Nutritional challenges in adult patients with cystic fibrosis pre and post lung transplantation

Abstract

Cystic fibrosis (CF) is one of the most common fatal hereditary disorders worldwide. 1 It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene codes for the CFTR protein, which functions as a channel for chloride ions and water across the membrane of cells that produce mucus, sweat, saliva, tears, and digestive enzymes. A mutation in this CFTR gene impairs the function of these channels, which leads to thicker and sticky mucus. Because the CFTR protein is present in all mucus producing organs in the body, CF is a multi-system genetic disorder affecting the lungs, pancreas, liver, intestine and other organs. 2,3 Symptoms of CF can differ due to the many different mutations in the CFTR gene, ranging from complete absence or mild dysfunction of CFTR. Depending on the impaired function of CFTR, patients may experience fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. 4 Therapy is directed to maintain and optimize pulmonary function and nutritional status, since these are key factors in survival. 5 Recent decades have shown a great improvement in survival of individuals with CF. 6 Registries in Europe, the USA, and Canada now demonstrate that median survival for CF is around 40-50 years. 7 This is much higher than the median age at death worldwide, which is only around 30 years 6,7 due to health inequalities and a dramatic variation in survival of CF individuals across Europe. 6,8 The increased survival in some Western countries can be explained by newborn screening programs, 9,10 centralized care 9 using evidence-based clinical and nutrition guidelines, 11 access to CF specific medications, and the introduction of drugs that modulate CFTR. 12,13 As the majority of children with CF now live into adulthood, the number of adults with CF is growing. This also means that more CF patients have different stages of pulmonary disease, with complex co-morbidity. Therefore, health care professionals need to be able to adapt the nutritional treatment of a CF patient across the life span. NUTRITIONAL CARENutritional status has a positive association with pulmonary function and survival in CF. 14 However, many patients suffer from an impaired nutritional status, due to a combination of increased nutrient demands, increased losses, and impaired nutritional intake. Insufficient nutritional intake is common in the CF population. There are many different reasons for this.First, CF patients often experience severe fatigue 15 which can contribute to a reduced food General introduction 20.