UK Bleeding Disorder Statistics UK Bleeding Disorder Statistics UK - - PowerPoint PPT Presentation

UK Bleeding Disorder Statistics UK Bleeding Disorder Statistics UK Bleeding Disorder Statistics UK Bleeding Disorder Statistics for 2007 for 2007 for 2007 for 2007

Dr CRM Hay Dr CRM Hay

Registrations: Registrations: -

• g

In 2007 In 2007 Total in Register Total in Register g Haem A Haem A Severe Severe 44 44 1,756 1,756 Total Total 137 137 6 303 6 303 Total Total 137 137 6,303 6,303 De De-

• registered 400+

registered 400+ Haem B Haem B Severe Severe 13 13 363 363 l l Total Total 34 34 1,340 1,340 VWD VWD male male 126 126 8,610 8,610 female female 229 229 female female 229 229 Total all diagnoses Total all diagnoses 959 959 23 629 23 629 Total all diagnoses Total all diagnoses 959 959 23,629 23,629

Density of Severe Haemophilia A & B population by patient post code area population ‐ by patient post code area

Density of Severe Haemophilia A & B Population ‐ by patient post code area – SE

Total Factor VIII / IX Usage (inc. inhibitor) by d patient post code area

Total Factor VIII / IX Usage (inc. inhibitor) by patient post code area ‐ London

Severe Haemophilia A – No. pts without inhibitor <18 years, treated by factor VIII issued by each centre

18 20 n 18 years

0 93 0 0001

14 16 ents less than

r = 0.93, p< 0.0001

10 12

• philia A patie

6 8 severe Haemo 4 6 IU issued to s 2 10 20 30 40 50 60 70 80 90 Million

• No. severe Haemophilia A patients less than 18 years treated

Severe Haemophilia A No pts lacking inhibitor ≥ 18 Severe Haemophilia A – No. pts lacking inhibitor ≥ 18 yrs, treated by factor VIII units issued by each centre

18 20 r more 14 16 nts 18 years or

r = 0.95, p < 0.0001

10 12 philia A patien 8 10 evere Haemop 4 6 IU issued to s 2 Million 10 20 30 40 50 60 70 80 90

• No. severe Haemophilia A patients 18 years or more treated

Severe haemophilia A and B lacking inhibitor using

Age 18 years or more

• nly one product during 2007: median usage

Kogenate-Helixate

Age 18 years or more Age less than 18 years

(413) [100,088‐122,912] (186) [95,134‐216,216]

Advate uct

(209) [102,118‐136,772]

(270) [152,383-195,000]

Refacto Produ

(21) [75,755‐188,391] (45) [90,840‐151,051]

Benefix

(179) [119,679‐173,250] ( ) [ ]

Plasma derived

(95) [67,680‐109,683] (19) [47,358‐286,463]

Plasma-derived Factor IX Median Usage (IU)

Severe Haemophilia A patients treated with FVIII products Severe Haemophilia A patients treated with FVIII products with no current inhibitor: median IU issued with no current inhibitor: median IU issued

(n=286) 250000

Cuzick’s trend p< 0.001

(n 286) (n=282) (n=184) (n=173) 200000 ent ( ) (n=173) (n=78) 150000 s per patie (n=266) (n=68) 100000 edian unit 50000 Me 0 9 10 19 20 29 30 39 40 49 50 59 60+ 0-9 10-19 20-29 30-39 40-49 50-59 60+ Age (years)

Factor VIII usage by UK Haemophilia Centres: 1989 2007 1989‐2007

400000000 350000000

Recombinant Plasma

Total 378M units in 2007

300000000 200000000 250000000 ts 150000000 200000000 Uni 100000000 50000000 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007

Year

Market Share of factor VIII concentrates known to Market Share of factor VIII concentrates known to have been used by UK Haemophilia Centres

CSL Behring (Plasma) 4% CSL Behring (Recombinant) 9% G if l (Pl ) 8% Hyate C Speywood (Plasma) 0% BPL (Plasma) 1% Grifols (Plasma) 8% Octapharma (Plasma) 1% Wyeth (Recombinant) 6% Baxter (Plasma) 0% Baxter (Recombinant) 37% Bayer (Recombinant) 34% Bayer (Plasma) 0%

Severe Haemophilia B: Pts lacking inhibitor < 18 d b f d yrs, compared to number of units issued

2 than 18

r= 0.78 r = 0 78

1.6 atients less t

r = 0.78

1.2 mophilia B pa ars 0.8 severe Haem yea 0.4 IU issued to Million 2 4 6 8 10 12 14 16 18 20

• No. severe Haemophilia B patients less than 18 years treated

Severe Haemophilia B: Pts lacking inhibitor ≥18 yrs d b f d

6

compared to number of units issued

5 tients 18

r = 0 86

4 philia B pa

r = 0.86

3 ere Haemo rs or more 2 ued to seve year 1 illion IU issu 5 10 15 20 25 Mi

• No. severe Haemophilia B patients 18 years or more treated

Severe Haemophilia B patients with no current h b d d inhibitor: median IU issued

(n=102) 160000 180000 (n=61) (n=26) 120000 140000 tient (n=111) 100000 120000 its per pat 60000 80000 Median un 20000 40000 M 20000 0-19 20-39 40-59 60+ Age (years)

Total factor IX units used by UK Haemophilia Centres 1989‐2007

60000000 70000000

Recombinant Plasma

Total 67.6M units in 2007

50000000 60000000 40000000 s 30000000 Units 20000000 10000000

1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007

Year

Market share of Factor IX concentrates known to have been used by UK Haemophilia Centres

BPL (Plasma) 4% Baxter (Recombinant) 32% CSL Behring (Plasma) 1% Grifols (Plasma) 4% ( ) % Octapharma (Plasma) 0% Wyeth (Recombinant) 59%

Von Willebrand’s Disease Von Willebrand’s Disease Von Willebrand s Disease Von Willebrand s Disease

Total registered Total registered M l M l Type 1 Type 1 2,223 2,223 Type 1A/B Type 1A/B 7 Male Male Female Female Type 1A/B Type 1A/B 7 Type 2 Type 2 95 95 T 2A T 2A 169 169 Type 2A Type 2A 169 169 Type 2B Type 2B 76 76 T 2D T 2D 1 Products used: Products used: -

• DDAVP + + +

DDAVP + + + Type 2D Type 2D 1 Type 2M Type 2M 88 88 Haemate P Haemate P 13.03M IU 13.03M IU Alphanate Alphanate 1.89M IU 1.89M IU Type 2N Type 2N 43 43 Type 3 Type 3 69 69 p p Wilfactin Wilfactin 1.04M IU 1.04M IU 8Y/Optivate 8Y/Optivate 0.75M IU 0.75M IU Subtotal Typed Subtotal Typed 2,795 2,795 Un Un-

• typed

typed 5,992 5,992 8Y/Optivate 8Y/Optivate 0.75M IU 0.75M IU yp yp

Principal causes of death 2007 Principal causes of death 2007 (Haemophilia A, B and VWD). (Haemophilia A, B and VWD).

Carcinoma

Carcinoma 17 (2 Liver) 17 (2 Liver)

Carcinoma

Carcinoma 17 (2 Liver) 17 (2 Liver)

Ischaemic Heart Disease

Ischaemic Heart Disease 13 13

Haemorrhage

Haemorrhage 16 16

C b l H h C b l H h 9

Cerebral Haemorrhage

Cerebral Haemorrhage 9

Liver failure

Liver failure 4

Liver failure

Liver failure 4

Infection

Infection 8 8

AIDS

AIDS 2 2

Haemophilia A, B and von Willebrand's Disease Pts p , (all severities) treated by UK Haemophilia Centres

20 15 10

r of Centres

10

Number

5

0 - 9 10 - 19 20 - 39 40 - 59 60 - 99 100 - 149 150 - 199 200 - 249 250 - 299

Number of Treated Patients

Severe Haemophilia A, B and von Willebrand's Disease pts treated by UK Haemophilia Centres

30 25 15 20

er of Centres

10 15

Numbe

5

0 - 9 10 - 19 20 - 39 40 - 59 60 - 99 100 - 149 150 - 199 0 9 10 19 20 39 40 59 60 99 100 149 150 199

Number of Severe Patients

Data Quality Data Quality Q y Q y

Commissioners consider that centres have a

Commissioners consider that centres have a

Commissioners consider that centres have a

Commissioners consider that centres have a contractual obligation to submit data to NHD. contractual obligation to submit data to NHD. DoH eq i es et n of data DoH eq i es et n of data

DoH requires return of data.

DoH requires return of data.

They are increasingly looking to NHD for data to

They are increasingly looking to NHD for data to support healthcare planning, benchmarking and support healthcare planning, benchmarking and reviews of service configuration. reviews of service configuration.

We therefore all have a duty to ensure the data

We therefore all have a duty to ensure the data is therefore as accurate as possible. is therefore as accurate as possible. is therefore as accurate as possible. is therefore as accurate as possible.

In the last year we have made great strides in

In the last year we have made great strides in improving data quality improving data quality improving data quality. improving data quality.

In the next year we intend to improve data

In the next year we intend to improve data further further further. further.

Data Quality Data Quality Data Quality Data Quality

Internal cross

Internal cross-

• checks:

checks: -

• Demographics checked with NSTS

Demographics checked with NSTS

Automatic reconciliation with HCIS system

Automatic reconciliation with HCIS system Automatic reconciliation with HCIS system Automatic reconciliation with HCIS system

Centre data compared with previous 2 years

Centre data compared with previous 2 years

No coding data (grossly unreliable) used

No coding data (grossly unreliable) used

No coding data (grossly unreliable) used

No coding data (grossly unreliable) used

Deaths cross

Deaths cross-

• checked with ONS

checked with ONS

Data queries raised

Data queries raised

Data queries raised

Data queries raised

Centres should check data annually

Centres should check data annually

D t le ning mp ign D t le ning mp ign

Data cleaning campaigns

Data cleaning campaigns

VWD, inhibitors, registrations of untreated

VWD, inhibitors, registrations of untreated Haem Haem A/B A/B

2 data chasers and the appointment of a data

2 data chasers and the appointment of a data quality officer. quality officer. q y q y

Will visit centres for training purposes and audit

Will visit centres for training purposes and audit