Www.hbregistry.org.uk 1 © Copyright Haemoglobinopathy Registry - Central Middlesex Hospital 2003-2004 Haemoglobinopathy Registry in the Clinical Management

London (North West)

Haemoglobinopathy Registry

1© Copyright Haemoglobinopathy Registry - Central Middlesex Hospital 2003-2004

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Haemoglobinopathy Registry in the Haemoglobinopathy Registry in the Clinical Management of Patients:Clinical Management of Patients:

Progress & UpdateProgress & Update

77thth July 2004 July 2004

Presented By:

Dr Mark LaytonAnnette Gilmore


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Haemoglobinopathy RegistryHaemoglobinopathy RegistryProject TeamProject Team

Hb Registry Board Dr M Layton Dr N Philpott Dr G Hughes Jean Griffiths Dr M Soljak Mr S James Dr M Alli Mr K Martin Annette Gilmore Priscilla Plocki

EHR Team: Prof. Sally C Davies Dr J Howard Annette Gilmore Sameer J Patankar


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North West London ParticipantsNorth West London Participants

Central Middlesex and Northwick Park: Prof. S C Davies Dr J Howard Dr S Allard

Ealing: Dr C Michie Dr N Philpott Dr G Abrahamson

Hammersmith: Prof. I Dokal Dr M Layton

West Middlesex: Dr G Hughes Dr M Sekhar Dr A Davies

Data Co-ordinators: Annette Gilmore Priscilla Plocki


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EU Participants:EU Participants:Hydroxyurea RegistryHydroxyurea Registry

Yves Beuzard Paris Christina Borgna-Pignatti Ferrara Maria Domenica Cappellini Milan Sally C. Davies London Lucia de Franceschi Verona Pierre Fondu Brussels Frederic Galacteros Creteil Renzo Galanello Cagliari Robert Girot Paris Dimitri Loukopoulos Athens Marianne de Montalambert Paris Eliazer Rachmilewitz Jerusalem Corrina McMahon Dublin

Co-ordinated by: Dr M Roberts-Harewood


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AimsAims

Local Accurate information for clinical practice, clinical

governance & audit, and planning services Collaboration and information sharing Impact on future research

General Study the natural history of the Haemoglobinopathies Evaluation of interventions - Hydroxyurea (HU) in SCD

patients


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EHR Research Ethics ProcessEHR Research Ethics Process

Data Protection Act Registration[Registration No. Z5730583]

MREC [Approval - MREC/99/2/4] LREC [Approval - BEC 612] Patient Information and Consent Forms Secure Access to Registry (PATS):

Password Access Encrypted Data Communications


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EHR Process (1)EHR Process (1)

Standardise Data Collection: Proformas (MDS & EDS) – Adobe Acrobat (PDF) Data Dictionary and Guidelines for Completion Proformas, Consent Forms, Guidelines, etc. –

External Secure Website Submitted to Registry by Post or Fax


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EHR Process (2)EHR Process (2)

Data Processing and Storage: Automated Data Validation & Processing Automated Data Log for Validation by Data

Collector (‘To Be Approved’ Report) After Final Validation (‘Final’ Report) – Data

Entered in to Patient Analysis & Tracking System (PATS Database) – Dendrite Clinical Systems

Data Outputs: Automated Reporting:

Clinical Reports (Microsoft Word) Data and Reports to Clinicians (Microsoft Excel)

(Data and Reports Returned on CD)


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Achievements of the RegistryAchievements of the Registry

Demographic Information for Monitoring & Service Provision

Individualized Clinical Patient Reports Monitoring and Evaluation of Hydroxyurea

Treatment Developed in Collaboration with EU and UK

Doctors Haemoglobinopathy Registry Website

www.hbregistry.org.uk

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© Copyright Haemoglobinopathy Registry - Central Middlesex Hospital 2003-2004

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European Haemoglobinopathy Registry (EHR) European Haemoglobinopathy Registry (EHR) Progress Report for CMH, Ealing, Hammersmith Progress Report for CMH, Ealing, Hammersmith

and West Middlesex Hospitals 31/03/2004and West Middlesex Hospitals 31/03/2004

Haemoglobinopathy Registry

0

100

200

300

400

500

CMH Ealing Hammersmith West Middlx

No

. of

Pat

ien

ts

No. of Active Patients Identified per HospitalNo. of Patients Consented to Hb RegistryNo. of Patients Data Collected

Note: Active patient ≡ Seen in hospital in last 36 months as IP or OPD.

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Central Middlesex Hospital Central Middlesex Hospital HaemoglobinopathyHaemoglobinopathy

Patients (538) by PCT of Residence Patients (538) by PCT of Residence

Har r ow 18%

Br ent 49%

UK 6%

Over seas 1%

Other London 11%

Westminster 1%

Kensington & Chelsea 0%

Hammer smith & Fulham

3%

Hil l ingdon 3%

Hounslow 1%

Eal ing 7%

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Ealing HospitalEaling HospitalHaemoglobinopathyHaemoglobinopathy


Eal ing 90%

Hounslow 5%


0%

Har r ow 0%

Br ent 1%

Hi l l ingdon 0%


Westminster 0%

Other London 1%

Over seas 0% UK 3%

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Hammersmith Hospital Hammersmith Hospital HaemoglobinopathyHaemoglobinopathy

Patients (173) by PCT of ResidencePatients (173) by PCT of Residence

Hi l l ingdon 3%

Har r ow 2%

Br ent 13%Eal ing 21%


27%

Hounslow 5%


Westminster 2%

Other London 12%

Over seas 2%

UK 9%

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West Middlesex Hospital West Middlesex Hospital HaemoglobinopathyHaemoglobinopathy


Hounslow 87%


Westminster 0%Other London 0%

Over seas 0%

UK 0%


5%

Eal ing 8%Hi l l ingdon 0%

Br ent 0%

Har r ow 0%

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CMH, Ealing, Hammersmith, West CMH, Ealing, Hammersmith, West Middx. Hospitals HaemoglobinopathyMiddx. Hospitals HaemoglobinopathyPatients (828) by PCT of Residence Patients (828) by PCT of Residence

Hounslow 7%

Har r ow 12%

Br ent 36%

Hil l ingdon 2%

Eal ing 17%


8%

UK 6%Over seas 1%

Other London 9%

Westminster 1%


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CMH, Ealing, Hammersmith, West CMH, Ealing, Hammersmith, West Middx. Hospitals Haemoglobinopathy Middx. Hospitals Haemoglobinopathy Patients (828) by PCT of ResidencePatients (828) by PCT of Residence

0

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150

200

250

300

No

. of

Pat

ien

ts

Patient's PCT of Residence

West Middlesex Hospital

Hammersmith Hospital

Ealing Hospital

Central Middlesex Hospital

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CMH, Ealing, Hammersmith,CMH, Ealing, Hammersmith,West Middx. HospitalsWest Middx. Hospitals

828 Patients by Age Group828 Patients by Age Group

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100

150

200

250

300

350

Central MiddlesexHospital

HammersmithHospital

Ealing Hospital West MiddlesexHospital

0-13 Years 14-20 Years > 20 Years

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828 Patients by Age Group 828 Patients by Age Group

22727%

12014%

48159%

0-13 Years 14-20 Years > 20 Years

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828 Patients by Hb Type 828 Patients by Hb Type

SS 53%

SC 21%

SBThal 10%

Other 6%Missing

Diagnosis 3%

Thal Syndromes 7%

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Hb SS Patients 16 Years and Older Hb SS Patients 16 Years and Older Currently on HydroxyureaCurrently on Hydroxyurea

Adult HbSS Patients on HU

0

20

4060

80

100

120140

160

180

Nu

mb

er o

f P

atie

nts

On Hydroxyurea

Adult HbSS Patients

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20

40

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120

140

160

180

Ealing Hammersmith West Middlesex Central Middlesex

Num

ber

of P

atie

nts

On Hydroxyurea

Adult HbSS Patients

Adult HbSS Patients On HydroxyureaEaling 12 2Hammersmith 67 10West Middlesex 5 0Central Middlesex 142 22

Adult HbSS Patients on HU

020406080

100120140160180

Nu

mb

er o

f P

atie

nts

On Hydroxyurea

Adult HbSS Patients

0

20

40

60

80

100

120

140

160

180

Ealing Hammersmith West Middlesex Central Middlesex

On Hydroxyurea

Adult HbSSPatients

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IndividualizedIndividualizedPatientPatientReportingReporting

Baseline Data Complete EHR

Initial Registration Proforma

Annual Follow-up Storage in PATS Clinical Reporting

in Word & Excel

Adult Patients with Sickle Cell DiseaseFirst Consultation Proforma

NHS No

Hosp No

Patient's postcode

Date of Birth / /Sex M F

8. First SCD presentation

Doctor's SignatureNurse's Signature

3. Date of consultation / /

9. Ethnic origin British Irish Any other white background

White/Black Caribbean White/Black African White/Asian Any other mixed background

Indian Pakistani Bangladeshi Any other Asian background within (c)

Caribbean African Any other black background within (d)

Chinese Any other ethnic group

18. Alpha Thal trait No Yes Not Known

19. No. of functional alpha genes

23. Specific antibodies

20. G6PD deficiency No Yes Not Known

21. RBC allo antibodies No Yes Not Known

22. RBC phenotyped No Yes Not Known

24. Diagnosis made by:1. Antenatal screening

2. Neonatal screening

3. Postnatal

4. Family screening

5. Symptomatic presentation

6. Routine/Other Assessment

7. Not known

wks gestation

25. Was PND offered? No Yes

27. Was PND accepted? No Yes

28. PNDResult

Months

29. Age at diagnosis

59. No. of dactylitis events58. Dactylitis No Yes Not Known

months60. Age 1st dactylitis event

Last name

First name

Consultant (surname)

MUST

16. Hb type SS SC SBTh+ SBTho S+HPFH Other 17. State

50669

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Monitoring ofMonitoring ofHydroxyureaHydroxyureaTreatmentTreatment

Baseline Data: Complete EHR

Initial Registration Proforma

Follow-up Data: Complete at each

patient OPD visit Data Stored in PATS Clinical Reporting in

Word & Excel

Keep copy in Hospital Notes with record of comments and full physical examination (if done)

Write in black ink, using clear capitals. Items in red MUST be completed. Circles must be completely filled in.

Blood Transfusion Exchange Venesection

Hosp. No.

Diagnosis Pain Other

Hospital CMH Other

Weight .HU Daily Dose mgs

WCC . 10^9/l

Hb . g/dl

MCV . fl

MCH . pg

g/dlMCHC .RDW .

10^9/lPlatelets

10^9/lNeuts .NRBC /100

%Retics .

Albumin g/L

mmol/LNa

mmol/LK .

mmol/LUrea .mol/LCreatinine

IU/LAST

IU/LALT

IU/LgGT

mol/LTotal Bilirubin

Total protein g/L

IU/LAlk. Phosphate

IU/LLDH

ng/LRed Cell folateng/LSerum B12

Ferritin ng/ml

mmol/lTIBCmmol/lSerum Fem/mHZPP

secsPTTK

PT . secs

Pain in community No Yes If Yes, describe

Date seen / /

HU Toxicity No Yes If Yes, describe

Other problems No Yes If Yes, describe

Examine No Yes Abnormalities

IP No YesState other hospital

State other diagnosis

HU dose Unchanged Increased Decreased Restarted Stopped

If Stopped give date / /

Episode No.

Signature of Doctor/Nurse

Next Appt

Were HU doses missed No Yes Unknown

If Yes, no. of IP days

No. units in No. mls outDate of LMP / /

RBC .

%Hb F .%Hb S .%Hb A2 .%Hb A .%Hb Other .

If Yes, no. of missed doses

Entered into PATS: .... / .... / .... Initials: .......

Items in boldare essential

30815

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MCV, Hb F, Neutrophils and Hb & HU Dose

1 1 Toxic Episodes

2 15 7 23 4 4 8 8 IP Days

0.0

20.0

40.0

60.0

80.0

100.0

120.0

140.0

22/0

8/199

5

+2 M

onth

s

+4 M

onth

s

+6 M

onth

s

+8 M

onth

s

+10

Mon

ths

+12

Mon

ths

+14

Mon

ths

+16

Mon

ths

+18

Mon

ths

+20

Mon

ths

+22

Mon

ths

+24

Mon

ths

+26

Mon

ths

+28

Mon

ths

+30

Mon

ths

+32

Mon

ths

+34

Mon

ths

+36

Mon

ths

+38

Mon

ths

+40

Mon

ths

+42

Mon

ths

+44

Mon

ths

+46

Mon

ths

+48

Mon

ths

+50

Mon

ths

+52

Mon

ths

+54

Mon

ths

+2 M

onth

s

+4 M

onth

s

+6 M

onth

s

+8 M

onth

s

+10

Mon

ths

+12

Mon

ths

+14

Mon

ths

+16

Mon

ths

+18

Mon

ths

+20

Mon

ths

+22

Mon

ths

+24

Mon

ths

+26

Mon

ths

MC

V (

fl),

Hb

F (

%),

Neu

tro

ph

ils

(10^

9/l)

an

d H

b (

g/d

l)

0

5

10

15

20

25

HU

Do

se (

mg

/kg

/day

)

Calculated dose (mg/kg/Day) MCV (fl) Hb F (%) Neutrophils (10^9/l) Hb (g/dl) Total Interval Markers HU Toxic Episodes IPDays

Patient XPatient XHaematological & Clinical ReportHaematological & Clinical Report

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Lessons Learned (1)Lessons Learned (1)

Central Registry Model Works Significant Resources Required to Complete

Collection and Processing/Reporting of Retrospective Data

Adequate Resources Required to Continue Longitudinal Follow-Up: Clinical Follow-Up and Research

Improved Patient Care: Compliance Follow-Up Monitoring & Evaluation Service Planning

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Lessons Learned (2)Lessons Learned (2)

Need to Improve the Efficiency of the Data Submission and Processing using Web based Technology: Online Data Submission Online Data Validation Online Data Retrieval Ability to View PATS Data Online

Require more IT Specialist Input: Web based PATS Server and Additional Software Development

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Contact DetailsContact DetailsDr Mark Layton

Haematology Department

Commonwealth Building

Hammersmith Hospital

Du Cane Road

London W12 0NN

Tel: 020-8383 1320

Fax: 020-8383 2175

email:[email protected]

Annette Gilmore

EHR Co-ordinator



Acton Lane

London

NW10 7NS

Tel: 020-8453 2135

Fax: 020-8965 1115


Sameer J Patankar

IT Consultant



email: [email protected]


www: http://www.hbregistry.org.uk

Dr J Howard



Acton Lane

London

NW10 7NS

Tel: 020-8453 2433

Fax: 020-8965 1115

email: [email protected]

Documents

Www.hbregistry.org.uk 1 © Copyright Haemoglobinopathy Registry - Central Middlesex Hospital 2003-2004 Haemoglobinopathy Registry in the Clinical Management