North West London Haemoglobinopathy Managed
Clinical Network
Lola OniProfessional Services Director/ LecturerBrent Sickle Cell & Thalassaemia Centre
Making a difference: service models and care pathways for babies with disease states
Sickle Cell Disorders In Sickle Cell Disorders In ChildrenChildren
Complications rarely occur before 3 months of age. Infrequent before 6 months of age.
1/3 of children develop symptoms by 1st birthday.2/3 of children develop symptoms by 2nd birthday.
10% risk of stroke in children with HbSS.
NHS PLAN (ENGLAND)NHS PLAN (ENGLAND)
“…effective and appropriate screening programmes for women and children including a nationally linked ante-naral and neonatal screening programme for haemoglobinopathy and sickle cell disease”
(DoH 2000:109)
Newborn Screening for sickle cell disease in England
Aim of newborn screening for
sickle cell disease:
To implement health care early in order to reduce handicap, mortality and morbidity
associated with sickle cell disease
NW London Network - PCTs
Brent Ealing Kensington & Chelsea Hammersmith & Fulham Harrow Hillingdon Hounslow Westminster
Nursing Model 1
Brent PCT All local HVs give normal results BSCTC provide carrier results BSCTC re-tests all unknown bands and
babies with a suspected disease state Follow up all children and adults with a
disease state in acute and community
Nursing Model 2
Hounslow, H & F, Ealing PCT
This will be covered by:
- Emma Quarshie, Hounslow PCT
- Cherril Ward, H & F PCT
- Brigid Offley-Shaw, Ealing PCT
Nursing Model 3
Harrow, K & C PCT
All local HVs give normal results Designated local HVs give carrier results BSCTC re-tests all unknown bands and
babies with a suspected disease state Follow up all children via local paediatric
clinic
Nursing Model 4
Westminster PCT
All local HVs give normal and carrier results BSCTC re-tests all unknown bands and
babies with a suspected disease state Follow up all children via local paediatric
clinic
Penicillin prophylaxis
Randomised controlled trial (Gaston et al 1986) Penicillin V reduces the mortality from
pneumococcal sepsis in children with SCD Incidence of pneumococcal infection reduces
after 5 years Therefore concentrate on early compliance
(give appropriate dose twice a day) Lifelong treatment is recommended
UK Forum Sickle Cell Paediatric Care Guidelines Summary 2005
4 key audit standards Penicillin prophylaxis (90% by 3/12, 99% by 6/12)
Pneumococcal immunisation (95% at 2 yrs)
Transcranial Doppler scanning annually from 3 years of age (90% by 2008, 99% by 2010)
Failsafe arrangements for DNA (95% by 2008, 99% by 2010)
Management of SCDManagement of SCD
Prophylactic Penicillin
Folic Acid
Immunisation against pneumococcal infections (Prevenar with primary immunisations at 2; 3; 4 months. Pneumovax at 2 years)
Parental Education
Symptomatic Treatment
Care Pathway - Summary
Newborn identification Parent notification, education, provision of
parent handbook community support, Commencement of prophylactic penicillin Referral for paediatric care
- Transcranial Doppler annually- Follow up of DNA
Pneumovax
UK THALASSAEMIA SOCIETY2005
Standard 1: Effective management of iron load - family education, monitoring
and treatment of complications Standard 2: Psycho-social management and
support strategy Standard 3: Effective management of an acute
illness on presentation to primary or secondary care