The role of the chronic care model in promoting the management of the patient with rare liver disease

ABSTRACT

Introduction: The chronic care model (CCM) provides a holistic approach for managing chronic illnesses. Patients with rare liver diseases (RLD) have complex needs, impaired quality of life and often life-threatening complications. Most RLD meet the criteria for a long-term chronic condition and should be viewed through the prism of CCM. We aimed to ascertain whether the CCM has been considered for the frequently-encountered RLD.

Methods: MEDLINE®/PubMed®/Cochrane/EMBASE were searched to identify publications relating to the use of the CCM for the management of six RLD. We identified 33 articles eligible for inclusion.

Results: Six, eleven, one, thirteen, two and zero studies, discussed individual components of the CCM for autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cirrhosis (PSC), Wilsons disease (WD), alpha-1 antitrypsin deficiency (A1AD) and lysosomal acid lipase deficiency (LALd) respectively. We have not identified studies using the full CCM for any of the aforementioned RLD.

Discussion: Unlike in common chronic conditions e.g. diabetes, there has been limited consideration of the use of CCM (or its components) for the management of RLD. This may reflect a reluctance of the clinical community to view these diseases as chronic or lack of healthcare policy investment in rare diseases in general.

KEYWORDS:

• Liver disease
• orphan liver diseases
• rare diseases
• chronic care model
• integrated care

Declaration of interest

M. Pericleous received speaker fees from Intercept Pharmaceuticals and is a member of the Ursofalk advisory board. C. Kelly is supported by the UK National Institute for Health Research. A. Ala has received speaker fees, unrestricted educational grants from Intercept pharmaceuticals, Alexion Pharmaceuticals, BMS and Dr Falk pharma. A. Ala is a member of the Wilson Therapeutics Advisory Group and is supported by the UK National Institute for Health Research. S. de Lusignan is supported by the Eli Lilly fund a Real-World Evidence Centre in diabetes; Astra-Zeneca diabetes study; and Takeda gastroenteritis. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

M. Pericleous received speaker fees from Intercept Pharmaceuticals and is a member of the Ursofalk advisory board. C. Kelly is supported by the UK National Institute for Health Research. A. Ala has received speaker fees, unrestricted educational grants from Intercept pharmaceuticals, Alexion Pharmaceuticals, BMS and Dr Falk pharma. A. Ala is a member of the Wilson Therapeutics Advisory Group and is supported by the UK National Institute for Health Research. S. de Lusignan is supported by the Eli Lilly fund a Real-World Evidence Centre in diabetes; Astra-Zeneca diabetes study; and Takeda gastroenteritis.