words Alison Hill photos Maria Reed
While his primary school friends fantasized about a life of fishing, football and surfing, Damian Jones was forced to accept he could never share their dreams. Growing up on Phillip Island with a rare disease and being ‘different’ from other kids is an isolating and sometimes lonely experience. It means a childhood dealing with teasing and intolerance, which sadly, often accompany the stigma of living with a disability. However, the Island, like most close knit communities, was like one big family. Damian grew up with many supportive friends who watched over him and became concerned with what was happening to his body. At the age of 11, Damian discovered that he had Fibrodysplasia Ossificans Progressiva (FOP). Due to the rareness of this condition, doctors failed to recognize the first sign, a shortened big toe. “No-one thought anything of it,” Damian recalls. Unable to participate in most childhood activities and being naturally shy, Damian had to find ways of entertaining himself. “I’ve always done my own thing really,” Damian reflects. “My bike was my main source of independence, and I was lucky enough to get my driver’s licence.” Growing up on a small acreage, I kept myself amused by helping out around the farm. Despite initial modifications to his car, Damian has now been forced to give up driving and relies on other people for transport. Put simply, FOP transforms soft tendons, ligaments, and skeletal muscle into hard bone, trapping its victims in a second skeleton. It is a rare genetic condition, with only 8 sufferers in Australia, and 500 worldwide. Damian’s initial symptoms were painful golf ball-sized lumps on his back. Suspecting cancer, his doctor referred him to the Children’s Hospital for further testing. Unaware of the danger, specialists ordered a bone biopsy, which Damian now realizes should not have been done, as any operation causes FOP to develop more aggressively. “Any attempt to remove the extra bone leads to an explosive growth of new bone. The slightest trauma such as bumps, bruises, childhood immunizations, or injections for dental work can cause the muscles to turn to bone,” says FOP specialist Dr Frederick Kaplan. Damian was diagnosed by Dr John Rogers, Australia’s FOP specialist. After the small lumps on his spine were discovered the deterioration accelerated,” says Damian. “It’s a feeling of constant pain when the muscle turns to bone, a sensation of heat in the area that’s hardening.” At the age of 16 he developed an interest in photography, but soon he was unable to lift the heavy equipment or hold it properly. Fortunately, with today’s lighter digital cameras, Damian has regained his interest. After trying his hand at different TAFE courses, dabbling in photography, computers and more recently, electronic engineering - he realized his heart wasn’t in it. Now 33 years old, Damians range of movement has become seriously limited. When he was 16, his spine
calcified, making it crooked, and his left shoulder stiffened, followed by parts of his arms. His jaw locked at 19, after which he lost partial movement in his legs. Eventually his left arm stiffened completely. As his joints progressively fuse he will become completely immobile, although he doesn’t know exactly when this will happen. Fiercely loyal to his family, who look after him, he knows it’s frustrating for them as well. He can’t sit in one position for any length of time, and he must constantly ask for assistance to rearrange his posture. So what do you do when you have FOP, and can hardly walk without the assistance of a walking frame, let alone get yourself out bed? Damian’s solution was to start his own business. Realizing he’d need the help and support of his family for the rest of his life, 13 years ago he built the Cowes’ Boarding Kennels and Cattery on his family’s 20-acre property. Originally renovating a disused building, he now operates a purpose-built facility, with accommodation for 20 dogs and 8 cats. Turning his love of animals into a business has been Damian’s salvation. “Sometimes you just feel forgotten, but feeling needed is a big bonus and the animals look up to you, they’re like your kids. I don’t wish to have kids, as there is a 98% chance they would develop FOP.” He has met other FOP suffers whose bodies have more limited movement then his own, and he says his strength has grown from watching other people survive. Damian survives on a disability pension and the earnings he makes from his business. “Being on a pension gets me around the bills. I can’t get any insurance from companies; they don’t want to cover me. ” There is still no known medical cure for this mysterious disease. Damian, however, has not given up hope, and he is excited that the gene responsible for FOP has been identified. “I’m slower physically now, so I have to keep my brain active by coming up with new ways of achieving my goals without asking for help.” What Damian longs for most is physical independence. Determined to make the most of his life, the 33-year-old is currently saving up for a specialized wheelchair, which will allow him a certain amount of personal freedom. The ‘Permobil’ C500 vertical wheelchair will allow him to sit and stand without assistance. While there are small government subsidies available, a wheelchair can cost anywhere up to $40,000. You get the impression that it would be unwise to feel sorry for Damian; he doesn’t have time for sympathy. “There are people who find it hard to accept what has happened to them, whether it is a disease or a self-inflicted problem. But you have to accept the challenges that you’ve been given and get on with life.” C
damian’s story
The Jones family
coast 32 Summer 07.indd 32
26/11/06 9:48:49 AM