Brave boy battles on

He is a courageous, charismatic 10-year-old who suffers from a rare and life-threatening condition.

The Bayswater boy is one of three children in Australia with asphyxiating thoracic dystrophy, also known as Jeune syndrome, an inherited disorder of bone growth characterised by a small chest, short ribs and shortened bones.

Every bone in Shannon's body is dislocated and his lungs are the size of a 20-week-old baby's, forcing him to breathe with the help of a ventilator.

He also has a curved spine, putting further pressure on his tiny lungs and leaving him in constant pain.

Shannon needs care 24 hours a day, seven days a week and his statistics checked hourly.

Every two hours he needs fluid suctioned from his airways to enable him to breathe more freely.

Shannon is one of the luckier children with Jeune syndrome. Most die as an infant or in early childhood.

Despite the odds stacked against him, Shannon is courageous and gets his fighting spirit from his mum Samantha.

The mother-of-four knows all too well the cruel reality that her son's time is limited and has fought for the quality of his life since the day he was born.

"He was born by caesarean and the minute he came out he turned blue," Ms Stobie said.

"He was rushed straight to Princess Margaret Hospital and was in the intensive care unit for two years.

"The doctors said he had a rare condition and that there was nothing they could do for him so we should take him home to pass away.

"I wouldn't tolerate that."

Despite Shannon having a cardiac arrest in hospital and a high chance of brain damage, the Hillcrest Primary School student continued to fight with his mum by his side.

Ms Stobie said contrary to doctors expectations, Shannon was resuscitated without brain damage.

"He only got seizures," she said.

"Everything else was fine mentally, Shannon's problems are physical.

"He's also got loss of vision, it happened quite rapidly and every time he would lose vision he would have a seizure; he needed glasses and since he got them the seizures stopped."

Determined to find a way to help her son, Ms Stobie contacted a surgeon in America where Jeune syndrome was more common.

He told her there was hope for Shannon and that a series of surgeries to give him titanium ribs so there would be more room in his chest for his lungs to grow was an option .

The pint-sized West Coast Eagles supporter has had one surgery but was unable to have more which needed to be done before he was three, so his lungs are not at full capacity to support his body.

Ms Stobie said Shannon needed further surgery because if he ran out of room in his lungs to breathe, that was it. "That is going to be the hardest time in my life," she said.

"I want the best quality of life for my son and I believe he should have further surgery to give him the best chance.

"His respiratory doctor feels that when Shannon is well enough for surgery then he'll be fine but they're not willing to take the chance."

Ms Stobie said that although it had not been an easy road for Shannon he continued to fight.

"He has had four respiratory arrests, and one cardiac arrest, the recent one which was last year we nearly lost him because of the humidity, he can't be too hot or too cold," she said.

"The humidity is changing and it's getting hotter and he can't cope with that."

Ms Stobie said Shannon needed refrigerated air conditioning so his temperature could be stabilised but despite medical support, living in a Department of Housing home and on a carer's pension it was out of reach.

"It's amazing that he's lasted this long and everyday is just very special for us that we've still got him," she said