An interview with Prof Siobhan Burns, Professor of Translational Immunology, Royal Free – London and Chair of ESID Clinical Working Party
Why do we need clinical guidelines for Rare Diseases?
Clinical guidelines are statements designed to help doctors and patients make decisions about specific clinical situations and are aimed to improve the quality and consistency of care provided to patients. Although commonplace in other many areas of medicine, clinical guidelines are relatively scarce for rare conditions such as primary immunodeficiency disorders (PID). A key task for the ESID Clinical working party (CWP) is the development of clinical guidelines for the diagnosis and management of PID that can be shared across European countries.
How are guidelines developed?
Clinical guidelines are developed by rigorous methodology that brings together available published evidence and expert opinion to give specific recommendations. The ESID CWP has recently updated their methodology for development of PID clinical guidelines. The first step in the process is to appoint an expert panel who will oversee a systematic literature search to develop a draft set of recommendations. These will then be circulated to a wider group of clinicians for suggestions, before the final guidelines are agreed at a consensus meeting of the expert panel. Through IPOPI, there will be patient representation on every expert panel to ensure that guidelines incorporate the view of patients.
How will guidelines be disseminated?
Both ESID and ERN-RITA have broad reach to clinicians and patients with PID. ESID and ERN-RITA have agreed that clinical guidelines for PID will be developed by the ESID CWP for adoption and circulation through both ESID and ERN-RITA networks.
What are the priorities?
There are many areas of PID that lack formal clinical guidelines. The CWP is already working on guidelines for vaccination in PID, management of Chronic Granulomatous disease and diagnosis and management of Thymic Disorders. In addition, the CWP is working with the ERN-RITA to develop guidelines for transition from paediatric to adult services and use of haematopoietic stem cell transplant for adults with PID. Going forward, the ESID CWP will consult clinicians and patients to prioritise a list of guidelines by development over the next three years.
Look out for a survey from the ESID CWP on this topic so that you can give your suggestions and comments!