Section 10 - Cystic Fibrosis Clinical Care Pathway

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d) Post-Intestinal Surgery Infants who have undergone surgery for meconium ileus may develop temporary disaccharide intolerance. As a precaution the use of a hydrolysed protein feed post surgery is sometimes indicated. Pregestimil (Mead Johnson) or Pepdite (Scientific Hospital Supplies) are both suitable infant formulae, being sucrose and lactose free and having a proportion of the fat in the form of medium chain triglycerides (MCTs). These feeds are prepared in the milk room at GOSH, and do require appropriate enzyme supplementation Infants may be discharged home on these products and the dietitian will advise the parents on feed preparation and prescription (via the General Practitioner). Thriving infants should be challenged with a standard infant formula within three months of the surgery. If this fails, sucrose and lactose free weaning advice may need to be given. e) Liver and Biliary Problems (see section 7.2) Tolerance of dietary fat and protein may be reduced in patients with liver or biliary involvement. If dietary restrictions are necessary, energy intake must still be maintained and adequate fat soluble vitamin status ensured. Introduction of ursodeoxycholic acid may aid absorption in these patients. 3.1.3 Hospital dietetic service The CF Unit at GOSH has a dietitian who specialises in the care of infants and children with cystic fibrosis. Her role is to plan dietary management, assess and monitor nutritional status and offer advice on the use of pancreatic enzyme and vitamin supplements. The dietitian visits the respiratory ward daily, routinely reviews CF patients attending the outpatient clinic and takes part in the assessment of CF patients attending for annual review. The following patient advice sheets are available from the dietitian • CF Trust booklets: Nutrition: Eating well with Cystic Fibrosis. A guide for feeding infants. • Getting the balance right between fat intake and enzymes • Calcium. Are you getting enough? • Information Sheet on Dietary Management of CF Related Diabetes • Ways to increase calories in your diet • Increasing the energy content of your diet 44
3.2 Pancreatic Insufficiency Pancreatic insufficiency is present in about 85-90% of the CF population. Patients who are homozygous for the common ∆F508 mutation have a 99% chance of being pancreatic insufficient. This can lead to a variety of manifestations, including steatorrhoea, malnutrition, fat-soluble vitamin deficiency, growth failure and rectal prolapse. Distal intestinal obstruction syndrome (DIOS) can also occur. Pancreatic elastase can be measured on a 1 cubic centimeter stool sample, at any age. Specimens are sent to virology (for the attention of David Cubbitt) at GOSH for analysis. Normal levels are in excess of 500µg/g. CF patients usually have levels below 200µg/g. In pancreatic insufficient CF patients undetectable levels are frequently reported. The gold standard for assessing pancreatic insufficiency is by direct sampling and measurement of the pancreatic juices in the second part of the duodenum after stimulation. In practice, this is rarely done and is restricted to specialist paediatric gastroenterology centres. 3.2.1 Pancreatic enzyme supplementation There is no standard enzyme dose. The correct dose is that which symptomatically corrects steatorrhoea, abdominal pain and decreases frequency and mass of stools. More enzymes may be needed with a fatty meal or if stools are loose, frequent, offensive, pale and oily. Completely normal stools may never be achieved in some cases. Infants: Enteric coated mini microsphere enzyme preparations (Creon 10000, Solvay) are started in young infants. The starting dose is usually a quarter capsule per feed. The gelatine capsule is opened and the granules mixed with a little soft food such as fruit puree and given at the start of the feed. Alternatively, it can be mixed with some breast or formula milk on a spoon but should not be added to the baby’s bottle. Children: patients either swallow the capsules whole (over five years) or empty the granules directly into their mouth. The granules should not be chewed or crushed as this destroys the enteric coating. High doses of enzyme have been linked to the occurrence of colonic strictures (fibrosing colonopathy, see section 3.7). The use of high strength preparations is now discouraged in children and an upper limit of 10,000 international Lipase Units/Kg body weight/day has been suggested. 45
Page 1: Handbook for the Management of Chil
Page 4 and 5: Contents Introduction 1. The Cystic
Page 6 and 7: Introduction Cystic fibrosis (CF) i
Page 8 and 9: 1.2 The Newly Diagnosed Patient The
Page 10 and 11: 15 or 16. Young adults are usually
Page 12 and 13: • Urine dipstick and analysis Inv
Page 14 and 15: Chrispin-Norman Chest X-ray Scoring
Page 16 and 17: at schools and nurseries, children
Page 18 and 19: prescription of twice daily chest p
Page 20 and 21: e reviewed at each clinic visit. Tr
Page 22 and 23: inclination of the device from the
Page 24 and 25: intensive physiotherapy prior to su
Page 26 and 27: Pseudomonas aeruginosa The acquisit
Page 28 and 29: epidemic fashion within individual
Page 30 and 31: When should it be prescribed? a) In
Page 32 and 33: 2.3.1 On the ward It is unnecessary
Page 34 and 35: only be used on children who are ab
Page 36 and 37: 2.5.2 Haemoptysis Occasional streak
Page 38 and 39: weeks then long term maintenance th
Page 40 and 41: ) Increased energy expenditure Ther
Page 42 and 43: Vitamin K is not routinely given as
Page 46 and 47: For cases of persistent steatorrhoe
Page 48 and 49: • Children may be reluctant to go
Page 50 and 51: The type of feed chosen will depend
Page 52 and 53: Other conditions which should be co
Page 54 and 55: Section Four: Psychosocial Services
Page 56 and 57: physiotherapy, self managed techniq
Page 58 and 59: ICA does not depend on contribution
Page 60 and 61: send them a certificate (Form AG2 o
Page 62 and 63: upon a clinical assessment, the fam
Page 64 and 65: 5.6 Population screening There has
Page 66 and 67: Section Six: Lung Transplantation 6
Page 68 and 69: 6.2 Surgical Aspects and Post-Opera
Page 70 and 71: Other complications following heart
Page 72 and 73: Diagnosis In the presence of glycos
Page 74 and 75: 7.2 Liver Disease Introduction Path
Page 76 and 77: a catastrophic event and may requir
Page 78 and 79: 7.5 Arthropathy Cystic fibrosis art
Page 80 and 81: Section Eight: Pharmacopoeia Drug I
Page 82 and 83: Gastrointestinal Individual Oral Do
Page 84 and 85: Mucolytics Individual nebulised dos
Page 86 and 87: Section Nine: Useful Names and Addr
Page 88 and 89: OR For antibiotics order Pari LC Pl
Page 90: Cystic Fibrosis Trust Booklets •

Section 10 - Cystic Fibrosis Clinical Care Pathway

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