan with two flat tires, but still trying to go 120 km/h. It takes a crazy amount of energy—but fortunately, it gives me energy too.”

Alex is naturally positive and prefers to focus on what he can still do. “The wheelchair has given me a lot of freedom back—I do all sorts of things. One month after I got the wheelchair last May, I went to a festival with it. A month later, I took my first flight. I just wanted to break through all those barriers right away. I even took wheelchair training, so I can use escalators at shopping malls. I’m always looking for solutions to stay part of society.”

Raising 100,000 euros in a week

To raise awareness and funds for research, Alex and others founded the Life4HSP Foundation earlier this month. Life4HSP “Because the disease is so rare, there was hardly any research into medications or quality-of-life improvements. There’s still so little known about it. That’s why we’ve united as patients—also internationally. A group of 1,000 people doesn’t interest pharmaceutical companies, but 800,000 patients worldwide is a different story.”

A week after the foundation’s launch, they had already raised over 100,000 euros. “We’ve already been able to set things in motion, though much more money is needed. Studies are underway. It’s still in its infancy, but at least something is happening. And that gives a lot of energy. And hope—hope that one day there will be a better treatment, or even a cure.”

What the future holds for him remains uncertain. “Doctors estimate that it will remain limited to my lower body and will eventually stabilize, but they can’t say anything with certainty. That’s the difficult part of this disease.”

Simply very happy

Still, Alex wouldn’t trade places with anyone. “If you ask me to rate my life, I’d give it a 9. That might sound strange, but everyone deals with something. One person loses a loved one, another is deeply unhappy in their job. Everyone has their share. I’m just really happy, despite everything. I try to make the most of life and always set a new goal on the horizon. That’s what I did with my circus dream, and that’s still what I do. I keep looking for opportunities.”

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