3Primary Prevention Interventions

• Public (Internet)

CONTENTS

1Background

2Cystic Fibrosis

2.1Introduction

2.2Search methodology

2.3Prevalence

2.4Hospital admissions

3Primary prevention interventions

4Secondary prevention interventions

5Further information

6References

© 2010 Public Health Wales NHS Trust.

Material contained in this document may be reproduced without prior permission provided it is done so accurately and is not used in a misleading context.

Acknowledgement to Public Health Wales NHS Trust to be stated.

1Background

This document has been produced to assist local health boards to implement the Welsh Assembly Government’s, Designed for people with chronic conditions, Service development and commissioning directives, Chronic respiratory conditions1, and should be read in conjunction with that publication.

A key action identified in chapter 2: Prevention – reducing the risks (p. 22)of the publication is evidence-based primary and secondary prevention1.

To supplement the evidence–base, and provide an overview of the topic, information with regard to prevalence (where available); hospital admissions (where information is available from Patient Episode Database Wales - PEDW); and links to additional information resources have been included. The links to the additional information resources is included to indicate where further details, or management and treatment guidance can be sought.

The information contained in this document is not exhaustive.

2Cystic Fibrosis

2.1Introduction

Cystic fibrosis (CF) is a inherited disease that affects the internal organs. It is a genetic disorder in which internal bodily secretions become thick and sticky, and hinder the function of organs, particularly the lungs and digestive system2.

The condition is caused by a faulty gene that controls the movement of salt and water in and out of cells in the body. CFoccurs when too much salt and too little water passes into cells and turns the body's secretions, which normally act as a lubricant, into a thick mucus. The mucus blocks tubes, ducts and passagewaysmaking them ineffective and prone to infection2.

Symptoms of CF include recurrent chest infections and poor growth, and can result in related health problems such as diabetes and infertility2.There is no cure for cystic fibrosis.

2.2Search methodology

Search terms used: primary prevention, secondary prevention, cystic fibrosis.

Search terms were kept broad to maximise retrieval of literature and search limits set to retrieve papers published between January 2003 to January 2010.

Electronic databases: Medline; Embase; Cochrane Database of Systematic Reviews; Database of Abstracts of Reviews of Effects; Cochrane Central Register of Controlled Trials and British Nursing Index.

Meta search engines: Turning Research Into Practice (TRIP); Google Scholar; SUMsearch.

Websites: NHS Evidence; International Network of Agencies for Health Technology Assessment (INAHTA); National Institute for Health and Clinical Excellence (NICE); National Horizon Scanning Centre and Map of Medicine; UpToDate.

2.3Prevalence

Cystic fibrosis is an inherited condition that affects over 8,500 children and young adults in the UK. Five babies are born with the condition each week. Median life expectancy has increased in recent decades and is currently in the mid-thirties2.

The faulty gene that causes cystic fibrosis is recessive;two separate genes, one from each parent, areneeded to inherit. It is possible to carry the disease and not suffer from it, 1 in 25 people in the UK carry the condition.A child born to parents who are both carriers of the gene will have a2:

• 25% chance that they will not inherit the genes from either parent,will not have cystic fibrosis or carry the condition;
• 50% chance that they will inherit a copy of the gene from eitherparent, they will not have cystic fibrosis but will be a carrier of the condition;
• 25% chance that they will inherit genes from each parent, and will have cystic fibrosis.

2.4Hospital admissions

Figure 1: Persons admitted to hospital in Walesin 2008 with a principal diagnosis of cystic fibrosis (ICD-10, E84) by Unitary Authority

Figure 2: Persons admitted to hospital in Walesin 2008 with a principal diagnosis of cystic fibrosis (ICD-10, E84) by Local Health Board

Table 1: Persons admitted to hospital in Walesin 2008 with a principal diagnosis of cystic fibrosis (ICD-10, E84)

Source: PEDW

Note:- denotes data not shown due to small counts (less than five admissions)

3Primary prevention interventions

Cystic fibrosis is an inherited disorder and there are no primary prevention measures, however, pre-conception counselling could avoid conceptions of CF foetus and antenatal screening identifies affected foeti (in reality the latter is secondary prevention)3, 4.

4Secondary prevention interventions

Secondary prevention consists of minimising the sequelae of CF5.

There are numerous systematic reviews and primary research on the topic and up to date reference lists may be found on the NHS Evidence,2009 annual evidence update on cystic fibrosis website3.

Effective neonatal screening programmes can reduce the risk of permanent lung damage, malnutrition and repeated chest infections. The additional benefits attributed to screening are avoidance of lengthy delays and frequent admissions that sometimes characterise early history, prior to diagnosis in infants6, 7. Nutritional benefits from screening were apparent in the findings of a Cochrane review8, and screening of lung function has potential, but needs long term follow up.

A number of guidelines exist for the management and treatment of patients with CF9 - 11.

A British Thoracic Society (BTS) statement on criteria for specialist referral, admission, discharge and follow-up for adults with respiratory disease, proposes good practice indicators in the management of respiratory disease for adults, and includes cystic fibrosis12.

5Further information

British Thoracic Society:

• Standards of Care Committee: BTS statement on criteria for specialist referral,admission, discharge and follow-up for adults withrespiratory disease. Thorax 2008; 63.

Cystic Fibrosis Trust:

Map of Medicine

• Cystic fibrosis in children
• Suspected cystic fibrosis
• Cystic fibrosis – management
• Cystic fibrosis – management of advanced disease

6References

1. Welsh Assembly Government. Designed for people with chronicconditions. Service development and commissioning directives. Chronic respiratory conditions. Cardiff: WAG; 2007. Available at: [Accessed 11th Mar 2010]
2. NHSClinical Knowledge summaries [Website]. Cystic fibrosis. Available at: [Accessed 8th Apr 2010]

1. NHS Evidence. Respiratory. 2009 annual evidence update on cystic fibrosis. Available at: [Accessed 8th Apr 2010]

1. Dequeker E et al. Best practice guidelines for molecular genetic diagnosis of cystic fibrosis and CFTR-related disorders – updated European recommendations. Eur J Hum Genet2009; 17:51-65. Available at: [Accessed 8th Apr 2010]

1. Ratjen F. Update in cystic fibrosis 2008. Am J Respir Crit Care Med 2009; 179:445-448. Available at: [Accessed 23rd Aug 2010]

1. Castellani C et al. European best practice guidelines for cystic fibrosis neonatal screening. J Cyst Fibros2009; 8:153-73

1. Mayell SJ et al. A European consensus for evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis. J Cyst Fibros2009; 8:71-8. Available at: [Accessed 8th Apr 2010]

1. Southern KW et al. Newborn screening for cystic fibrosis. Cochrane Database Syst Rev2009, Issue 1. Art. No.: CD001402. DOI: 10.1002/14651858.CD001402.pub2. Available at: [Accessed 8th Apr 2010]

1. Kerem E et al. Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 2005; 4: 7-26. Available at: 8th Apr 2010]

1. Bott J et al. Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient. Thorax 2009; 64(Suppl 1). Available at: [Accessed 8th Apr 2010]

1. Cystic Fibrosis Trust. Antibiotic treatment for cystic fibrosis.Report of the UK Cystic Fibrosis Trust Antibiotic Working Group. 3rd ed. Bromley: Cystic Fibrosis Trust; 2009. Available at: 8th Apr 2010]

1. British Thoracic Society Standards of Care Committee. BTS statement on criteria for specialist referral,admission, discharge and follow-up for adults withrespiratory disease. Thorax 2008;63(Suppl I):i1–i16. doi:10.1136/thx.2007.087627. Available at: [Accessed 19th Apr 2010]