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1. 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
3. Perceptions of Risk Brothers
The Black man has normal haemoglobin AA
The other brother carries the Hb S gene.Brothers
The Black man has normal haemoglobin AA
The other brother carries the Hb S gene.
4. Sickle Cell Disorders In Children
5. 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)
6. 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
8. Blood spot at 5 8 days of age
9. NW London Network - PCTs Brent
Ealing
Kensington & Chelsea
Hammersmith & Fulham
Harrow
Hillingdon
Hounslow
Westminster
10. Outer London PCTs
Bedfordshire
North & East Hertfordshire
11. 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
12. 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
13. 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
14. 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
15. 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
16. 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)
18. 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
19. 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
20. 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
21. Acknowledgements
Graphics:
Professor Sally C Davies, consultant haematologist
Cynthia Gill, independent practitioner