Every 72 hours, a baby is born in the UK suffering from Hirschsprung’s disease — a life threatening bowel obstruction, usually diagnosed within days of birth.
It can be treated by surgery, but the outcome for some children is poor, and they go on to suffer lifetime incontinence.
Now a team of researchers at the University of Liverpool and Alder Hey Children’s Hospital is embarking on a radical study in the hope that stem cells isolated in the laboratory and transplanted into the bowel may hold the key to a cure.
Funded with a grant of £122,840 from Action Medical Research, the two year project is being led by consultant paediatric surgeon Mr Simon Kenny and developmental biologist Dr David Edgar.
Hirschsprung’s disease results in a lack of nerve cells in the rectum. It cannot be predicted before birth but is usually diagnosed soon after. The condition affects about one in 5,000 babies in the UK every year, but the surgical techniques used to remove the diseased section of bowel can have poor long-term outcomes.
Simon Kenny said that the Touching Tiny Lives grant from Action Medical Research was a ‘brilliant boost’ to the team, making the study possible by funding a research fellow and a technician, as well as the general study costs over the next two years.
He said, “It is marvellous that Action Medical Research has recognised the significance of this study, not just on Hirschsprung’s disease, which causes untold misery in families, but also on potential stem cell therapy treatments for other conditions, such as Parkinson’s disease.
“It is very early days, but we have already demonstrated that the cells we believe can stimulate new nerve growth can be isolated in the laboratory, and if these can be safely transplanted into the bowel of an infant suffering from Hirschsprung’s disease, we could theoretically form the nerve endings needed to overcome the problem.”
Simon Kenny at Alder Hey hospital in Liverpool. The hospital has a dedicated Hirschsprung’s disease clinic
Alder Hey has a dedicated Hirschsprung’s clinic and the follow-up of patients over the past two decades has shown that surgical techniques are not always successful.
Simon Kenny said, “By following the progress of our patients we are able to see that for many, surgical intervention is not a success.”
“Bowel incontinence can have a profound effect on a child’s life, their personality and social development. Incontinence is a private, secret thing and not something that is openly discussed outside the immediate family.
“Surgery to relieve the obstruction in the bowel is currently the only answer we have, but we estimate that around ten per cent of children who have had surgery go on to require a lifetime colostomy and up to three quarters of children have continence problems.
“I would like to think that our research can lead to better treatments and a better quality of life for children born with Hirschsprung’s disease.”
The University of Liverpool has a stem cell consortium, of which the Hirschsprung’s study is now a part. It is a centre of excellence for stem cell research in the UK and the hope is that this study may have ‘spin off ’ applications into other fields.
Simon Kenny added, “I’m really pleased that Action Medical Research has recognised the potential of this study in giving it financial support. It really does make all the difference to us as a research team and, looking ahead, may be the key to transforming the lives of hundreds of children in the future.”
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This article was printed from http://www.action.org.uk/touching_lives/2005/03/hirschsprungs/
It first appeared in our Touching Lives magazine in March 2005.