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A Healthy Dose of Science
Sarah Gaskin

SCIENCE, once such an intangible term, is now increasingly finding its way into people’s lives. Science can play a role in almost anything – politics, history, even the fine arts, and not all science is necessarily to do with laboratory rats and nasty experiments. Medicine is one branch of science where thousands of research papers annually contribute to saving lives and helping the sick become well again. Studies add to existing knowledge of not only symptoms and better treatments but early detection and prevention of illness.

One of many diseases undergoing research is Cystic Fibrosis (CF). CF is a genetic disease caused by the mutation of the CFTR (Cystic Fibrosis Transmembrane Regulator) gene on chromosome 7. For every 2,500 live births in Australia each year, one of these babies will have CF. Half of the CF population will die before the age of forty. Research is being conducted worldwide, namely in Canada, Italy and the United States, on the genetic and phenotypic (visible symptoms) implications of the disease. In Australia, studies are under way at the Royal Prince Alfred Hospital in Sydney, Royal Children’s Hospital in Melbourne, Princess Margaret Hospital in Perth, the Adelaide Children’s Hospital, and also at the Royal Children’s Hospital in Brisbane.

This report concentrates on the studies being done by the James Fairfax Department of Nutrition, situated at the Children’s Hospital Westmead, Sydney. Their most significant research is concerned with the effects CF has on nutrition and life expectancy of children and teenagers with the disease.

The James Fairfax Department has been conducting research for the past twenty years. Over that period, they have produced over eighty papers on the effects of this disease. Although it may appear at times that the main purpose of research is for public, and even world-wide, recognition, it must be stressed that the benefits these studies have on those suffering from the disease are huge. New findings are constantly being incorporated into detection and early treatment.

A current study by the Department is investigating the rate that calories are being used up (resting energy expenditure) in girls with CF. Previous findings by this department have shown that children with CF, particularly females, burn up calories at a faster rate than children without CF. This is considered clinically important as it may account for the higher degree of malnutrition usually evident in female adolescent and adult CF populations. It is thought, though not yet proved, that the reason why females have such a high degree of malnutrition is a result of the hormonal changes that occur at puberty.

This particular study is measuring resting energy expenditure in females with and without the disease, before and after the onset of menstruation. It looks at those who have commenced menstruating on two occasions during their menstrual cycle. The data obtained will assist in indicating what extra levels of energy intake are required to obtain a healthy weight for females suffering from CF. Whilst this all seems very clinical, the implications of this study will eventually help in improving the nutritional status and growth of those with CF, and will ultimately enhance their survival and quality of life.

Research such as this one are conducted every day. The repetition of such experiments may seem tedious, time consuming, and costly. However, whilst appearing to be unsustainable, research projects such as these are made worthwhile through the willingness of the patients from the Children’s Hospital Westmead CF clinic, female volunteers without CF, those providing funding such as the James Fairfax Foundation, and of course, the researchers themselves. Those working in the department at Westmead Children’s Hospital put much time and effort into such studies. The research they have conducted is not only recognised at home, but also world-wide, placing Australia at the forefront of research involving CF.


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