National Standards of Care in

Adult Sickle Cell Disorders in

the UK.
Dr Ade Olujohungbe
Sickle Cell Society Working Group Chair

September 2008

Standards of Care in SCD: Working Towards Better Care February 2008

 Overview
• History
• Objectives
• Standards development & processes
• Next steps
• Critical success factors

Standards of Care in SCD: Working Towards Better Care February 2008

 Why have standards in
the UK?
• Increasing patient population
– Newborn Screening
– Immigration patterns
• Concerns about patient safety
– Wellcome trust workshop/ SC NCEPOD grading of care
society
– NCEPOD 7
1

• Remove “postcode lottery” 1

17
– Access to high cost drugs 1

– Inequalities in health care

• Commissioning Resource
• Flagship in partnership working Good practice
Clinical improevment
– Medical community 14 Organisational improvement
Clin & org improvement
– Voluntary sector < Satisfactory

– Industry Insufficient info

Standards of Care in SCD: Working Towards Better Care February 2008

 Why are we doing this?
• Variability of standards across the UK
– Wellcome-Trust Sickle Cell Report (2005)
highlighted key areas of concern for service
users

• Compliment other related initiatives

– SCD & Thalassaemia NHS Screening
– Thalassaemia Standards (2005)
– SCD in Childhood: Standards and Guidelines
(2006)
Standards of Care in SCD: Working Towards Better Care February 2008
Standards of Care in SCD: Working Towards Better Care February 2008
 Objectives

Standards AND Education

To agree and publish national

To produce an educational
standards of care for adults with
resource for health care providers
SCD, endorsed by the Department
and service users, as well as a
of Health and UK Forum on
benchmark of acceptable standards
Haemoglobin Disorders by July
of care
2008

Standards of Care in SCD: Working Towards Better Care February 2008

 Who is involved?

Haematology
specialists

Service Haematology
users counselling

Working
group

Social work Primary care

Psychology

Standards of Care in SCD: Working Towards Better Care February 2008

 Working group members
• Dr Kofi Anie • Sister Matty Asante Owusu
• Professor Elizabeth Anionwu • Dr Shivan Pancham
• Dr Karl Atkin • Dr Norman Parker
• Dr Wale Atoyebi • Professor John Porter
• Dr Moji Awogbade • Ms Elizabeth Quarcoopome
• Dr Lorna Bennett (Deputy • Dr David Rees
Chair) • Dr Kate Ryan
• Dr Claire Chapman • Dr Farrukh Shah
• Dr Phil Darbyshire • Dr Joan St John
• Mrs Verna Davis • Ms Stephanie Sulaiman
• Dr Bernard Davis • Professor Swee Lay Thein
• Dr Moira Dick • Ms Iyamide Thomas
• Dr Joanna Howard • Dr Christine Wright
• Dr Mark Layton • Dr Josh Wright
• Mr Anthony Mason • Dr Anne Yardumian
• Dr Asa'ah Nkohkwo
• Ms Ogo Okoye  Editorial Team Member
• Dr Adebayo Olujohungbe
(Chair)
Standards of Care in SCD: Working Towards Better Care February 2008
 Terms of reference
• To act as a body of experts for development of standards of care
for adults with sickle cell disease (SCD)
• To ensure project funding is subject to robust scrutiny, wholly
transparent and open competition
• To ensure industry partnerships adhere to codes of conduct
prescribed by the Health Coalition Initiative and the Association of
British Pharmaceutical Industry
• To formulate standards of care with demonstrable user
representation and consultation so that the views of adults with
SCD are reflected
• To take account of key developments that are relevant to the
operational context of the standards which include appropriate
legislation and other bodies of work
• To ensure that the role of the Sickle Cell Society is clearly
described and acknowledged in the finished document
• To ensure that the process of resolving differences of opinion is
clearly communicated to the whole of the group and agreed

Standards of Care in SCD: Working Towards Better Care February 2008

 Process
Convene Editorial Team
Working Literature review/
Group: searches editing
Development process Full
of Standards Writing groups Manuscript
structure, develop draft Series of
content and copy Editorial Team
format Meetings

?
?
Working Production ?
Group and Implementatio
and Audit
independent n
review launch ?
We are ?
here ?

Standards of Care in SCD: Working Towards Better Care February 2008

 Who is it aimed at?
SCD Adult Standards of Care

SCD Community Health Care Community

Consultant Haematologists/
Service Users
Specialist Nurses

Carers GPs/ Practice Nurses

Psychologists/ Haemoglobinopathies
Social Services
Counsellors

A&E Consultants/ Nurses/

Expert Patients
Paramedics

Commissioners/ NHS Payors

Standards of Care in SCD: Working Towards Better Care February 2008

 Standards contents
• Forward • Managing Chronic
• Executive Summary Complications
• Methodology • Pregnancy,
• Contraception &
Summary of Standards
Fertility
• SCD: Overview
• Issues in Blood
• Organization of Care & Transfusion
Commissioning SCD
• Surgery & Specific
Services
Therapies
• Managing Acute
• Appendices
Complications
• Glossary

Standards of Care in SCD: Working Towards Better Care February 2008

 “Six Principal Standards”

1. Pain management
a) 2hr target
2. Acute complications
a) Vital signs
3. Detecting and managing chronic complications
a) screening
4. Setting up networks of care with user involvement
a) Organisation of care levels
5. Education and training
a) Patients
b) Health care workers/ Commissioners
6. Adequate resources
a) Audit
b) Access to high cost interventions

Standards of Care in SCD: Working Towards Better Care February 2008

 Levels of evidence (Agency for Healthcare
Research and Quality 1992)

• (A) requires at least one randomised trial as part of body

of literature of overall good quality and consistency
addressing specific recommendation
• (B) Requires availability of well conducted clinical studies
but no randomised clinical trials on topic of the
recommendations
• (C) Requires evidence from expert committee reports or
opinions and or clinical experience of respected authorities.
Indicates absence of directly applicable studies of good
quality.

Standards of Care in SCD: Working Towards Better Care February 2008

 Recommendations for best practice
Iron Overload.

• Not frequently recognised in intermittent users in SCD.

• Limited evidence of worsening clinical outcomes in SCD.
• Accurate transfusion history required.
• Start chelation >20 transfusions or LIC >7mg/g dry weight
(C).
• Serial Serum Ferritin trends in “steady state” useful
• Adjunctive quantitative hepatic iron measurements T2*MRI
(C).

Standards of Care in SCD: Working Towards Better Care February 2008

 1. Pain management

• Commonest presentation to Hospital and core

component of resource utilisation.
• Rapid assessment.
• Effective analgesia within 30 mins ( Rees et al
2003).
• Pain control within 2 hrs with continuous
evaluation fundamental to success.

Standards of Care in SCD: Working Towards Better Care February 2008

 2.Acute complications

• At AED, Assessment for acute and potentially life

threatening complications e.g. Chest Syndrome.
• Basic observations ( TPR) recorded regularly
• Competent clinical expertise “on hand” to managed these
complications

Standards of Care in SCD: Working Towards Better Care February 2008

 3.Chronic complications

• Regular outpatient follow up

– Mechanism for contacting defaulters at 1o care level
• Screening for complications e.g. PHT , renal, iron overload
• Screening for other medical complications e.g. prostrate,
DM.
• Combined specialist clinics in Pregnancy.

Standards of Care in SCD: Working Towards Better Care February 2008

 Indications for transfusion
therapy in SCD

Acute intervention Long-term management

• Anaemia • Heart failure
• Stroke • Prophylaxis against
• Acute chest syndrome recurrent stroke
• Preoperative (in some cases) • Cardiopulmonary
• Acute multiple-organ failure – Chronic pulmonary
syndrome hypertension
• Preoperative – Refractory congestive
• Malaria-associated severe heart failure
hemolytic anemia • Hydroxyurea non-
responders
• Previous splenic
sequestration in children
aged ≤2–3 years
• Chronic pain February 2008
Standards of Care in SCD: Working Towards Better Care
 Transfusional iron overload in
SCD
30

25
Iron (mg/g dry weight)

20 R=0.795

15

10

0
0 20 40 60 80 100 120 140 160

Transfusion duration (months)

Whole body iron is a major determinant of morbidity and

mortality in SCD
Standards of Care in SCD: Working Towards Better Care February 2008
Harmatz P et al. Blood 2000;96:76–79, permission pending
 Comparison of chelators

Property DFO1,2 Deferiprone3,4 Deferasirox5,6

Usual dose 25–60 75 20–30
(mg/kg/day)
Route s.c., i.v. Oral Oral
(8–12 hours, 3 times daily once daily
5 days/week)

Half-life 20–30 minutes 3–4 hours 8–16 hours

Excretion Urinary, faecal Urinary Faecal

Main Local reactions, Gastrointestinal Gastrointestinal

adverse ophthalmological, disturbances, disturbances, rash, mild
effects auditory, growth agranulocytosis/ non-progressive
in PI retardation, allergic neutropenia, creatinine increase,
arthralgia, elevated elevated liver enzymes,
liver enzymes ophthalmological,
auditory
Status Licensed Licensed outside Licensed
US/Canada

Standards of1 Care in SCD: Working Towards 2Better Care February 2008
Olivieri NF, et al. Blood. 1997;89:739-61. Deferoxamine [package insert]. Novartis; 2002. 3Kushner JP, et al. Hematology. 2001;47-61.
4
Deferiprone [package insert]. Apotex Europe Ltd; 1999. 5Cappellini MD, et al. Blood. 2006;107:3455-62. 6Deferasirox [package insert]. Novartis; 2005.
 4.Care networks

• Access to a wide range of services

• Close to home where appropriate
• Facilitated access to specialist clinics for complex care
• User representation at all times

Standards of Care in SCD: Working Towards Better Care February 2008

 Access to care

Service
User

Multidisciplinary working
Carers/ Family

Education/ EPP

COMMUNITY
SCaT Centres/ Primary Care/ Social
Services

HOSPITAL
Local Hospital Unit/ SCD Specialist Centre

Standards of Care in SCD: Working Towards Better Care February 2008

 Managed clinical network:
high prevalence (part I)

SCD SCD
Specialist Specialist
Centre Centre

SCD
Specialist SCaT Centre
Centre

Standards of Care in SCD: Working Towards Better Care February 2008

 Managed clinical network:
high prevalence (part II)
Local Authority

Specialist
Primary
Voluntary Services
Care
Sector Network
Services
(part I)

Social
Services

Standards of Care in SCD: Working Towards Better Care February 2008

 Hub & spoke model: low
prevalence
Local Authority

Local
Voluntary Hospital
Sector Unit

SCD
Specialist
Centre/ SCaT
Centre

Local Primary
Hospital Care
Unit Services

Social
Services

Standards of Care in SCD: Working Towards Better Care February 2008


SERVICES
A&E/ Out-patients/ Phlebotomy
In-patient beds
Day unit
Prescribing
Pain management
Psychology service
Genetic counselling services
Interpreting & advocacy services
Social work service
Stroke risk screening/TCD
Stroke care
Surgery
Urological & renal services
SCD obstetric care
High dependency care and ITU
Specialist liver service
SCD respiratory services
Pulmonary hypertension
SCD ophthalmology
Erythrocytopheresis
Diagnostic imaging
Lab. support (diagnostics &
transfusion)
Annual clinical reviews
Standards of Care in SCD: Working Towards Better Care
Required services:
summary
SPECIALIST CENTRES LOCAL HOSPITAL COMMUNITY
  
 (supported by specialist input)  (to manage common uncomplicated
presentations)
 (extended hours)  (extended hours) Day assessment/ treatment area
  Nurse prescribing
  Home care pain service
  (access only) 
 (access only)  (access only) Genetic counselling
  
 (access only)  (access only) Social work service

 (inc. emergency management,  (inc. ongoing transfusion & iron Stroke rehabilitation
specialist diagnostics & initiating chelation)
transfusion)
 (complex surgery inc orthopaedic  (low risk surgery in liaison with
services) specialist centre team)
 (inc. access to dialysis and transplant)


 (access only)

 (screening & access to tertiary centres)
 (inc. retinopathy screening)
 (&/or facilities for manual red cell
exchange)
  (CT/MRI)
 (appropriate CPA-accredited support)  (appropriate CPA-accredited support)

February 2008
 5.Education and training

• Education of patients, carers and health professionals on

SCD.
• Accessible protocols and policies in clinical areas.
• CPD documentation
• Competency assessment
• Support for Expert patient programmes

Standards of Care in SCD: Working Towards Better Care February 2008

 6. Adequate resources
Commissioning

• Specialist commissioning for SCD services

• Evidence based
• Equal access to high cost drugs and
interventions.
• Audit & peer review.
• Service improvement

Standards of Care in SCD: Working Towards Better Care February 2008

 Exjade funding status, sickle cell disease
July 2008

5% 7% 4%
9%

1%

74%

Funding not approved Approved on a named patient basis

Approved under Service Level Agmt Trust funding
Funding decision pending Funding not requested

Total number of PCOs recorded: 210

Market Survey

Standards of Care in SCD: Working Towards Better Care February 2008

 TCD in SCD
• No strong evidence in Adults
• In paediatrics 90% of sickle cell centres should have
capability of offering annual TCDs to children with SCD
from age of 3yrs by 2008.
• This should increase to 99% by 2010.
• Development and commissioning is vital.
• What Next?

Standards of Care in SCD: Working Towards Better Care February 2008

 Research Development

• Paucity of grade A evidence in Adult SCD.

• Clinical trials network centrally funded with research and
administrative staff e.g. LRF, MRC, NCRI.
• Not reliant on goodwill of clinicians and support staff

Standards of Care in SCD: Working Towards Better Care February 2008

 Critical success factors
• Peer review and endorsement
• Production planning
• Successful launch/ high awareness
• Implementation
• Audit and measurable improvement

Standards of Care in SCD: Working Towards Better Care February 2008

 A poisoned Chalice? 

Standards of Care in SCD: Working Towards Better Care February 2008

 Questions?

Standards of Care in SCD: Working Towards Better Care February 2008