Proteus Syndrome

Nine year old Jordan was born with a rare genetic condition which, in it’s extreme form affects less than one hundred people in the world. It is believed that Proteus Syndrome is the same condition that Joseph Merrick, the Elephant Man, suffered from.

It randomly affects different parts of the body causing bone, sinew and tissue to grow uncontrollably. Jordan’s legs have become so deformed that doctors say he shouldn’t be able to stand far less play football, which he does. Jordan lives in Bexhill-on-Sea with mum Tracy and dad Dean.

Proteus Syndrome is not an inherited condition, it’s caused by a, little understood, fault which occurs in the genes of the developing embryo. Because it’s so rare there is unsettled medical opinion on the best treatment for it.

Boy with Proteus Syndrome

Previous operations have removed sections of bone overgrowth from Jordan’s legs, but each surgical operation has high risks. As well as a long recovery period, surgery dramatically increases the risk of deep-veined thrombosis. DVT is the biggest cause of death in children with Proteus Syndrome. This gives mum Tracy a real dilemma, more surgery will be traumatic for Jordan and risk DVT, but doing nothing will allow the condition to worsen and will, almost certainly, confine Jordan to a wheelchair, a situation that would distress him a great deal.

Proteus Legs

Jordan attends a mainstream school where his physical deformities are accepted. His mental faculties are not impaired at all, and he does well in his lessons. Once a week, he has a physiotherapy session, because his joints are at continual risk of freezing up. It is essential that he is kept active and, to aid this, Tracy has raised the money to have a bike specially designed and built for him.

Soon, Jordan and Tracy will fly to America to visit the National Institute of Health in Washington D.C. The institute is one of the few places, in the world, that specialises in treating Proteus sufferers. For Jordan, his biggest hope is that they find a way to keep him walking.

It wasn’t until Jordan was 2 years old, that a doctor suggested he may have Proteus Syndrome. Tracy looked this up on the World Wide Web and found a support group and an email for the founder, Kim Hoag who suggested they fly to America to see Dr. Leslie Biesecker. Since this first diagnosis, Jordan has been part of Dr. Biesecker’s research programme into Proteus Syndrome.

Proteus Syndrome
Deformed Hand

While in America, Jordan will visit a Proteus family conference, he last attended one four years ago, it’s a rare opportunity to meet other children with the same condition. Of the 35 people in Dr. Biesecker’s research programme, eight families have flown into be at the conference. Because of the nature of the condition, each child with Proteus Syndrome is affected differently.

Kim Hoag’s son Alex died when he was 9 years old from DVT. This made doctors far more aware of the massive risk of blood-clots in children with Proteus, and made Tracy even less sure of the best way forward for Jordan.

Calcified Tissue
Extra Tissue Growth on Foot

With the conference over, Jordan now faces a week of intensive tests. During his last visit, Dr. Biesecker recommended major surgery for Jordan’s legs. This was carried out in England and was successful in the short term, but since then Jordan’s legs have continued to grow and deform. Of all the doctors Jordan will see this week, Dr. Laura Tulsey, the orthopaedic surgeon, is the most important as she will decide what, if any, surgery Jordan will require. Te following day Jordan needs to be anaesthetised for a two hour MRI scan to assess how far the condition has progressed in his neck and spine. After the MRI scan, a CT scan and several X-Rays, doctors warn that he may need spinal surgery in the future.

Dr. Tulsey explains that the new bone growth or calcification around and over the knee is slowly freezing his knee. She further confides that the surgeons are correct and that if something is not done soon he will stop walking. She has come up with a relatively simple procedure to deal with the problem. A metal staples placed on the side of the bone that is growing uncontrollably, should stop the affected side growing allowing the leg to straighten itself naturally. This will avoid the invasive surgery that Tracy and Jordan have been dreading.

Back home, Jordan’s doctor has agreed to the staple surgery and Tracy is thankful that the tests have shown no indication of the condition spreading to Jordan’s face.

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Jordan: The Boy with Proteus Syndrome
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