National Haemoglobinopathy Registry. Annual Report 2015/16. mdsas

National Haemoglobinopathy Registry Annual Report 2015/16 mdsas

National Haemoglobinopathy Registry Annual Report (2015/16) 3

1 Introduction CHAPTER 1 This 2015/16 data update follows the same format as the 2014/15 data update in providing stakeholders of the NHR with an update on patient numbers for Sickle Cell and Thalassaemia in England. All patient data entered on the NHR by 31st March 2016 is included in this data update. An extra chapter has been included in this Annual Report titled Information Requests. Detailed in this chapter are the information requests received by the NHR from various stakeholders. These published information requests demonstrate the range of information that is available from the NHR. If you would like to make an information request to the NHR please contact support@mdsdas.com The removal of inactive patients from reporting is necessary to maintain an accurate portrayal of patient populations. Figure 1.1 details the number of patients that have been removed from reporting since the function to update the status of patients was added to the NHR. We encourage users of the NHR to update the status of their patients accordingly. The next NHR Annual Report for 2016/17 will be published towards the end of 2017. Figure 1.1 Patient status updates Status Update No. No Longer Seen 382 Transferred to other centre 249 Patient Died 140 Total 771 4

CHAPTER 12 Sickle Cell Reports Sickle Cell Reports Page No. Figure 2.1 Sickle Cell registrations by centre 6-7 Figure 2.2 Sickle Cell patients by diagnosis type chart 8 Figure 2.3 Sickle Cell patients by ethnicity 8 Figure 2.4 Map of Sickle Cell registrations by commissioning hub 9 Table 2.4 Table of Sickle Cell registrations by commissioning hub 9 Figure 2.5 Sickle Cell Patients by current Age 10 Figure 2.6 Sickle Cell therapy type chart 10 Figure 2.7 Sickle Cell iron chelation type chart 11 Figure 2.8 Map of patients with TCD monitoring by commissioning hub 12 Table 2.8 Table of patients with TCD monitoring by commissioning Hub 12 5

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 2.1 Sickle Cell registrations by centre 2015/16 Centre Patients Kings College Hospital 1007 Guy s and St Thomas 936 Newham University Hospital 674 North Middlesex 565 Royal London 503 Queens Hospital (BHR) 472 Homerton Hospital 346 North West London Hospitals NHS Trust (Central Middlesex Hospital) 335 Queen Elizabeth Hospital, Woolwich 328 The Whittington Hospital NHS Trust 280 Manchester Childrens Hospital 275 Croydon Health Services NHS Trust 270 Manchester Royal Infirmary 261 Birmingham - City Hospital 259 Birmingham Children s 255 Imperial College Healthcare NHS Trust (Hammersmith Hosptial) 247 Lewisham Healthcare NHS Trust 246 University College London Hospitals 238 St Georges Healthcare NHS Trust, London 229 Imperial College Healthcare Trust (St Mary s) 147 Coventry University Hospital 136 St James s University Hospital, Leeds 128 Nottingham University Hospitals 127 Whipps Cross University Hospital 122 University Hospitals Leicester 114 6

Sickle Cell Reports Figure 2.1 Sickle Cell registrations by centre 2015/16 Centre Patients University Hospitals Bristol NHS Trust 98 Luton and Dunstable Hospital 92 Sheffield Teaching Hospitals 77 Milton Keynes Hospital 77 St Helier University Hospital Trust 75 Sheffield Childrens Hospital 64 Darent Valley hospital 58 Addenbrooke s Hospital Cambridge 53 Southampton University Hospital Trust 46 Oxford Children s Hospital 46 New Cross Hospital, Wolverhampton 45 Royal Victoria Infirmary, Newcastle upon Tyne 40 Royal Liverpool University Hospital 39 Northampton General Hospital 37 East and North Herts NHS Trust - Lister and QE2 Hospital 37 Bradford 32 Medway Maritime hospital Paediatric 32 Kettering General Hospital NHS foundation Trust 30 Derby 30 Oxford University Hospitals NHS Trust 26 Alder Hey Childrens Liverpool 26 Royal Berkshire NHS Foundation Trust 22 James Cook University Hospital, Middlesbrough 20 Gloucestershire Royal Hospital 14 Princess Royal University Hospital 13 Hull and East Yorkshire Hospitals NHS Trust 9 Basildon and Thurrock University Hospital NHS FT 6 Sickle & Thalassaemia Centre 2 East Lancashire Hospitals nhs Trust 1 7

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 2.2 Sickle Cell diagnosis type chart 2015/16 Figure 2.3 Sickle Cell patients by ethnicity 2015/16 8

Sickle Cell Reports Figure 2.4 Map of Sickle Cell registrations by commissioning hub 2015/16 Table 2.4 Table of Sickle Cell registrations by commissioning hub 2015/16 Region No. Patients Region No. Patients London 6804 West Midlands 695 North West 602 Yorkshire & Humber 539 East Midlands 415 East of England 188 Thames Valley & Wessex 140 South West 112 South East Coast 90 North East & Cumbria 60 9

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 2.5 Sickle Cell Patients by current Age 2015/16 Figure 2.6 Sickle Cell therapy type chart 2015/16 10

Sickle Cell Reports Figure 2.7 Sickle Cell iron chelation type chart 2015/16 11

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 2.8 Map of patients with TCD monitoring by commissioning hub 2015/16 Table 2.8 Table of patients with TCD Monitoring by commissioning hub 2015/16 Region No. Patients Region No. Patients London 1816 West Midlands 264 North West 233 Yorkshire & Humber 223 Thames Valley & Wessex 74 East of England 56 South East Coast 47 North East & Cumbria 14 East Midlands 118 12

CHAPTER 13 Thalassaemia Reports Thalassaemia Reports Page No. Figure 3.1 Thalassaemia registrations by centre 14-15 Figure 3.2 Thalassaemia patients by diagnosis type 16 Figure 3.3 Thalassaemia patients ethnicity table 16 Figure 3.4 Map of Thalassaemia registrations by commissioning hub 17 Table 3.4 Table of Thalassaemia registrations by commissioning hub 17 Figure 3.5 Thalassaemia patients by age group 18 Figure 3.6 Thalassaemia therapy type chart 18 13

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 3.1 Thalassaemia registrations by centre 2015/16 Centre Patients The Whittington Hospital NHS Trust 140 Birmingham Children s 120 University College London Hospitals 120 Manchester Childrens Hospital 59 Manchester Royal Infirmary 48 Bradford 42 Birmingham - City Hospital 39 North Middlesex 38 Imperial College Healthcare Trust (St Mary s) 34 Royal London 32 University Hospitals Leicester 31 Queens Hospital (BHR) 30 St James s University Hospital, Leeds 27 Newham University Hospital 25 Kings College Hospital 23 Coventry University Hospital 22 Luton and Dunstable Hospital 20 Imperial College Healthcare NHS Trust (Hammersmith Hosptial) 18 Sheffield Teaching Hospitals 16 University Hospitals Bristol NHS Trust 16 North West London Hospitals NHS Trust (Central Middlesex Hospital) 14 Sheffield Childrens Hospital 13 St Georges Healthcare NHS Trust, London 12 14

Thalassaemia Reports Figure 3.1 Thalassaemisa registrations by centre 2015/16 Centre Patients Nottingham University Hospitals 10 Lewisham Healthcare NHS Trust 8 Southampton University Hospital Trust 8 East Lancashire Hospitals nhs Trust 8 Homerton Hospital 7 Guy s and St Thomas 7 Derby 6 Royal Victoria Infirmary, Newcastle upon Tyne 5 Addenbrooke s Hospital Cambridge 5 Whipps Cross University Hospital 4 Queen Elizabeth Hospital, Woolwich 3 James Cook University Hospital, Middlesbrough 3 Royal Berkshire NHS Foundation Trust 3 Royal Liverpool University Hospital 3 New Cross Hospital, Wolverhampton 3 Oxford University Hospitals NHS Trust 3 East and North Herts NHS Trust - Lister and QE2 Hospital 3 Northampton General Hospital 2 Croydon Health Services NHS Trust 2 Alder Hey Childrens Liverpool 1 15

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 3.2 Thalassaemia patients by diagnosis type 2015/16 Figure 3.3 Thalassaemia patients ethnicity table 2015/16 16

Thalassaemia Reports Figure 3.4 Map of Thalassaemia registrations by commissioning hub 2015/16 Table 3.4 Table of Thalassaemia registrations by commissioning hub 2015/16 Region No. Patients Region No. Patients London 505 West Midlands 184 North West 119 Yorkshire & Humber 110 East Of England 28 South West 19 Thames Valley & Wessex 14 North East & Cumbria 6 East Midlands 49 17

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 3.5 Thalassaemia patients by age group 2015/16 Figure 3.6 Thalassaemia therapy type chart 2015/16 18

CHAPTER 14 Serious Events Serious Event Reports Page No. Figure 4.1 Reported serious events by financial year 2011/12-2015/16 19 Figure 4.2 Reported serious events - Sickle Cell 2015/16 20 Figure 4.3 Total reported serious events - Sickle Cell (All Years) 20 Figure 4.4 Reported serious events - Thalassaemia 2015/16 21 Figure 4.5 Total reported serious events- Thalassaemia (All Years) 21 Figure 4.1 Reported serious events by financial year 2011/12-2015/16 19

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 4.2 Reported serious events - Sickle Cell 2015/16 Figure 4.3 Total reported serious events - Sickle Cell (All Years) 20

Serious Events Figure 4.4 Reported serious events - Thalassaemia 2015/16 Figure 4.5 Total reported serious events- Thalassaemia (All Years) 21

CHAPTER 51 Information Requests 2015/16 Figure 5.1 New born patients registration status Diagnosis Year No. Born Registered within 365 days Sickle Cell 2011 275 88 Sickle Cell 2012 240 125 Sickle Cell 2013 193 102 Sickle Cell 2014 129 98 Sickle Cell 2015 66 64 Thalassaemia 2011 24 6 Thalassaemia 2012 27 16 Thalassaemia 2013 19 13 Thalassaemia 2014 11 10 Thalassaemia 2015 7 7 Other 2011 31 13 Other 2012 12 6 Other 2013 11 7 Other 2014 9 7 Other 2015 3 3 Figure 5.2 Sickle Cell patients on hydroxyurea by age Diagnosis Age Patients % Hydroxyurea % Sickle Cell (Hb SS) <5 516 8.4% 10 1.9% Sickle Cell (Hb SS) 5-17 2160 35% 243 11.3% Sickle Cell (Hb SS) 18+ 3488 56.6% 620 17.8% Total 100% 873 14.2% 22

Information Requests Figure 5.3 Sickle Cell and Thalassaemia transfusion status Diagnosis Patients Transfusion Data % Iron Chelation % Sickle Cell 9770 637 6.6% 245 2.6% Thalassaemia 1041 473 45.4% 513 49.2% Figure 5.4 Cardiac Values (Annual Review) Cardiac Values No. Results <8ms 20 8-12ms Moderate Loading 34 13-20ms Mild Loading 53 >20ms No Loading 280 Figure 5.5 Thalassaemia patients by age and region Region Age Range Thalassaemia Patients East Midlands 0-14 11 East Midlands 15+ 38 East Of England 0-14 18 East Of England 15+ 10 London 0-14 92 London 15+ 414 North East & North Cumbria 0-14 4 North East & North Cumbria 15+ 4 North West 0-14 50 North West 15+ 59 South West 15+ 16 Thames Valley and Wessex 0-14 3 Thames Valley and Wessex 15+ 11 West Midlands 0-14 105 West Midlands 15+ 79 Yorkshire & Humber 0-14 39 Yorkshire & Humber 15+ 70 Total Patients 1023 23

National Haemoglobinopathy Registry Annual Report (2015/16) Figure 5.6 East Midlands patients map 24

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