Wednesday, October 21, 2009

Extraordinary People - The Tiniest Boy in Britain

Extraordinary People - The Tiniest Boy in Britain - EP17

Five’s acclaimed documentary strand continues with the story of two-year-old Alex Connerty, Britain’s only primordial dwarf. Alex was born with an extremely rare condition that has stunted his development and means that he will grow to be no taller than three feet. This film follows Alex and his family as they travel to the US to meet the world’s only expert in primordial dwarves, in the hope that he can tell them what their son’s future holds.

Alex Connerty from Liverpool suffers from a remarkable genetic disorder called primordial dwarfism, which results in smaller physical development from the womb onwards. This condition, barely understood by doctors, affects an estimated 100 people in the world, and is so rare that it can be difficult to diagnose. So little is known about the disorder that doctors cannot even predict how long Alex will live – whether it be two, five or ten years. It is known, however, that primordial dwarves almost never live beyond 30 years, and that they are at greater risk of internal organ problems.

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This film meets Alex’s parents, Sue and John, as they talk frankly about living with a condition that has baffled medical experts. Sue explains that she knew something was wrong with Alex even during pregnancy. She had three miscarriages before conceiving him, so was especially alarmed when Alex did not move in the womb. “I bought a listening device so I could hear his heartbeat just to put my mind at rest,” she says.

Doctors assured Sue and John that Alex was simply a small baby; however, at 30 weeks, a scan revealed that Alex had stopped growing. Sue was rushed into hospital for an emergency Caesarean. “I was devastated – I thought I had lost another baby,” she says. Almost one third of primordial dwarves die at birth or soon after – but, against the odds, Alex survived. For the first three months of his life, he was kept in intensive care as he battled stomach and lung problems. Then a visiting geneticist examined Alex and came to a conclusion that his parents had never expected to hear. “She said, ‘your son is a primordial dwarf’,” John recalls. “I didn’t understand what she was on about. She tried to explain. [When] she told me he would never be bigger than three foot, I laughed in her face.”

After the news sank in, John and Sue had to come to terms with the terrible consequences of Alex’s condition. Aside from his curtailed life span, he would never be able to have children, and was at risk of slow development, spinal curvature and breathing problems. The couple were further staggered to learn that there was little information their doctor could give them. “He literally said, ‘I don’t know the answer’ to most of our questions,” Sue says. “We were flabbergasted.”

What is known is that Alex is in severe danger of suffering a ruptured blood vessel in his head – a common occurrence in primordial dwarves, whose brains often develop faster than their skulls can accommodate. This is why the Connerty family have decided to travel to Seattle tomeet the only world expert in primordial dwarfism : Dr Charles Scott. In Seattle, Alex has access to a type of MRA scanner unavailable in the UK, which may help give John and Sue the answers they need about their son’s future development.

While in the US, the Connertys also find time to visit the ‘Little People of America’ convention, where they meet the parents of other primordial dwarves, and discover that they are not alone in coping with this incredible and disturbing condition. “Our son will walk with giants for the rest of his life,” Sue says. “But to us he is a titan, and already people stand in his shadow –his family among them.”

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