Gastrointestinal and Accessory Organ Problems. The case study is titled “Paul’s Adaptation to Cystic Fibrosis.” Paul is a 12-year-old boy with cystic fibrosis. He is hospitalized with pneumonia, and he has difficulty breathing. His stools are large and frequent, and they contain undigested food material. Questions for Analysis: 1. What is cystic fibrosis? Account for the clinical effects of the disease as evidenced by Paul’s appearance and symptoms. 2. What are the basic goals of the treatment of cystic fibrosis? Why is vigorous nutrition therapy a primary part of treatment? 3. Describe the role of enzyme replacement therapy in this aggressive nutrition support. 4. Why does Paul require therapeutic doses of multivitamins, including fat-soluble vitamins?
Please complete all questions in the case study in Ch.18: Gastrointestinal and Accessory Organ Problems. The case study is titled “Paul’s Adaptation to Cystic Fibrosis.” Paul is a 12-year-old boy with cystic fibrosis. He is hospitalized with pneumonia, and he has difficulty breathing. His stools are large and frequent, and they contain undigested food material. Questions for Analysis: 1. What is cystic fibrosis? Account for the clinical effects of the disease as evidenced by Paul’s appearance and symptoms. 2. What are the basic goals of the treatment of cystic fibrosis? Why is vigorous nutrition therapy a primary part of treatment? 3. Describe the role of enzyme replacement therapy in this aggressive nutrition support. 4. Why does Paul require therapeutic doses of multivitamins, including fat-soluble vitamins?
The Correct Answer and Explanation is:
Answers to the four case-study questions
| # | Answer |
|---|---|
| 1 | Cystic fibrosis (CF) is an autosomal-recessive disorder caused by mutations in the CFTR gene. Defective chloride transport makes all exocrine secretions thick and sticky. In Paul this manifests as chronic lung disease (pneumonia, dyspnea) and pancreatic duct obstruction that blocks digestive-enzyme flow. The pancreatic insufficiency leaves fats, protein, and carbohydrate only partly digested, so he is thin, easily fatigued, constantly hungry, and passes large, frequent, foul-smelling stools containing undigested food (steatorrhea). |
| 2 | Treatment goals are to (a) preserve lung function and control infection, (b) maintain normal growth and nutritional status, and (c) prevent or manage complications (e.g., diabetes, liver disease). Vigorous nutrition therapy is indispensable because resting energy expenditure is high, work of breathing is increased, and malabsorption robs the child of calories and nutrients needed for growth, tissue repair, and immune competence. Adequate calories, protein, and essential fatty acids directly correlate with better pulmonary outcomes and survival. |
| 3 | Pancreatic enzyme replacement therapy (PERT) supplies enteric-coated capsules of lipase, protease, and amylase that are swallowed with every meal and snack. By breaking macronutrients into absorbable molecules in the small intestine, PERT sharply reduces steatorrhea and abdominal pain, improves weight gain and linear growth, and allows high-energy diets (often 120–150 % of the RDA) to be meaningfully absorbed. |
| 4 | Paul needs therapeutic (2–4 × RDA) doses of multivitamins, especially fat-soluble A, D, E, K. Because fat absorption is impaired, these vitamins are poorly taken up in their natural form. Water-miscible or encapsulated formulations circumvent the defect and prevent deficiencies that would otherwise lead to night-blindness (A), rickets (D), neuropathy (E), or coagulopathy (K). |
Integrated explanation
Cystic fibrosis is fundamentally a disease of obstructive secretions: the same thick mucus that clogs Paul’s bronchi also blocks his pancreatic ducts. Without pancreatic enzymes, macronutrients—even when supplied in generous amounts—simply pass through the gut, so despite a “large appetite” he remains underweight with minimal muscle bulk. The undigested fat in his intestines attracts water, producing the voluminous, greasy stools described. Malnutrition then weakens respiratory muscles and compromises immunity, compounding the risk of pulmonary infections such as the pneumonia that precipitated this hospitalization.
Because nutrition and pulmonary status are so tightly linked, modern CF care treats food almost like a medication. Energy needs typically run 30–50 % above those of healthy peers, and high-fat, high-protein meals are encouraged. Yet for those calories to be useful, they must be absorbed—hence the central role of pancreatic enzyme replacement therapy. Taken in adequately titrated doses (usually 500–2 000 lipase units per kilogram per meal), PERT recreates near-normal intraluminal digestion, allowing the child to convert the extra calories into growth, fat stores, and respiratory-muscle strength. Better nutrition, in turn, improves pulmonary mechanics and immune responses, reducing hospitalization rates.
Micronutrients also deserve special attention. Fat-soluble vitamins enter the body in micelles that require both bile and pancreatic lipase; in CF, this pathway is impaired, so deficiencies are common unless water-miscible or high-dose preparations are provided. Vitamin A maintains epithelial integrity in the airways, vitamin D supports bone mineralization (important because chronic steroid use and inactivity heighten osteoporosis risk), vitamin E protects cell membranes from oxidant damage during lung inflammation, and vitamin K is essential for normal coagulation. Together with aggressive airway clearance, antibiotics, and regular monitoring, this comprehensive nutritional strategy has transformed CF from a uniformly fatal pediatric illness into a chronic condition compatible with adult life.
