OBJECTIVES: Primary sclerosing cholangitis (PSC) is a rare chronic disease that presents challenges to both patients and clinicians. This study aimed to explore the experiences of patients with PSC regarding their disease and healthcare. DESIGN: A series of semi-structured interviews was completed with patients with PSC, including questioning their experiences of living with PSC and its related healthcare. SETTING: Participants were recruited from communities in England, Scotland and Wales, with advertisement via PSC Support (UK disease-specific charity support group). PARTICIPANTS: 18 patients aged between 21 and 72 years were interviewed
10 were male (56%), and all were of Caucasian ethnicity. Inclusion criteria were as follows: adults, self-identifying as having a diagnosis of PSC, and currently under National Health Service treatment for this disease. Patients with a history of liver transplantation were excluded. RESULTS: Participants confirmed the ongoing physical and psychological burden of PSC and its related healthcare. Living with PSC was described as a journey
the timeline of events was important to patients, with particular challenges identified along the way. These included difficulties in obtaining a diagnosis and accessing timely and knowledgeable medical care. Overcoming the unusual combination of uncertainties that PSC presents was of particular concern to participants
these differ from those observed in more common chronic diseases with established treatment pathways. Hidden complexities within chronic illness behaviour in PSC were described, including a noteworthy fragile doctor-patient relationship and dependence on the specialist. These produce additional challenges for the optimal clinical management of such patients by generalists and specialists. CONCLUSIONS: This study complements the existing literature on the ongoing high burden of PSC, with added value from in-depth discussions with patients themselves. Priorities for further work have been identified, including the need for improved risk stratification tools to allow individualised management and prognostication, as well as improving access to knowledgeable care while maintaining a strong doctor-patient relationship.