QLexNursing

A. Water-soluble vitamins (Fat-soluble vitamins A, D, E, K in water-miscible form)

🫁 Children with cystic fibrosis have exocrine pancreatic insufficiency, leading to malabsorption of fat-soluble vitamins (A, D, E, and K).

🫁 These vitamins are essential for vision (A), bone health (D), neurological function (E), and clotting (K)—all of which are at risk due to fat malabsorption.

🫁 The vitamins should be given in a water-miscible form, which allows for absorption without the need for pancreatic enzymes.

🫁 This supplementation is lifelong and essential for growth, immune function, and overall development in children with CF.

B. Dornase alfa

🫁 Dornase alfa (Pulmozyme) is a recombinant human deoxyribonuclease that breaks down DNA in thick pulmonary secretions, improving airway clearance.

🫁 It is a standard part of respiratory management in CF to reduce the viscosity of sputum and prevent recurrent infections and airway obstruction.

🫁 Administered via nebulizer, it should be given daily or twice daily, depending on severity.

🫁 Dornase alfa improves pulmonary function, reduces exacerbations, and enhances quality of life in CF patients with chronic obstructive lung disease.

D. Pancreatic lipase (Pancreatic enzyme replacement therapy – PERT)

🫁 Children with CF need pancreatic enzyme replacement therapy (PERT) because their pancreatic ducts are blocked, preventing normal secretion of digestive enzymes.

🫁 Pancreatic lipase, amylase, and protease are replaced to allow for digestion and absorption of nutrients, especially fats and proteins.

🫁 This therapy helps reduce steatorrhea (foul-smelling, frothy stools), promotes weight gain, and improves nutritional status.

🫁 Pancreatic enzymes must be taken with every meal and snack, and doses are based on body weight and stool consistency.