North West London Haemoglobinopathy Managed Clinical Network Lola Oni Professional Services Director/ Lecturer Brent Sickle Cell & Thalassaemia Centre Making a difference: service models and care pathways for babies with disease states

Perceptions of Risk Guardian Newspaper May 1998

Sickle Cell Disorders In Children 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) “…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

Blood spot at 5 – 8 days of age

NW London Network - PCTs Brent Ealing Kensington & Chelsea Hammersmith & Fulham Harrow Hillingdon Hounslow Westminster

Outer London PCTs  Bedfordshire  North & East Hertfordshire

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 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)

Transcranial Doppler measurement of cerebral blood flow Fast flow predicts brain damage

Management 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 SOCIETY 2005 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

Acknowledgements Graphics: Professor Sally C Davies, consultant haematologist Cynthia Gill, independent practitioner