Survival of infants born with Down's syndrome: 1980-96

The improved life expectancy of people with Down's syndrome as a result of the greater availability of surgery and advances in medical care has been w idely documented. However, there has been no evaluation of survival in the Australian Down's syndrome population since the 1980s. This study aimed to evaluate the changes in survival from birth in cases of Down's syndrome not ified to the Birth Defects Registry in Western Australia. Babies born with Down's syndrome between 1980 and 1996 (inclusive) and registered with the B irth Defects Registry were studied. Survival status was obtained in several ways. Cases were stratified into three cohorts for comparison. Survival cu rves were constructed using the methods of Kaplan and Meier. For infants bo rn during 1980-96, survival to 1 year is now > 91%, and 85% can expect to s urvive until the age of 10 years. Although survival in those with heart dis ease showed improvement over the period studied, overall this was still a s trong predictor of mortality. Survival in Aboriginal children with Down's s yndrome was significantly poorer than in non-Aboriginal children, mirroring the pattern in the general population. Mortality was greater in females an d in those with a low birthweight. There was no statistically significant d ifference in the survival between those born in metropolitan and in rural a reas. There has been a considerable improvement in survival of infants born with Down's syndrome in Western Australia. This improvement is similar to findin gs in recent international studies. The difference in survival between Abor iginal and non-Aboriginal children is particularly disturbing. These findin gs are useful for both clinicians and families who need to plan for the lon g-term care of these children.