Huichuan Lai

Cystic fibrosis (CF) is a genetic disease that affects more than 30,000 Americans. Malnutrition and lung disease reduce survival of cystic fibrosis patients; 20% of deaths occur before 20 years of age and half die before mid 30’s. CF is caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene, resulting in defective chloride transport and production of viscid secretions of low water content in exocrine glands. Major clinical manifestations of CF include malnutrition, growth faltering, nutrient deficiencies, airway obstruction and recurrent respiratory infections, leading to pulmonary failure.

Our research group investigates how nutrition affects treatment, quality of life and survival in people with cystic fibrosis (CF), one of the most common genetic diseases in the US. The ultimate goal of this research is to develop personalized nutrition therapy and evidence-based clinical practice guidelines to improve clinical care and health outcomes of CF. Since 2012, we have been conducting a prospective multi-center clinical study referred to as FIRST (Feeding Infants Right… from the Start) in six CF centers located in five states (WI, IL, IN, MA and UT), that follows a birth cohort of 180 children with CF from the neonatal period to 6 years of age (2024). FIRST has expanded to include many ancillary studies such as breast milk composition, gut microbiome with sibling control, probiotic supplementation, chest computed tomography and lung clearance index, and nutrigenomics and pharmacogenomics using whole genome sequencing-derived genotype data. The goals of FIRST are to identify optimal feeding regimens during infancy, to predict non-responders of vitamin D supplementation, to resolve the longstanding controversy of essential fatty acid supplementation, and to advance our understanding of the gut microbiome and its relationship to obesity, malnutrition, diabetes and liver diseases in children with CF.