Section 10 - Cystic Fibrosis Clinical Care Pathway

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Mucolytics Individual nebulised dosage Doses < 5 years > 5 years Per Day 3% Saline 3-5 ml 3-5 ml Pre-physiotherapy Parvolex (n-acetylcysteine) 0.5 ml 1 ml or + + Pre-physiotherapy Mistabron (mesna) 3 ml NaCl 2.5 ml NaCl DNase (Pulmozyme) 2.5 mg 2.5 mg Once daily / alternate days Non Enteric-Coated Enzymes Per Capsule (BP Units) (for tube feeders & those unable to swallow) (>1 hour before physiotherapy) Protease Lipase Amylase Appearance Pancrex V Capsules 160 2950 3300 Clear capsules ‘125 (Paines & Byrne)’ containing powder. Enteric Coated Standard Preparations Nutrizym GR (Merck) 650 10000 10000 Olive green/yellow opaque capsules Pancrease (Cilag) 330 5000 2900 White opaque capsules. Containing microspheres. Creon 10000 600 10000 8000 Brown/translucent capsules 84 containing microspheres
Prescribable Nutritional Supplements Used in Cystic Fibrosis Product Manufacturer Comments Glucose polymer powders Caloreen Nestlé Maxijul Scientific Hospital All provide about 4kCal/g powder. Supplies (SHS) Can be made into a solution with Polycal Nutricia water or mixed in with food Polycose Abbott Glucose polymer liquids Maxijul liquid SHS Flavoured concentrated glucose polymer Polycal liquid Nutricia solutions. Can be drunk neat, diluted Milk shakes 1kCal/ml: Ensure Abbott Fresubin Fresenius with a fizzy drink or used to make jelly or instant puddings 1.5kCal/ml: Liquid in cans or cartons with a straw Ensure plus Abbott Various flavours (savoury and sweet) Entera Fresenius Fortisip Cow & Gate Powder: Calshake Fresenius Scandishake SHS 600 kCal (1 sachet + 240ml milk) Yogurt style Fortifresh Nutricia 1.5kCal/ml Non-milk based supplements Fortijuce Nutricia 1.5 kCal/ml Enlive Abbott 1.25 kCal/ml Provide Xtra Fresenius 1.25 kCal/ml This list is not comprehensive but mentions the most commonly used products 85
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Handbook for the Management of Chil
Page 4 and 5:
Contents Introduction 1. The Cystic
Page 6 and 7:
Introduction Cystic fibrosis (CF) i
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1.2 The Newly Diagnosed Patient The
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15 or 16. Young adults are usually
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• Urine dipstick and analysis Inv
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Chrispin-Norman Chest X-ray Scoring
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at schools and nurseries, children
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prescription of twice daily chest p
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e reviewed at each clinic visit. Tr
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inclination of the device from the
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intensive physiotherapy prior to su
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Pseudomonas aeruginosa The acquisit
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epidemic fashion within individual
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When should it be prescribed? a) In
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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 44 and 45: d) Post-Intestinal Surgery Infants
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 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 •
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Section 10 - Cystic Fibrosis Clinical Care Pathway

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