Fibrodysplasia ossificans progressiva

IN THE OPENING scene of last night's beautifully shot Horizon (BBC2), the camera waited patiently in a blowy, snow-swirled carpark as a middle-aged man walked into shot with his shuffling companion. It appeared at first sight to be his elderly father, but was, in fact, his young son. The father had to pick him up and set him down again in order for him to change direction, like a clockwork car on the last turn of its key. The boy walked bow-legged with a stick, so stiff that he seemed frozen. And, as it turned out, he was.

In victims of fibrodysplasia ossificans progressiva (FOP), healthy muscle is turned to bone. Victims wake up immobile from a Medusa-like nightmare unable to move their joints again as their wrists become permanently cricked, their arms pinned across their chests. Due to an unnatural brace of bone that had sprouted inside the crook of her elbow, one woman sat with her arms in a permanent hug about her tummy, her face, too, was cast in an unwilling rictus of forbearance. One young man was folded in half at the waist. He strode downhill with the top of his head pointing forward like a rugby player at the back of the scrum.

There was a sparse beauty of speech in those in close proximity to the disease, as if the intensity of experience had somehow crystallised their language. One doctor seeking a cure described how FOP "girds the chest wall with bands and sheets and plates of bone." A mother spoke of the "ribbons of bone" which swaddled her daughter's ribcage, compress- ing her lungs to the extent that she couldn't cough. The theme of opposites was incorporated cleverly and subtly. Still images of skeletons and scalpels flashed on the screen in a white negative before the colour flowed back, a subliminal reminder of the fundamental bodily inversion that FOP brings about. It was a film which made your jaw drop open before you caught yourself with the irony. It was a lovely film in which explanation and entertainment, those ever strange bedfellows, wrestled for the covers. Not for the first time in the history of Horizon, the resulting struggle kept the audience wide awake. It was easy, on the other hand, to sneer at The Day Britain Turned Disco (ITV), a programme screened to coincide with the 21st anniversary of the release of the film Saturday Night Fever. It featured, among other things, Simon Bates introducing the Yorkshire regional final of the British Disco Dancing Championships. Twenty years on, it seems implausible that people watched this stuff, but such a superior attitude ignores the fact that this decade the populace watched an entire series about a Welsh toilet cleaner who couldn't drive. And enjoyed it. There was a fabulous lack of irony from some of the contributors. "If you don't know what you're doing, then obviously you look like a bit of an idiot, don't you?" remarked one woman at a disco dancing class. Unfortunately, amid a flurry of arms and legs, her fellow pupils then demonstrated the opposite; that it was only at the point when you knew what you were doing that you looked like a complete idiot.

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