Quality Standards for Diabetes Care Toolkit

Cystic fibrosis

The Standards of Care for Cystic Fibrosis (CF) in New Zealand by the Cystic Fibrosis Association of New Zealand (2011) suggest the following:

The primary place of CF care should be at a hospital with a CF clinic closest to where the person with cystic fibrosis lives.

People with cystic fibrosis should be seen at least four times a year in a dedicated CF clinic.

If attending a smaller local clinic, the makeup of the health professional team may vary; therefore, there needs to be a well-defined relationship with a Regional CF Centre.

A regional multidisciplinary team should review all people with cystic fibrosis at least annually or more frequently as dictated by individual needs:

patients may travel to regional cystic fibrosis centres, or

combined clinics may be held locally.

Core members of the regional centre multidisciplinary team are involved.

Core members of the regional centre multidisciplinary team at a minimum should include:

CF clinician

nurse specialist

specialist physiotherapist

dietitian.

Between visits regional CF centre support can be augmented through the use of the TelePaediatrics service video link system.

The extent of regional clinic contact is determined by an individual’s need rather than geography.

All people with cystic fibrosis have an annual review – usually regional CF centre involvement occurs then.

It is impractical to expect all specialist services can be provided outside regional CF centres:

access to specialist services must be available to all patients depending on need and regardless of location

the requirement of a regional CF centre is extensive and includes onsite availability from sub-specialists experienced in CF complications including endocrinology

people with cystic fibrosis-related diabetes should have access to, and an established working relationship with, an endocrinologist and diabetes service with knowledge and expertise in the management of CFRD.

The UK Cystic Fibrosis Trust recommends the following for people with cystic fibrosis-related diabetes:

‘Ideally the CF Clinician and Diabetologist should carry out a combined general and CFRD Annual Review. In practice, it is usually more convenient for the CFRD Annual Review to be carried out at a different appointment to the general CF Annual Review. The aim of the CFRD Annual Review is to screen for, and if necessary; treat early complications, check that diabetic treatment is adequate and appropriate, to assess nutritional management and to address adherence issues, diabetic education and psychosocial issues’ (The UK Cystic Fibrosis Trust Diabetes Working Group 2004, p 32).

Insulin treatment will improve hyperglycaemia and help to prevent catabolic weight loss in CF particularly during intercurrent infections. High dietary energy intake is recommended including high fat and high complex carbohydrates (International Society for Pediatric and Adolescent Diabetes 2000). The American Diabetes Association recommends as part of their Clinical Care Guideline for Cystic Fibrosis-related Diabetes Management that ‘patients with CFRD should be managed by a multidisciplinary team of health professionals with expertise in CF and diabetes. The diabetes team should be intimately familiar with CFRD, recognising differences between this and type 1 and type 2 diabetes pathophysiology and treatment. Good communication between diabetes and CF care providers is essential. Poor team communication and inadequate or conflicting information from health care providers have been identified as significant sources of stress for patients with CFRD’ (Moran et al 2010, pp 2702–3).