Section 10 - Cystic Fibrosis Clinical Care Pathway

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For cases of persistent steatorrhoea the following should be checked: • Is the child actually taking their enzymes with all meals and snacks? • Check stated dose per meal and snack against the reported daily total. • How are they taken? • Ideally, the dose should be split through a meal (half at the start, half in the middle) but failing this they can be taken at the beginning of the meal. The enteric coated preparations should not be crunched or mixed in with the whole feed. • Is the amount of enzyme altered with the fat content of the meal? • Has a previous increase in enzyme dose improved digestion? If not, has an H2 receptor antagonist (e.g. ranitidine) or proton pump inhibitor (e.g. omeprazole) been tried? • Are the enzymes stored correctly? Enzymes should be stored away from heat as this denatures the enzyme. Are the enzymes used still in date? • Are the enzymes taken with large volumes of fluid? Although it is important that children with cystic fibrosis drink plenty of fluids, taking enzymes with large volumes of fluid can result in the enzymes being washed too quickly through the gut leaving food but little enzyme for digestion. Stool microscopy can be undertaken to check for the presence of unabsorbed fat (stool elastase levels are unaffected by exogenous enzyme administration). Foods not requiring enzymes include: • Fruit (except avocado) • Vegetables (except potatoes, beans, peas and olives) • Sugar, jam, honey or syrup • Fruit juice, fizzy drinks, squash or water • Sorbet or fruit lollies • Special products such as glucose polymer powders and liquids and fruit juice based supplements e.g. Fortijuce. 46
3.3 Growth and Puberty Close monitoring of linear growth and weight should be carried out at each clinic visit. Measurements should be plotted on appropriate centile charts and height velocity calculated from measurements made over six monthly intervals. Infants with CF often grow poorly in the first year of life, particularly before the diagnosis is made. Catch-up growth usually occurs once treatment is established so that expected height for age is reached by five years. Growth during middle childhood is usually normal but a period of slow growth is often noted in the pre-pubertal period, leading to a decline in the centile rank around this age. The majority of patients with CF show some delay of growth in late childhood and this problem is exacerbated by the late onset of puberty that is commonly associated with CF. Children do tend to keep growing for longer than normal so they often regain their original height centile. The final height in the majority of patients therefore tends to fall within the normal range although often less than that expected from mid-parental height. Pubertal development should also be monitored. Patients with delayed puberty should be referred to the growth clinic. Estimation of bone age (x-ray of left hand and wrist) is best performed at the endocrine clinic itself rather than before referral. Depending on individual circumstances, some patients will be offered hormone therapy to aid onset of puberty. This is usually of great psychological benefit and does not appear to reduce final height. CF itself does not involve a primary abnormality of growth hormone secretion. Growth problems are usually related to poor nutrition, recurrent infections and disturbed lung function. Optimising CF therapy is therefore very important in the management of those with a falling height centile for age. Bone density (DEXA) scans should be performed at annual review in those with delayed onset of puberty and any patient on long term oral steroids. 3.4 Gastrostomy Tube Feeding Long term supplementary tube feeding may be necessary for some children with CF. At GOSH a gastrostomy is preferred to nasogastric feeding for the following reasons: • Nasogastric (NG) tubes may be dislodged with physiotherapy and coughing. • The presence of an NG tube may irritate the nose and throat and can be difficult to pass in individuals with nasal polyps. 47
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 44 and 45: d) Post-Intestinal Surgery Infants
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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