Researchers Monitor Rare Immune Disorder in Aboriginal Children
 Severe combined immunodeficiency (SCID) is a group of rare genetic disorders
that interfere with the immune system's ability to fight against infections.
It is a serious, life-threatening condition that affects newborns.
Within the first few months of life, infants with SCID suffer from chronic
or recurrent diarrhea, pneumonia, ear infections and other serious infections.
Severe wasting begins soon after infections and diarrhea start.
Early diagnosis of SCID is critical. A child who receives treatment before
serious infections occur has the greatest chance of recovery. Without
prompt treatment, nearly all SCID patients will die within their first
year.
Estimates of the incidence of SCID are approximately 1 in 75,000-100,000
live births. At present, there is no Canadian data available on the incidence
of SCID; however, it appears that the rate is higher in Canada's aboriginal
population.
Determining the Incidence of SCID
Dr. Louise Pelletier and Dr. Rosemarie Ramsingh, researchers at Health
Canada's First Nations and Inuit Health Branch, are working to help determine
the incidence of SCID in Canada's aboriginal populations.
According to Dr. Pelletier, "This information is especially important
because infants with SCID may develop serious complications after receiving
BCG (bacilli Calmette-Guerin) vaccine, which is given to newborns in some
aboriginal populations to fight tuberculosis."
The researchers will use data collected through the Canadian Paediatric
Surveillance Program (CPSP), which is a joint project of Health Canada's
Centre for Infectious Disease Prevention and Control, and the Canadian
Paediatric Society. The CPSP gathers data from 2,400 paediatricians and
paediatric sub-specialists each month to monitor rare diseases and conditions
in Canadian children.
The Paediatric Surveillance Program will be asked to report any child
less than two years of age suspected of suffering from SCID. These reports
will also include information on Aboriginal ancestry of the infant, gender,
age at diagnosis of SCID, the main SCID symptoms displayed, and treatment
and outcome.
"Information about the incidence of SCID is necessary to help us
make the best decision - one based on scientific evidence - about the
risks versus the benefits of offering the BCG vaccine to aboriginal children
on reserves with tuberculosis," explains Dr. Pelletier. "The
research will also increase the awareness of all Canadian peadiatricians
about this severe disease and the importance of prompt diagnosis and treatment."
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