Malabsorption Syndromes – Exocrine Pancreatic Insufficiency (EPI)

Malabsorption of macronutrients (fat, protein, carbohydrate) is a result of a problem in one or more of the steps in the intestinal hydrolysis and subsequent transport of nutrients. Impaired secretion of pancreatic enzymes (EPI), changes in gastric, duodenal, liver, bile or gallbladder physiology or secretion as well as disturbed GI transit, motility or critical quantitative loss of intestinal mucosa all lead to malabsorption.

Malabsorption Syndromes are life-threatening conditions broadly impacting all macronutrients with each macronutrient critical to improved outcomes and survival. In addition, malnutrition impacts lung disease further complicating care for people with CF and progressive pulmonary diseases like bronchiectasis.

  • Fat malabsorption leads to reduced caloric intake, weight loss, fat soluble vitamin deficiencies as well as LCPUFA fatty acid abnormalities particularly omega-3 (e.g. DHA/EPA) leading to chronic inflammation, compromised immune function and reduced survival.
  • Protein malabsorption decreases muscle or fat-free mass, cachexia, impacts lung function, bone mineralization and results in GI pain.
  • Carbohydrate malabsorption results in GI symptoms and distress which leads to reduced appetite, changes to gastric emptying and alterations in gut microbiota.

Treatment of Malabsorption Syndromes rarely eliminates maldigestion, significant fatty acid abnormalities remain (e.g. long-chain polyunsaturated fatty acids) and undesirable GI symptoms as well as malnutrition continue.

There is a critical need for more effective treatments that can impact a broad spectrum of macronutrients which is necessary for improved outcomes and survival.

LCPUFAs (docosahexaenoic acid [DHA] eicosapentaenoic acid [EPA]): Beneficial Fats

LCPUFAs are the densest source of calories of any macronutrient. Evaluation of LCPUFA levels in plasma and red blood cells is a critical measure of nutritional status. People with CF have a profound deficiency in LCPUFA levels.