Fibrodysplasia Ossificans Progessiva
Luciana Wulkan loves to dance and race around like many other little girls her age. But the seven-year-old is living in the shadow of a terrible disease – she is gradually being turned into a human statue by a very rare medical condition.
Luciana has fibrodysplasia ossificans progressiva (FOP), an affliction that turns muscle into bone, leaving victims frozen into position, Eventually, this extra bone-growth crushes the internal organs, causing death. The incurable condition is so rare, only 200 people in the world have it and Luciana is one of only 45 sufferers in the UK.
When Luciana Wulkan was born she appeared to be perfectly normal except that her big toes were shorter than the other toes. As her mother, Pina, would learn later this is one of the main clues when diagnosing fibrodyspasia ossificans progressiva (FOP). Pina takes Luciana to the Nuffield Orthopaedic Centre in Oxford, one of the few hospitals in the world that specialises in FOP, to meet Professor Paul Wordsworth who plans to X-Ray her whole upper body to assess how far the disease has spread.
Last year, Luciana developed a dangerous new bone growth under her chin. This could have caused her jaw to lock leaving her unable to eat or drink. In similar cases, other FOP sufferers have had to have their teeth removed. The X-Ray reveals that this growth appears to have subsided and is no longer an immediate cause for concern.
Childhood and adolescence will be the most difficult times for her, as FOP seems to be most active during growth periods. For severe flare-ups short-term treatment with steroids is a last resort.
As Luciana’s condition escalates, she may contort and freeze into a set position. If she’s lucky she may be able to choose that position for herself, if she isn’t her body will choose for her. Professor Paul Wordsworth explains that as an individual gets more and more ossification of the muscles they may have to make a very difficult decision about what they do with their life. Basically, before the body locks completely they must decide if they will spend their life sitting or standing.
Pina goes to Bellingham in Northumberland to visit Robert Kinghorn, who at 55 years old is one of the oldest surviving FOP sufferers in the UK. Robert’s body is almost completely frozen. He has a small amount of movement in his fingers and ankles but otherwise his body is totally rigid. He has elected to be in a standing position which he believes is preferable.
Robert Kinghorn was fortunate as he had an orthopaedic surgeon who made him a special back and leg brace to help him maintain his upright stance when his back began to bend. He is unable to walk and requires a customised trolley to move around. The picture to the right does not convey the complete lack of mobility experienced by Robert. On this custom trolley he looks like a shop mannequin being transported!
Pina then travels to Wigan in Lancashire to meet 28 year old Adrian Berry who, while diagnosed with FOP as a child, didn’t experience a rapid deterioration of the condition until he was in his teens. His back is badly bent by the FOP but he has managed to adapt well and still manages to work in his allotment where he enjoys the space and sense of freedom.
With question marks over her future, Pina wants Luciana to experience as many activities as possible while she still can. She takes her horse-riding which Luciana loves. One of the few chances Luciana has of escaping the confines of her body is her weekly hydrotherapy session. While nothing can be done to prevent flare-ups, hydrotherapy keeps her joints mobile and healthy. For the time being, Luciana retains complete freedom in her lower body, but as she gets older the condition will, undoubtedly, spread throughout her limbs.
Another activity planned by Pina will be a trip of a lifetime for Luciana as they go to Key West in Florida to swim with the dolphins at a dolphin therapy centre that caters for children with special needs.
CREDITS: All of the above information came from the UK television’s Channel 5 series entitled “Extraordinary People”
IFOPA is the International Fibrodysplasia Ossificans Progressiva Association, a non-profit support organisation for families dealing with FOP.