Section 10 - Cystic Fibrosis Clinical Care Pathway

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epidemic fashion within individual CF centre patient populations. Thus all B. cepacia isolates must be sent for genomovar typing. There is circumstantial evidence that social contact outside of hospital is important in spread of the epidemic strain within and between clinics. Fortunately the prevalence of Burkholderia cepacia in the CF clinic at GOSH remains very low. We do not have many infected patients and have no evidence for cases of clinic associated transmission. Cross infection is by coughing and contamination with infected sputum and in keeping with policies elsewhere, children infected with Burkholderia cepacia are seen in separate clinics. The following practical guidelines are in use should an individual, admitted to the ward, be found to be Burkholderia cepacia positive on sputum culture: Antibiotic Guidelines Nebulised and IV colistin should not be used if B. cepacia is the sole pathogen in the sputum. Consideration can be given to using nebulised ceftazadime (although the taste is unpleasant). Antibiotic therapy should be guided by sensitivities. Infective exacerbations may be treated with oral chloramphenicol, co-trimoxazole or ciprofloxacin. Nursing Guidelines • Consideration should be given to admitting patients to a ward other than Badger. • Patients with Burkholderia cepacia must sleep in their own rooms. • Chest physiotherapy must be carried out in the patient’s room only. • No other patients with CF (including others with cepacia infection) should go into that room (e.g for lung function testing). • Equipment (e.g.lung function equipment) must not be shared. If this is not possible then the Burkholderia cepacia positive child should use the equipment last, and the cleaning instructions must be followed after use. • Sputum pots and tissues must be disposed of immediately and hands must be washed immediately afterwards. • Staff dealing with all CF patients and coming into contact with sputum must wear gloves and plastic aprons and pay careful attention to hand washing before and after each treatment. They should be seen last on ward rounds. Social Guidelines • All children must be encouraged to cover the mouth when coughing. • Children can mix socially with other non-CF patients as long as they cough away from patients. Contact with other CF patients should be avoided. 28
• Intimate contact between patients should be avoided as this carries a high risk of spread. • Nursing staff must take responsibility where young children are concerned with regard to the amount of socialising allowed (e.g. children who do not cover their mouth when coughing). Stenotrophomonas maltophilia This organism is becoming more prevalent among CF patients. There is conflicting evidence as to whether it is associated with a decline in lung function. Current opinion is that it does not. Therefore only those patients who are chronically infected AND have clinical deterioration should be treated with anti-Stenotrophomans therapy. This is usually with cotrimoxazole (depending on sensitivities). There is no evidence for cross infectivity and therefore additional strict isolation protocols are not necessary. Methicillin-resistant Staphylococcus aureus (MRSA) A small number of patients will be colonised with MRSA. If sputum is found to be positive for MRSA then the child must be swabbed according to the MRSA screening protocol. Nasal carriage is treated with Mupirocin. In addition, family members should be swabbed and treatment arranged if found to be positive. If MRSA is suspected to be causing symptoms then treat according to sensitivities. Consider nebulised vancomycin preceded by nebulised salbutamol. For acute exacerbations include either teicoplanin or vancomycin. Linezolid may become useful in the future but at present experience is limited. The hospital policy on MRSA must be followed. Patients should be seen last on ward rounds and in an isolation area in the outpatient department. Atypical Myocobacteria Consider treating the mycobacteria if patients do not respond to conventional antibiotic therapy. Treatment length should be 6 to 12 months. Aspergillus Fumigatus (see <strong>Section</strong> 2.5) 2.2.2 Macrolides There is emerging evidence for the beneficial use of azithromycin in children with CF. In addition to the antibacterial properties it is thought that azithromycin may work by a variety of anti-inflammatory mechanisms. 29
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 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 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 •
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Section 10 - Cystic Fibrosis Clinical Care Pathway

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