Scientists are hoping a pioneering Europe-wide research project into a rare degenerative brain disorder affecting young people will help them come closer to a cure.
This week is Huntington’s Awareness Week and experts in Sheffield announced they are leading a unique initiative aimed at helping those with Juvenile Huntington’s Disease (JHD).
JHD is thought to affect just 300 people in the UK and the research team is collecting data from 35 young people across the continent with the condition in a bid to learn more
Consultant clinical geneticist Dr Oliver Quarrell, who is leading the study for Sheffield Children’s NHS Foundation Trust, said: “We are extremely pleased to be leading this scheme across Europe and hope that by honing the information we get from families we can improve our patient assessments.
“As there are so few patients with JHD it’s taken a lot of work to find enough patients for us to monitor, but now our study is big enough to show important results. Hopefully this can lead to patients with JHD taking part in trials which could eventually yield a cure.”
Huntington’s Disease (HD) is an inherited, degenerative brain disorder which can be passed down from one generation to the next. Every child of a parent who carries the HD gene has a 50% chance of inheriting the abnormal gene.
A child who inherits the HD gene will eventually develop the illness, although usually not until adult life. The research team said HD affects one in 10,000 people and five in 10,000 people are at risk. HD can start at any age but begins most commonly between 35 and 55. It is only found in people under 20 in 5% of cases.
They said JHD is slightly different to adult HD so any treatments developed from adult trials need to be assessed before being given to children. Sheffield’s JHD scheme is part of the European Huntington’s Disease Network which also runs many projects and drug trials on “classic” or adult HD patients.
The JHD patients, who are aged up to 29, are assessed at regional centres near their homes and are living in areas including Sweden, Ireland, England, Italy, Poland and Holland.
Source: The Press Association
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