A Paediatric Sickle Cell Service

Essential Components
Dr Moira Dick
Consultant Community Paediatrician
Kings College Hospital
Lambeth PCT

1981-7 Belgrave Children’s Hospital/KCH

1990- 2003 STH
1994- date KCH
Belgrave
Hospital
for Children

First
paediatric
sickle cell
clinic UK 1970
 Sickle cell services SE
London
1958 selective screening paediatric
admissions KCH
2 paediatric clinic Belgrave Hospital (KCH)
1981 prenatal diagnosis KCH
1982 selective neonatal screening (cord blood)
1988 universal neonatal screening Camberwell
6 paediatric clinic STH
1994 3 borough specialist nurse counsellor
team
+ universal neonatal screening
2000 universal ante-natal screening (LSL)

2005 universal neo-natal screening England

Sickle cell disease
 chronic condition
acute exacerbations
unpredictable
severity of pain can be unbearable
occasionally life threatening
extremely variable
in UK many children of 1st generation
immigrant families
Essential steps
main aim to ensure child grows into
self managing and coping adult
Essential steps
Identify child
 antenatal screening &
counselling
 neonatal screening programme
 registration in paediatric clinic
Katy’s story Screening but
Born 1992 no antenatal
counselling

3rd child
2 elder
brothers
Aim of antenatal
screening
• To offer timely antenatal sickle cell and
thalassaemia screening to all women (and
couples) to facilitate informed decision
making
• The offer includes the offer of, uptake of,
reporting of prenatal diagnosis and any
subsequent action by the end of 12 weeks
of pregnancy
South East
London
Sickle Cell
and
Thalassaem
ia Centre
Aim of neonatal screening
programme
• To achieve the lowest possible childhood
death rate and to minimise childhood
morbidity from sickle cell disorders
Objectives:
• Process tests in a timely manner
• Identify and arrange timely follow up of
infants identified as needing further
investigation
• Offer treatment and start parental
education in a timely manner
Criteria for screening
programmes
• The condition should be an important
health problem
• The natural history of the condition should
be understood
• There should be a simple, safe, precise
and validated screening test
• There should be an effective treatment
and evidence of early treatment leading to
better outcomes
Katy’s story Screening but
Born 1992 no antenatal
counselling

Neonatal
screening

3rd child
2 elder
brothers
Early treatment
• Penicillin prophylaxis

• Conjugate pneumococcal immunisation

• Education eg palpation of spleen,

recognition of other signs

• Offer of regular blood transfusions in those

at risk of stroke ( transcranial doppler
screening)
Pneumococcal
prevention
Good evidence
Adherence
Practical considerations eg prescribing
Education & support
Evidence that added value in reducing
morbidity & mortality
Most evidence based on provision of care
in clinic
setting
Blood transfusion for
children at risk of stroke
Good evidence for transcranial doppler
scans in HbSS to identify those most at risk
Good evidence that blood transfusion can
prevent the majority of strokes and can
prevent recurrence
•Newborn screening programme
•Working group
•Executive summary
•Detailed guidance

www.sickleandthal.org.uk
Standards (1)
 Penicillin prophylaxis
ii) 90% of infants should have been
prescribed Penicillin V (or alternative) by
3 months. 99% of infants should have
been offered and prescribed Penicillin V
or alternative) by 6 months
iii) Any parental refusal should be
documented
Standards (2)
 Pneumococcal immunisation
ii) 95% of infants should have completed
the primary prevenar ( conjugated
pneumococcal vaccine) course by 15
months
iii) 95% should be given Pneumovax
(polysaccharide antigen) at 2 years of
age (24-27 months) and 5 yearly
thereafter
Standards (3)
Transcranial Doppler scanning (TCD)
 90% of sickle cell centres should have
the capability of offering annual TCDs to
children with SCD from the age of 3 years
by 2008 and 99% should have this
capability by 2010
Katy’s story Screening but
Born 1992 no antenatal
counselling

Neonatal
screening

3rd child Prophylaxis

2 elder Regular follow u
brothers
No TCD
Katy
aged 5 (1997) had TIA & then acute stroke
right hemiplegia
MRI cerebral infarct left hemisphere
TCD showed raised velocities left MCA
On transfusion programme
mild learning difficulties

Adams RJ, McKie VC, Hsu L, et al. Prevention of a

first stroke by transfusions in children with sickle cell
anemia and abnormal results on transcranial Doppler
ultrasonography. N Engl J Med. 1998;339:5-11.
Aspects of outpatient
management
• Primary and secondary prevention
• Recognition of acute/chronic problems
• Screening for complications
• Specific treatments eg Hydroxyurea, BMT
Other concerns
immigration
housing
schooling
future pregnancies
admissions to hospital/time off work
travel

+ all other complications

Essential steps
Follow up

minimum standards for out patients

education & support
guidelines for acute complications
comprehensive but flexible
meets individual and family’s needs
procedure for those lost to follow up
transition to adult service
Minimum standards
 frequency of
attendance
frequency & nature of
investigations
continuity of
professionals
advice & education
how to manage at
home
when to come to
hospital
Psychological support*
 painful, chronic and life threatening
condition
nocturnal enuresis common
good evidence for cognitive behavioural
therapy
necessary for assessing children who are
at risk or who have had stroke

*very few units have clinical or health

psychology
Acute management
 majority of care is ambulatory
if get this right, inpatient admissions will be
very few
need to have local protocols
detailed guidance is available
pathways for transfer eg to PICU
care plans for frequent attenders + those
approaching transition

www.sickleandthal.org.uk
Standards (4)
Failsafe arrangements
ii)the sickle cell centres in conjunction with
local paediatric units should have
continuing responsibility for children with
SCD identified on the newborn screening
programme and should maintain a list
iii)by 2008 95% ( 99% by 2010) should have
robust FU arrangements and the capability
to track children
Organisation of service
Primary care
Community clinics
Hospital clinics
Paediatric A/E
Paediatric wards /day units for transfusion
Sickle cell centres – inreach/outreach
Follow up for DNA

Good liaison between primary/community/acute –

general & specialist
Care pathways
Multidisciplinary team
Sickle cell centres
• Hospitals with large population of sickle
cell children (but may not have the whole
range of services: MRI, TCD, PICU)

• Hospitals in areas with few local patients

which act as a centre of expertise and can
provide MRI, HDU/PICU and ?TCD

Informal arrangements already exist and

proposal is to build on these but ensure
coverage across England
Role of local unit Role of sickle
Named paediatrician centre
Named paediatrician or
haematologist
Routine OP Designated clinic
Annual review/TCD
Hydroxyurea/BMT
Acute management of Management of
painful episode/anaemia ACS/stroke
Provision HDU/PICU
Follow up Register/audit/research/
training
Final thoughts
 the sort of paediatric service to be set up will
depend on numbers of children , expertise of
staff and available resource and geography
paediatricians (acute & community) are key
to its success and training needs for junior
staff recognised
specialist nurses and psychologists are
essential
sickle cell centres still need to be developed
availability of TCD not yet universal