'Sharing data saves lives' campaign

In January 2014 PID UK joined other leading charities and organisations across the UK to support a campaign to raise awareness of the importance of sharing data from medical records with medical researchers who use it to help them understand the causes of disease and to find better treatments.

Of-course everyone needs to make their own choice about their own personal data, but PID UK hopes the majority of people will be happy for their data to be used to help researchers find discoveries that can improve and save lives for other people affected by PID.

Here are two examples where data from PID patients is already being used to drive forward medical advances through involvement in patient registries.

The UKPID registry

The UK Primary Immunodeficiency (UKPID) registry exists as a sub-register of a larger European “ESID” registry. The purpose of the registry is to collate data on rare primary immunodeficiencies (PIDs) in the UK and Europe. These rare disorders, although genetic and monogenic in some cases are rarely homogenous in their presentation, with many patients with the same gene defect presenting in different ways. The variability adds to the difficulty in monitoring outcomes as part of clinical trials and good quality longitudinal data can only really be attained within a registry.

Reducing variability in care

The vast majority of PID patients who have antibody deficiency require replacement immunoglobulin (Ig) therapy. The data from the registry to date has provided evidence that a therapeutic threshold of 7 g/l of immunoglobulin (Ig) is a minimum target will keep patients out of hospital with serious infectious complications. This knowledge helps us to ensure that patients on Ig replacement therapy are treated to this minimum standard and reduces variability in care.  Additional data enquiry has helped to provide evidence on higher doses of Ig therapy on a personalised basis.

Driving up standards for diagnosis and clinical care

Aggregation of experience from across Europe requires standardisation to assess patient outcomes and to drive up the quality of patient care. For example simple measures such as how CT scans should optimally be reported is not uniform. The Chest CT group and substudy in antibody deficiency as part of ESID is set to use the patients in the registry to address this issue. The NBS (Nijmegen Breakage Syndrome) criterion substudy has similarly made use of the registry to validate the clinical diagnostic criterion. By doing so the standards of diagnostic and clinical care with these conditions is improved.

You can find out more about the UKPID registry here.

UK CGD registry

The UK Chronic Granulomatous Disease (CGD) registry is a sub-register of the ESID European register. The enrolment of patients onto the register facilitates the collection of substantial amounts of data regarding diagnosis, complications, treatments and outcome of the UK cohort of patients with CGD. The collection of this data enables detailed analyses of patient management, and changing trends in outcome over time.

Studies using this data have shown that mortality and complications increase with age, and the incidence of complications increases over time, despite appropriate prophylactic therapy. However, in recent years, mortality has improved in childhood. Furthermore, outcome of CGD in childhood is the same whether conventional prophylactic treatment or haematopoietic stem cell transplantation (HSCT) is employed. Detailed analysis has demonstrated that in the paediatric age-range, patients with CGD have a reduced quality of life when compared with age-matched controls, although this normalises following HSCT.

Following the establishment of this registry, and as a result of the information collected, the approach to treatment has changed in the UK and in Europe, with more rapid consideration of HSCT following diagnosis. Additionally, having identified X-linked patients, a carrier study is now being performed on female relatives who possess the mutated gene.

The NHS and your data

The NHS is creating a new system to share information from patient records to improve care and research, while protecting each person’s confidentiality.

Everybody in England can choose how information from their patient record is shared in this new system.

Every time you visit an NHS hospital or your GP, information about your health is recorded and stored in your patient record. The NHS uses this information to help provide the best clinical care for you.

Because your patient record contains personal information about your health, it must be handled very carefully and accessed safely and securely, protecting your confidentiality. Information from your record can also be used to improve healthcare delivery for all patients, and by health researchers who use it to help them understand the causes of disease and to find better treatments.


In February 2014, NHS England announced that it planned to delay the collection of data from GP surgeries until the autumn and that it will work with patients and professional groups to ensure information is accessible and reaches all sections of the community in order to build confidence in the initiative.

This delay should allow for fuller communications to ensure people are aware of how their data will be collected and used, the safeguards that are in place, and how to exercise their right to object.  PID UK hopes that systems will be put in place that are trusted and understood by everyone.