Bravery of humans who are 'tin soldiers' trapped in a second skeleton

Imprisoned by a second skeleton, 53-year-old Thozamile Mciki is a “tin soldier” with a heart.

Mciki, of Gugulethu in Cape Town, is the oldest known patient in Africa with fibrodysplasia ossificans progressiva (FOP).

He is one of 900 people in the world and 16 in SA who have the rare genetic condition that  slowly turns muscle, ligament and tissue into bone.

Those born with it eventually form a second skeleton. Injury and even inflammation spark rapid growth, leading to the joints locking over time, sending the person into a spiral towards immobility.

“We are human beings. Our bodies are hard, like tin soldiers, but we still have a heart,” Mciki said.

Mciki stars in a documentary, Tin Soldiers, which premiered at the Jozi Film Festival yesterday.

“This movie is an educational tool to doctors, parents and would-be parents. It will help unearth the secrets of this condition,” he said.

Mciki’s parents discovered he had FOP when he fell off the bed as at toddler.

“My parents told me that I got this sickness that is causing the hardening of the body.”

He lived his childhood as a spectator.

“I couldn’t do what other children could do. I couldn’t play soccer or ride a bike. I couldn’t go swimming because my arms are fixed together. I cannot stretch my arms. It was difficult to play with other children. Most of the time I watched others play.”

Dr Christiaan Scott, the head of paediatric rheumatology at the Red Cross War Memorial Children’s Hospital in  Cape Town,   treats most of SA’s FOP patients.

He said a person’s  mobility became progressively limited from early on.

“You will have a six-year-old going for their first day at school without being able to move their neck or pick up their arms  at all. We have just diagnosed a  boy whose arms and neck are already completely fixed.

He is only four. He falls over and bumps his head as he can’t move his arms to protect himself when he falls. Head injuries at a young pre-scholar age are a real issue,” said Scott.

People with FOP are born with malformed big toes. This is often missed by doctors. Instead, biopsies are done when a person starts to show signs of the disease.

Scott said biopsies actually sped up the effects of the condition. He said the documentary would raise awareness that could lead to more doctors diagnosing FOP at birth.

There were therapies in the pipeline that  could become available in the “next year or two”, he said.

“There are two clinical trials already fully enrolled and they hope to publish some time next year.

“If those studies are positive, we may be able to intervene, and we feel very positive that we will be able to intervene and actually prevent children from developing any kind of FOP, or at least slowing down the progression of this disease significantly.”

The documentary’s producer and director, Odette Schwegler, was struck by every patient’s “incredible ability to adapt”.

“I interviewed a mother 10  years ago and she was telling me her son was five years old when he was diagnosed. I asked: ‘How do you explain to a five-year-old that he has this condition and that it was going to rob him of movement?’

“She said: ‘I said to him, you know how your back is tall and stiff like a tin soldier, it’s going to get more like that. Tin soldiers, like all soldiers, are brave and strong and can get through anything.’ These patients are all brave and strong.”