European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre

Abstract A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the multidisciplinary team (MDT), which should include consultants, clinical nurse specialist, microbiologist, physiotherapist, dietitian, pharmacist, clinical psychologist, social worker, clinical geneticist and allied healthcare professionals, all of whom should be experienced in CF care. Members of the MDT are also expected to keep up to date with developments in CF through continued professional development, attendance at conferences, auditing and involvement in research. Specialists CF Centres should also network with other Centres both nationally and internationally, and feed Centre data to registries in order to further the understanding of the disease. This paper provides a framework for the specialist CF Centre, including the organisation of the Centre and the individual roles of MDT members, as well as highlighting the value of CF organisations and disease registries.

[1]  M. Stanton,et al.  Cystic fibrosis mortality and survival in the UK: 1947–2003 , 2007, European Respiratory Journal.

[2]  L. Dupont,et al.  End of life care for patients with cystic fibrosis. , 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[3]  Physiotherapy for people with Cystic Fibrosis: from infant to adult , 2009 .

[4]  D. Peckham,et al.  376* The role of electronic patient records (EPR) in improving service efficiency and clinical performance in a regional adult UK centre , 2011 .

[5]  G. Cutting,et al.  Environmental allergies and respiratory morbidities in cystic fibrosis , 2013, Pediatric pulmonology.

[6]  Nasr Sz.  Cystic fibrosis in adolescents and young adults. , 2000 .

[7]  S. Kelsey,et al.  The prognostic value of exercise testing in patients with cystic fibrosis. , 1992, The New England journal of medicine.

[8]  J. Bradley,et al.  Physical training for CF. , 2002 .

[9]  L. Saiman,et al.  Infection control recommendations for patients with cystic fibrosis: Microbiology, important pathogens, and infection control practices to prevent patient-to-patient transmission. , 2003, American journal of infection control.

[10]  R. Garrod,et al.  Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient , 2009, Thorax.

[11]  P. Flume.  Pneumothorax in cystic fibrosis , 2011, Chest.

[12]  Charles A. Johnson,et al.  Factors influencing outcomes in cystic fibrosis: a center-based analysis. , 2003, Chest.

[13]  F. Moran,et al.  INCONTINENCE IN ADULT FEMALES WITH CYSTIC FIBROSIS: A NORTHERN IRELAND SURVEY , 2003, International journal of clinical practice.

[14]  N. Blow.  Glycobiology: A spoonful of sugar , 2009, Nature.

[15]  M. Bryon,et al.  Transition from Paediatric to Adult Care: Psychological Principles , 2001, Journal of the Royal Society of Medicine.

[16]  Naomi A Mckoy,et al.  Active cycle of breathing technique for cystic fibrosis. , 2012, The Cochrane database of systematic reviews.

[17]  R. Tattersall,et al.  Posture and cystic fibrosis. , 2003, Journal of the Royal Society of Medicine.

[18]  H. Shwachman,et al.  Massive hemoptysis in cystic fibrosis. , 1970, The Journal of pediatrics.

[19]  B. Button,et al.  Physiotherapy for cystic fibrosis in Australia: Knowledge and acceptance of the Consensus Statement recommendations , 2013, Respirology.

[20]  Chantal Darquenne,et al.  Aerosol deposition in health and disease. , 2012, Journal of aerosol medicine and pulmonary drug delivery.

[21]  P. Flume,et al.  Transition programs in cystic fibrosis centers: Perceptions of team members , 2004, Pediatric pulmonology.

[22]  L. Warnock,et al.  Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis. , 2015, The Cochrane database of systematic reviews.

[23]  S. Patton,et al.  Survey of professionals' expectations of developmental task achievement of cystic fibrosis self‐care in children , 2005, Pediatric pulmonology.

[24]  C. Schans,et al.  Conventional chest physiotherapy compared to other forms of chest physiotherapy for cystic fibrosis , 2003 .

[25]  M. Macek,et al.  Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries. , 2010, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[26]  G. Trimarchi,et al.  Stunting is an independent predictor of mortality in patients with cystic fibrosis. , 2013, Clinical nutrition.

[27]  M. Desai,et al.  Compliance of Clinical Microbiology Laboratories in the United States with Current Recommendations for Processing Respiratory Tract Specimens from Patients with Cystic Fibrosis , 2006, Journal of Clinical Microbiology.

[28]  W. Robinson.  Palliative and end-of-life care in cystic fibrosis: what we know and what we need to know , 2009, Current opinion in pulmonary medicine.

[29]  S. Madge.  Growing up and growing older with cystic fibrosis. , 2006, Journal of the Royal Society of Medicine.

[30]  D. Lomas,et al.  Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional study , 1998 .

[31]  G. Sawicki,et al.  Advance care planning in adults with cystic fibrosis. , 2008, Journal of palliative medicine.

[32]  A. Bush,et al.  Cystic fibrosis in the 21st century , 2005 .

[33]  A. Webb,et al.  How a clinical psychologist manages the problems of adults with cystic fibrosis. , 2005, Journal of the Royal Society of Medicine.

[34]  K. Khair,et al.  Multidisciplinary teams in the United Kingdom: problems and solutions. , 2000, Journal of pediatric nursing.

[35]  J. J. Díaz Martín,et al.  Treatment compliance in children and adults with cystic fibrosis. , 2008, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[36]  S. Madge,et al.  Technological and behavioral strategies to reduce treatment burden and improve adherence to inhaled antibiotics in cystic fibrosis. , 2011, Respiratory medicine.

[37]  M. Samuels,et al.  BTS guidelines for home oxygen in children , 2009, Thorax.

[38]  A. Quittner,et al.  Psychological interventions for people with cystic fibrosis and their families. , 2008, The Cochrane database of systematic reviews.

[39]  L. Stark,et al.  Developmental and psychosocial issues in cystic fibrosis. , 2011, Pediatric clinics of North America.

[40]  L. Morrison,et al.  Oscillating devices for airway clearance in people with cystic fibrosis. , 2014, The Cochrane database of systematic reviews.

[41]  R. Frontini,et al.  EAHP survey 2010 on hospital pharmacy in Europe: parts 4 and 5. Clinical services and patient safety , 2013 .

[42]  G. Sawicki,et al.  Cystic Fibrosis and Transition to Adult Medical Care , 2010, Pediatrics.

[43]  J. Bradley,et al.  Physical training for cystic fibrosis. , 2002, The Cochrane database of systematic reviews.

[44]  B. Stack,et al.  Cystic Fibrosis in Adolescents and Young Adults , 1979, Scottish medical journal.

[45]  T. Hart.  The Burkholderia cepacia Complex: Suggestions for Prevention and Infection Control , 2004 .

[46]  E. Kerem,et al.  Standards of care for patients with cystic fibrosis: a European consensus. , 2005, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[47]  P. Sterk,et al.  Oxygen‐assisted exercise training in adult cystic fibrosis patients with pulmonary limitation to exercise , 1991, International journal of rehabilitation research. Internationale Zeitschrift fur Rehabilitationsforschung. Revue internationale de recherches de readaptation.

[48]  S. Carr,et al.  A comparison of the prevalence of urinary incontinence in girls with cystic fibrosis, asthma, and healthy controls , 2006, Pediatric pulmonology.

[49]  C. van der Schans,et al.  Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis. , 2000, The Cochrane database of systematic reviews.

[50]  K. Dwan,et al.  Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. , 2015, The Cochrane database of systematic reviews.

[51]  R. Loddenkemper,et al.  European curriculum recommendations for training in adult respiratory medicine: crossing boundaries with HERMES , 2008, European Respiratory Journal.

[52]  Carla Colombo,et al.  The implementation of standards of care in Europe: state of the art. , 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[53]  K. Lowton.  'A bed in the middle of nowhere': parents' meanings of place of death for adults with cystic fibrosis. , 2009, Social science & medicine.

[54]  L. Denehy,et al.  Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis , 2003, Thorax.

[55]  E. Main.  Airway clearance research in CF: the ‘perfect storm’ of strong preference and effortful participation in long-term, non-blinded studies , 2013, Thorax.

[56]  S. Madge.  Challenges for Nurses , 2006 .

[57]  K. Carson,et al.  Exercise improves lung function and habitual activity in children with cystic fibrosis. , 2012, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[58]  C. Schans,et al.  Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. , 2005, The Cochrane database of systematic reviews.

[59]  D. Rosen.  Transition of young people with respiratory diseases to adult health care. , 2004, Paediatric respiratory reviews.

[60]  M. Bryon,et al.  A model for transition from pediatric to adult care in cystic fibrosis. , 2002, Journal of pediatric nursing.

[61]  N. Maffulli,et al.  Musculoskeletal involvement in cystic fibrosis. , 1999, Bulletin (Hospital for Joint Diseases (New York, N.Y.)).

[62]  H. Kanazawa,et al.  Phenotypic differences between asymptomatic airway hyperresponsiveness and remission of asthma. , 2011, Respiratory medicine.

[63]  K. Hardy,et al.  Transition programs in cystic fibrosis centers: Perceptions of patients , 2002, Pediatric pulmonology.

[64]  W. Mackenzie,et al.  Guidelines for the management of pregnancy in women with cystic fibrosis. , 2008, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[65]  B. Button,et al.  Physiotherapy in infants and young children with cystic fibrosis: current practice and future developments. , 2004, Journal of the Royal Society of Medicine.

[66]  Report of the UK Cystic Fibrosis Trust Microbiology Laboratory Standards Working Group , 2010 .

[67]  Naomi A Mckoy,et al.  Active cycle of breathing technique for cystic fibrosis. , 2016, The Cochrane database of systematic reviews.

[68]  G. Latchford,et al.  Motivational interviewing for adherence problems in cystic fibrosis , 2010, Pediatric pulmonology.

[69]  M. Götz,et al.  Parents' recollection of the initial communication of the diagnosis of cystic fibrosis. , 1996, Pediatrics.

[70]  H. Quinton,et al.  Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. , 2013, The Journal of pediatrics.

[71]  K. Slifer,et al.  Using behavioral interventions to assist with routine procedures in children with cystic fibrosis. , 2010, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[72]  B. Button,et al.  Structure and function of the mucus clearance system of the lung. , 2013, Cold Spring Harbor perspectives in medicine.

[73]  J. Regnis,et al.  Benefits of continuous positive airway pressure during exercise in cystic fibrosis and relationship to disease severity. , 1993, The American review of respiratory disease.

[74]  J. Bradley,et al.  Non-invasive ventilation for cystic fibrosis. , 2003, The Cochrane database of systematic reviews.

[75]  S. Bell,et al.  Transition of adolescents with cystic fibrosis from paediatric to adult care , 2011, The clinical respiratory journal.

[76]  S. Roberts,et al.  The sexual health of adolescents with cystic fibrosis. , 2005, Journal of the Royal Society of Medicine.

[77]  J. Carlin,et al.  Association of body composition and lung function in children with cystic fibrosis , 2005, Pediatric pulmonology.

[78]  M. Stern,et al.  From registry to quality management: the German Cystic Fibrosis Quality Assessment project 1995–2006 , 2008, European Respiratory Journal.

[79]  Martin Stotz,et al.  Lactate in cystic fibrosis sputum. , 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.