What if your child’s overeating was so uncontrollable
that it led to eating out of the garbage, ransacking the kitchen
and getting arrested for stealing food?
These scenarios are a daily reality for parents of children
with Prader-Willi syndrome (PWS) – a complex and currently
untreatable genetic disorder characterized by compulsive eating,
food foraging, massive weight gain and delayed mental development.
In the first study of its kind, University of British Columbia
clinical professor of pediatrics Dr. Jean-Pierre Chanoine
will test the effectiveness of a long-acting form of octreotide,
a drug similar to the hormone somatostatin, in reducing patients’
insatiable appetites. The drug is known to counteract ghrelin
– a recently discovered hormone that stimulates appetite.
In patients with PWS, ghrelin levels are three to five times
that of normal eaters.
“This illness is a nightmare for families,” says
Chanoine, who is head of the endocrinology and diabetes unit
at BC Children’s Hospital (Children’s) and a member
of the BC Research Institute for Children’s & Women’s
Health. “It means 24-hour supervision to keep the child
away from food and constant guidance to regulate behaviour.
Most patients face a bleak prognosis of massive obesity and
life-threatening related illnesses.”
In a year-long trial that starts this month at Children’s,
Chanoine will enroll 10 subjects aged 10-17 years who have
PWS. They will be given regular doses of octreotide or a placebo
to determine if the drug can decrease ghrelin levels and reduce
appetite.
A condition that affects an estimated 1 in approximately
15,000 children worldwide, PWS is caused by an abnormality
in a chromosome that is normally contributed by the father.
The syndrome affects males and females equally and is known
in all ethnicities. More than one-third of patients with PWS
weigh more than 200 per cent of their ideal body weight. Many
people with PWS experience complications of obesity such as
diabetes, respiratory problems and heart failure.
Genetic screening to confirm a diagnosis of PWS is available.
For more information on the study, contact Dr. S. Almazan
or Dr. Chanoine at 604.875.2624.
BC Children’s Hospital is an agency of the Provincial Health
Services Authority. The BC Research Institute for Children’s
& Women’s Health is a partnership of UBC and Children’s.
NB. Editors: A parent of an adolescent with
PWS is available for interviews. An electronic image of a
young child with PWS is also available.
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