Understanding Primary Biliary Cholangitis

PBC treatment and management guidelines

It is not easy to identify the National Policy introducing recommendations for primary biliary cholangitis, so, for this reason; the essay is supported by guidelines from different resources to explain the essence of this long-term rare condition. Guidelines are generally defined as “systematically developed statements to assist practitioners and patients to make decisions about appropriate health care for specific circumstances.” Guidelines are “tools” to help the decision-makers to make better decisions and therefore both development and implementation strategies must be focused on the “end-user” decision-makers. It aims to make integrated care management approaches more consistent and supported self-care and self-management (WHO, 1997).

Primary biliary cholangitis (PBC) is an autoimmune liver disease in which a cycle of immune-mediated biliary epithelial cell injury (The British Society of Gastroenterology, 2017). PBC is a progressive disease, which can destroy some bile ducts and bile can no longer flow through them. When this process occurs, bile builds up in the liver and starts to damage the liver cells and causing inflammatory and scarring. Over the years, fibrosis becomes widespread, the liver is less able to repair itself and leading to cirrhosis. This condition can cause liver failure (British Liver Trust, 2007).

According to the European Association for the Study of the Liver guidelines (2017), etiology of PBC is unknown, but they believe that, genetic factors combined with environmental circumstances could play a crucial role. In the UK, PBC affects 20 thousand people and is most prevalent in women the age of over 50, but a spectrum of disease is recognized in adult male sex and younger than age 45 (The British Society of Gastroenterology, 2017). Diagnostically, PBC should be considered when the unexplained repeated elevation of serum alkaline phosphatase (ALP) and gamma-glutamyltransferase. Moreover, autoantibody status should be checked, because most patients have antimitochondrial antibody (AMA) in their blood (British Liver Trust 2007). In order to establish a full picture of the liver condition it might be required biopsy or MRI scans (EASL, 2017).

There is no cure for PBC at the moment, however, there are treatments available to halt condition process and ameliorate symptoms, which further reduce the quality of life. (EASL 2017) Evidence-based advice for PBC is drug therapy to prevent disease progression. In the first instants, it is recommended ursodeoxycholic acid. If the patient’s body responds well to UDCA then a patient will need to continue taking it for the rest of their life. However, when the patient does not respond to UDCA it may be recommended obeticholic acid. This medication improved liver biochemistry in the vast majority of cases and was found to help almost 50% of patients (The British Society of Gastroenterology, 2017). In light of the gathered data from the above guidance, treatment for PBC will not alleviate the symptoms however; these are treatable and improved if the right guidance is followed. Tiredness and itching are generally the first symptoms to appear while jaundice is usually associated with a later stage of the disease. All the national frameworks provide the recommendation for managing the symptoms and determine medication and alternative ways to improve the condition of the patient. An example of managing the symptoms can be shown on itching. It is a common symptom of PBC, although not all patients will have it. There are medications available such as bile sequestrates, antibiotics or oral opiate antagonists. However, they might not be suitable or effective for everyone (Hegade et al., 2015).

So, EASL (2017) suggested that, some practical measures can be helpful to manage these symptoms. The guidance indicates alternative ways such as use emollients or oatmeal extract, get cold baths or shower and consider that food or other allergies could be the cause of itching. According to EASL (2017), scratching the itch has become an addiction, in this case, it may help professional psychological advice.

NICE recommends in the guideline, Patient Experience in Adult NHS Services: Improving the Experience of Care for People Using Adult NHS service’’ that clear, consistent, evidence-based, tailored information is provided to patients throughout all the stages of their care. The factors such as knowledge, information, consistency, a positive approach, simplification, and repetition lead to a positive diagnosis experience.

A critical review of the article /article point of view

Many articles very deeply arguing about primary biliary cholangitis and explain the problematic character of this disease. One of the articles contains Orphanet Journal of Rare Diseases (2008) which features the complex information applying to primary biliary cholangitis in the article entitled, Primary biliary cirrhosis’’

According to the journal, PBC was described in 1851 by Addison and Gull. The label 'Primary biliary cirrhosis' was adopted in 1949, even though not all patients were cirrhotic at diagnosis. (Ahrens and Kunkel 1949) Nowadays primary biliary cirrhosis is now known as primary biliary cholangitis. It is a chronic and slowly progressive cholestasis liver disease of autoimmune etiology characterised by the destruction of the intrahepatic bile ducts results in periportal inflammation called cholestasis that might lead to liver failure (Kumagi and Heathcote, 2008). PBC prevalence among women over 40, but it can affect every one of all ages and gender. The disease ratio among females to males is 9:1 (Pandit and Samant, 2019). The highest incident and prevalence rate came from the United Kingdom, Scandinavia, Canada, and the United States, whereas the lowest rate came from Australia. There is no clear worldwide evidence to support the concept of "a polar-equatorial gradient" as it has been reported for other autoimmune conditions, but it may also be the case in PBC (Kumagi and Heathcote, 2008).

Kumangi and Heathcote sustain the same point of view as EASL that the pathogenesis of primary biliary cirrhosis is related to the interaction between genetic predisposition and environmental triggers. Familial predisposition to PBC is not rare. It has been reported that familial PBC is related to maternally inherited factors and that the disease tends to present earlier in the second generation (Kumagi and Heathcote, 2008). Pandit and Samant reaffirm the judgment of Kumagi and Heathcote of the environmental triggers include toxic waste, cigarette smoking and various xenobiotics (e.g., Escherichia coli, Mycobacterium gordonae, Novosphingobiumaromaticivorans). These environmental triggers induce the autoimmune reaction in genetically susceptible patients. The authors of the article,, Primary biliary cirrhosis’’ did not mention how pregnancy can be related to PBC. However, British Liver Trust (2007) indicated that, pregnancy could trig the PBC in their guidance. Occasionally PBC comes to light during or just after pregnancy. It is not clear whether pregnancy may spark it off or because pregnant women are under close medical supervision.

The journalists very widely describe treatment for PBC and mention about three groups of drugs that have been evaluated in PBC: immunomodulators, antifibrotics, and anticholestatics. Currently, the first-line therapy is ursodeoxycholic acid (UDCA), an anti cholestatic. Patients with PBC will develop complications related to cirrhosis. These patients should be evaluated for a liver transplant ( Pandit and Samant, 2019). According to the article liver transplant used to be a common, gold standard treatment for primary biliary cirrhosis. However, the proportion of patients with PBC who undergo liver transplantation has recently decreased. The assessment for liver transplantation in PBC is indicated when the patients show features of end-stage liver failure. However, the other resources provide the information that liver transplant can be recommended before the organ failure (PBCers Organization, 2019).

Patients with primary biliary cirrhosis can be asymptomatic or present with jaundice, pruritus, and fatigue and also can suffer from other autoimmune conditions such as Hashimoto thyroiditis, CREST syndrome, Sjogren syndrome, Rheumatoid arthritis, celiac disease. Osteoporosis, renal tubular acidosis, various skin conditions are also common in PBC patients (Zetterman, 2015). Therapies for symptoms and coexisting condition should be continuously evaluated rather than on an ad hoc basis, and it is important to re-evaluate symptoms and response to therapy. There is also a risk of recurring symptoms on stopping therapy and most patients require treatment long term. The symptoms of PBC typically do not correlate with disease severity and do not improve with UDCA therapy (The British Society of Gastroenterology, 2017).

Role of the nurse in self-care

By the long-term condition, we can understand a medical condition that cannot currently be cured but managed with the use of medication and other therapies. Having a long-term condition can admit a significant effect on a person’s life and their care givers. People with long term disease required personal-cantered design and delivered treatment and care are plans (British Medical Association, 2016).

Everyone responds in individual ways and the impact of diagnosis will vary according to the effect of the symptoms on the person’s life circumstances. This includes social, economic, psychological, cognitive and cultural issues for that person, as well as the physical aspects of the condition. All of these factors can have a profound impact on a person’s well-being and self-esteem (Alliance Scotland and Scottish Government, 2008).

Personal-centred care is fundamental in the management of the long-term condition and has been defined as treating patients as individuals and enabling them to make a decision. Personal- centred care aims to provide a system of care that understands the person’s health and wellbeing additionally, facilitates self-care and self-management (British Medical Association, 2016).

In the United Kingdom, there is a drive by the government to manage more effectively individuals with long-term conditions in primary care. This includes emphasising self-care with patients becoming experts at managing their condition (McHugh et al., 2009).

Self-care can be defined as the care taken by individuals towards improving or maintaining their health and wellbeing. Self-management is the process that enables the patients to manage their condition. Self-management is the responsibility of the individual, but it does not mean the individual is doing it alone. Successful self-management relies on a person having access to the right information, education, support, and services. It also depends on professionals understanding and embracing a person-centred, empowering approach in which the individual is the leading partner in managing their own life and condition (Alliance Scotland and Scottish Government, 2008). The nurses promote and support self- care and self-management among patients having LTC as PBC, have to act alongside NMC standards, based on the evidence-based approach and build a therapeutic relationship with the individuals and their families to achieve a positive outcome. According to Rees and Williams (2009), when patient-orientated interventions were added to professional and organizational interventions, patient health outcomes were improved. Nurses have an effective role in educating patients and facilitating adherence to treatment. The role of the nurses is supporting and encourages individuals to take control of their health. They play a significant role in promoting self-care what enables individuals to improve their knowledge about PBC and develop the skills and ability to maintain as good conditions as possible for a longer time (Medacs, 2013). They worked to ensure patient follow-up and referrals as necessary and described themselves as lynchpins in the management of long-term conditions. Nurses also recognised that, the patients would engage at different levels in the self-management process (Journal of Clinical Nursing, 2016). Personalised care planning, self-care and self- management support work together as part of one process of care delivery that promotes patient empowerment and choice, supporting people to be more independent and in control of their conditions and to become more actively engaged (DH, 2010). More proactive, preventive and person-centred approaches can improve patient experience, reduce unscheduled use of hospital care and reduce the cost of hospitalisation (DH, 2010).

Primary biliary cholangitis affects people in very individual ways. It is very hard to identify what impact PBC can have on people's life. The nurse must emphasise the personal-centred approach, work with the patient and recognise where the person needs support. The support may relate to an appropriate diet to manage the gastrological problems. A common symptom of PBC such a low level of energy, the nurse can review the individual’s day-to-day habits and establish an action plan to improve tiredness. This condition has many different symptoms and comorbidities therefore the nurse should be wise and very careful to support the patient (McHugh et al., 2009).

eHealth intervention/ strategy

Self-care and self-management might be challenging for people with long-term conditions and health care professionals because everyone is very specific personage and required a unique personal-care approach. In this situation, very helpful can be eHealth strategy or intervention that is useful in finding, recording, managing, and transmitting information to support health care, in particular, to make decisions about patient care.

Royal College of Nursing provides the World Health Organisation (WHO) definition of eHealth as "the cost-effective and secure use of information and communications technologies in support of health and health-related field, including health care services, health surveillance, health literature, and health education, knowledge and research" (Royal College of Nursing, 2020).

eHealth is the use of information, computers, and telecommunications in support of meeting the needs of patients and the health of citizens (The Scottish Government 2008).

Electronic health (eHealth) tools incorporate many opportunities for patients to increase their engagement through focused disease-specific learning, options to receive regular feedback and frequent reinforcement (peripheral monitoring devices). Additional inbuilt support functions that assess progress provide goal-setting and problem-solving, aim to increase the patient’s skills and confidence in managing their health problems. Supplementary motivational interviewing and cognitive behavioural components can also be provided via the internet, mobile device or telephone (Parker et al., 2017).

The benefits that eHealth can help bring about are the same as those sought in Better Health Better Care. Sharing information for the benefit of patients is a key responsibility of NHS services to provide good quality services and help protect vulnerable individuals. eHealth strategy is about improving patient safety and effectiveness through information. This will directly improve the outcomes of professional care, and at the same time provide essential support for effective self-care and health improvement by people in Scotland (The Scottish Government, 2008).

PBC Foundation released the new and improved app, which is available free to download on mobile devices. According to the PBC Foundation, this app can be used to record symptoms on a day-to-day basis and biochemistry results. It is very helpful to gain full access to important pieces of information, well as allow access to the PBC Compendium and EASL Lay guidelines. The application can be personalised to the patient, their journey, priorities, and values. The same way the tool becomes a good implement appropriable to self- care and helps better manage the patient’s condition on a daily basis (UK-PBC, 2019).

As part of a patient community, the PBC Foundation can drive forward progress in PBC simply by recording data on the unmet needs we face every day. The patients are becoming part of the international patient registry. PBC Foundation is able to directly influence medical research and help pharmaceutical companies discover new treatments to help PBC sufferers. Furthermore, the information the patient is recording can be shared with an adequate clinician to assist with the individual’s ongoing appointments. All records are safely stored, anonymised and protected by digital partner MTC Media (PBC Foundation, 2019).

The opportunities presented by digital to truly empower people and put them in control of their own health and wellbeing are immense. Moreover, significantly reduce complexity, open up access to information, automate back end processes and support effective evidence-based decision making by front-line professionals (Scottish Government, 2018).

Conclusion

There is not set up one main policy which can direct the right pathway to treat and manage primary biliary cholangitis. However, the European Association for the Study of the Liver and British Liver Trust provides complex guidelines apply to this long-term condition. Furthermore, some journals published very interesting articles that can expand knowledge about PBC. According to evidence-based data primary biliary cholangitis is a rare autoimmune liver disease prevalent among women over 40. This condition is slow, progressive destruction of small bile ducts of the liver, causing bile other toxins to build up in the liver (Pandit and Samant, 2019). The diagnosis of PBC is based on a persistent elevation of serum alkaline phosphatase, the presence of antimitochondrial antibodies, liver biopsy and MRI scan. There is no cure for PBC, so treatment and management of PBC aim to prevent end-stage liver disease and ease problematic symptoms. There are medications to slow down or even stop the destructive process of PBC. Recently for management of this condition are used ursodeoxycholic acid and obeticholic acid. It is some possibility of a liver transplant; however, this procedure is recommended in the end-stage of the disease (EASL, 2017).

Primary biliary cholangitis is a long-term disease that is a complex condition and each person will experience PBC differently (EASL, 2017). The National Health Service (NHS) Improvement Plan sets out the government’s strategy for improving the care of people with long-term conditions by moving towards a patient-centred approach. It is recommended that care should be focused on primary care settings but with improved partnerships and communications across all health and social care agencies (McHugh et al., 2009).

For people with complex needs, it has been accepted that care should focus on the well-being and management of the condition. The nurses are placed to provide care to all people with long-term conditions, from health promotion, prevention and encouraging self-care to caring for people with more complex needs, comorbidities, and end-of-life care. The nurses are an essential element of the multidisciplinary team and should take the lead in delivering complex care to people with the long-term disease while maintaining a focus on developing self-care, empowerment, community engagement and acting as an advocate for clients and their care givers (Journal of Clinical Nursing, 2016).

The new tool that is developing for patients and MDT seems to be very helpful to manage well-being and self-care in the long-term condition. It is an eHealth strategy and it allows to the empowerment of people and put them in control of their health, wellbeing and let them better organise self-care. There are the opportunities to significantly reduce complexity, open up access to information, automate back end processes and support effective evidence-based decision making by front-line professionals (The Scottish Government, 2018). Given the fundamental of health care information and communications technologies (ICT), it is important that nursing staff, from front line workers to leaders, embrace the agenda and play a key role in it, meeting the needs of the profession, patients and health care.

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