Improving gut function in Hirschsprung Disease by adding new neurons

Drs Lincon Stamp and Marlene Hao, from the University of Melbourne’s Department of Anatomy and Neuroscience and Centre for Stem Cell Systems, have received a competitive international grant from the REACH Hirschsprung Disease Foundation.

Hirschsprung Disease is a genetic disorder, occurring in about 1:5000 new born children. These children have an obstructed large intestine, caused by the absence of neurons within the intestine that control expulsion of faeces.

Current treatment is to surgically remove the non-functioning segment of large intestine. In a large proportion of cases, the child continues to have debilitating bowel disorders, primarily faecal incontinence and periods of constipation.

Lincon and Marlene are exploring an alternative therapy to surgery. Through studies using rat models, they have already performed a colostomy and created a temporary stoma to by-pass the obstruction of the gut. They have also already recolonised the bowel of mice with Hirschsprung Disease with new neurons, created from neural stem cells isolated from the bowel. Muscle movement recordings have shown a full functional recovery of the gut.

Through the $US 30,000 grant from REACH, Lincon and Marlene aim combine these procedures with the idea of fully restoring colonic function.

“If this can be done, and our experiments so far indicate that it can be, then the procedures should be readily transferable to human infants.” Said Dr Lincon Stamp.

“This would significantly improve the quality of life of children suffering from Hirschsprung Disease” added Dr Marlene Hao.

REACH Hirschsprung Disease Foundation is a non-profit organization committed to improving the lives of children and families affected by Hirschsprung Disease.