Child and Youth Cystic Fibrosis Clinical Network

Purpose of the Network

The purpose of this Clinical Network (CN) is to support all multidisciplinary teams who work across primary, secondary and tertiary services, to deliver best practice care in the treatment of cystic fibrosis. The network will work closely with Cystic Fibrosis NZ and PSNZ Respiratory Special Interest Group

The Clinical Reference Group (CRG) members are:
Dr Cass Byrnes, Respiratory Consultant, Starship (co-Chair)
Dr Peter McIlroy, Paediatrician, Nelson  (co-Chair)
Dr Julian Vyas, Respiratory Consultant, Starship
Jan Tate, Cystic Fibrosis Clinical Nurse Specialist (CNS), Starship
Vivienne Isles, Paediatric CNS, Christchurch
Jane Bollard, CEO, Cystic Fibrosis NZ (CFNZ)
Sue Lovelock, Social Worker, CFNZ
Fiona Leighton, Dietitian, Christchurch
Rebecca Scoones, Physiotherapist, Starship
Julie Graves, Dietitian, Tauranga
Rebecca Landreth, Pharmacist, Dunedin 

The CRG is supported by:

Karyn Sanson, RN, MHPrac Facilitator/Operational Project Manager NZCYCN
Mollie Wilson, CEO PSNZ

The CRG has approved the Terms of Reference which can be viewed here. 

The first set of goals will be a phased review of the current clinical guidelines in order to produce current best practice guidelines which are nationally applicable regardless of the centre providing care. These guidelines will be informed by the Standards of Care document produced in conjunction with Cystic Fibrosis NZ and will also support equity of access to specialty and multidisciplinary care for all children and young people with Cystic Fibrosis in New Zealand.

As many clinicians are aware the document is a large one and will be broken down into topics and settings in order to progress it. The first will be infection control in the inpatient and outpatient settings. In addition a parallel workstream will be working on the requirements for patient review at diagnosis, and annually.

If you have ideas or would like to be involved on any of the related workstreams please don't hesitate to make contact with us.

Cystic Fibrosis New Zealand Referral Form

Cystic Fibrosis New Zealand (CFNZ) is a not-for-profit organisation which provides support and advocacy for families living with cystic fibrosis. Please use this link to the referral form to ensure families have full access to the facilities and support provided by CFNZ.

Cystic Fibrosis information for newly diagnosed infants

• A handout for families on how to give pancreatic enzymes can be viewed or dowloaded here

• A handout for families on how to give salt to their child can be viewed or downloaded here

Additional information about newly diagnosed Cystic Fibrosis can be found on the Cystic Fibrosis NZ website:

• https://www.cfnz.org.nz/get-support/resources/guides/

• https://www.cfnz.org.nz/life-with-cf/new-diagnosis/

Cystic Fibrosis Clinical Guidance

All guidelines on Cystic Fibrosis have been made available on the Starship Website. Please see https://starship-org.nz/health-professionals/ to search for the guidelines you want, which include:

• Annual review for children and young people with Cystic Fibrosis

• Antibiotic allergy in Cystic Fibrosis

• Antibiotic treatment for respiratory exacerbations in Cystic Fibrosis

• Aspergillus lung disease in Cystic Fibrosis

• Dermatological involvement with Cystic Fibrosis

• End of life care in Cystic Fibrosis

• Investigation of respiratory physiology in Cystic Fibrosis

• Lung Transplant Assessment for a child with Cystic Fibrosis

• Non infective respiratory complications in Cystic Fibrosis

• Nutrition care and management of Cystic Fibrosis

• Other complications of Cystic Fibrosis

• Psychological screening of young people with Cystic Fibrosis

• Regular three monthly clinic review for children and young people with Cystic Fibrosis

• Sleep and sleep disordered breathing in Cystic Fibrosis

Infection control practices for children and young people with Cystic Fibrosis.

The infection Control recommendations can be found on the Starship website. These recommendations were based on the following:

Background to the recommendations

It is well recognised that transmission of pathogens can occur between individuals with Cystic Fibrosis (CF) and that these pathogens can be associated with increased morbidity and mortality. As a result, infection control policies have been developed by hospitals throughout New Zealand (NZ) and around the world to prevent the transmission of pathogens between individuals with CF. However, policies vary from hospital to hospital and these differing policies can generate controversy and anxiety among members of the CF community, including persons with CF, their families and their medical team.

In 2014 the Cystic Fibrosis Foundation (USA) published updated guidelines on Infection Prevention and Control Guideline for people with CF, their families and healthcare professionals. The updated guidelines were developed by an interdisciplinary committee who reviewed existing literature, graded the evidence and reached a consensus based on the level of evidence and expert practice of team members. The release of these guidelines provided the incentive for clinics in NZ to again look at their Infection Control Guidelines for care of CF patients and to see whether the recommendations from the above guidelines were appropriate for the NZ setting and should be adopted.

It was therefore timely that The Child & Youth Cystic Fibrosis Clinical Network was established late in 2014 with the aim of developing nationally coordinated care for children and young people with CF. The decision was made by this group to look at infection control in the hospital setting as the first priority and to produce recommendations which were nationally applicable regardless of the centre providing care.

Purpose of the recommendations

The focus of these recommendations is to provide guidance on best practices to prevent children and young people with Cystic Fibrosis (CF) from transmitting and/or acquiring respiratory tract pathogens from others with CF while in the hospital setting.

These recommendations are intended for use by all healthcare personnel involved with the care of children and young people with CF in New Zealand. They apply to inpatient settings, outpatient clinics and other ambulatory care areas including diagnostic and therapeutic areas.

The goal of these recommendations is to reduce the risk of transmission and acquisition of CF pathogens between individuals, while recognising that the risk is unlikely ever to be minimised to zero.

Methodology of the recommendations

A group of health professions across a number of disciplines involved in the care of children and young people with Cystic Fibrosis (CF) in New Zealand were approached to participate in the development of these recommendations. It was accepted by the group the Cystic Fibrosis Foundation Guideline Infection Prevention and Control Guideline for Cystic Fibrosis: 2013 Update had sufficient scientific vigour and expert opinion from those involved in all fields of CF care that it was unlikely that any new information would be obtained by further systemic review and review of the evidence. Guidelines from the UK and Australia were also reviewed.

It was however, recognised that NZ has its own unique environment with a much smaller population of people with CF leading to smaller outpatient clinic sizes and inpatient numbers. In addition the NZ model of care for CF means that most children and young people are cared for by an age-specific team at their local hospital. Though this is not the recommended international agreed model of care where children and young people have some form of oversight from a specialised centre, NZ is small enough that there is well established links between the smaller and larger centres which allows communication and cooperation between the CF care teams. As a result each recommendation from the CF Foundation was examined for fit with the NZ environment. Final recommendations were reached by simple consensus of the group.