Transcript Standards of CF Care in Europe

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?
• 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
ECFS Consensus Conference
Artimino March 2004
Artimino, Italy March 2001
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
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
We discussed standards of care in the
cellars of Artimino – while ‘accompanying
persons’ had other interests!
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
The Consensus Group were unanimous on
the need for CF 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
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
The CF Team
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
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
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
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
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
Psychologist & Social Worker
• 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
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Extra support at
various times
Access to local
services and benefits
Complements and
works with
psychologist
CF Pharmacist
•Flucloxacillin, Ciproxin
•Nebulised antibiotic
•4 courses I.V. antibiotics/yr
•Bronchodilator
•Inhaled steriods
•Pulmozyme
•Gastrostomy
•Enteral feeds
•Vitamins
•Enzymes
•UDCA & taurine
•Insulin
• Monitor and advise
on taking complex
drug regimens in most
effective and
acceptable way
• Inhaled drugs
• IV antibiotics
• Oral drugs
• Patient education
• Drug interactions
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
Weekly CF Centre Meeting
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
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
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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
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
Inpatient facilities required
• 3-5 beds per 50 patients –
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Not all patients appreciate
segregation!!!
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
Isolate unless recent
infection status known
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
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
Home care – Nurse, Dr & Family
• Home IV – CF Nurse
• Flushing TIVADs
• Close supervision of home
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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
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
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CF Centre
c
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with a CF interest
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
local consultant
“should not be allowed to result
in suboptimal care”.
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
The ‘Annual Review’
“Success of
treatment will
depend on a
complete
assessment of the
patient and then
continuing
attempts to obtain
normal bodily
function”
Crozier 1974
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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
Annual Review should be
performed at a CF centre
Annual Review
Physiotherapist
• Assess methods,
competence and
frequency
• Spirometry
• Use of bronchodilator,
rhDNase & antibiotics
• Bronchodilator
reversibility testing
• Review / arrange
service of nebuliser
• Exercise testing (?)
Dietitian
• 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 Review
Laboratory & X-rays
• Sputum for Non-TB
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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 (?)
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!
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
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
Neonatal CF screening
After neonatal screening/diagnosis
• 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
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
All adults with CF should
but carefully regular
attend a Specialist CF Centre
monitoring
Specific problems for discussion or referral
• Prolonged exacerbations
• Deterioration RFTS not
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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
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problems
Transplant assessment
Osteoporosis
Renal disease
CF related ENT problems
Role for many other specialists
• Gastroenterology
• Surgeons – Transplant and others
• Hepatology
• Diabetes
• Bone disease
• ENT surgeon
• Obstetrics & Gynaecology
• Fertility
• Genetic counselling
• X-rays & imaging
Staff member
(per 50 patients)
Specialist
Paediatric Centre
Specialist
Adult 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
Pharmacist
Secretary
0.3
1.0
0.3
1.0
Data Clerk
0.1
0.1
US CF Foundation’s “FATAL FLAWS”
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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
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
Rest
Best
• 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
Successful CF doctors
[Inspired by “The Bell Curve”. Gawande G, New Yorker, Dec 6th 2004]
• 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
Standards of care for patients with cystic fibrosis: a
European Consensus
Kerem, Conway, Elborn, Heijerman et al for the Consensus Committee
J of Cystic Fibrosis 2005;4:7
• To define standards for the
routine evaluation,
monitoring and treatment
of patients with CF in
Europe
• A basis for auditing CF
care
• Provides data for
comparison between CF
Centres
Standards of care for patients with
cystic fibrosis: a European Consensus
Kerem, 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