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Canberra Hospital and Health ServicesClinical Guideline Cystic Fibrosis: Management and Care of the Patient (Infants, Children and Adolescents)Contents

Contents....................................................................................................................................1

Guideline Statement.................................................................................................................2

Background........................................................................................................................... 2

Key Objective........................................................................................................................ 2

Alerts.....................................................................................................................................2

Scope........................................................................................................................................ 2

Section 1 – Care of the Inpatient with Cystic Fibrosis...............................................................3

1.1 Care considerations on admission...................................................................................3

1.2 Infection control considerations.....................................................................................3

1.3 Pharmalogical Considerations.........................................................................................5

1.4 Vitamin Supplementation...............................................................................................7

1.5 Involvement of the Cystic Fibrosis Team.........................................................................9

1.6 At Discharge..................................................................................................................10

Section 2 – Cystic Fibrosis outpatients – process for review when unwell.............................10

Implementation...................................................................................................................... 12

Related Policies, Procedures, Guidelines and Legislation.......................................................12

References.............................................................................................................................. 13

Search Terms.......................................................................................................................... 13

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Do not refer to a paper based copy of this policy document. The most current version can be found on the ACT Health Policy Register

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Guideline Statement

BackgroundThis guideline has been developed to guide the care of the infant, child or adolescent with Cystic Fibrosis (CF).

Cystic Fibrosis is an inherited genetic disease that severely affects the lungs, the digestive system (pancreas and liver) and sometimes the reproductive system. CF affects the exocrine glands, which produce body fluids such as saliva, sweat, mucus, tears and enzymes. When a person has CF, their mucus glands secrete very sticky mucus that clogs up tiny air passages in the lungs that can cause infection due to tapped bacteria. Repeated infections and blockages can cause irreversible lung damage. Digestive enzymes needed to breakdown food are not secreted by the pancreas, which results in problems with nutrition, and people who suffer from CF often need to eat foods high in kilojoules, fats, sugars and salt.

Key ObjectiveThis guideline aims to provide an overview of the baseline care required during an admission of a paediatric or adolescent patient with CF and to provide a safe and standard process for review of infants, children and adolescents with CF when they become unwell.

Alerts Infection control considerations are an important aspect of the care of the patient with CF, as these patients are at risk of colonisation with organisms that cause respiratory morbidity and accelerate respiratory decline.

Back to Table of Contents

Scope

This guideline pertains to staff caring for a paediatric or adolescent patient with CF admitted to (inpatient) or known to the paediatric (outpatient) service. This document applies to the following Canberra Hospital Health Services (CHHS) staff working within their scope of practice: medical staff registered nurses and enrolled nurses student nurses allied health




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Section 1 – Care of the Inpatient with Cystic Fibrosis

1.1 Care considerations on admissionOn patient admission, the following should be undertaken or considered: Full set of vital signs. Measure weight and height, then twice weekly weights. Plot serial centiles. Check Full Blood Count (FBC), Liver Function Test (LFT), Urea, Electrolytes, Creatinine

(UEC); Blood Sugar Level (BSL); Blood Cultures and Coagulation profile. Once the patient is stable check Vitamins A, D & E, Immunoglobulin g E, Aspergillus

precipitins. Sputum Microscopy Culture Sensitivity (MCS) – obtain recent results for comparison.

Include ‘Cystic Fibrosis’ in the patient history section of pathology requests. Chest X-ray if there are new symptoms or an X-ray has not been performed in the last 12

months. Inform the Nutrition Department (Extension 42567) of admission in order for menus,

nourishing mid-meal extras and salt supplementation to be implemented. If the patient receives enteral feeds, formula and equipment can also be arranged by

contacting the Nutrition Department. Inform physiotherapy of the admission. During business hours Monday to Friday the

senior Paediatric Physiotherapist should be contacted to review the patient. Over the weekend or after hours (until 2100) the weekend/evening physiotherapist will review the patient.

Inform CF team, paediatric respiratory physician, dietitian, social worker, physiotherapist, psychologist and CF Nurse of admission.

Consider contacting the paediatric endocrine team, the paediatric gastroenterologist, and/or genetics team for input/management.

1.2 Infection control considerationsStaff should refer to the Healthcare Associated Infections Procedure on the Policy Register (http://inhealth/PPR/default.aspx).

Standard precautions Standard precautions are work practices that are required to maintain the basic level of infection prevention and control. Standard precautions are recommended for the treatment and care of all patients, all body fluids, secretions and excretions (excluding sweat), regardless of whether they contain visible blood, non-intact skin and mucous membranes.

Standard precautions must be used for the treatment and care of all patients, regardless of their known or perceived infectious status and include: Hand Hygiene (HH) before and after every patient contact appropriate use of Personal Protective Equipment (PPE) safe use and disposal of sharps environmental cleaning cleaning and reprocessing of share patient equipment respiratory hygiene and cough etiquette



http://inhealth/PPR/default.aspx

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aseptic technique, and safe waste and linen handling and disposal.

Special considerations for patients with specific infectionsMulti-resistant organisms including MRSA, PseudomonasRefer to Additional Requirements for the Care of Children with a Multi-resistant Organism (MRO) in the Healthcare Associated Infections Procedure .

MROs can be spread from person to person or from contact with contaminated equipment or the environment.

The following requirements apply to patients known to or suspected of having a MRO: They must be placed in contact precautions in a single room with dedicated facilities and

where possible dedicated equipment. If the MRO is in the patient’s sputum, healthcare workers are also required to wear a

surgical mask when attending to the patient. The patient should be encouraged to stay within their room. CF patients with an MRO in their sputum and a cough must wear a surgical mask when

out of their room. Patients unable to effectively manage their respiratory secretion must remain within

their rooms. They should be discouraged from socialising with other children, particularly

immunosuppressed children. Patients with a MRO should not socialise with other non MRO CF patients, including in

the school room and playroom. They should not play with toys in the hospital playroom. Where possible hospital toys

should be limited and toys that are used must be appropriately cleaned after use. Best practice is for the patient’s own toys to be brought in from home and kept in their room.

If a patient colonised with an MRO attends school it is important that they are placed in a defined area away from other children. The area must be well cleaned after the school attendance.

Only one CF patient with an MRO can attend the hospital school at a time. Adolescents with an MRO may need to be allocated time in the activities room when no

other patients are allowed to use the room. The patients room must be cleaned on a daily basis. For repeat test and clearance requirements refer to Multi- resistant Organism Screening

and Clearance of the Healthcare Associated Infections Procedure .

Burkholderia cepacia Burkholderia cepacia complex (B. cepacia) consists of different species of bacteria that are found in the natural environment. Some of these species pose serious risks to the health of a person with CF. Burkholderia bacteria are often resistant to many antibiotics, which makes them difficult to treat once they infect the lungs. However, some species may be successfully treated with combinations of antibiotics.



http://inhealth/PPR/PP%20Archive/Healthcare%20Associated%20Infections%20Procedure.docx


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Basic infection prevention and control practices reduce the risk of acquiring or spreading B. cepacia. These bacteria pose very little medical risks to healthy people.

Patients colonised with B. cepacia will be cared for in a single room with a dedicated bathroom/ ensuite and have dedicated medical equipment. The general management of CF patients with B. cepacia is the same as above for the patient with a MRO.

If a patient with B. cepacia has appointments in other areas of the hospital e.g. respiratory laboratory, the receiving area must be pre-warned to avoid inadvertent contact with other susceptible patients.

Patient with B. cepacia who need to attend specific CF outpatient clinics should be placed in a defined area/consultation room, to limit contact with other CF patients. Note:For repeat test and clearance requirements refer to Multi-resistant Organism Screening and Clearance of the Healthcare Associated Infections Procedure. Clearance requires three consecutive negative sputum specimens for B. cepacia over a 12 month period.

Pseudomonas aeruginosaTo avoid premature acquisition, it is recommended that patients colonised with P. aeruginosa should be separated from other CF patients who are yet to acquire the organism.

Respiratory viruses [Respiratory Syncitial Virus (RSV), parainfluenza, Influenza etc]Additional droplet precautions are recommended for CF patients with symptoms of respiratory viral infections. When respiratory viral infections are suspected or proven, droplet precautions are implemented in addition to standard precautions and include single room with dedicated facilities. The patient must remain in droplet precautions until asymptomatic.

1.3 Pharmalogical ConsiderationsEmpiric Treatment for Tune up or Major Exacerbation – Antibiotics for InpatientsSpecimens (sputum, cough swab or blood cultures) should be obtained for microscopy and culture as soon as possible. If no culture results are available, use two antibacterial agents: Tobramycin starting dose 10mg/kg/dose up to 15mg/kg IV once daily (max 660mg).

o If the patient is >20% over their Lean Body Weight (LBW), use their LBW to calculate the dose (Ref: AMH CDC).

o If the patient has received IV tobramycin in the recent past, start with the dose they received at the end of their last admission, assuming therapeutic levels were attained.

o Adjust the dose as required according to therapeutic drug levels (see section below).AND Piperacillin with tazobactam (Tazocin®) 75-100mg/kg/dose (piperacillin component max

4g) IV q4(maximum 16 grams/day).




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IV therapy should continue for 10-14 days. Request Antimicrobial Stewardship (AMS) approval before Day 3 of therapy as per AMS Clinical Procedure.

In cases of penicillin allergy, an AMS code is required within the first 24hours. Use: Ceftazidime 50mg/kg/dose (max 2 grams) IV q6h (Ref: AMH CDC). Meropenem 40mg/kg/dose (max 2 grams) IV q8h (Ref: AMH CDC).

Tobramycin Therapeutic Drug Monitoring Tobramycin should be monitored after the first dose using the Area Under the Curve (AUC) calculation. The AUC should be checked evey time a dose is changed and then once weekly if the patient is stable. Target AUC is 100mg/L/hr with a range of 90-110mg/L/hr and target Cmax is 20-40mg/L. Tobramycin levels should be checked 2 and 6 hours after the infusion was commenced. Ideally tobramycin should be given at 0600 so that monitoring can be undertaken at

08:00 and 1200. This allows pathology to determine the levels and the ward pharmacist to calculate the

AUC to make a dose recommendation within the same day. Tobramcyin should be given in 50mL of normal saline and infused over 30 minutes. The exact time infusion was commenced, finished and the exact time the levels where

taken must be recorded on the medication chart and in progress notes so that the AUC can be calculated.

Directed Treatment – Antibiotics for InpatientsIf Methicillin Sensitive Staphylococcus aureus isolated from sputum culture in past 3 months, replace above regimen with: Flucloxacillin 50mg/kg/dose (max 2grams) IV q4-6h OR oral 25mg/kg (max 1gram) q6h

(Ref: AMH CDC). If the isolate is MRSA, substitute vancomycin for flucloxacillin, refer to Therapeutic

Guidelines: Antibiotic for information on paediatric dosing and monitoring.

If Stenotrophomonas maltophilia isolation from sputum culture in past 3 months, add: Trimethoprim/sulfamethoxazole (Bactrim™) 4mg/kg bd. [0.5mL/kg of (200 mg/40 mg

per 5 mL) mixture, maximum 20mL bd]. (Ref: MIMS Online). Note that 10mL of mixture = one 80/400mg tablet, and 20mL = one 160/800mg (DS)

tablet. Dose refers to Trimethoprim content.

If Pseudomonas aeruginosa isolated from sputum culture in past 3 months: Treat with two antipseudomonal antibiotics as for Empiric Therapy (as above). Eradication treatment of Pseudomonas aeruginosa.

The aim of this approach is to eradicate or delay early onset of pseudomonas colonisation of the CF lung after initial positive culture. For patients that are well use: Oral ciprofloxacin 15mg/kg (max 750mg) q12h for a further 2-3 weeks.

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Nebulised Tobramycin (Tobra-Day ™)(vial 500mg/5ml) - 80 mg nebulised bd. Children > 6 years:

Nebulised Tobramycin (Tobi Solution™) (vial 300mg/5ml) – 300 mg nebulised bd (every 12 hours, and not <6 hours apart) for 28 days (Ref: MIMS Online).OR

Tobramycin powder for inhalation (Tobi Podhaler™): 112 mg (4 caps) (inhaled) twice daily 12 hrs for 2 weeks. The first dose should be given under supervision prior to discharge, or in Paediatric Outpatients (Ref: MIMS online).

If child is unwell, or Pseudomonas is persistent despite above treatment seek advice from CF physician for either a repeat course or IV antibiotics as per above ‘Empiric Treatment for Major Exacerbation’ regimen.

Adjunct therapyMacrolide antibiotic an anti-inflammatory agentThe Paediatric Respiratory Consultant may prescribe azithromycin (a macrolide antibiotic) for use as an anti-inflammatory agent in CF patients (recommended for use in patients >6yrs of age). 10mg/kg/dose if body weight <15kg three times per week (M,W,F). 250mg if 15-40kg three times per week (M,W,F). 500mg if >40kg three times per week (M,W,F).

When macrolides are used in this way, a non tuberculous mycobacterium (nTM) cultures should be checked annually. nTM needs to be specified on the path form rather than just mycobacterium.

If prescribing for an inpatient, the AMS team will approve azithromycin prescriptions provided for the above criteria or in discussion with the admitting staff specialist for other circumstances. AMS Approval should be sought before Day 3 of initiation per the CHHS AMS Procedure.

Hypertonic saline inhalation5mL of solution (Hypersal ™) nebuslised pre physio twice a day. Commence at 3% hypertonic saline, and grade up to 6% hypertonic saline over a period of 3 weeks.

Hypertonic saline may cause or aggravate bronchoconstriction, therefore is often admistered following brochodilator therapy.

1.4 Vitamin SupplementationVitamin supplementation is divided into two categories:

A) Prophylactic supplementationBaseline supplementation of fat soluble vitamins is necessary in all patients who are pancreatic insufficient (PI), and may also be necessary in those who are pancreatic sufficient (PS) as guided by blood results. Factors that may contribute to deficiencies include poor



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nutritional intake, malabsorption, increased utilisation as a result of increased oxidative stress, liver disease, late diagnosis and poor adherence to therapy.

Recommended starting doses for Vitamin Supplementation in individuals with CF

Age Vitamin A (Units) Vitamin D (Units) Vitamin E (Units) Vitamin K (Units)

0-12 months 1500-2000 400-1000 40-80 150-500

1-3 years 1500-2500 400-1000 50-150 150-500

4-7 years 2500-5000 400-1000 150-300 300-500

8-18 years 2500-5000 400-1000 150-500 300-500

Adults 2500-5000 400-1000 150-500 300-500

In newly diagnosed infants the following doses provide fat soluble vitamin supplementation in line with the recommendations above (except for vitamin K): 0.45mL Pentavite (contains vitamin A and D) + 0.5mL Micelle E. This provides 1620 units

of vitamin A, 405 IU vitamin D and 78 IU vitamin E.

Please note vitamin levels need to be checked to ensure adequacy of supplementation. This needs to take place during a period of clinical stability (vitamins A and E act as antioxidants and will be lower during periods of oxidative stress).

VitABDECK is a CF specific multivitamin which is commonly used with children, adolescents and adults. Infants can be given an opened capsule of VitABDECK, or part thereof, or a combination of Pentavite and Micelle E can be used (as above).

Current dosing recommendations for VitABDECK multivitamins

Age Dosage

0 – 1 yr 1 cap daily

1 – 10 yrs 1 -2 caps daily

> 10 yrs 2 caps daily

B) Treatment of Vitamin DeficiencyIf fat soluble vitamin deficiencies are detected on routine blood tests then additional quantities of these vitamins will be required.

Vitamin A and E can be given in liquid form using Micelle A+E or Micelle E. Vitamin D can also be provided separately in liquid form. It is important to check levels 3 months after commencing replacement therapy as a guide to adherence and/or therapeutic response.

Excessive vitamin intake can be hazardous. Treatment threshold for vitamin deficiencies are a Vitamin E level of 12 umol/L [normal range 12-36 umol/L] and a vitamin A level of 0.8



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umol/L [range 0.8-2.5 umol/L]. Vitamin K supplementation is guided by the paediatric gastroenterologist.

Vitamin A and E dosage for paediatric patients with vitamin deficiency

Age Dose Available as Dosage

Vitamin A 0 – 5 yrs

> 5 yrs

5,000 units / day

10,000units / day

Micelle A & E*

Liquid

2 mls / day

4 mls / day

(recheck – 3 mths)

Vitamin E 1 – 5 yrs

> 5 yrs

25 units / kg / day

250 units / day

Micelle E**

Liquid

0.15 mls / kg / day

2.0 mls / day

(recheck – 3 mths)

1.5 Involvement of the Cystic Fibrosis TeamIt is an expectation that the admitting team ensures engagement of the other members of the CF team by making timely referrals to the teams involved in care of patients with Cystic Fibrosis, including the paediatric respiratory physicians, paediatric gastroenterology physician, and the genetics team (for newly diagnosed patients).

Physiotherapy Referral to physiotherapy should be made when the patient initially presents to the Emergency Department or on direct admission to the ward. During business hours Monday to Friday, the paediatric physiotherapist should be contacted to review the patient. Over the weekend or after hours (until 2100) the weekend/evening physiotherapist will review the patient. Frequency of physiotherapy intervention will then be decided by the reviewing therapist.

Physiotherapy for people with CF incorporates physiotherapy techniques to assist with airway clearance, thoracic mobility, posture and exercise to maximise cardiorespiratory fitness, strength and bone density. The goal of chest physiotherapy in CF is to enhance mucociliary clearance and impede the development of progressive suppurative lung disease.

Nutrition (dietitian)Advising the Nutrition Department when a patient with CF is admitted allows appropriate menus, mid-meal extras and salt supplementation to be commenced. Assessment by a dietitian will then allow an individualised plan to be formulated.

Nutritional intervention is an important component of the multidisciplinary care recommended for CF patients. The goals of nutrition management of CF patients are: to meet increased energy needs with the goal of normal growth in children and good

nutritional status in adults to meet salt and vitamin needs to promote clients’ understanding of CF related nutrition issues and support developing

independent management in young people



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to promote positive attitudes to food and body image, and to assist management of complications affecting nutritional requirements or intake.

Pancreatic Enzymes should be ordered as per the patient/family. Referral to the dietitian can be made if education is required regarding the matching of enzymes to dietary fat intake (this is also reviewed in CF clinic nutrition reviews).

Social Worker and PsychologyContact during first business hours of the admission unless urgent issues arise.Social workers and psychologists can help with many of the practical and emotional issues that may be experienced as a result hospitalisation and a chronic illness.

Outpatient Care Patients with CF should be reviewed at 3 monthly intervals. Referral to the Paediatric Outpatient Department (POPD) should be made on discharge as guided by medical staff.

CF NurseContact during first business hours of the admission. A message should be left with the CF Nurse (Extension 47374) for further co-ordination of the patient’s care.

1.6 At Discharge Reconcile discharge with admission medications. Ensure adequate prescription of medications. Ensure follow-up plan established including physiotherapy program and clinic

appointment and communicated to family and patient if age relevant. Ensure appointment for Pulmonary Function Tests made and communicated to family

and/or patient.


Section 2 – Cystic Fibrosis outpatients – process for review when unwell

Alert: If there is any concern that a child or adolescent is acutely unwell, the parents or guardian will be instructed to call an Ambulance 000 and come directly to the Emergency Department.

During business hours (Monday to Friday 0830 – 1700 hours)If a child or adolescent with cystic fibrosis becomes unwell, the parent/guardian or adolescent will have been educated to contact the Paediatric CF nurse or the Paediatric Outpatient Clinic Nurse on 61747374 or phone the switchboard and ask to page the Paediatric CF nurse on Extension 54587.



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The immediate role of the nurse is to make an assessment over the phone according to set criteria if applicable:

Respiratory, for example: cough increased work of breathing decreased exercise tolerance fever change in colour and volume of sputum produced, and/or chest pain.

Gastroenterological symptoms, for example: abdominal Pain steatorrhoea gastrostomy/enteral tube button concerns, and/or change in bowel habits e.g. bowels not opened for a longer period than usual.

Endocrine, for example: unstable blood sugar levels – refer to paediatric Diabetes Educator or Paediatric

Endocrinologist on call.

The clinic nurse should inform the paediatric registrar on duty. The registrar will consult with the patient’s respiratory paediatrician if necessary or if unavailable, the paediatrician on call.

The clinic nurse will liaise with the medical team to arrange a suitable time for the child to present to the Paediatric Outpatient Clinic.

On arrival the clinic nurse will: obtain weight and vital signs (including oxygen saturations, respiratory and pulse rate

and temperature) and record on appropriate Paediatric Early Warning Score (PEWS) chart

obtain sputum sample if possible place child in a safe and appropriate place for observation and isolation if required negotiate with the Paediatric Day Stay Unit as necessary to determine the best area for

the child to be assessed.

After hours (weekends, public holidays, from 1700 - 0830 hours on week days)Parents will have been educated to contact the paediatric registrar on call via the Canberra Hospital switchboard on 6244 2222 stating that their child has Cystic Fibrosis.

The registrar will make an assessment over the phone. If required, the registrar will notify the Emergency Department and arrange to meet the child and family in this department.

It may be determined as safe and appropriate for the child to present to the Outpatient Clinic the next day. If so, the parent should telephone the clinic nurse after 0830 hours on Doc Number Version Issued Review Date Area Responsible PageCHHS16/246 1 16/12/2016 01/06/2018 WY&C 11 of 15


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61747374 who should then arrange a suitable time for the patient to be reviewed in consultation with the paediatric registrar.

Note: Some concerns may be safely and appropriately managed by the General Practitioner (GP) this is to be decided by the paediatric registrar. The paediatric registrar should liaise with the GP and with the patient’s respiratory paediatrician.


Implementation

The development of this document will be communicated to staff via staff in services .

Any education regarding Patients with Cystic Fibrosis will be based on this Clinical Guideline.

Information on how to access and utilise all Clinical Guideline is included in orientation for all new staff to the unit.


Related Policies, Procedures, Guidelines and Legislation

LegislationHuman Rights Act 2004

Policies, Procedures and GuidelinesClinical Procedure – Healthcare Associated InfectionsClinical Procedure – Antimicrobial StewardshipClinical Policy – Medication HandlingClinical Policy – Vital Signs and Early Warning Scores




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References

Australian Medicines Handbook – Children’s Dosing Companion (AMH CDC) (2016), Adelaide, South Australia

Button, B. M., Wilson, C., Dentice, R., Cox, N. S., Middleton, A., Tannenbaum, E.& Moran, F. (2016). Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline. Respirology, 21(4), 656-667

The Children’s Hospital at Westmead, Sydney, Australia, Practice Guideline for Cystic Fibrosis Manual - CHW [Internet, last updated 1st April, 2015, date viewed 27th June, 2016 ], Available from: http://www.schn.health.nsw.gov.au/_policies/pdf/2015-8000.pdf

Cystic Fibrosis Australia, North Ryde, Australia, Standards of Care for Cystic Fibrosis [Internet, last updated 2008], available from http://www.cysticfibrosis.org.au/media/wysiwyg/CF-Australia/PDF_files/CFA_Standards_of_Care_journal_31_Mar_08.pdf

NHMRC, Australia, Guideline – Clinical Guidelines for the Prevention and Control of Infection in Healthcare, [Internet, last updated 2010], available from http://www.nhmrc.gov.au/guidelines-publications/cd33

Sydney Children’s Hospital, Sydney, Australia, Practice Guideline for Cystic Fibrosis: Guidelines for Management – SCH [Internet, last updated 27th September, 2015, date viewed 27th June, 2016 ], Available from: http://www.schn.health.nsw.gov.au/_policies/pdf/2015-7018.pdf

Zobell, J. et al (2013) Optimization of Anti-pseudomonal Antibiotics for Cystic Fibrosis Pulmonary Exacerbations: II Cephalosporins and Penicillins, Pediatric Pulmonology, 48:107-122


Search Terms

Cystic Fibrosis, Child, Infection, Infant, Respiratory, Adolescent, Paediatric, Lung, Pancreas, Enzymes




http://www.schn.health.nsw.gov.au/_policies/pdf/2015-7018.pdf


http://www.nhmrc.gov.au/guidelines-publications/cd33

http://www.cysticfibrosis.org.au/media/wysiwyg/CF-Australia/PDF_files/CFA_Standards_of_Care_journal_31_Mar_08.pdf

http://www.cysticfibrosis.org.au/media/wysiwyg/CF-Australia/PDF_files/CFA_Standards_of_Care_journal_31_Mar_08.pdf


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Disclaimer: This document has been developed by Health Directorate, Canberra Hospital and Health Services specifically for its own use. Use of this document and any reliance on the information contained therein by any third party is at his or her own risk and Health Directorate assumes no responsibility whatsoever.

(to be completed by the HCID Policy Team)Date Amended Section Amended Approved ByEg: 17 August 2014 Section 1 ED/CHHSPC Chair



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cystic fibrosis - management and care of the patient · web viewcystic fibrosis is an inherited...

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