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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d) Post-Intestinal Surgery<br />
Infants who have undergone surgery for meconium ileus may develop<br />
temporary disaccharide intolerance. As a precaution the use of a hydrolysed<br />
protein feed post surgery is sometimes indicated. Pregestimil (Mead Johnson)<br />
or Pepdite (Scientific Hospital Supplies) are both suitable infant formulae,<br />
being sucrose and lactose free and having a proportion of the fat in the form<br />
of medium chain triglycerides (MCTs). These feeds are prepared in the milk<br />
room at GOSH, and do require appropriate enzyme supplementation<br />
Infants may be discharged home on these products and the dietitian will<br />
advise the parents on feed preparation and prescription (via the General<br />
Practitioner). Thriving infants should be challenged with a standard infant<br />
formula within three months of the surgery. If this fails, sucrose and lactose<br />
free weaning advice may need to be given.<br />
e) Liver and Biliary Problems (see section 7.2)<br />
Tolerance of dietary fat and protein may be reduced in patients with liver or<br />
biliary involvement. If dietary restrictions are necessary, energy intake must<br />
still be maintained and adequate fat soluble vitamin status ensured.<br />
Introduction of ursodeoxycholic acid may aid absorption in these patients.<br />
3.1.3 Hospital dietetic service<br />
The CF Unit at GOSH has a dietitian who specialises in the care of infants<br />
and children with cystic fibrosis. Her role is to plan dietary management,<br />
assess and monitor nutritional status and offer advice on the use of pancreatic<br />
enzyme and vitamin supplements. The dietitian visits the respiratory ward<br />
daily, routinely reviews CF patients attending the outpatient clinic and takes<br />
part in the assessment of CF patients attending for annual review.<br />
The following patient advice sheets are available from the dietitian<br />
• CF Trust booklets: Nutrition: Eating well with <strong>Cystic</strong> <strong>Fibrosis</strong>. A guide for<br />
feeding infants.<br />
• Getting the balance right between fat intake and enzymes<br />
• Calcium. Are you getting enough?<br />
• Information Sheet on Dietary Management of CF Related Diabetes<br />
• Ways to increase calories in your diet<br />
• Increasing the energy content of your diet<br />
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