The amazing little girl dubbed 'Jigsaw Kid' ... with five spleens, back-to-front liver and two left lungs
By David Wilkes
On the outside, Bethany Jordan looks like any healthy little girl - cheeky grin included.But on the inside, she has been pieced together differently from everyone else. Dubbed the 'Jigsaw Kid', Bethany was born with five spleens, a hole in the heart, a diseased and back-tofront liver, a right lung formed as if it was a left one, and her stomach on the wrong side.
'Jigsaw Kid': Bethany Jordan, six, has a body like no other in the world Doctors warned her parents Lisa and Robert there was little chance she would survive birth. But she did, and six years later is still defying the odds.
Bethany, of Stourbridge, West Midlands, suffers from Ivemark Syndrome, a very rare disorder characterised by a poorly-formed cardiovascular system and having organs in the wrong place.
It means she cannot stand up to some of the normal rigours and strains of an average child. When she exercises too much, Bethany's heart can be seen beating through her back. There are so few sufferers - one in 150,000 - and each case is so different, that very little is known about the condition. The cause is thought to be genetic.
Yesterday her mother said: 'Underneath her skin everything is back to front and jumbled up. 'We have to really watch her because she gets tired very quickly because of her condition.' Her five spleens - each much smaller than a healthy one - do not work effectively at filtering her blood, leaving her at risk of harmful infections.
'It means that if she gets ill it takes a lot longer for her to get over it because she has a very low immune system,' said Mrs Jordan. Doctors at Birmingham's Women's Hospital discovered Bethany's condition before she was born during routine pregnancy scans.
They were not able to find a spleen and warned her parents she could also be suffering from Down's Syndrome. Doctors also thought Bethany could be brain-damaged, leaving her family with the agonising decision of whether to terminate the pregnancy. Later tests showed her brain was normal and Mrs Jordan chose to go ahead with the birth.
Bethany was born at nearby Wordsley Hospital, weighing 4lb 15oz. She spent ten days in an incubator before being allowed home. A month later she was taken back to hospital after her heart failed and in the ensuing months had operations on her liver, a collapsed lung and on the hole in her heart, before rejoining her parents and brothers Drew, 19, Joshua, 17, and Reece, 13, at home again.
Dr Patrick McKiernan, Bethany's liver consultant at Birmingham Children's Hospital, said: 'No two children who have this condition would be alike, but treatment for Bethany's syndrome is getting better and better.
'We only need to see her twice a year now, which is great news for her and her family. She will eventually-need a liver transplant but at the moment we are just waiting to see when that will be. 'She is doing very well despite all of her problems, I think she's a very tough little girl.'
Ivemark Syndrome sufferers are slightly smaller than other children their age because of the calories they burn off by their hearts having to work harder. Mrs Jordan, 37, said Bethany's condition only made her even more precious to her and Mr Jordan, 44.
'You have to treasure every moment and her story should be an example of that to all mums,' she said. 'Sometimes she does get a bit worried and says she doesn't want to be special. But she is a real fighter, she is my star and I love her the way she is - I always will.'
On the outside, Bethany Jordan looks like any healthy little girl - cheeky grin included.But on the inside, she has been pieced together differently from everyone else. Dubbed the 'Jigsaw Kid', Bethany was born with five spleens, a hole in the heart, a diseased and back-tofront liver, a right lung formed as if it was a left one, and her stomach on the wrong side.
'Jigsaw Kid': Bethany Jordan, six, has a body like no other in the world Doctors warned her parents Lisa and Robert there was little chance she would survive birth. But she did, and six years later is still defying the odds.Bethany, of Stourbridge, West Midlands, suffers from Ivemark Syndrome, a very rare disorder characterised by a poorly-formed cardiovascular system and having organs in the wrong place.
It means she cannot stand up to some of the normal rigours and strains of an average child. When she exercises too much, Bethany's heart can be seen beating through her back. There are so few sufferers - one in 150,000 - and each case is so different, that very little is known about the condition. The cause is thought to be genetic.
Yesterday her mother said: 'Underneath her skin everything is back to front and jumbled up. 'We have to really watch her because she gets tired very quickly because of her condition.' Her five spleens - each much smaller than a healthy one - do not work effectively at filtering her blood, leaving her at risk of harmful infections.
'It means that if she gets ill it takes a lot longer for her to get over it because she has a very low immune system,' said Mrs Jordan. Doctors at Birmingham's Women's Hospital discovered Bethany's condition before she was born during routine pregnancy scans.
They were not able to find a spleen and warned her parents she could also be suffering from Down's Syndrome. Doctors also thought Bethany could be brain-damaged, leaving her family with the agonising decision of whether to terminate the pregnancy. Later tests showed her brain was normal and Mrs Jordan chose to go ahead with the birth.
Bethany was born at nearby Wordsley Hospital, weighing 4lb 15oz. She spent ten days in an incubator before being allowed home. A month later she was taken back to hospital after her heart failed and in the ensuing months had operations on her liver, a collapsed lung and on the hole in her heart, before rejoining her parents and brothers Drew, 19, Joshua, 17, and Reece, 13, at home again.
Dr Patrick McKiernan, Bethany's liver consultant at Birmingham Children's Hospital, said: 'No two children who have this condition would be alike, but treatment for Bethany's syndrome is getting better and better.
Precious gift: Bethany's mother Lisa cradles her newborn daughter
'We only need to see her twice a year now, which is great news for her and her family. She will eventually-need a liver transplant but at the moment we are just waiting to see when that will be. 'She is doing very well despite all of her problems, I think she's a very tough little girl.'
Ivemark Syndrome sufferers are slightly smaller than other children their age because of the calories they burn off by their hearts having to work harder. Mrs Jordan, 37, said Bethany's condition only made her even more precious to her and Mr Jordan, 44.
'You have to treasure every moment and her story should be an example of that to all mums,' she said. 'Sometimes she does get a bit worried and says she doesn't want to be special. But she is a real fighter, she is my star and I love her the way she is - I always will.'
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