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

Perceptions of RiskPerceptions of Risk

Guardian Newspaper May 1998

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

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 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)

Transcranial Doppler measurement of cerebral blood flow

Fast flow predicts brain damage

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

Acknowledgements

Graphics:

Professor Sally C Davies, consultant haematologist

Cynthia Gill, independent practitioner