Luciana Wulkan is a seven year old girl who lives a happy life in Bolton, England. Like other seven year olds she is a member of the Rainbows, loves going to school and seeing her friends and really, really wants to go horse riding. However unlike most young girls she understands the meaning of the words Fibrodysplasia Ossificans Progressiva, an extremely rare genetic condition that affects just 450 people worldwide.
FOP is a degenerative disease that slowly paralyzes the body. The faulty gene triggers muscles, tendons, ligaments, and other connective tissues to turn into bone. Bridges of extra bone form across the joints in characteristic patterns, progressively restricting movement. FOP is a disease in which the body produces not just too much bone, but an extra skeleton that immobilizes the joints of the body and turns sufferers into living statues.
Children with FOP appear normal at birth except for congenital malformations of the great toe. During the first or second decade of life, children form painful fibrous nodules over the neck, back, and shoulders which mature into bone in a process known as heterotopic ossification. FOP then progresses along the trunk and limbs of the body. Any surgical attempt to remove the extra bone results in fresh bone formation. People who have FOP experience different rates of new bone formation; in some the progress is rapid, while in others it is more gradual. In each case, the exact rate of progression is unpredictable but always debilitating.
To show what the future holds for Luciana, the film also includes Adrian a 28 year old FOP sufferer whose body has been ravaged by the condition, and 53 year old Robert Kinghorn who is frozen in an upright condition. Meeting these characters will give the viewer a clear understanding of what Luciana’s future could hold.
In America research into which gene is responsible for FOP is coming tantalizingly close to completion and with it the potential key to stopping FOP in its tracks. But will it come in time to prevent Luciana becoming as physically affected as Adrian?
The Girl Who’s Muscles Are Turning To Bone will be a window on to Luciana’s world and her journey as she learns to live with this terrible condition, ultimately however it is a film about a special girl, not about disability
For more information on Luciana's disease you can visit the website of the International Fibrodysplasia Ossificans Progressiva Association here.