Acta Scientiarum Polonorum Technologia Alimentaria

Cystic fibrosis (CF) was originally described as an exclusively digestive disease causing early death in infants due to extreme malnutrition before any sign of respiratory illness had developed. Once the pathological etiology was established, the suggestion was logical to introduce pancreatic enzyme therapy. One of the key findings in the care for people with CF was that energy is the magic cornerstone, which should be provided by food, rich in taste and calories. The next important step was to introduce enteral feeding using nasogastric tube and subsequently via gastrostomy. The help of psychologists is also invaluable in behavioural feeding problems at any age but especially in pre-school children. Since the earliest years, fat soluble vitamins were supposed to be problematic in CF because of the fat malabsorption. Their supplementation has been introduced and nowadays new specific preparations are available. Essential fatty acids and ω-3 long chain polyunsaturated fatty acids – increasingly have been demonstrated to be beneficial. Finally, feeding of newborns and infants has received the required attention. In many countries diagnosis is made at an early age thanks to systematic newborns screening. This is as efficient as the subsequent follow-up. At first stages breast-feeding should be continued. When it becomes impossible or not efficient, industry now puts a special formula (Cystilac) at our disposal, containing more energy, partially hydrolyzed protein, MCT fat, more salt, calories and vitamins, needing less enzyme substitution.