Helping Emma To Live Without Pain: Pertness' Disease | Action Medical Research

Helping Emma to live without pain: Perthes' disease

Perthes’ disease is a condition that affects the hip joint. Children with Perthes’ are normally diagnosed after they start to experience pain in their hip or after developing a limp, usually around the age of five. More boys than girls are affected. Some children with Perthes’ develop permanent deformities or arthritis in later life.

The pain and difficulty in movement these children experience seem to result from a poor blood supply to the top of the thigh bone, which then softens and breaks down.

Currently, it’s not possible to predict which children will develop Perthes’ disease. By the time they’re diagnosed, their thigh bone is often already damaged.

New research: identifying who’s at risk and why?

Researchers in Hull have teamed up with colleagues in Southampton to develop screening guidelines to help identify children who are at risk of getting Perthes’ disease. This vital research is being funded by children’s charity Action Medical Research.

Professor Michael Fagan, of the University of Hull, is leading the team developing the screening guidelines. He’s also investigating possible preventative treatments that could spare many children from the pain caused by Perthes’.

Professor Fagan explains: “Over time, the hip can become stiff, and walking can become difficult and painful. Normally, just one leg is affected. If children have the condition for a long time, that leg may look shorter and thinner than the other.”

Current treatment promotes the natural healing process to prevent any permanent deformity or disability.

“Sometimes all that’s necessary is to monitor a child’s progress. Interventions include physiotherapy, bed rest, crutches, a plaster cast or special leg brace, and surgery. Treatment varies depending on the severity of a child’s condition, but even bed rest can be difficult for a child who is otherwise well,” explains Professor Nicholas Clarke, University of Southampton, joint investigator on the project.

The picture to the right shows an A-frame abduction brace used as current treatment.*

The team is investigating how the shape and position of the hip joint influence the process of the disease using highly sophisticated, three-dimensional computer modelling techniques.

They are using the same techniques to study whether certain activities and exercises, like horse riding or swimming, might have the potential to stop the disease from becoming established.

Emma’s story

Born by Caesarean, Emma was a happy, lively baby with no immediate problems. “She was a fidget before she was born so I knew she’d be an active child,” says her mum. She excelled at Tumble Tots as a toddler and later loved running, learned to swim at four and did well at trampoline club.

Then one day when she was just six Emma complained of pain in her left leg and had difficulty standing up. Four weeks later, she was still favouring her right leg and was referred by the GP for an X-ray.

A subsequent MRI scan confirmed that Emma has Perthes’ disease which affects the hip joint and usually affects just one leg. The hip can become stiff and walking can be difficult and painful. Some children develop permanent deformities or arthritis in later life.

Following Emma’s diagnosis of Perthes’ she had surgery to release a tendon in her groin. She needed to wear a hip brace for six weeks, which was a struggle particularly at school. Two years later she underwent a surgical procedure, during which her femur bone was cut and re-angled to improve hip function. Temporary plates were inserted to help correct the angle of her hip, and further painful surgery was needed to remove them.

 “Early on it was really, really tough. Emma became very sad and disruptive at school,” says her mum. Having shown such early promise as an athlete, Emma now concentrates more on music.

“She’s such a lively spirit and is very brave. Her dad is very positive, but I’m more cautious about Emma’s future. She has got a lot more growing to do which may mean more problems and even more pain!”

“I hope that the new research being funded by Action will make sure that other children like Emma don’t end up in the same boat. If something could’ve been done for her earlier, it would’ve saved a lot of pain, disappointment and heartache,” says Theresa.

Read more about the Perthes’ disease research here. And read more about Emma’s story in our supporter magazine Touching Lives.

You can also read Emma's own letter on living with Perthes on our donation page.

* Picture from: HK Kim, Legg-Calvé-Perthes disease. J Am Acad Orthop Surg. 18, 676-686.

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