Nutritional management of chronic disease — branching viva

Branch 1 — The cystic fibrosis child with a falling body mass index

The consultant presents a ten-year-old with cystic fibrosis and pancreatic insufficiency whose body mass index has drifted down despite a large appetite, with bulky greasy stools and two pulmonary exacerbations in the last year. ^[2]

Examiner probes:

1. What are the two mechanisms driving his malnutrition and what in the history points to each? Increased demand from the work of breathing and chronic infection, and malabsorption from pancreatic insufficiency with the steatorrhoea and the falling body mass index despite a good appetite. ^[2]
2. What is the energy and fat strategy and why is the fat not restricted? The energy target is commonly one hundred and twenty to one hundred and fifty per cent of the standard for age, with a high fat intake that is not restricted, because fat is calorie-dense and the malabsorption is managed with enzymes rather than by avoiding fat. ^[2]
3. What is the pancreatic enzyme and vitamin management? Pancreatic enzyme replacement at roughly five hundred to one thousand units of lipase per kilogram per meal titrated to the symptoms, with fat-soluble vitamin supplementation of vitamins A, D, E and K to prevent the deficiencies of malabsorption. ^[2]
4. When would you escalate the feeding route? When the oral intake cannot sustain the body mass index despite optimised enzymes and supplements, overnight gastrostomy feeds are used early rather than late, because the lung function and the nutritional status move together. ^[2]