“Jigsaw girl” shows constant improvement

Ivemark syndrome: Bethany`s rare case
Bethany suffers from the rare Ivemark syndrome, which is characterized by poorly formed cardiovascular system and wrong arrangement of organs inside the body.

Just one out of 1,50, 000 people are found to suffer from this disease, and each case is different. Genetic factors are thought to be behind this disorder.

“Doctors said her insides were like a jigsaw. If she does any exercise you can see her heart beating through her back.” says Lisa, Bethany`s mother.

Lisa explains that Bethany is at a higher risk of developing harmful infections because her spleens do not work effectively to filter her blood. This would mean, she added, if she ever gets ill it will take her much longer to get well due to low immune system.

Bethany`s story
During pregnancy scans in 2003, Doctors at Birmingham Women`s Hospital could not find any spleen in her, and told her parents, Lisa (37) and Robert Jordan (44), that the child may suffer from Down`s syndrome and brain damage.

Still, the parents went ahead and Lisa gave birth to Bethany. The girl was born with 5 spleens, weighed 2.2 kgs (4lb 15 oz), and spent her first 10 days in an incubator. She underwent some heart and liver operations.

Doctors positive about Bethany
Bethany`s liver consultant, Dr. Patrick Mckiernan at the Birmingham Children`s Hospital, said that Bethany’s health is getting better.

He said, “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."