Pathogenesis: We have developed a model of the pathogenesis of malnutrition in cystic fibrosis. It consists of the relationship between nutrient balance and nutrient requirement. The validation has been conducted with respect to energy, but the same general principals can be applied to any nutrient. A patient with CF either loses weight or fails to grow normally if their absorbed energy intake is less than their total daily energy expenditure. Multiple factors have the potential to contribute to reduced energy intake including, anorexia, gastroeosophageal (GE) reflux leading to vomiting and hence food loss, as well as maldigestion. Another more recently recognized source of energy loss, is glucosuria as a result of CF related diabetes (CFRD). Conversely, lung inflammation appears to be related to increases in resting metabolic rate (RMR). Acute exacerbations of the chronic lung disease increases RMR which returns to a basal level some weeks after the inflammation is treated. In clinically stable patients with CF, RMR rises in a quadratic fashion as lung function falls. When FEV(1)is >85% predicted RMR is not different from controls, but it rises in a curvilinear fashion as FEV(1)falls. Initially it appears that patients adapt to their increased RMR by reducing their activity so their total daily energy expenditure (TDEE) is often no higher than controls. But this is by no means always the case. Furthermore good lung care requires CF patients to be involved in aerobic activities, hence their TDEE would rise. Although there has been considerable interest as to whether the genetic defect has an energy wasting effect, it appears genetic factors have little or no effect on RMR.
Treatment: This starts with making an energy diagnosis. First, a 3 day faecal fat balance study is conducted. This provides information with regard to intake as well as to maldigestion. In addition a history of GE reflux is sought, since it can readily be treated with H(2)-blockers. If significant fat malabsorption exists, efforts are made to improve pancreatic enzyme dose and function. The possibility of CFRD also needs to be considered. We measure the RMR of the patient using open circuit indirect calorimetry. Recommendations for diet therapy are based on estimated TDEE, which is determined from RMR taking into account faecal losses. Diet therapy places the emphasis on increasing the fat content of the diet. We have conducted a study to determine whether or not oral supplements help increase TDEE and they did not; they merely replaced food energy. Conversely, nocturnal gastrostomy supplemental feeding, while reducing voluntary food energy intake by about 20%, does result in a significant increase in total daily energy intake. Our target is to achieve a completely normal nutritional status. Long term follow-up of these patients has shown significantly better survival in patients who achieve normal nutritional status. The advent of lung transplantation has added another dimension. In our experience, following a successful lung transplant, most patients no longer need their supplemental gastrostomy feeding.
Summary: Our clinic policy is to encourage a high fat diet (35-40% total energy) and our patients grow normally in height and weight until their lung disease deteriorates significantly. Patients who develop a negative energy balance seldom if ever respond to diet therapy and hence are candidates for supplemental nocturnal gastrostomy feeds. Gastrostomy fed patients constitute 3 to 5% of our total CF population of approximately 590 patients.
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