Density of Severe Haemophilia A & B

Transcription

1 UK Bleeding Disorder Statistics for 2007 Dr CRM Hay

2 Registrations: - Haem A Haem B VWD In 2007 Total in Register Severe 44 1,756 Total 137 6,303 De-registered 400+ Severe Total 34 1,340 male 126 8,610 female 229 Total all diagnoses ,629

3 Density of Severe Haemophilia A & B population bypatientpostcodearea post area

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

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

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

7 Severe Haemophilia A No. pts without inhibitor <18 years, treated by factor VIII issued by each centre IU issued to severe Haemo ophilia A patie ents less than 18 years Million r = 0.93, p< No. severe Haemophilia A patients less than 18 years treated

8 Severe Haemophilia A N o. pts lacking inhibitor 18 yrs, treated by factor VIII units issued by each centre 20 Million IU issued to s evere Haemop philia A patien nts 18 years or more r = 0.95, p < No. severe Haemophilia A patients 18 years or more treated

9 Severe haemophilia A and B lacking inhibitor using only one product during 2007: median usage Kogenate-Helixate (413) [100, ,912] (186) [95, ,216] Age 18 years or more Age less than 18 years Produ uct Advate Refacto (209) [102, ,772] (21) [75, ,391] (45) [90, ,051] (270) [152, ,000] Benefix (95)[67, ,683] (179) [119, ,250] Plasma-derived Factor IX (19) [47, ,463] Median Usage (IU)

10 Severe Haemophilia A patients treated with FVIII products with no current inhibitor: median IU issued (n=286) Cuzick s trend p<0.001 s per patie ent (n=282) (n=184) (n=173) (n=78) Me edian unit (n=266) (n=68) Age (years)

11 Factor VIII usage by UK Haemophilia Centres: Recombinant Plasma Total 378M units in ts Uni Year

12 Market Share of factor VIII concentrates known to have been used by UK Haemophilia Centres CSL Behring (Plasma) 4% BPL (Plasma) 1% CSL Behring (Recombinant) 9% Gif Grifols (Plasma)8% Hyate C Speywood (Plasma) 0% Octapharma (Plasma) 1% Wyeth (Recombinant) 6% Baxter (Plasma) 0% Bayer (Recombinant) 34% Baxter (Recombinant) 37% Bayer (Plasma) 0%

13 Severe Haemophilia B: Pts lacking inhibitor < 18 yrs, compared to number of units issued atients less than 18 mophilia B pa ars severe Haem yea r= 0.78 r= IU issued to 0.4 Million No. severe Haemophilia B patients less than 18 years treated

14 Severe Haemophilia B: Pts lacking inhibitor 18 yrs compared to number of units issued 6 tients 18 philia B pa Mi illion IU issu ued to seve ere Haemo year rs or more r= No. severe Haemophilia B patients 18 years or more treated

15 Severe Haemophilia B patients with no current inhibitor: hb median IU issued (n=102) its per pat tient (n=111) (n=61) (n=26) Median un Age (years)

16 Total factor IX units used by UK Haemophilia Centres Recombinant Plasma Total 67.6M units in Units Year

17 Market share of Factor IX concentrates known to have been used by UK Haemophilia Centres Baxter (Recombinant) 32% BPL (Plasma) 4% CSL Behring (Plasma) 1% Grifols (Plasma) 4% Octapharma (Plasma) 0% Wyeth (Recombinant) 59%

18 Von Willebrand s Disease Total registered Type 1 2,223 Male Type 1A/B 7 Female Type 2 95 Type 2A 169 Type 2B 76 Products used: - Type 2D 1 DDAVP +++ Type 2M 88 Haemate P 13.03M IU Type 2N 43 Alphanate 1.89M IU Type 3 69 Wilfactin 1.04M IU Subtotal Typed 2,795 8Y/Optivate 0.75M IU Un-typed 5,992

19 Principal causes of death 2007 (Haemophilia A, B and VWD). Carcinoma 17 (2 Liver) Ischaemic Heart Disease 13 Haemorrhage 16 Cerebral Haemorrhage 9 Liver failure 4 Infection 8 AIDS 2

20 Haemophilia A, B and von Willebrand's Disease Pts (all severities) treated by UK Haemophilia Centres Number of Centres Number of Treated Patients

21 Severe Haemophilia A, B and von Willebrand's Disease pts treated by UK Haemophilia Centres Numbe er of Centres Number of Severe Patients

22 Data Quality Commissioners consider that centres have a contractual obligation to submit data to NHD. DoH requires es return of data. They are increasingly looking to NHD for data to support healthcare planning, benchmarking and reviews of service configuration. We therefore all have a duty to ensure the data is therefore as accurate as possible. In the last year we have made great strides in improving data quality. In the next year we intend to improve data further.

23 Data Quality Internal cross-checks: checks: - Demographics checked with NSTS Automatic reconciliation with HCIS system Centre data compared with previous 2 years No coding data (grossly unreliable) used Deaths cross-checked checked with ONS Data queries raised Centres should check data annually Data cleaning campaigns VWD, inhibitors, registrations of untreated Haem A/B 2 data chasers and the appointment of a data quality officer. Will visit centres for training purposes and audit