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A Paediatric Sickle Cell ServicEssential Components
Dr Moira DickConsultant Community PaediatricianKings College HospitalLambeth PCT
1981-7 Belgrave Childrens Hospital/KCH1990- 2003 STH
1994- date KCH
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Sickle cell diseasechronic condition
acute exacerbations
unpredictableseverity of pain can be unbearable
occasionally life threatening
extremely variablein UK many children of 1st generation
immigrant families
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Essential steps main aim to ensure child grows into
self managing and coping adult
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Essential stepsIdentify child
antenatal screening &counselling neonatal screening programme registration in paediatric clinic
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Katys storyBorn 1992
3rd child2 elderbrothers
Screening buno antenatal
counselling
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Aim of antenatal
screeningTo offer timely antenatal sickle cell and
thalassaemia screening to all women (and
couples) to facilitate informed decisionmaking
The offer includes the offer of, uptake of,reporting of prenatal diagnosis and any
subsequent action by the end of 12 weeksof pregnancy
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South EastLondonSickle CellandThalassaemia Centre
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Aim of neonatal screening
programmeTo achieve the lowest possible childhood
death rate and to minimise childhoodmorbidity from sickle cell disorders
Objectives:
Process tests in a timely manner
Identify and arrange timely follow up of
infants identified as needing furtherinvestigation
Offer treatment and start parentaleducation in a timely manner
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Criteria for screening
programmesThe condition should be an important
health problem
The natural history of the condition shouldbe understood
There should be a simple, safe, preciseand validated screening test
There should be an effective treatmentand evidence of early treatment leading tobetter outcomes
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Katys storyBorn 1992
3rd child2 elderbrothers
Screening butno antenatal
counselling
Neonatal
screening
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Early treatment Penicillin prophylaxis
Conjugate pneumococcal immunisation
Education eg palpation of spleen,recognition of other signs
Offer of regular blood transfusions in thoseat risk of stroke ( transcranial dopplerscreening)
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Pneumococcal
preventionGood evidence
Adherence
Practical considerations eg prescribing
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Education & supportEvidence that added value in reducing
morbidity & mortality
Most evidence based on provision of carein clinic
setting
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Blood transfusion for
children at risk of strokeGood evidence for transcranial doppler
scans in HbSS to identify those most at risk
Good evidence that blood transfusion canprevent the majority of strokes and canprevent recurrence
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Newborn screening programmeWorking group
Executive summaryDetailed guidance
www.sickleandthal.org.uk
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Standards (1) Penicillin prophylaxis
ii) 90% of infants should have been
prescribed Penicillin V (or alternative) by3 months. 99% of infants should havebeen offered and prescribed Penicillin Vor alternative) by 6 months
iii) Any parental refusal should bedocumented
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Standards (2) Pneumococcal immunisation
ii) 95% of infants should have completed
the primary prevenar ( conjugatedpneumococcal vaccine) course by 15months
iii) 95% should be given Pneumovax
(polysaccharide antigen) at 2 years ofage (24-27 months) and 5 yearlythereafter
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Standards (3)Transcranial Doppler scanning (TCD)
90% of sickle cell centres should have
the capability of offering annual TCDs tochildren with SCD from the age of 3 yearsby 2008 and 99% should have thiscapability by 2010
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Katys storyBorn 1992
3rd child2 elder
brothers
Screening butno antenatal
counselling
Neonatal
screening
ProphylaxisRegular follow
No TCD
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Katyaged 5 (1997) had TIA & then acute strokeright hemiplegiaMRI cerebral infarct left hemisphere
TCD showed raised velocities left MCAOn transfusion programmemild 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.
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Aspects of outpatient
management Primary and secondary prevention
Recognition of acute/chronic problems
Screening for complications Specific treatments eg Hydroxyurea, BMT
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Other concernsimmigration
housing
schooling
future pregnancies
admissions to hospital/time off work
travel
+ all other complications
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Essential stepsFollow up
minimum standards for out patientseducation & support
guidelines for acute complications
comprehensive but flexible
meets individual and familys needs
procedure for those lost to follow up
transition to adult service
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Psychological support*painful, chronic and life threatening
condition
nocturnal enuresis commongood evidence for cognitive behavioural
therapy
necessary for assessing children who areat risk or who have had stroke
*very few units have clinical or health
psychology
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Acute managementmajority of care is ambulatoryif get this right, inpatient admissions will be
very few
need to have local protocolsdetailed guidance is available
pathways for transfer eg to PICU
care plans for frequent attenders + thoseapproaching transition
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Standards (4)Failsafe arrangements
ii)the sickle cell centres in conjunction with
local paediatric units should havecontinuing responsibility for children withSCD identified on the newborn screeningprogramme and should maintain a list
iii)by 2008 95% ( 99% by 2010) should haverobust FU arrangements and the capabilityto track children
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Organisation of servicePrimary careCommunity clinicsHospital clinics
Paediatric A/EPaediatric wards /day units for transfusionSickle cell centres inreach/outreachFollow up for DNA
Good liaison between primary/community/acute general & specialistCare pathwaysMultidisciplinary team
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Role of local unit Role of sickle
centreNamed paediatrician Named paediatrician orhaematologist
Routine OP Designated clinic
Annual review/TCDHydroxyurea/BMT
Acute management of
painful episode/anaemia
Management of
ACS/strokeProvision HDU/PICU
Follow up Register/audit/research/
training
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Final thoughts the sort of paediatric service to be set up will
depend on numbers of children , expertise ofstaff and available resource and geography
paediatricians (acute & community) are keyto its success and training needs for juniorstaff recognised
specialist nurses and psychologists areessential
sickle cell centres still need to be developed
availability of TCD not yet universal