Abstract
Achievement of optimal nutritional status within the first 1000 days of life for a child with cystic fibrosis (CF) is of paramount importance, with an emphasis on favorable early life growth trajectories that best optimize pulmonary and extrapulmonary health. The 'first 1000 days' framework emphasizes that environmental, sociocultural and nutritional exposures during this period can have life-long consequences for physical, cognitive, social and emotional health and development. Optimal nutrition encompasses not just physical growth, but the provision of nutrients and optimal feeding throughout the preconception, pregnancy and first 1000-day period to ensure lifelong healthy development and aging. For children with CF (cwCF), the first 1000 days is marred by a myriad of complications, exposing a unique nutritional fragility within this critical developmental window. Conversely, as life expectancy increases for people with CF (pwCF), overnutrition is becoming increasingly prevalent and the widespread uptake of disease-modifying drugs challenges clinicians to take a nuanced and personalized approach to lifelong nutritional care. This review explores early disease manifestations of CF and their impact on early life growth and nutrition in the modern era of CF. This review also considers how we might theoretically view early life nutrition in CF from a lens which takes into consideration well-known frameworks such as 'the first 1000 days' and 'developmental origins of health and disease'.