In pursuit of excellence: an integrated care pathway for C1 inhibitor deficiency

Abstract

There are estimated to be approximately 1500 people in the United Kingdom with C1 inhibitor (C1INH) deficiency. At BartsHealth National Health Service (NHS) Trust we manage 133 patients with this condition and we believe that this represents one of the largest cohorts in the United Kingdom. C1INH deficiency may be hereditary or acquired. It is characterized by unpredictable episodic swellings, which may affect any part of the body, but are potentially fatal if they involve the larynx and cause significant morbidity if they involve the viscera. The last few years have seen a revolution in the treatment options that are available for C1 inhibitor deficiency. However, this occurs at a time when there are increased spending restraints in the NHS and the commissioning structure is being overhauled. Integrated care pathways (ICP) are a tool for disseminating best practice, for facilitating clinical audit, enabling multi-disciplinary working and for reducing health-care costs. Here we present an ICP for managing C1 inhibitor deficiency.

Figure 1

BartsHealth National Health Service (NHS) Trust cohort of patients with C1INH deficiency under regular review (includes 11 paediatric patients). (a) Diagnosis. (b) Acute treatment plan (prescribed medication and setting for emergency treatment – C1INH concentrate includes Berinert, Cinryze and Ruconest). Patients who have both icatibant and C1INH concentrate are advised to take one or the other treatment, depending on the nature and circumstances of their attack.

Figure 2

C1 inhibitor deficiency process map.