Challenges with optimizing nutrition in cystic fibrosis

ABSTRACT

Introduction. Optimizing nutrition remains the cornerstone of therapy for patients with cystic fibrosis (CF) since it is associated with better pulmonary function and survival. However, a significant proportion of patients still fail to achieve normal growth and nutritional status.

Areas covered. This review describes the current challenges in providing effective nutritional therapy in CF with a focus on the current issues related to energy imbalance, dietary composition, adherence to nutritional recommendations, pancreatic enzyme replacement therapy, and the effects of modulators of the CF transmembrane conductance regulator.

Expert opinion. CF is a multisystemic disease that requires a personalized nutritional approach with accurate evaluation of energy balance. There is an urgent need for evidence-based recommendations on the dietary composition, in consideration of the increasing prevalence of overweight, diabetes and the potential effects of fatty acids on inflammation and immune response. More research into new pancreatic enzyme formulations is also required.

KEYWORDS:

• Cystic fibrosis
• nutrition
• diet
• adherence
• new therapies

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewers Disclosure

Peer reviewers on this manuscript have no relevant financial relationships or otherwise to disclose.