European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre
Jazyk angličtina Země Nizozemsko Médium print
Typ dokumentu časopisecké články, přehledy
PubMed
24856776
PubMed Central
PMC7105239
DOI
10.1016/j.jcf.2014.03.009
PII: S1569-1993(14)00084-8
Knihovny.cz E-zdroje
- Klíčová slova
- CF Centre, Continuing professional development, Multidisciplinary team,
- MeSH
- cystická fibróza komplikace diagnóza terapie MeSH
- dítě MeSH
- dospělí MeSH
- dostupnost zdravotnických služeb organizace a řízení MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- nemocnice speciální organizace a řízení MeSH
- předškolní dítě MeSH
- společnosti lékařské MeSH
- standardní péče * MeSH
- týmová péče o pacienty organizace a řízení MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- předškolní dítě MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- Geografické názvy
- Evropa MeSH
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.
Adult Cystic Fibrosis Unit St James's Hospital Leeds UK
Copenhagen CF Centre Rigshospitalet University Hospital Copenhagen Denmark
Cystic Fibrosis Centre University Hospital Leuven Belgium
Cystic Fibrosis Europe Belgium
Department of Microbiology Papworth Hospital NHS Foundation Trust Papworth Everard Cambridge UK
Department of Pediatrics Institute of Mother and Child Warsaw Poland
Department of Respiratory Medicine Royal Brompton Hospital Sydney Street London UK
Dutch Cystic Fibrosis Foundation The Netherlands
Gartnavel General Hospital West of Scotland Adult CF Unit Glasgow UK
Gothenburg CF Centre Queen Silvia Children's Hospital Göteborg Sweden
HagaZiekenhuis Department of Pulmonology and Cystic Fibrosis The Hague The Netherlands
Paediatric and Adult CF Units Leeds Teaching Hospitals Trust UK
Zobrazit více v PubMed
Dodge J.A., Lewis P.A., Stanton M., Wilsher J. CF mortality and survival in the UK: 1947–2003. Eur Respir J. 2007;29:522–526. PubMed
Mahadeva R., Webb K., Westerbeek R.C., Carroll N.R., Dodd M.E., Bilton D. Clinical outcome in relation to care in Centres specialising in cystic fibrosis: cross sectional study. BMJ. 1998;316:1771–1775. PubMed PMC
Johnson C., Butler S.M., Konstan M.W., Morgan W., Wohl M.E. Factors influencing outcomes in cystic fibrosis. A center-based analysis. Chest. 2003;123:20–27. PubMed
Kerem E., Conway S., Elborn S., Heijerman H., for the Consensus Committee Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros. 2005;4:7–26. PubMed
Bell S.C., Robinson P.J. Cystic fibrosis standards of care Australia. In: Fitzgerald D.S., editor. Cystic Fibrosis Australia; Sydney, NSW: 2008.
2nd ed. Cystic Fibrosis Trust; London: 2011. Standards for the clinical care of children and adults with cystic fibrosis in the UK. [ https://www.cysticfibrosis.org.uk/media/82070/CD_Standards_of_Care_Dec_11.pdf]
Colombo C., Littlewood J. The implementation of standards of care in Europe: state of the art. J Cyst Fibros. 2011;10(Suppl. 2):S7–S15. PubMed
Loddenkemper R., Haslam P.L., Séverin T., Annesi-Maesano I., Chuchalin A., Coles C. European curriculum recommendations for training in adult respiratory medicine: report of the HERMES Task Force. European Respiratory Society. Breathe. 2008;5(1):80–93.
Madge S., Khair K. Multi-disciplinary teams in the United Kingdom: problems and solutions. J Pediatr Nurs. 2000;15(2):131–134. PubMed
Madge S.L. National consensus standards for nursing children and young people with cystic fibrosis. Paediatr Care. 2002;14(1):32–35.
Geller D.E., Madge S.L. Technological and behavioural strategies to reduce treatment burden and improve adherence to inhaled antibiotics in cystic fibrosis. Respir Med. 2011;105(Suppl. 2):S24–S31. PubMed
Madge S. Challenges for nurses. In: Bush A., Alton E.W.F.W., Davies J.C., Griesenbach U., Jaffe A., editors. Cystic fibrosis in the 21st century. vol. 34. Karger; Basel: 2006. pp. 286–292. (Progress in respiratory research).
Madge S. Growing up and growing older with cystic fibrosis. J R Soc Med. 2006;99(Suppl. 46):23–26. PubMed
Bolyard D.R. Sexuality and cystic fibrosis. MCN Am J Matern Child Nurs. 2001;26:39–41. PubMed
Roberts S., Green P. Sexual health of adolescents with cystic fibrosis. J R Soc Med. 2005;98(Suppl. 45):7–16. PubMed PMC
Arias Llorente R.P., Bousono Garcia C., Diaz Martin J.J. Treatment compliance in children and adults with cystic fibrosis. J Cyst Fibros. 2008;7:359–367. PubMed
Nasr S.Z. Cystic fibrosis in adolescents and young adults. Adolesc Med. 2000;11:589–603. PubMed
Madge S., Bryon M. A model for transition of care in cystic fibrosis. J Pediatr Nurs. 2002;17:283–288. PubMed
Flume P.A., Taylor L.A., Anderson D.L., Gray S., Turner D. Transition programs in cystic fibrosis centers: perceptions of team members. Pediatr Pulmonol. 2004;37:4–7. PubMed
Bryon M., Madge S. Transition from paediatric to adult care: psychological principles. J R Soc Med. 2001;94(Suppl. 40):5–7. PubMed PMC
Flume P.A., Yankaskas J.R., Ebeling M., Husley T., Clark L.L. Massive hemoptysis in cystic fibrosis. Chest. 2005;128:729–738. PubMed
Flume P.A., Strange C., Ye X., Ebeling M., Husley T., Clark L.L. Pneumothorax in cystic fibrosis. Chest. 2005;28:720–728. PubMed
Mackie A.D., Thornton S.J., Edenborough F.P. Cystic fibrosis-related diabetes. Diabet Med. 2003;20:425–436. PubMed
Lowton K. ‘A bed in the middle of nowhere’: parents' meanings of place of death for adults with cystic fibrosis. Soc Sci Med. 2009;69:1056–1062. PubMed
Sands D., Repetto T., Dupont L.J., Korzeniewska-Eksterowicz A., Catastini P., Madge S. End of life care for patients with cystic fibrosis. J Cyst Fibros. 2011;10:S37–S44. PubMed
International Physiotherapy Group for Cystic Fibrosis (IPGCF) 4th ed. 2009. Physiotherapy for people with cystic fibrosis: from infant to adult. [ http://www.ecfs.eu/ipg_cf/booklet]
Darquanne C. Aerosol deposition in health and disease. J Aerosol Med Pulm Drug Deliv. 2012;25(3):140–147. PubMed PMC
Button B.M., Button B. Structure and function of the mucus clearance system of the lung. Cold Spring Harb Perspect Med. 2013;3(8) [pii: a009720] PubMed PMC
van der Schans C.P., Prasad A., Main E. Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis. Cochrane Database Syst Rev. 2000;2 doi: 10.1002/14651858.CD001401. [CD001401] PubMed DOI
Lannefors L., Button B.M., McIlwaine M. Physiotherapy in infants and young children with cystic fibrosis: current practice and further developments. J R Soc Med. 2004;97(S44):8–25. PubMed PMC
Main E., Prasad A., van der Schans C.P. Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. Cochrane Database Syst Rev. 2005;1 doi: 10.1002/14651858.CD002011.pub2. [CD002011] PubMed DOI PMC
Elkins M., Jones A., van der Schans C.P. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database Syst Rev. 2006;2 doi: 10.1002/14651858.CD003147.pub3. [CD003147] PubMed DOI
Bott J., Blumenthal S., Buxton M., Ellum S., Falconer C., Garrod R. Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient. Thorax. 2009;64(Suppl. 1):i1–i51. PubMed
Morrison L., Agnew J. Oscillating devices for airway clearance in people with cystic fibrosis. Cochrane Database Syst Rev. 2009;1 doi: 10.1002/14651858.CD006842.pub2. [CD006842] PubMed DOI
Holland A.E., Button B.M., on behalf of the International Physiotherapy Group for Cystic Fibrosis Physiotherapy for cystic fibrosis in Australia: knowledge and acceptance of the consensus statement recommendations. Respirology. 2013;18:652–656. PubMed
McKoy N.A., Saldanha I.J., Odelola O.A., Robinson K.A. Active cycle of breathing technique for cystic fibrosis. Cochrane Database Syst Rev. 2012;12 doi: 10.1002/14651858.CD007862.pub3. [CD007862] PubMed DOI
Main E. Airway clearance research in CF: the ‘perfect storm’ of strong preference and effortful participation in long-tem, non-blinded studies. Thorax. 2013;68:701–702. PubMed
Parasa R.B., Maffulli N. Musculoskeletal involvement in cystic fibrosis. Bull Hosp Jt Dis. 1999;58:37–44. PubMed
Tattersall R., Walshaw M.J. Posture and cystic fibrosis. J R Soc Med. 2003;96(S43):18–22. PubMed PMC
Nixon P.A., Orenstein D.M., Kelsey S.F., Doershuk C.F. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med. 1992;327:1785–1788. PubMed
Bradley J.M., Moran F. Physical training for cystic fibrosis. Cochrane Database Syst Rev. 2008;1 doi: 10.1002/14651858.CD002768.pub2. [CD002768] PubMed DOI
Paranjape S.M., Barnes L.A., Carson K.A., v Berg K., Loosen H., Mogayzel P.J., Jr. Exercise improves lung function and habitual activity in children with cystic fibrosis. J Cyst Fibros. 2012;11:18–23. PubMed
Heijerman H.G., Bakker W., Sterk P.J., Dijkman J.H. Oxygen-assisted exercise training in adult cystic fibrosis patients with pulmonary limitation to exercise. Int J Rehabil Res. 1991;14:101–115. PubMed
Henke K.G., Regnis J.A., Bye P.T. Benefits of continuous positive airway pressure during exercise in cystic fibrosis and relationship to disease severity. Am Rev Respir Dis. 1993;148:1272–1276. PubMed
Holland A.E., Denehy L., Ntoumenopoulos G., Naughton M.T., Wilson J.W. Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax. 2003;58:880–884. PubMed PMC
Moran F., Bradley J.M., Piper A.J. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2013;4 doi: 10.1002/14651858.CD002769.pub4. [CD002769] PubMed DOI
Moran F., Bradley J.M., Boyle L., Elborn J.S. Incontinence in adult females with cystic fibrosis: a Northern Ireland survey. Int J Clin Pract. 2003;57:182–183. PubMed
Prasad S.A., Balfour-Lynn I.M., Carr S.B., Madge S.L. A comparison of the prevalence of urinary incontinence in girls with cystic fibrosis, asthma, and healthy controls. Pediatr Pulmonol. 2006;41:1065–1068. PubMed
Edenborough F.P., Borgo G., Knoop C., Lannefors L., Mackenzie W.E., Madge S. Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros. 2008;7:S2–S32. PubMed
European Cystic Fibrosis Society International Physiotherapy Group for Cystic Fibrosis. https://www.ecfs.eu/ipg_cf
Morrison L., McIntosh L., Freeman A., on behalf of the Association of Chartered Physiotherapists in Cystic Fibrosis . 2010–2011. ACPCF National Audit of Clinical Standards of Care in CF.
Pedreira C.C., Robert R.G., Dalton V., Oliver M.R., Carlin J.B., Robinson P. Association of body composition and lung function in children with cystic fibrosis. Pediatr Pulmonol. 2005;39:276–280. PubMed
Yen E.H., Quinton H., Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013;162:530–535. PubMed
Stern M., Wiedemann B., Wenzlaff P., on behalf of the German Cystic Fibrosis Quality Assessment Group From registry to quality management: the German Cystic Fibrosis Quality Assessment project 1995–2006. Eur Respir J. 2008;31:29–35. PubMed
Vieni G., Faraci S., Cillura M., Lombardo M., Traverso G., Cristadoro S. Stunting is an independent predictor of mortality in patients with cystic fibrosis. Clin Nutr. 2013;32:382–385. PubMed
Zhou J., Garber E., Desai M., Saiman L. Compliance of clinical microbiology laboratories in the United States with current recommendations for processing respiratory tract specimens from patients with cystic fibrosis. J Clin Microbiol. 2006;44:1547–1549. PubMed PMC
Report of the UK Cystic Fibrosis Trust Microbiology Laboratory Standards Working Group. 1st ed. September 2010. Laboratory standards for processing microbiological samples from people with cystic fibrosis, September 2010. [ https://www.cysticfibrosis.org.uk/media/82034/CD_Laboratory_Standards_Sep_10.pdf]
MiQ 24. Qualitätssicherungskommission der Deutschen Gesellschaft für Hygiene und Mikrobiologie (DGHM); 2006. Atemwegsinfecktionen bei Mukoviszidose. (Mikrobiologisch-infektiologische Qualitätsstandards).
Cystic Fibrosis Trust . Suggestions for prevention and infection control. 2nd ed. November 2004. Pseudomonas aeruginosa infection in people with cystic fibrosis. (Report of the UK Cystic Fibrosis Trust Infection Control Group).
Cystic Fibrosis Trust . Report of the UK Cystic Fibrosis Trust Infection Control Group. April 2008. Methicillin-resistant Staphylococcus aureus (MRSA)
Cystic Fibrosis Trust . Report of the UK Cystic Fibrosis Trust Antibiotic Working Group. 3rd ed. May 2009. Antibiotic treatment for cystic fibrosis.
Saiman L., Siegel J., the Cystic Fibrosis Foundation Consensus Conference on Infection Control Participants Infection control recommendations for patients with cystic fibrosis: microbiology, important pathogens and infection control practices to prevent patient-to-patient transmission. Infect Control Hosp Epidemiol. 2003;24:S6–S52. PubMed
Cystic Fibrosis Trust . Report of the UK Cystic Fibrosis Trust Infection Control Group. 2nd ed. September 2004. The Burkholderia cepacia complex. Suggestions for prevention and infection control.
Redfern J., Webb K. Benefits of a dedicated cystic fibrosis pharmacist. J R Soc Med. 2004;97(Suppl. 44):2–7. PubMed PMC
Frontini R., Miharija-Gala T., Sykora J. EAHP survey 2010 on hospital pharmacy in Europe: parts 4 and 5. Clinical services and patient safety. Eur J Hosp Pharm. 2013;20:69–73.
Medicines management in NHS hospitals. The Audit Commission; London: 2001. A spoonful of sugar. [ http://www.audit-commission.gov.uk/SiteCollectionDocuments/AuditCommissionReports/NationalStudies/nrspoonfulsugar.pdf]
European Society of Clinical Pharmacy Clinical pharmacy overall goal. http://www.escpweb.org/cms/Clinical_pharmacy
UKCF Trust Pharmacy standards of care. 2011. https://www.cysticfibrosis.org.uk/search.aspx?keywords=pharmacy%20standards
Ernst M.M., Johnson M.C., Stark L.J. Developmental and psychosocial issues in cystic fibrosis. Child Adolesc Psychiatr Clin N Am. 2010;19:263–283. PubMed PMC
Oxley H., Webb A.K. How clinical psychologist manages the problems of adults with cystic fibrosis. J R Soc Med. 2005;98(Suppl. 45):37–46. PubMed PMC
Jedlicka-Köhler I., Götz M., Eichler I. Parents' recollection of the initial communication of the diagnosis of cystic fibrosis. Pediatrics. 1996;97:204–209. PubMed
Anderson D.L., Flume P.A., Hardy K.K., Gray S. Transition programs in cystic fibrosis centers: perceptions of patients. Pediatr Pulmonol. 2002;33:327–331. PubMed
Tuchman L.K., Schwartz L.A., Sawicki G.S., Britto M.T. Cystic fibrosis and transition to adult medical care. Pediatrics. 2010;125:566–573. PubMed
Patton S.R., Graham J.L., Holsclaw D., Jr., Varlotta L. Survey of professionals' expectations of developmental task achievement of cystic fibrosis self-care in children. Pediatr Pulmonol. 2005;40:135–140. PubMed
Towns S.J., Bell S.C. Transition of adolescents with cystic fibrosis from paediatric to adult care. Clin Respir J. 2011;5:64–75. PubMed
Rosen D.S. Transition of young people with respiratory diseases to adult health care. Paediatr Respir Rev. 2004;5:124–131. PubMed
Robinson W.M. Palliative and end-of-life care in cystic fibrosis: what we know and what we need to know. Curr Opin Pulm Med. 2009;15:621–625. PubMed
Sawicki G.S., Dill E.J., Asher D., Sellers D.E., Robinson W.M. Advance care planning in adults with cystic fibrosis. J Palliat Med. 2008;11:1135–1141. PubMed PMC
Glasscoe C.A., Quittner A.L. Psychological interventions for people with cystic fibrosis and their families. Cochrane Database Syst Rev. 2008;3 [CD003148] PubMed
Ward C.M., Brinkman T., Slifer K.J., Paranjape S.M. Using behavioral interventions to assist with routine procedures in children with cystic fibrosis. J Cyst Fibros. 2010;9:150–153. PubMed PMC
Duff A.J., Latchford G.J. Motivational interviewing for adherence problems in cystic fibrosis. Pediatr Pulmonol. 2010;45:211–220. PubMed
Collaco J.M., Morrow C.B., Green D.M., Cutting G.R., Mogayzel P.J., Jr. Environmental allergies and respiratory morbidities in cystic fibrosis. Pediatr Pulmonol. 2013;48:857–864. PubMed PMC
Mehta G., Macek M., Jr., Mehta A. Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries. J Cyst Fibros. 2010;9(Suppl. 2):S5–S21. PubMed
Shaw N., Peckham D., Conway S., Denton M. Financial savings following the introduction of a cystic fibrosis electronic. J Cyst Fibros. 2010;9:S116.
Etherington C., Conway S., Peckham D. The role of electronic patient records (EPR) in improving service efficiency and clinical performance in a regional adult UK centre. J Cyst Fibros. 2011;10:S96.
The future of cystic fibrosis care: a global perspective
Organization of Patient Management and Fungal Epidemiology in Cystic Fibrosis