Section 10 - Cystic Fibrosis Clinical Care Pathway
Section 10 - Cystic Fibrosis Clinical Care Pathway
Section 10 - Cystic Fibrosis Clinical Care Pathway
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<strong>Section</strong> Three:<br />
Nutritional Management<br />
3.1 Nutritional Management<br />
Importance of nutritional status<br />
Malnutrition is not an inevitable consequence of CF and can be prevented or<br />
corrected. Poor nutritional status can lead to:<br />
• Stunted growth, delayed onset of puberty/sexual development.<br />
• Weight loss due to loss of adipose tissue and muscle wasting (including<br />
the respiratory muscles). This can eventually impair the ability to cough<br />
leading to an increase in respiratory infections and decline in lung<br />
function. Appetite may be further affected by the large amounts of<br />
mucus swallowed due to inadequate coughing.<br />
• Poor body image, leading to depression and school absenteeism.<br />
• Decreased immune function.<br />
• Specific signs of certain nutrient deficiencies (e.g. of fat soluble vitamins<br />
or essential fatty acids).<br />
Patients with CF may have altered nutritional requirements as a consequence<br />
of three interrelated factors: malabsorption, increased energy expenditure and<br />
poor intake.<br />
a) Malabsorption<br />
Increased stool losses of fat, fat soluble vitamins and protein often occur in<br />
CF due to one or more of the following:<br />
• Pancreatic enzyme deficiency (affecting >85% of patients).<br />
• Low intestinal pH, secondary to lack of pancreatic bicarbonate and over<br />
production of gastric acid. Some patients with persistent malabsorption<br />
may benefit from H2 receptor antagonists such as ranitidine taken 30<br />
minutes before meals or proton pump inhibitors such as omeprazole<br />
once daily.<br />
• Excessive, viscous intestinal mucus presenting a physical barrier to<br />
nutrient absorption.<br />
• Bile salt abnormalities (both quantitative and qualitative) due to<br />
decreased ileal bile reabsorption and increased stool losses.<br />
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