CFWW/CFE Conference CRETE 2005

1. Standards of care for patients with cystic fibrosis: a European Consensus CFWW/CFE ConferenceCRETE 2005 Dr Jim Littlewood

2. Concerns of UK families and patients CF Trust questionnaire 2000 • What standard of care should they expect? • Facilities & services that should be available? • Person responsible for their care? • ‘Shared-care’ arrangements often unclear • Local Clinics lacked support of CF Specialist and ‘team’ • Poor communication between Specialist Centre & local CF Clinic • Specialist adult services inadequate • Cross-infection issues

3. ECFS Consensus ConferenceArtimino March 2004 Artimino, Italy March 2001

4. European Cystic Fibrosis Society • Initiative by Gerd Döring and the European CF Society • Organised by Christine Dubois • 36 Representatives from many countries • To agree a consensus on Standards of Care for people with CF

5. Format of the meeting • In Artimino at the 16th century La Ferdinanda Medicean Villa • 6 round table groups each considered four questions • Answers presented to the whole Consensus meeting • Discussion and eventual agreement by Consensus Group

6. We discussed standards of care in the cellars of Artimino – while ‘accompanying persons’ had other interests!

8. A European Consensus • To “define standards for the routine evaluation, monitoring and treatment of patients with CF in Europe” • Basis for audit of care • Provide data for comparison

9. The Consensus Group were unanimous on the need forCF Centre care • CF a complex disease requiring an holistic approach • Center care by a team of health care professionals is essential for optimal patient management and outcome • Specialist care in dedicated CF Centres is associated with improved survival and QOL • Frequent clinical evaluations by physicians and other health workers specifically trained in CF care and early interventions

10. Specialist CF Centres • MINIMUM OF 50 PATIENTS • Specialist CF staff • In a major teaching hospital • Treat all CF problems • 24 hour access for patients • Various specialist facilities - contacts with other experts • Liaison with a transplant centre Centre treated patients have better prognosis

11. The CF Team

12. The CF Centre Director • The leader of a team • Main interest CF • Expert on CF care • Patients’ advocate • Audits performance • Monitors outcomes • Establish network of CF expertise in other hospital departments • Secure adequate facilities in which to work • Communication, education, research

13. Another CF Specialist • Senior, permanent doctor • 30% of time on CF • Expert in modern CF care • Interest in Pulmonology or Gastroenterology • Expert cover when Director is away

14. CF Nurse Specialist • Almost full-time CF • Wide knowledge of modern CF care • Advocate for every patient • Support and advice for families and patients • Patient and family liaison with Center • Education and research • Major role organising home IV antibiotics

15. Physiotherapist • Regular supervision and treatment in the clinic and ward • Individualised, reasonable, optimal effective regimen • Advice on airway clearance • Inhalation therapy – technique and tolerance • Physical education • Routine spirometry hide

16. Dietitian/Nutritionist • Ensure provision of appropriate nutrition from infants to adults • Dietary assessments • Use of pancreatic enzymes • Adequate vitamin supplements • Advice in both the clinic and ward • Intestinal absorption • Enteral feeding • CF-related diabetes

17. Comprehensive assessment of emotional state Annual Review (?) Inclusion in context of CF team Transition Transplantation Helping team with routine care and at crisis times Families emotional and practical needs Extra support at various times Access to local services and benefits Complements and works with psychologist Psychologist & Social Worker

18. Monitor and advise on taking complex drug regimens in most effective and acceptable way Inhaled drugs IV antibiotics Oral drugs Patient education Drug interactions CF Pharmacist • Flucloxacillin, Ciproxin • Nebulised antibiotic • 4 courses I.V. antibiotics/yr • Bronchodilator • Inhaled steriods • Pulmozyme • Gastrostomy • Enteral feeds • Vitamins • Enzymes • UDCA & taurine • Insulin

19. Microbiologist & Laboratory • Recognition of typical (usual) CF bacteria • Confirmation of B.cepacia etc. at CF Laboratory • Mycobacteria other than M. tuberculosis • Typing of bacteria • Different behaviour of antibiotics in CF • Antibody levels • Aspergillus cultures and antibodies Minimum diagnostic service

20. Weekly CF Centre Meeting

21. CF Centre requires • 24-HOUR ACCESS FOR PATIENTS • Reliable sweat tests • Venous access expertise (TIVAD) • Written guidelines for complications • Bronchoscopy service • X-rays & Scanners • Lung function testing • Nasogastric & gastrostomy service • Gene mutation testing

22. Other Functions of a CF Centre • Provision of specialised procedures, diagnostic & laboratory facilities • Clinical research, CF meetings & publication • Arrangements for transition to adult care • Liaison with transplant centre • CF education, monitoring, data collection • Patient involvement in service • Psychological support - CF related

23. Outpatient clinic • Every 1-3 months • Designated CF sessions • Adequate clinic rooms • CF doctor & CF Nurse • Others staff accessible • Weight, Height (& Skull) • Throat swab or sputum • Respiratory function tests • History & Examination • Review treatment • Discussion

24. Outpatient clinic • A high standard of hygiene by all staff • Separate clinics for patients with and without chronic P. aeruginosa • Separate day for B.cepacia complex and MRSA • “Day case” facilities • Organise home IV antibiotics • Separate offices for staff • 24 hour access by phone or direct contact Patients should be segregated according to their microbiological status

25. Inpatient facilities required • 3-5 beds per 50 patients – immediate admission when needed • Single rooms, en suite facilities, • Parents’ accommodation • No patient mixing anywhere • Clear infection control policy • B.cepacia & MRSA in separate cubicles on separate ward • Education and recreation facilities Not all patients appreciate segregation!!! Isolate unless recent infection status known

26. Inpatients • Physiotherapist - physical treatment, monitoring overnight O2 saturation • Weekly sputum cultures & Spirometry • Skill with venous access & TIVADs • Exercise facilities • Clear treatment PROTOCOLS for IV antibiotics and common complications

27. Inpatients • Review by Pharmacist, Dietitian and Social worker • CF related diabetes • Treatment of DIOS • Expertise to start home IV, enteral feeding, & oxygen • Review of all aspects care • Clear treatment plan • Summary within 10 days

28. Home care – Nurse, Dr & Family • Home IV – CF Nurse • Flushing TIVADs • Close supervision of home treatment is essential • Physiotherapy • Communication with GP • Instruction and support in the home • Enteral and gastrostomy feeding, • Diabetes management All CF Centres should offer home IV service

29. Shared care for CF at a local “CF Clinic” • 20+ patients at local Clinic • For children - not adults • Special CF clinic sessions • Physiotherapist, dietitian & nurse with a CF interest • Shared care arranged as- - Alternate visitsto CF Clinic and Specialist CF Centre - Periodic visitsto Local CF Clinic by CF Specialist +/- team from centre - Annual Reviewby staff at the Specialist CF Centre • Day-to-day responsibility of local consultant c CF Centre “should not be allowed to result in suboptimal care”.

30. Transition to adult care • “Seamless process” • Flexible 16-19 years • Close paediatric & adult liaison • Ideally common treatment protocols and infection control policies • Various patterns of transfer. • Joint adolescent clinic sessions with staff from both units works well

31. “Success of treatment will depend on a complete assessment of the patient and then continuing attempts to obtain normal bodily function” Crozier 1974 Detailed history of events over the past year Full physical examination Wt & Ht plotted on growth chart Assessment of knowledge of Cystic Fibrosis Check vaccination status The ‘Annual Review’ Annual Review should be performed at a CF centre

32. Assess methods, competence and frequency Spirometry Use of bronchodilator, rhDNase & antibiotics Bronchodilator reversibility testing Review / arrange service of nebuliser Exercise testing (?) Nutritional review Current diet Wt profile and changes over time Adequacy and knowledge of pancreatic enzyme therapy Energy supplements and enteral feeds Vitamins supplements CF related diabetes Annual ReviewPhysiotherapist Dietitian

33. AnnualReviewLaboratory & X-rays • Sputum for Non-TB mycobacteria • Aspergillus RAST precipitins, IgE, IgA, IgM, IgG, other inflammatory markers • Pseudomonas antibodies • Chest X-ray • Full blood count, urea and electrolytes, liver function tests, vitamins ADE (K), clotting, • Faecal elastase 1 • Fat absorption – micro’ • Glucose tolerance test (>10 yrs) • Liver ultrasound considered (?) • DEXA scan for bone density from 10 yrs (?)

34. Annual Review – Results, Decisions & Discussions • Director personally reviews clinical & chest X-ray scores • Usually discusses with appropriate team members and plans therapy • Discusses findings and suggestions with patient or parents • Director writes full letter and report to referring consultant, patient’s doctor & copy to patient or parents • Data entered into CF Centre and National Databases • Fix next year’s appointment!

35. For new patients at their first Annual Review • Repeat sweat test • Genotype if not already done • Confirm pancreatic insufficiency with EL1 (even if on enzymes) • Provide CF Centre’s literature • Meet members of the CF Team Mistakes in diagnosis and unnecessary enzyme treatment must be identified

36. Neonatal screening • Good methods now well tested • Major proven nutritional and respiratory advantages • Early CF centre referral and treatment essential • Genetic advice All newborn infants should be screened for CF

37. Neonatal CF screening

38. Full explanation with a parent/relative present Written information Meet nurse specialist and others of the CF team Admit ill infants Start regular treatment – antibiotics, enzymes, vitamins, physiotherapy Regular follow up with staff contact numbers All adults treated at specialist CF centre “Shared care” for some children Family doctor & health visitor informed Patient informed of the national CF organisation Genetic advice for family Sweat test all siblings After neonatal screening/diagnosis

39. Hospital care for adults with CF • Complex, changing, developing treatment • CF age-related new problems – CFRD, bones, fertility • Need staff with wide experience of CF • Advice for dealing with diagnosis in adult life • Transplant issues • Advice on atypical CF – individualised but carefully regular monitoring All adults with CF should attend a Specialist CF Centre

40. Prolonged exacerbations Deterioration RFTS not responding to R/. Pneumothorax Haemoptysis (>20ml) ABPA Significant atelectasis Respiratory/cardiac failure Meconium ileus Acute surgery Elective major surgery GI bleeding Liver/gall bladder complications. Jaundice Insertion of TIVADs For gastrostomy Unexplained wt loss or FTT Glucose intolerance Vasculitis, Arthropathy Pregnancy, fertility issues Serious psychological problems Transplant assessment Osteoporosis Renal disease CF related ENT problems Specific problems for discussion or referral

41. Role for many other specialists • Gastroenterology • Surgeons – Transplant and others • Hepatology • Diabetes • Bone disease • ENT surgeon • Obstetrics & Gynaecology • Fertility • Genetic counselling • X-rays & imaging

43. US CF Foundation’s“FATAL FLAWS” • No regular team meetings • Insufficient clinic time to see patients • Too many patients per clinic • Unreliable sweat test technique • Incomplete 24 hr expert CF coverage • No adult care plan • No backup physician • Less than 50 patients

44. There is room for improvement in CF care • Wide variation between the “best” and the “rest” • Respiratory function (FEV1) differs by 10% • Median age of death varies from 23.2 to 28.2 yrs • Improving “rest” to the “best” would increase life expectancy by 7 years • These differences now receiving much attention • National CF registries are essential to show these differences Best Rest

45. [Inspired by “The Bell Curve”. Gawande G, New Yorker, Dec 6th 2004] Successful CF doctors • Lead and support a caring expert CF Team • Well informed & have wide experience • Focused and aggressive • Think hard and often about their patients • Never satisfied with present treatment • Inventive – tries new approaches • Push patients with treatment – kindly! • Capacity to learn and adapt rapidly • Involve expertise of other departments and colleagues in a large hospital • Successfully enlist support of hospital managers • Do research with staff and publish results • >50 PATIENTS ESSENTIAL

46. Standards of care for patients with cystic fibrosis: a European ConsensusKerem, Conway, Elborn, Heijerman et al for the Consensus Committee J of Cystic Fibrosis 2005;4:7 • To definestandardsfor the routine evaluation, monitoring and treatment of patients with CF in Europe • A basis forauditing CF care • Provides data forcomparisonbetween CF Centres

47. Standards of care for patients with cystic fibrosis: a European ConsensusKerem, Conway, Elborn, Heijerman et al for the Consensus Committee J. of Cystic Fibrosis 2005;4:7-26 “A Roadmap to Best Care” for people with Cystic Fibrosis Littlewood. Editorial. J of Cystic Fibrosis 2005;4:1-5