11th July 2012
Families and clinicians came together at a special event at Alder Hey Children’s NHS Foundation Trust on Thursday 12th July, aiming to raise awareness of Hirschsprung’s disease.
Hirschsprung’s is a life threatening, congenital condition where the nerve supply of an area of the bowel has not developed properly. This causes a blockage of the large intestine due to improper muscle movement in the bowel. Usually discovered either within the first few days or weeks of birth, the condition is rare and only occurs in around 1 in 5,000 children. Clinicians from the Department of General Surgery at Alder Hey are leading the way into research into the condition.
Consultant Paediatric Surgeon, (from the Department of General Surgery) Simon Kenny (left) pictured with young Jacob Jones, his baby brother Isaac and parents Sara and Gareth.
The event at Alder Hey has been organised by the family of Jacob Jones who was born with Total Colonic Hirschsprung’s disease in 2007. Now five years old, Jacob has been through a number of surgical interventions including five major surgeries and continues to have ongoing care at Alder Hey.
Mum Sara said: “When Jacob was born I thought we would never meet another family who had been through what we had. That’s why we set up the Champs Appeal which aims to raise awareness of Hirschsprung’s Disease and how it affects children and families.
“Since then we have linked up with thousands of families just like us. They all have lots of questions to ask and so it made sense to hold a conference at Alder Hey where Jacob is being treated and also where some essential research into future treatment is taking place.”
Pioneering stem cell research at Alder Hey in association with the University of Liverpool is aiming to improve the lives of children like Jacob. Consultant Paediatric Surgeon, Simon Kenny, part of the General Surgery at Alder Hey said: “Life-saving surgery is necessary soon after birth to remove the diseased part of the bowel, but many of these babies experience on-going bowel problems as they grow up. At Alder Hey we have subspecialised in the care of these children and as such we have high individual levels of surgical experience.
“Most babies with Hirschsprung’s disease become ill within the first couple of weeks of life. We have been able to isolate the stem cells that form the nervous system in the bowel from babies with Hirschsprung’s disease. We believe it may one day be possible to transplant a baby’s own stem cells into the bowel wall, to re-grow the missing nerve supply and improve the function of the bowel. Certain stem cells have the ability to turn into the right sort of nerves. When these cells are transplanted into experimental sections of bowel in the laboratory, they stimulate contractions. The research is investigating how these stem cells might behave if transplanted into humans, by studying what controls their growth and development. This is important, as uncontrolled growth could cause cancer. The knowledge gained might also allow the cells’ behaviour to be manipulated, to make any future transplants more effective.”
Nearly 100 guests attending the event had the chance to hear more information about the disease and this groundbreaking research. Surgeons, Colin Ballie and Simon Kenny from the Department of General Surgery spoke about the latest advancements in research into Hirschsprung’s Disease.
Sara concludes: “We hope that this event will help to raise awareness of the condition and show other families how much information and support is available.
“Jacob has been through a really rough time and I’m really thankful for what everyone at Alder Hey has done. They haven’t just looked after Jacob but have looked after me and my family too!”
For more information about the Department of General Surgery click here