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Abstract
Background: Progressive familiar intrahepatic cholestasis (PFIC) is a group of rare cholestatic liver diseases whose main features are itching, jaundice and even liver failure. Many studies have been reported in clinical reports, but the care of PFIC is rarely described. Purpose: To provide a summary and recommendations for the setup of strategies for PFIC patient care. Material and methods: A non-systematic review of PFIC nursing research until July 12, 2020. Use online search engines (PubMed, Medline, Embase, Web of Science, and CINAHL) to find PFIC nursing-related content, sort and summarize nursing-related content. Results: As a rare disease, there is limited research in PFIC nursing. The main care measures of PFIC including nursing interventions and evaluation tools. Nursing intervention is divided into general symptom care and nursing care of postoperative complications. Evaluation tools mainly focus on quality of life assessment and evaluation of itching. Conclusions: Nurses should pay attention to the use of scales when caring for PFIC patients, and observe whether specific nursing measures can help improve patients’ quality of life. PFIC nursing interventions and evaluation tools should be tailored according to PFIC clinical manifestation. A multidisciplinary collaborative approach is encouraged for proper management and tailoring therapy according to clinical manifestation.
Highlights
Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive genetic disease with an incidence of about 1/50,000 to 1/100 000 [1]
PFIC nursing interventions and evaluation tools should be tailored according to PFIC clinical manifestation
As a type of cholestasis, PFIC is divided into 6 types according to different mutation genes encoding biliary transport-related proteins, and they are designated as PFIC types 1 to 6
Summary
Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive genetic disease with an incidence of about 1/50,000 to 1/100 000 [1]. Progressive familiar intrahepatic cholestasis (PFIC) is a group of rare cholestatic liver diseases whose main features are itching, jaundice and even liver failure. Many studies have been reported in clinical reports, but the care of PFIC is rarely described. Results: As a rare disease, there is limited research in PFIC nursing. The main care measures of PFIC including nursing interventions and evaluation tools. Conclusions: Nurses should pay attention to the use of scales when caring for PFIC patients, and observe whether specific nursing measures can help improve patients’ quality of life. PFIC nursing interventions and evaluation tools should be tailored according to PFIC clinical manifestation. A multidisciplinary collaborative approach is encouraged for proper management and tailoring therapy according to clinical manifestation
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