Cystic fibrosis: management of non-respiratory manifestations

Abstract

This document has been developed to update the previous guidelines on the Nutritional Management of Cystic Fibrosis (Cystic Fibrosis Trust, 2002). This is a comprehensive support document for dietitians working with infants, children and adults with cystic fibrosis (CF).The Nutrition Consensus Working Group recognises that practice may differ between Specialist CF Centres and between paediatric and adult care; this document represents what the current Nutrition Consensus Working Group and those who have contributed to the document consider to be best clinical practice.The endorsement process of the document by the Cystic Fibrosis Trust has included reviews by relevant experts in addition to peer reviews. This document has been developed independently of any funding body. Table 1: Recommended dietetic staffing levels 2475 patients 150 patients 250 patients Adult centre (WTE) 0.5 1.0 2.0 Paediatric centre (WTE) 0.5 1.0 1.5Whilst these are helpful recommendations, exposure to a critical mass of patients is essential to ensure staff are experienced enough to understand the complexity of management. For example a Specialist Centre may have 0.5 WTE for 75 patients but if that 0.5 WTE comprises a number of individuals contributing 0.1 or 0.2 WTE they will be unable to develop the appropriate expertise. Incremental changes in staffing numbers do not necessarily occur with patient numbers greater than 250. 24 The CF Trust Standards of Care 24 also make the following recommendations:A Specialist CF Dietitian must:Be registered with Health and Care Professions Council.Be a member of the Cystic Fibrosis Dietitians' Group.Have specialist knowledge and be experienced in the care of children and/or adults with CF.Maintain CPD through attendance at study days and meetings such as national and international CF conferences. Roles and responsibilities of Specialist CF Dietitians include:Providing regular dietetic support and advice to people with CF and their parents/carers in an outpatient and inpatient setting.Ensuring that dietetic practice is evidence-based and reflects current research, clinical guidelines and consensus views.Providing appropriate advice and education to people with CF and their carers on the nutritional management of CF. This should be individualised advice taking into account age, clinical and nutritional status.Acting as a resource on the nutritional training, development, education and support of other allied health and medical professionals working in CF.Taking part in dietetic and multi-professional CF research and audits.Evaluating clinical practice identifying improvements where necessary.The CF Trust Standards of Care 24 also suggests that a Specialist CF Dietitian should:See all people with CF for a nutritional annual review (PS and PI).See all inpatients at least twice per week or more frequently if appropriate.Be available at every clinic visit and ensure all PI patients are seen at every clinic visit and those who are PS are seen as necessary.Current Service Specification and the Commissioning for...