Stirling, June 2009

Newborn Sickle Screening Programme – Clinical Pathways in Scotland.

EA Chalmers

Royal Hospital for Sick Children, Glasgow.

Stirling, June 2009

Newborn Sickle Screening Programme.

• Objectives:– To achieve lowest possible childhood death rate

• Early infant mortality• Mortality in children <5yrs: <4/1,000 person yrs of life

– To minimise childhood morbidity from sickle cell disorders

– Accurate diagnosis of infants with HbSS, HbSC, HbSD & HbS/beta thalassaemia

– Offer treatment and start parental education in a timely manner

Stirling, June 2009

Newborn Sickle Screening Programme - Clinical Care Pathways

• Identification of cases– Newborn blood spot screening

programme

• Referral of “affected” cases– Named regional lead clinicians– Confirmation of diagnosis

• Timing

• Repeat testing

• Communication to parents

• Provision of clinical services

Stirling, June 2009

Sickle cell disease – Clinical Services

• Sickle Cell disease in Childhood – Standards & Guidelines for Clinical Care (DOH/Sickle Cell Society)

• Delivery of healthcare:– In community/at home– Acute care/local hospitals– Specialist care

• Paediatric haematology• PICU/HDU• Transcranial doppler• Transfusion & chelation/MRI monitoring• Hydroxycarbamide

Stirling, June 2009

Sickle Cell Disease – Paediatric Clinical Services in Scotland

– Patient numbers• Existing cases – Approx. 50 children• New cases – Approx. 5 per annum

– Geography• Glasgow, Edinburgh, Aberdeen,

Dundee

– Staffing• Medical, nursing, other

– Clinical services• Acute/local• Specialist

Stirling, June 2009

Newborn Sickle Screening – Results of Mapping Exercise

• 4/13 boards reported experience of neonatal testing/diagnosis– Confirmation of diagnosis

• <3 months

– Initiation of treatment• <3 months

– Follow up arrangements• Paediatric haematology

Stirling, June 2009

Sickle Cell Disease – Paediatric Clinical Services in Scotland

• Sickle Cell disease in Childhood – Standards & Guidelines for Clinical Care– Equitable comprehensive care programme

• National standards for TCD scanning– Training support, audit

• Models for care in Scotland– Managed clinical network– National paediatric specialist service

• Clinical network arrangements– Need for “tertiary” support/advice– Transition & adult services