Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study

Abstract

,4 and the UK-PBC Consortium Primary biliary cirrhosis (PBC) has a complex clinical phenotype, with debate about the extent and specificity of frequently described systemic symptoms such as fatigue. The aim of this study was to use a national patient cohort of 2,353 patients recruited from all clinical centers in the UK to explore the impact of disease on perceived life quality. Clinical data regarding diagnosis, therapy, and biochemical status were collected and have been reported previously. Detailed symptom phenotyping using recognized and validated symptom assessment tools including the PBC-40 was also undertaken and is reported here. Perception of poor quality of life and impaired health status was common in PBC patients (35% and 46%, respectively) and more common than in an age-matched and sex-matched community control group (6% and 15%, P < 0.0001 for both). Fatigue and symptoms of social dysfunction were associated with impaired perceived quality of life using multivariate analysis. Fatigue was the symptom with the greatest impact. Depression was a significant factor, but appeared to be a manifestation of complex symptom burden rather than a primary event. Fatigue had its greatest impact on perceived quality of life when accompanied by symptoms of social dysfunction, suggesting that maintenance of social networks is critical for minimizing the impact of fatigue. Conclusion: The symptom burden in PBC, which is unrelated to disease severity or ursodeoxycholic acid response, is significant and complex and results in significant quality of life deficit. The complexity of symptom burden, and its lack of relation to disease severity and treatment response, suggest that specific approaches to symptom management are warranted that address both symptom biology and social impact. (HEPATOLOGY 2013;58:273-283)

Quality of Life and Physical Activity after Liver Transplantation. Literature Review.

“…This refers in particular to those subjects with liver cirrhosis complicated by hepatic encephalopathy, ascites or pruritus (Arguedas et al, 2003;Les et al, 2010;Marchesini et al, 2001;Wunsch et al, 2013). However, several HRQOL parameters are also impaired in non-cirrhotic patients with chronic liver diseases, such as those suffering from primary biliary cirrhosis (PBC) (Mells et al, 2013;Selmi et al, 2007;Huet et al, 2000). QOL improved following liver transplantation in all domains compared to the preoperative status (Sainz-Barriga et al, 2005;Aberg et al, 2012;Dupuis-Girod et al, 2010;Sirivatanauksorn et al, 2012).…”

Pathogenesis of Primary Biliary Cirrhosis and Its Fatigue

“…Fatigue is associated with many chronic inflammatory conditions, but appears to be a particular problem in patients with PBC [5,41]. Patients describe it as a feeling of physical exhaustion rather than simple tiredness, and often liken it to the sensation of their ‘batteries running down', rendering them unable to carry out simple day-to-day tasks.…”

“…Patients describe it as a feeling of physical exhaustion rather than simple tiredness, and often liken it to the sensation of their ‘batteries running down', rendering them unable to carry out simple day-to-day tasks. Fatigue is the most frequently reported symptom in PBC with more than 40% of patients suffering from moderate to severe fatigue [41]. Interestingly, fatigue is variable even within the PBC cohort but although a proportion of patients do not experience fatigue, these remain within a minority: fatigue is a significant problem for many PBC patients.…”

“…It is however difficult to conclude whether the depression is a result of having to live with fatigue or a contributing factor. The observation that depression is rarely seen as a sole associated feature in PBC fatigue, being seen in contrast most frequently when sleep disturbance and autonomic dysfunction are also present individually or in combination, would point to depression being a consequence of overall symptom burden and resulting social isolation rather than a cause of fatigue in PBC [4]. …”

“…The condition affects approximately 30 per 100,000 of the population in the UK [2]. Whereas the traditional approach to considering the disease has been to focus exclusively on the risk of progression to end-stage liver disease requiring transplant or resulting in the death of patients, it is now clear that there are additional impacts on patients including through the development of characteristic cholestatic itch and fatigue [3,4]. The aim of this review is to explore current thinking regarding the pathogenesis of PBC, and to explore the mechanisms thought to underpin the characteristic fatigue of PBC, the symptom in PBC with seemingly the greatest impact on patients and their quality of life [5].…”