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Dr Jim Littlewood Dr Jim Littlewood CFWW/CFE Conference CFWW/CFE Conference CRETE 2005 CRETE 2005 Standards of care for Standards of care for patients with cystic patients with cystic fibrosis: a European fibrosis: a European Consensus Consensus

CFWW/CFE Conference CRETE 2005

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Standards of care for patients with cystic fibrosis: a European Consensus. CFWW/CFE Conference CRETE 2005. Dr Jim Littlewood. Concerns of UK families and patients CF Trust questionnaire 2000. What standard of care should they expect? Facilities & services that should be available? - PowerPoint PPT Presentation

Text of CFWW/CFE Conference CRETE 2005

Page 1: CFWW/CFE Conference CRETE 2005

Dr Jim LittlewoodDr Jim Littlewood

CFWW/CFE ConferenceCFWW/CFE ConferenceCRETE 2005CRETE 2005

Standards of care for Standards of care for patients with cystic patients with cystic fibrosis: a European fibrosis: a European


Page 2: CFWW/CFE Conference CRETE 2005

Concerns of UK families and patients Concerns of UK families and patients

CF Trust questionnaire 2000CF Trust questionnaire 2000

• What standard of care should they expect? What standard of care should they expect?

• Facilities & services that should be available?

• Person responsible for their care?Person responsible for their care?

• ‘Shared-care’ arrangements often unclear

• Local Clinics lacked support of CF Specialist Local Clinics lacked support of CF Specialist and ‘team’and ‘team’

• Poor communication between Specialist Centre & local CF Clinic

• Specialist adult services inadequateSpecialist adult services inadequate

• Cross-infection issues

Page 3: CFWW/CFE Conference CRETE 2005

Artimino, Italy March 2001

ECFS Consensus ECFS Consensus ConferenceConference

Artimino March 2004Artimino March 2004

ECFS Consensus ECFS Consensus ConferenceConference

Artimino March 2004Artimino March 2004

Page 4: CFWW/CFE Conference CRETE 2005

European Cystic Fibrosis European Cystic Fibrosis SocietySociety

• Initiative by Gerd Döring and the European CF Society

• Organised by Organised by Christine DuboisChristine Dubois

• 36 Representatives from many countries

• To agree a consensus To agree a consensus on Standards of Care on Standards of Care for people with CFfor people with CF

Page 5: CFWW/CFE Conference CRETE 2005

Format of the meetingFormat of the meeting• In Artimino at the In Artimino at the

1616thth century La century La Ferdinanda Ferdinanda Medicean VillaMedicean Villa

• 6 round table groups each considered four questions

• Answers presented Answers presented to the whole to the whole Consensus meetingConsensus meeting

•Discussion and eventual agreement by Consensus Group

Page 6: CFWW/CFE Conference CRETE 2005

We discussed standards of care in We discussed standards of care in the cellars of Artimino – while the cellars of Artimino – while

‘accompanying persons’ had other ‘accompanying persons’ had other interests!interests!

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A European A European ConsensusConsensus

•To “define standards for the To “define standards for the routine evaluation, routine evaluation, monitoring and treatment of monitoring and treatment of patients with CF in Europe”patients with CF in Europe”

•Basis for audit of care

•Provide data for comparison

Page 9: CFWW/CFE Conference CRETE 2005

The Consensus Group were The Consensus Group were unanimous on the need forunanimous on the need for CF Centre CF Centre


• CF a complex disease requiring an holistic CF a complex disease requiring an holistic approachapproach

• Center care by a team of health care professionals is essential for optimal patient management and outcome

• Specialist care in dedicated CF Centres is Specialist care in dedicated CF Centres is associated with improved survival and QOLassociated with improved survival and QOL

• Frequent clinical evaluations by physicians and other health workers specifically trained in CF care and early interventions

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Specialist CF CentresSpecialist CF Centres•MINIMUM OF 50


• Specialist CF staff

• In a major teaching In a major teaching hospital hospital

• Treat all CF problems

• 24 hour access for 24 hour access for patients patients

• Various specialist facilities - contacts with other experts

• Liaison with a Liaison with a transplant centre transplant centre

Centre treated Centre treated patients have better patients have better prognosisprognosis

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The CF TeamThe CF Team

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The CF Centre DirectorThe CF Centre Director• The leader of a teamThe leader of a team

•Main interest CF

• Expert on CF careExpert on CF care

• Patients’ advocate

• Audits performanceAudits performance

•Monitors outcomes

• Establish network of CF Establish network of CF expertise in other expertise in other hospital departmentshospital departments

• Secure adequate facilities in which to work

• Communication, Communication, education, researcheducation, research

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Another CF SpecialistAnother CF Specialist

•Senior, permanent Senior, permanent doctor doctor

•30% of time on CF

•Expert in modern Expert in modern CF careCF care

•Interest in Pulmonology or Gastroenterology

•Expert cover when Expert cover when Director is awayDirector is away

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CF Nurse SpecialistCF Nurse Specialist• Almost full-time CFAlmost full-time CF

•Wide knowledge of modern CF care

• Advocate for every Advocate for every patientpatient

• Support and advice for families and patients

• Patient and family Patient and family liaison with Centerliaison with Center

• Education and research

•Major role organising Major role organising home IV antibioticshome IV antibiotics

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PhysiotherapistPhysiotherapist• Regular supervision

and treatment in the clinic and ward

• Individualised, Individualised, reasonable, optimal reasonable, optimal effective regimeneffective regimen

• Advice on airway clearance

• Inhalation therapy Inhalation therapy – – technique and technique and


• Physical education

• Routine spirometryRoutine spirometry


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Dietitian/NutritionistDietitian/Nutritionist• Ensure provision of Ensure provision of

appropriate appropriate nutrition from nutrition from infants to adultsinfants to adults

•Dietary assessments

• Use of pancreatic Use of pancreatic enzymes enzymes

• Adequate vitamin supplements

• Advice in both the Advice in both the clinic and wardclinic and ward

• Intestinal absorption

• Enteral feedingEnteral feeding

• CF-related diabetes

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Psychologist & Social Psychologist & Social WorkerWorker

• Comprehensive Comprehensive assessment of assessment of emotional stateemotional state

• Annual Review (?)

• Inclusion in Inclusion in context of CF teamcontext of CF team

• Transition

• TransplantationTransplantation

•Helping team with routine care and at crisis times

• Families emotional Families emotional and practical and practical needsneeds

• Extra support at various times

• Access to local Access to local services and services and benefitsbenefits

• Complements and works with psychologist

Page 18: CFWW/CFE Conference CRETE 2005

CF PharmacistCF Pharmacist

•Monitor and advise Monitor and advise on taking complex on taking complex drug regimens in drug regimens in most effective and most effective and acceptable wayacceptable way

• Inhaled drugs

• IV antibioticsIV antibiotics

•Oral drugs

• Patient educationPatient education

•Drug interactions

•Flucloxacillin, CiproxinFlucloxacillin, Ciproxin

•Nebulised antibioticNebulised antibiotic

•4 courses I.V. antibiotics/yr4 courses I.V. antibiotics/yr


•Inhaled steriodsInhaled steriods



•Enteral feedsEnteral feeds



•UDCA & taurineUDCA & taurine


Page 19: CFWW/CFE Conference CRETE 2005

Microbiologist & Microbiologist & LaboratoryLaboratory

• Recognition of typical Recognition of typical (usual) CF bacteria (usual) CF bacteria

• Confirmation of Confirmation of B.B. cepaciacepacia etc. at CF etc. at CF LaboratoryLaboratory

•Mycobacteria other than Mycobacteria other than M. tuberculosisM. tuberculosis

• Typing of bacteriaTyping of bacteria

• Different behaviour of Different behaviour of antibiotics in CFantibiotics in CF

• Antibody levelsAntibody levels

• Aspergillus cultures and Aspergillus cultures and antibodiesantibodies

Minimum diagnostic Minimum diagnostic serviceservice

Page 20: CFWW/CFE Conference CRETE 2005

Weekly CF Centre MeetingWeekly CF Centre Meeting

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CF Centre requires CF Centre requires


•Reliable sweat tests

•Venous access expertise (TIVAD)Venous access expertise (TIVAD)

•Written guidelines for complications

•Bronchoscopy serviceBronchoscopy service

•X-rays & Scanners

•Lung function testingLung function testing

•Nasogastric & gastrostomy service

•Gene mutation testingGene mutation testing

Page 22: CFWW/CFE Conference CRETE 2005

Other Functions of a CF Other Functions of a CF CentreCentre

•Provision of specialised procedures, Provision of specialised procedures, diagnostic & laboratory facilitiesdiagnostic & laboratory facilities

•Clinical research, CF meetings & publication

•Arrangements for transition to adult careArrangements for transition to adult care

•Liaison with transplant centre

•CF education, monitoring, data collectionCF education, monitoring, data collection

•Patient involvement in service

•Psychological support - CF relatedPsychological support - CF related

Page 23: CFWW/CFE Conference CRETE 2005

Outpatient clinicOutpatient clinic • Every 1-3 monthsEvery 1-3 months

• Designated CF sessionsDesignated CF sessions

• Adequate clinic roomsAdequate clinic rooms

• CF doctor & CF NurseCF doctor & CF Nurse

• Others staff accessibleOthers staff accessible

•Weight, Height (& Skull)Weight, Height (& Skull)

• Throat swab or sputumThroat swab or sputum

• Respiratory function Respiratory function teststests

• History & ExaminationHistory & Examination

• Review treatmentReview treatment

• DiscussionDiscussion

Page 24: CFWW/CFE Conference CRETE 2005

• A high standard of hygiene by all staffA high standard of hygiene by all staff

• Separate clinics for patients with and without chronic P. aeruginosa

• Separate day for Separate day for B.B. cepacia cepacia complex complex and MRSAand MRSA

• “Day case” facilities

• Organise home IV antibioticsOrganise home IV antibiotics

• Separate offices for staff

• 24 hour access by phone or direct 24 hour access by phone or direct contactcontact

Outpatient Outpatient clinicclinic

Patients should be segregated Patients should be segregated according to their microbiological according to their microbiological statusstatus

Page 25: CFWW/CFE Conference CRETE 2005

Inpatient facilities Inpatient facilities requiredrequired

• 3-5 beds per 50 patients 3-5 beds per 50 patients – immediate admission – immediate admission when needed when needed

• Single rooms, en suite facilities,

• Parents’ accommodationParents’ accommodation

• No patient mixing anywhere

• Clear infection control Clear infection control policypolicy

• B.cepacia & MRSA in separate cubicles on separate ward

• Education and Education and recreation facilitiesrecreation facilities

Not all patients appreciateNot all patients appreciate segregation!!!segregation!!! Isolate unless recent Isolate unless recent

infection status knowninfection status known

Page 26: CFWW/CFE Conference CRETE 2005


•Physiotherapist - physical Physiotherapist - physical treatment, monitoring treatment, monitoring overnight Oovernight O22 saturation saturation

•Weekly sputum cultures & Spirometry

•Skill with venous access & Skill with venous access & TIVADsTIVADs

•Exercise facilities

•Clear treatment PROTOCOLS Clear treatment PROTOCOLS for IV antibiotics and common for IV antibiotics and common complicationscomplications

Page 27: CFWW/CFE Conference CRETE 2005

Inpatients Inpatients • Review by Review by

Pharmacist, Dietitian Pharmacist, Dietitian and Social worker and Social worker

• CF related diabetes

• Treatment of DIOS Treatment of DIOS

• Expertise to start home IV, enteral feeding, & oxygen

• Review of all aspects Review of all aspects carecare

• Clear treatment plan

• Summary within 10 Summary within 10 daysdays

Page 28: CFWW/CFE Conference CRETE 2005

Home care – Nurse, Dr & Home care – Nurse, Dr & Family Family

• Home IV – CF NurseHome IV – CF Nurse

• Flushing TIVADs

• Close supervision of Close supervision of home treatment is home treatment is essentialessential

• Physiotherapy

• Communication with Communication with GPGP

• Instruction and support in the home

• Enteral and Enteral and gastrostomy feeding, gastrostomy feeding,

• Diabetes management

All CF Centres All CF Centres should offer should offer home IV servicehome IV service

Page 29: CFWW/CFE Conference CRETE 2005

Shared care for CF at a local “CF Shared care for CF at a local “CF

Clinic”Clinic” • 20+ patients at local Clinic20+ patients at local Clinic• For children - not adults• Special CF clinic sessionsSpecial CF clinic sessions• Physiotherapist, dietitian &

nurse with a CF interest• Shared care arranged as- Shared care arranged as- - Alternate visits to CF Clinic

and Specialist CF Centre - Periodic visits to Local CF

Clinic by CF Specialist +/- team from centre

- Annual Review by staff at the Specialist CF Centre

• Day-to-day responsibility of Day-to-day responsibility of local consultantlocal consultant

cCF Centre

““should not be allowed to should not be allowed to result in suboptimal care”.result in suboptimal care”.

Page 30: CFWW/CFE Conference CRETE 2005

Transition to adult careTransition to adult care• ““Seamless process”Seamless process”

• Flexible 16-19 years

• Close paediatric & Close paediatric & adult liaison adult liaison

• Ideally common treatment protocols and infection control policies

• Various patterns of Various patterns of transfer.transfer.

• Joint adolescent clinic sessions with staff from both units works well

Page 31: CFWW/CFE Conference CRETE 2005

The ‘Annual Review’The ‘Annual Review’

““Success of Success of treatment will treatment will depend on a depend on a complete complete assessment of assessment of the patient and the patient and then continuing then continuing attempts to attempts to obtain normal obtain normal bodily function”bodily function”

Crozier 1974Crozier 1974

• Detailed history of Detailed history of events over the past year events over the past year

• Full physical examination

• Wt & Ht plotted on Wt & Ht plotted on growth chartgrowth chart

• Assessment of knowledge of Cystic Fibrosis

• Check vaccination statusCheck vaccination status

Annual Review should be Annual Review should be performed at a CF centreperformed at a CF centre

Page 32: CFWW/CFE Conference CRETE 2005

Annual ReviewAnnual Review

Physiotherapist Physiotherapist DietitianDietitian

• Assess methods, Assess methods, competence and competence and frequencyfrequency

• Spirometry

• Use of Use of bronchodilator, bronchodilator, rhDNase & rhDNase & antibioticsantibiotics

• Bronchodilator reversibility testing

• Review / arrange Review / arrange service of nebuliser service of nebuliser

• Exercise testing (?)

• Nutritional reviewNutritional review

• Current diet

•Wt profile and Wt profile and changes over timechanges over time

• Adequacy and knowledge of pancreatic enzyme therapy

• Energy supplements Energy supplements and enteral feedsand enteral feeds

• Vitamins supplements

• CF related diabetesCF related diabetes

Page 33: CFWW/CFE Conference CRETE 2005

AnnualAnnual ReviewReviewLaboratory & X-Laboratory & X-


• 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 Full blood count, urea and electrolytes, liver function electrolytes, liver function tests, vitamins ADE (K), tests, vitamins ADE (K), clotting, clotting,

• Faecal elastase 1Faecal elastase 1

• Fat absorption – micro’Fat absorption – micro’

• Glucose tolerance test Glucose tolerance test (>10 yrs)(>10 yrs)

• Liver ultrasound Liver ultrasound considered (?)considered (?)

• DEXA scan for bone DEXA scan for bone density from 10 yrs (?)density from 10 yrs (?)

Page 34: CFWW/CFE Conference CRETE 2005

Annual Review – Annual Review – Results, Decisions & DiscussionsResults, Decisions & Discussions

• Director Director personally personally reviews reviews clinical & chest X-ray scoresclinical & chest X-ray scores

•Usually discusses with appropriate team members and plans therapy

• Discusses findings and Discusses findings and suggestions with patient or suggestions with patient or parentsparents

•Director writes full letter and report to referring consultant, patient’s doctor & copy to patient or parents

• Data entered into CF Centre Data entered into CF Centre and National Databasesand National Databases

• Fix next year’s appointment!

Page 35: CFWW/CFE Conference CRETE 2005

For new patients at their For new patients at their first Annual Reviewfirst Annual Review

• Repeat sweat testRepeat sweat test

•Genotype if not already done

• Confirm pancreatic Confirm pancreatic insufficiency with EL1 insufficiency with EL1 (even if on enzymes)(even if on enzymes)

• Provide CF Centre’s literature

•Meet members of the Meet members of the CF TeamCF Team

Mistakes in diagnosis Mistakes in diagnosis and unnecessary and unnecessary enzyme treatment must enzyme treatment must be identifiedbe identified

Page 36: CFWW/CFE Conference CRETE 2005

Neonatal screeningNeonatal screening•Good methods now Good methods now

well tested well tested

•Major proven Major proven nutritional and nutritional and respiratory respiratory advantagesadvantages

•Early CF centre Early CF centre referral and referral and treatment essentialtreatment essential

•Genetic adviceGenetic advice

All newborn infants should be All newborn infants should be screened for CFscreened for CF

Page 37: CFWW/CFE Conference CRETE 2005

Neonatal CF screening

Page 38: CFWW/CFE Conference CRETE 2005

• Full explanation with a Full explanation with a parent/relative presentparent/relative present

•Written information

•Meet nurse specialist Meet nurse specialist and others of the CF and others of the CF team team

• Admit ill infants

• Start regular treatment Start regular treatment – antibiotics, enzymes, – antibiotics, enzymes, vitamins, vitamins, physiotherapyphysiotherapy

• Regular follow up with staff contact numbers

• All adults treated at All adults treated at specialist CF centrespecialist CF centre

• “Shared care” for some children

• Family doctor & Family doctor & health visitor health visitor informedinformed

• Patient informed of the national CF organisation

• Genetic advice for Genetic advice for family family

• Sweat test all siblings

After neonatal screening/diagnosisAfter neonatal screening/diagnosis

Page 39: CFWW/CFE Conference CRETE 2005

Hospital care for adults with Hospital care for adults with CFCF

• Complex, changing, Complex, changing, developing developing treatment treatment

• CF age-related new problems – CFRD, bones, fertility

• Need staff with wide Need staff with wide experience of CFexperience of CF

• Advice for dealing with diagnosis in adult life

• Transplant issuesTransplant issues

• Advice on atypical CF – individualised but carefully regular monitoring

All adults with CF shouldAll adults with CF should attend a Specialist CF Centreattend a Specialist CF Centre

Page 40: CFWW/CFE Conference CRETE 2005

Specific problems for discussion or Specific problems for discussion or referralreferral

• Prolonged exacerbations

• Deterioration RFTS not Deterioration RFTS not responding to R/.responding to R/.

• Pneumothorax

• Haemoptysis (>20ml)Haemoptysis (>20ml)


• Significant atelectasisSignificant atelectasis

• Respiratory/cardiac failure

• Meconium ileusMeconium ileus

• Acute surgery

• Elective major surgeryElective major surgery

• GI bleeding

• Liver/gall bladder Liver/gall bladder complications. Jaundicecomplications. Jaundice

• Insertion of TIVADs

• For gastrostomyFor gastrostomy

• Unexplained wt loss or FTT

• Glucose intoleranceGlucose intolerance

• Vasculitis, Arthropathy

• Pregnancy, fertility Pregnancy, fertility issuesissues

• Serious psychological problems

• Transplant assessmentTransplant assessment

• Osteoporosis

• Renal diseaseRenal disease

• CF related ENT problems

Page 41: CFWW/CFE Conference CRETE 2005

Role for many other Role for many other specialistsspecialists

•Gastroenterology Gastroenterology

•Surgeons – Transplant and others



•Bone diseaseBone disease

•ENT surgeon

•Obstetrics & GynaecologyObstetrics & Gynaecology


•Genetic counsellingGenetic counselling

•X-rays & imaging

Page 42: CFWW/CFE Conference CRETE 2005

Staff member Staff member (per 50 patients)(per 50 patients)

Specialist Specialist Paediatric CentrePaediatric Centre

Specialist Specialist Adult CentreAdult Centre

Consultant 1 0.5 0.5

Consultant 2 0.2-0.3 0.2-0.3

Staff Grade 0.4 0.6

CF Sp.Registrar 0.5 0.5

CF Nurse 1-1.5 1.0 -1.5

Physio 0.5 – 1.0 1.0

Dietitian 0.4 0.4

Social worker 0.4 0.4

Psychologist 0.4 0.4







Data Clerk 0.1 0.1

Page 43: CFWW/CFE Conference CRETE 2005

US CF Foundation’sUS CF Foundation’s “FATAL “FATAL FLAWS”FLAWS”

• No regular team meetingsNo regular team meetings

• Insufficient clinic time to see patients

• Too many patients per clinicToo many patients per clinic

• Unreliable sweat test technique

• Incomplete 24 hr expert CF coverageIncomplete 24 hr expert CF coverage

• No adult care plan

• No backup physicianNo backup physician

• Less than 50 Less than 50 patientspatients

Page 44: CFWW/CFE Conference CRETE 2005

There is room for improvement in CF There is room for improvement in CF

carecare • 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


Page 45: CFWW/CFE Conference CRETE 2005

Successful CF doctorsSuccessful 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 resultsDo research with staff and publish results

[Inspired by “The Bell Curve”. Gawande G, New Yorker, Dec 6th 2004]


Page 46: CFWW/CFE Conference CRETE 2005

Standards of care for patients with cystic Standards of care for patients with cystic fibrosis: a European Consensusfibrosis: a European Consensus

Kerem, Conway, Elborn, Heijerman et al for the Consensus Committee

J of Cystic Fibrosis 2005;4:7

• To defineTo define standards for the for the routine evaluation, routine evaluation, monitoring and treatment of monitoring and treatment of patients with CF in Europepatients with CF in Europe

• A basis forA basis for auditing CF careCF care

• Provides data forProvides data for comparison between CF between CF CentresCentres

Page 47: CFWW/CFE Conference CRETE 2005

Standards of care for patients Standards of care for patients with cystic fibrosis: a European with cystic fibrosis: a European

ConsensusConsensusKerem, Conway, Elborn, Heijerman et al for the

Consensus Committee J. of Cystic Fibrosis 2005;4:7-26

“ “A Roadmap to Best A Roadmap to Best Care” Care”


people with Cystic people with Cystic FibrosisFibrosis

Littlewood. Editorial. J of Cystic Fibrosis 2005;4:1-5