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

Journal article : Review

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.

Authors: Pericleous, M; Kelly, C; Ala, A; De Lusignan, S

• Publication status: Published
• Peer review status: Peer reviewed
• Publisher: Taylor and Francis
• Journal: Expert Review of Gastroenterology and Hepatology
• Volume: 12
• Issue: 8
• Pages: 829-841
• Publication date: 2018-07-13
• Acceptance date: 2018-07-03
• DOI: 10.1080/17474124.2018.1497483
• EISSN: 1747-4132
• ISSN: 1747-4124
• Pmid: 29976101
• Language: English
• Keywords: chronic care model; rare diseases; integrated care; orphan liver diseases; liver disease; FFR
• Subtype: Review