SICKLE CELL DISEASE IN B & D DR I R GRANT 15 FEB 2012
� 250,000 children born every year with SCD � About 0.15% African Americans are homozygous for SCD � 8% have sickle cell trait � 12,000 with SCD in UK. Most in London � About 600+ patients with SCD in BHR
� A structural globin chain variant � HbSS, HbS-β 0 Thal, HbS-β + Thal, HbSC � Median age at death is 42yrs for men and 48yrs for women. � Pulmonary complications commonest cause of death accounting for 28% of all deaths.
� The codon determining the amino acid at position β6 has changed from GAG coding for glutamic acid to GTG coding for valine.
HbS Structure HbS Structure
� Vaso-occlusive Crisis � Infection including aplastic crisis � Splenic sequestration � Acute chest syndrome � Neurological complications: TIA, CVA and silent infarcts � Delayed growth and puberty � Enuresis (bedwetting) � Priapism (painful erections) � Death
� Vaso-occlusion � Splinting of ribs � Hypoventilation due to pain and analgesia � Plate atelectasis of lung � Pulmonary infarction 2 0 to thrombosis � Fat embolism � Infection � Presenting with chest pain, dyspnoea and falling oxygen saturations
� Acute splenic sequestration � Rapid enlargement of spleen, fall in haemoglobin � Commonest in second six months of life � Majority of episodes before 2 years of age � Treated with top-up transfusion � Recurrent in ~50% � Chronic splenomegaly � 10% of patients with HbSS � May cause hyposplenism leading to poor growth
� Low, falling Hb with reticulocytopenia � May be overt accompanying viral infection � Erythema infectiosum, arthropathies, abdominal pain � Parvovirus B19 (IgM usually positive) � Treatment with blood transfusion support � Warn siblings, pregnant women
� Sickle cell disease commonest cause of childhood stroke � Increased risk by 280x � Presentation diverse - hemiplegia, limp, reduced consciousness, fits � IQ reduction of 4 points � Cognitive/learning difficulties � Significant morbidity + mortality
� Haemoglobinopathies – life-long � Significant morbidity and mortality � On the increase- are current services adequate to meet demands of patient load � Significant community/psychological,social and Significant community/psychological,social and welfare support � Management � Prevention � Surveillance � Treatment of complications
Services in BHRUT
BACKGROUND � Increasing number of patients living with haemoglobinopathy resident in Barking and Dagenham. This reflects demographic increases in high prevalence population groups. � 2009-10 acute trust register identified 400 children and adults in B&D. � 2012 Adults 353 B and D 156: Paed 292 B and D 170 � (2008-09 PbR non-elective activity with primary diagnosis ‘sickle cell admission’ was £255,000 for 210 admissions = £1214 per admission.) � Current gap in community service pathway for care of haemoglobinopathies.
HOSPITAL SERVICE � BHRUT have 3 haematologists with special interest in SCD � Paediatric and adult OP and IP service able to deliver all aspects of care and treatment for deliver all aspects of care and treatment for patients affected with SCD � Good positive feedback on inpatient provision � Universal Antenatal screening (introduced 2003) performed and counselling given to all couples who could have a child with SCD
COMMUNITY SERVICE � What has been lacking is a community service which will prevent admissions and improve holistic care to patients with Sickle Cell Disease � ONEL have committed to this from 1 st April 2012 � ONEL have committed to this from 1 April 2012 � Provides seamless care between community and hospital � Helps provide social, psychological, counselling and medical care in the community and prevents hospital admissions
HAEMOGLOBINOPATHY SERVICES BHRUT � 2003 DH review showed BHRUT to be significantly under resourced compared to rest of London � Recommendation of DH in 2003 we needed Recommendation of DH in 2003 we needed 2 haemoglobinopathy counsellors � Concerns that BHRUT would not be able to support the introduction of neonatal and prenatal screening for haemoglobinopathy
� DH supplied monies to support 1 haemoglobinopathy nurse specialist plus admin support � This was on the basis of about 60 patients � Now have > 600 � DH wrote letter of commendation to haem dept in 2004 congratulating them on the professional way they had introduced the screening programmes � We have the medical infrastructure but community infrastructure still lacking.
� Huge amounts of work done on medical side with much appreciation from patients. Protocols, databases in place � Peer review imminent which we will fail and Peer review imminent which we will fail and be seriously criticised based on the nurse, counsellor and pyschology support if we do not get a proper community service.
BHR HAEMOGLOBINOPATHY NUMBERS MAY 2012 � Adult database - 353 � B&D 156 � Redbridge 55 � Havering 62 � Other Newham/ Waltham Forest 50
BHR HAEMOGLOBINOPATHY NUMBERS MAY 2012 � Paediatric database - 292 � B&D -170 � Redbridge 59 � Havering 36 � Other Newham/ Waltham Forrest/ South West/ East Essex -14
PREDICTED GROWTH RATES � 3% for UK � BHR - New baby numbers alone - 10% increase 08 to 09 (plus paediatric and adult immigration) immigration)
NEW BIRTH RATES � B&D 1 in 297 babies born are affected by a significant haemoglobinopathy � Redbridge - 1 in 1454 � Havering - 1 in 7601 � 24 new babies seen at BHR in 2009 (B&D 16/ Redbridge 6/ Havering 2)
CARRIER RATES � B&D 1in 14 - the highest carrier rate of any PCT in the country!!!! � Redbridge - 1 in 32 � Havering - 1 in 50
HAEMOGLOBINOPATHY TEAM CURRENTLY � Dr Claire Hemmaway (Haemoglobinopathy lead) 2.25 clinics per week � Dr Grant (1 clinic a week) � Christine Williams (0.3WTE) � Anne Marie - clerical support 1WTE � Jane Mattison (0.6 WTE) � Pam Jones (2 days a week - welfare benefits advice) � An enormous amount of goodwill from both paediatric and adult cancer nurse specialists
NEWHAM SERVICE (COMMUNITY SERVICE) NOT A SPECIALIST CENTRE � EQUIVALENT NUMBERS � Own Community Building � Service manager Band 8a � Specialist nurse counsellor � Community health visitor � Adult Haemoglobinopathy nurse specialist (Band 7) � Adult Haemoglobinopathy community nurse (Band 6) � Paediatric Haemoglobinopathy nurse (Band 6) � Psychologist (Band 7) � Social worker (Band 7) � Family liason officer � 2 clerical staff �
INDIVIDUALS LIVING WITH HAEMOGLOBINOPATHY � Individuals living with a haemoglobinopathy require multi-disciplinary support in acute and maintenance periods. � They should have as a baseline an annual � They should have as a baseline an annual review and have regular blood tests, medication reviews, immunisation and psychological assessment and support. � At the point of diagnosis they, and their families, may need additional support and counselling.
Key Documents � Sickle cell disease in childhood – standards and guidelines for clinical care (Oct 2010) � Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK (2008) � National Service Framework for Children and Young People. (2004) � Healthy Children Programme. � A Sickle Crisis? (2008)
Local network of care including community care, local hospital (LH) and � specialist centre (SHT) with links to paediatric intensive care. Annual review of all children by SHT with first appointment by 3months of age, � including screening, education and prevention review. Annual trans-cranial doppler scan (TCD) on all children from 2yrs old. � Local voluntary sector support. � Named paediatrician and/or paediatric haematologist at LH and SHT. � Good communication with general practice and community nursing teams. � Expert patient program. � Coordination of community needs through community paediatric services. � Local authority aware and supporting specific needs. � Transition policy and specific transition review in place at 15-16yrs of age. � Specific adolescent clinic for formal review and handover. �
� Rapid assessment at A&E with first dose of analgesia with 30mins and pain control within 2hrs. Specialist support to manage complications. complications. � Regular outpatient follow-up with screening. � Education and training. � Clinical leadership with clinical and nursing experts and patient and public involvement.
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