Impact of vCJD on Haemophilia Practice

Dr Gerry Dolan

Nottingham Comprehensive Care Centre

UK

The UK issue – late 1990s

vCJD may be transmitted by blood products.

There may be a large number of infectious, asymptomatic individuals in the UK donor population.

Haemophiliacs in the UK treated with plasma-derived concentrates may have been exposed and continue to be exposed to transfusion transmittedvCJD.

The UK issue – 1990s

What can be done to minimise the risk to patients from this new TTI risk?

The UK issue – 1990s

History in haemophilia care of devastation caused by transfusion transmitted infection – HIV.

Difficulties caused by delayed acceptance of the risk of TTI and of delayed actions.

Potential to improve safety for patients.

The UK issue – 1990s

Incomplete knowledge

Meeting of UKHCDO in 1997

Spongiform Encephalopathy Advisory Committee

(SEAC)

Centre for Surveillance of CJD

Meeting of UKHCDO in 1997

New Disease

British Disease

Long incubation period may be many infected blood donors

Experimental evidence for TTI

Large number of UK haemophilic population had received and were still treated with plasma products derived from UK donors.

Reducing risk - 1

As soon as possible – stop using UK plasma-derived products.

Recombinant products treatment of choice.

Plasma-derived products – use non-European plasma USA plasma sourced products preferred.

Consequences

BPL and SNBTS stop production of FVIII/FIX

Source and import USA plasma

Long delay and interruption of supply of other products FVII

FXI

Patient Response

Alarm among patients (1st wave)

Confidence undermined.

Increased pressure for recombinant products.

Impact

UKHCDO/Haemophilia Society – had for many years argued for the introduction of recombinant products – based on safety.

‘Failure of pathogen inactivation steps to deal with all infective agents and new/emerging pathogens’

vCJD

Impact

Department(s) of Health – accepted the case for recombinant FVIII/IX for all children.

Children recombinant products.

Adults USA plasma-derived FVIII/IX.

The inevitable!

2000 BPL notified UKHDCO of batches of factor concentrate prepared in 1996/7 and used before 1998 – donor subsequently diagnosed with vCJD.

Many patients treated with these batches

? response

No clear evidence of vCJD transmitted by blood products but some experimental evidence.

No test for donor or recipient infection.

Long incubation period.

No treatment.

Not tell patients v Tell them everything

Write to all patients irrespective of whether they had received implicated batches or not and ask if they wanted to know:-

1. Yes and discuss in person

2. Yes and inform by letter

3. No – don’t want to know.

Impact on patients

Considerable concern

may be infected with vCJD

recombinant products not available

Recombinant for all

Undoubtedly increased political pressure to fund recombinant coagulation factor concentrates in the UK.

From April 1st 2005 – all patients with haemophilia A and B will be treated with recombinant products.

Unresolved issue

If these recipients of UK pooled plasma products have been exposed to vCJD – what risk do they pose to others?

‘Guidance’

Department of Health

Advisory Committee on Dangerous Pathogens

Spongiform Encephalopathy Advisory Committee

Risk assessment conducted by Det Norske Veritas Consulting.

‘Guidance’

Patient 1 receiving blood during an operation in 1997 –died of VCJD 6 years later.

Patient 2 – received blood from donor who subsequently developed vCJD. Patient died of other causes but at autopsy PrPsc in spleen.

Categorisation of patients at risk

1. Symptomatic patients

2. Asymptomatic patients at risk from familial forms of CJD linked to genetic mutations.

3. Asymptomatic patients potentially at risk from iatrogenic exposure.

Precautions

Single use instruments where possible.

All procedures performed in operating theatre.

Procedures should be performed at the end of list.

Minimum healthcare personnel involved.

Liquid repellent operating gown over a plastic apron.

Mask,goggles or full-face visor.

Precautions

Surgical instruments

High and medium risk procedures CNS, posterior eye

lymphoid

olfactory epith

DESTROY instruments

Precautions

Endoscopy

CNS

GI and olfactory if biopsy performed

Quarantine/destroy

Other initiatives

Research/surveillance

Autopsy

Recipients of potentially contaminated products.

Impact of vCJD in Haemophilia care in UK

Caused great anxiety and distress to patients in the UK.

Potential to severely reduce the quality of medical and surgical care to all patients.

Impact of vCJD in Haemophilia care in UK

Massive increase in extra clinics, administration and record keeping for haemophilia centre staff.

Potential to stigmatise the haemophilic population (again)

Impact of vCJD in Haemophilia care in UK

The Recombinant for all programme should ensure that this will not happen again.