At his age, Thozamile Mciki from Gugulethu is the oldest person in Africa living with fibrodysplasia ossificans progressiva (FOP), a rare disorder in which muscle and connective tissue such as tendons and ligaments is gradually replaced by bone, becoming ossified. This bone forms outside the skeleton, constraining movement.
But when looking at this wheelchair-using 53-year-old from a distance, one would be forgiven for mistaking him for a child. He has a tiny, skewed and stiff body. When sitting close to him, it becomes evident that his body has turned into bone.
The average life expectancy of an FOP patient is 40 years, but some live longer. Fortunately for the kind and talkative Thozi, as he is affectionately known, he is one of them.
There is no flexibility in his body at all. He seems to be sitting across his wheelchair although he is sitting straight up. Mciki’s upper body is immobile from neck to waist. His hips and legs are completely stiff.
Mciki was diagnosed with FOP at the age of seven, at the Red Cross War Memorial Children’s Hospital in Cape Town in 1973. He says the disease was so rare at the time that even the doctors had no clue. “They concentrated on removing lumps that were developing in my body. Each time a lump was removed, a muscle which will later turn into bone would develop,” says Mciki.
He says his parents died not knowing his condition.
Estimates put the number of cases of FOP worldwide at about 5 000, with around 800 to 900 in South Africa. But only about 17 cases have been recorded in the country, Mciki being one of them.
Mciki says this is about to change. He is pinning his hopes on a documentary that he featured in, called Tin Soldiers, which he regards as more than just a film to raise awareness about this rare condition. “It is very educational. After watching it, people will be aware and be able to notice the symptoms,” he says.
Set in Brazil, the United Kingdom, North America and South Africa, this is a film about the real stories and daily struggles of FOP patients and the effect it has on their families.
It took Odette Schwegler nine months to shoot Tin Soldiers, starting in August 2018. The documentary was screened at the 2019 Jozi Film Festival in October and then at the Hollywood Independent Filmmaker Awards Festival.
Schwelger was filming a documentary for investigative journalism television programme Carte Blanche about rare diseases in 2010 when she learnt there was a meeting taking place in Brazil for patients from Africa and South America with FOP.
“This condition is very depressing. You need help on even basic things. It takes away your independence. It locks joints. Look at me, I need someone to wash me, at my age,” says Mciki.
The screening of Tin Soldiers has already yielded the desired outcomes, he says. “After the film was shown in Johannesburg at the awards, a 4-year-old was diagnosed with FOP in Alexandra after a family watched the movie. They immediately realised the symptoms and took the child to the doctor. There are more such cases. Imagine, there are people in rural areas with no internet. Those people are very clueless. That is where this film must be shown and be made freely available.”
Mciki says his parents started to notice something unusual about his bones when he fell from his bed at the age of eight months.
“I was told it was when they started to notice that my body was different. But they had no clue what I had. Even when the doctors tried to explain to them, they never understood.”
He says that although his bones were stiff, that never dampened his spirit. “I went to normal schools up to the high school level. I was always among the top learners.” And he did not end there. “I finished high school and went on to enrol at the University of South Africa for a Bachelor of Commerce degree.”
But, again, his condition dashed his dream in 1991. “I had to drop out just after a few days as I could not stay on the campus because I became a burden to other students. They always had to assist me when I had to walk to the toilet, shop, library, and to and from the class.”
He says his condition deteriorated in 2008. “My joints were completely locked. And that resulted in this wheelchair.” His sister, Nomaindia Mciki, had to give up work to care for him.
Mciki attributes his long life to his eagerness to learn more about the condition. It has earned him the role of FOP ambassador. He has been to many countries, attending conferences, where he has met experts. This made him an obvious interviewee for Tin Soldiers.
On 29 May 2016, Mciki travelled to the UK, where FOP patients and experts from around the world discussed ways to find a prevention drug to help prolong the lifespan of FOP sufferers.
South African FOP Association chairperson Chris Scott – an associate professor at the University of Cape Town and head of paediatric rheumatology at the Red Cross children’s hospital, who also features in the film and has been working with FOP patients for 10 years – says this is a “horrible” disease that shortens patients’ life expectancy.
“It starts to show in patients as young as four. They develop lumps on their backs. Those lumps then turn into bones. As the patient grows older, it becomes difficult to move their muscles as the body becomes stiff from neck to toe.
“Patients then die early from complications arising from not being able to breathe because of muscle constriction. This is indeed a horrible disease,” he says.
There is no cure for this rare disease. Experts are still trying to find one.
Tin Soldiers is currently being screened only at festivals. The team is still trying to secure broadcast rights.