LIVER DISEASES
Hepatic encephalopathy is a potentially life-threatening neuropsychiatric condition that occurs among patients with liver dysfunction. It remains underdiagnosed and under-treated, resulting in poor quality of life for patients and a high burden on those who care for them. It is a debilitating condition that affects up to 40% of patients across Europe who suffer from advanced chronic liver disease. Despite the severity of hepatic encephalopathy, it remains underdiagnosed and under-treated, resulting in poor quality of life for patients and their carers.
SYMPTOMS:
HE is characterized by a loss of brain function, resulting in mild to severe incapacity, and is sometimes subtle and difficult to notice. HE occurs when the liver doesn’t remove sufficient toxins from the blood and is estimated to affect up to 200,000 people in Europe.
Symptoms and signs of hepatic encephalopathy may include:
- difficulty thinking
- personality changes
- poor concentration
- problems with handwriting or loss of other small hand movements
- confusion
- forgetfulness
- poor judgment
- a musty or sweet breath odor
- confusion
- drowsiness or lethargy
- anxiety
- seizures
- severe personality changes
- fatigue
- confused speech
- shaky hands
- slow movements
Hepatic Encephalopathy (HE) still on top of ELPA’s agenda
The second ELPA Educational Training day on June 24, 2020 was the occasion to reflect again on Hepatic Encephalopathy thanks to Dr. Teresa Casanovas Taltavull’s presentation about optimising outcomes in patients with hepatic encephalopathy (HE), a complication of cirrhosis. She also started her session quoting the article ‘Hepatic encephalopathy: a call to action to optimize patient outcomes’ from 2016 about HE campaign written by an ELPA representative.
Hepatic encephalopathy (HE) is a reversible syndrome of impaired brain function occurring in patients with advanced liver diseases. ELPA, being conscious of the importance of this topic, has never stopped putting it in the core of its policy and scientific activities.
On 26th February 2019, for example, the European Liver Patients’ Association (ELPA) held a policy roundtable entitled ‘The burden of Liver disease in Europe: The case of Hepatic Encephalopathy’ in the European Parliament. The event, co-hosted by some Members of the European Parliament (MEPs), brought together policymaker and high-level EU stakeholders in liver disease as well as related fields such as brain.
The reference document for the roundtable was ELPA’s new policy white paper focused on the key challenges facing people with hepatic encephalopathy (HE) in Europe and the policy actions which are required to secure a better future for people with hepatic encephalopathy (HE).
At the end of the meeting, there was broad support for the policy recommendations outlined in the new policy white paper and a commitment to pursue these recommendations in the 2019-2024 mandate.
ELPA’s engagement on HE will not end!
TREATMENT:
Reduction of plasma ammonia remains the central therapeutic strategy for HE, but there is a need for newer novel therapies. The two most often used medicines to treat HE are lactulose and certain antibiotics.
If you are, or a person that you know is, suffering from the symptoms described above and are/is, contact your doctor or a liver patient organization in your country. European Liver Patients’ Association is an umbrella organization with the national patient organizations as members. List of our members can be found here.
While the ‘fight΄ to change the name and what the disease stands for, has been going on for years,the disease has reached alarming conditions.Non- Alcoholic Fatty Liver Disease prevalence exceeds 25% in European adults, being higher in those with metabolic syndrome component(s)-related comorbidities.
We will not analyse the different aetiologies of Non- Alcoholic Fatty Liver disease or how it has described by different experts as NO single factor can explain the whole spectrum of disease.
What we want to emphasize is that the disease has alarming trends and serious effects on our daily lives as patients or patient representatives and not only as it has psychosocial negative influences to children and adolescents (stigmatization, labelling, bulling etc)
The problem and proposed solutions
Reveal / documentation of the problem
- The ELPA working groupon Non- Alcoholic Fatty Liver disease / NASH together with the ELPA scientific committee and the ELPA board support, haveconducted a survey.
ELPA ‘Socrates Survey’
Background:Non-alcoholic fatty liver disease (NAFLD) is excessive fat build-up in the liver without another clear cause such as alcohol use.
Aim: To evaluate the knowledge of the disease in the general population and their own opinion on the etymology of the acronymous of the disease (NAFLD)
Methods: A total of 650 people from 8 different countries were participating in this survey. All those who took part were over 18 years of age and it was done with their consent. Due to the Covid pandemic, the ELPA network was used, it was in a form of an electronic multiple-choice questionnaire translated into 8 different languages. Danish, Spanish, English, Hebrew, Arabic, French, German and Greek.
Duration: 1 Year (2021- 2022)
We named it so because we used the exact same method that this great Greek figure used to get results.’ The technique of simple questions.This time from patients to patients.
The survey was designed and evaluated by the ELPAs scientific committee and the ELPAs NAFLD/NASH group
In explaining what the acronym NAFLD means and whether it is a terminology that expresses them if they were patients, they did not agree with the term. To the question why they do not agree? The answer was that mentioning alcohol automatically stigmatizes people.
- The ELPA working group on Non- Alcoholic Fatty Liver disease / NASH together with the ELPA scientific committee and the ELPA board support, have conducted a questionnaire
This time the questionnaire was among patient representatives & associations from 20 countries,KOL, the European Patients Academy on Therapeutic Innovation (EUPATI)and some other associations as Obesity, Paediatric Associations etc.
We wanted to assess the implications or the impact of this disease and its name of ΝON-Alcoholic….Τhe result of this questionnaire highly anticipated:
The Non – Alcoholic disease is a stigmatizing disease. If a child has the disease and is overweight then he can become a victim of marginalization, bullying and teasing. It can make your life difficult in all areas.
Plans/ projects to solve the problem
- STOP the progression of the disease. Programs based on awareness of a proper nutrition and exercise
- Fatty Liver Future? Step out!!!
Program involving parents (Parents Association), school trainers-exercise programs, nutritionists. Strengthen the role of the school nurses.
Knowledge, education. Using an electronic platform where everyone can get the right, correct information according to the guidelines.
Young patients to share their experience and how they deal with these (videos,lectures… )
- Manifesto of the disease
Nonalcoholic steatohepatitis (NASH) is liver inflammation and damage caused by accumulation of fat in the liver. NASH falls under umbrella of non-alcoholic fatty liver disease (NAFLD) and is considered to be its most severe form due to the abnormal amount of fat in liver cells.
NASH can cause scarring of the liver (cirrhosis) and irreversible damage that leads to liver failure. It affects every age group and is considered to be a “lifestyle” disease as it is closely related to the obesity and diabetes. Although it can affect all the populations usually older people, people with diabetes and people with body fat concentrated in the abdomen are more likely to have NASH.
SYMPTOMS:
Most of the patients don’t know that they have NASH as the symptoms often occur after several years. However, possible symptoms of nonalcoholic steatohepatitis, and later cirrhosis, include:
- Abdominal swelling (ascites)
- Fatigue
- Enlarged blood vessels just beneath the skin’s surface
- Enlarged breasts in men
- Enlarged spleen
- Red palms
- Yellowing of the skin and eyes (jaundice)
RISK FACTORS:
Those patients that already suffer one of the conditions below are more likely to develop NASH.
- Diabetes
- High cholesterol
- High triglycerides
- Poor diet
- Metabolic syndrome
- Sleep apnea
- Underactive thyroid (hypothyroidism)
PREVENTION:
NASH is considered to be caused by the “western” lifestyle, thus unhealthy diet and sedentary culture. To reduce risk of nonalcoholic fatty liver disease one is advised to adopt a healthy diet rich in fruits, vegetables, whole grains and healthy fats, exercise regularly and maintain a healthy weight.
TREATMENT:
There is still no treatment for NASH and the best way of dealing with the condition is for the patient to implement lifestyle changes. Similar to the prevention, a patient should adopt a healthy diet, exercise and monitor his weight. If the patient suffers from other diseases such as diabetes then a part of the addressing NASH is also treating diabetes, or some other disease/condition that is increasing risk of NASH.
If you are, or a person that you know is, suffering from the symptoms described above contact your doctor or a liver patient organization in your country. European Liver Patients’ Association is an umbrella organization with the national patient organizations as members. List of our members can be found here.
ELPA’s WORK IN AREA OF NASH/NAFLD SO FAR:
- 1st EUROPEAN NASH-NAFLD POLICY SUMMIT REPORT, Brussels 2017 –https://elpa.eu/1st-european-nash-nafld-policy-summit-report-31st-may-2017/
- ELPA participated at NASH roundtable during AASLD, USA 2017
- NASH Council Meeting, USA 2017 https://static1.squarespace.com/static/53bafd3ce4b0ae714af7153f/t/5a2ec269c83025678abc75d7/1513013867120/nash-council-2017-meeting-report-web.pdf
- ELPA became and still is a part of NASH Council, formed by Global Liver Institute (GLI), US 2017 – https://www.globalliver.org/news/2017/12/6/nash-news?rq=korenjak
- ELPA participate at EASL NASH Summit event, Geneva 2018- https://elpa.eu/easl-nafld-summit-2018-geneva/
- ELPA participated at NAFLD Policy Research – http://regist2.virology-education.com/presentations/2019/EUNASH/02_Lazarus.pdf
- Roundtable on NASH in Paris 2019 – https://www.paris-nash.org/data-2019/Program-PARIS-NASH-MEETING-2019.pdf
- NASH patient expert meeting, Berlin 2019
ELPA organized ELPA Symposium focus on NAFLD and NASH as a part of EASL ILC Programme, Vienna 2019 – https://elpa.eu/elpa-symposium-19-nafld-and-nash-from-the-patients-perspective/ - ELPA organised Pre-Summit on NAFLD patient perspective meeting, Seville 2019 – https://elpa.eu/elpa-pre-summit-on-nafld-patient-perspective-meeting-held-on-26th-september-2019-in-seville-spain/
- ELPA created special working group focused on NAFLD and NASH, May 2019
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Hepatocellular carcinoma (HCC) is one of and is the most common types of liver cancer. Other types of liver cancer – intrahepatic cholangiocarcinoma and hepatoblastoma, are much less common. HCC is considered to be one of the leading causes of cancer-related death worldwide. It is associated with liver cirrhosis in western populations, but can occur in non-cirrhotuics especially in eastern populations with hepatitis B virus (HBV) infection.
POSSIBLE SYMPTOMS
Most people don’t exhibit symptoms in the early stages of HCC. However, when signs and symptoms do appear, they might be related to cancer or to chronic liver disease.
RISK FACTORS
The risk of hepatocellular carcinoma is higher in people with cirrhosis caused by infection with HBV, HCV, alcohol abuse or accumulation of fat in the liver (NAFLD/NASH). Chronic liver disease due to HBV or HCV or alcohol accounts for the majority of HCC cases, whilst the incidence of nonalcoholic fatty liver disease has been rising and it is also associated with the development of HCC. [1]
DIAGNOSIS
Hepatocellular carcinoma (HCC) is usually diagnosed through screening program to detect early HCC using ultrasounds every 6 months in people with cirrhosis. Once a lesion has been identified, the diagnosis of HCC is made on the presence of characteristic features of an HCC using dynamic imaging tests such as CT and MRI with contrast that should confirm the diagnosis in a patient deemed to be at risk of the disease.
PROGNOSTIC MODELS AND HCC STRATIFICATION
Barcelona Clinic Liver Cancer (BCLC) is the most used prognostic model of HCC. This classification categorize prognosis of HCC in 5 groups and guide the management of hepatocellular carcinoma.
Abbreviations: PST: performance status (0-2), Child-Pugh, A, B, C. classification cirrhosis score, N: extrahepatic lymphatic nodules, M: extrahepatic metastasis.
CURRENT TREATMENT MODALITIES
Treatments of hepatocellular carcinoma include, in early stages 0 or A:
-
- Resection (removing the cancer and a part of healthy tissue that surrounds it)
- Liver transplant surgery (remove the entire liver and replace it with a liver from a donor, cadaveric or living donor)
- Destroying cancer cells with heat or cold (ablation)
If the HCC is classified as intermediate stage, treatment is named loco-regional and include:
-
- Delivering chemotherapy or radiation directly to cancer cells (TACE, Transarterial chemo-embolization or similar)
In more advanced HCC treatment will be systemic such as:
-
- Targeted drug therapy. Targeted drugs, such as sorafenib, regorafenib, nivolumab etc. may help slow the progression of the disease in people with advanced liver cancer.
In more advanced HCC treatment will be systemic such as:
Due to the HCC characteristics, patient has two diseases cirrhosis and cancer and the treatment usually will be escalating through the stages, patients care would be on a multidisciplinary team based to improve management and assess and optimize risks.
If you are, or a person or you know someone that is suffering from the symptoms described above and might be considered to be affected by the risk factors that might lead to the development of HCC, contact your doctor or a liver patient organization in your country. European Liver Patients’ Association is an umbrella organization with the national patient organizations as members. List of our members can be found here.
[1] Review of hepatocellular carcinoma: Epidemiology, etiology, and carcinogenesis, Yezaz Ahmed Ghouri, Idrees Mian, and Julie H. Rowe, MD, Journal of Carcinogenesis 2017; 16: 1.
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Bile Duct Cancer (Cholangiocarcinoma)
VIRAL HEPATITIS
Hepatitis B is a treatable liver disease caused by the hepatitis B virus (HBV), which can cause both acute and chronic infection of the liver. Acute hepatitis B infection lasts less than six months and most people who get hepatitis B as adults have an acute infection. However, if one’s immune system can’t fight the infection then the disease develops into a chronic hepatitis B infection, which may last a lifetime, possibly leading to serious illnesses such as cirrhosis and liver cancer.
The possibility that infection becomes chronic depends also on the age of the person infected. For example, children less than 6 years of age who become infected with the hepatitis B virus are the most likely to develop chronic infections. 80–90% of infants infected during the first year of life develop chronic infections; and 30–50% of children infected before the age of 6 years develop chronic infections, whereas in adults less than 5% of otherwise healthy persons who are infected as adults will develop chronic infection; and 20–30% of adults who are chronically infected will develop cirrhosis and/or liver cancer.[1]
A CHRONIC HBV INFECTION CAN LEAD TO COMPLICATIONS SUCH AS:
- Liver cirrhosis (scarring of the liver)
- Liver cancer or Hepatocellular carcinoma
- Liver failure
- Other conditions such as kidney disease or inflammation of blood vessels, are extrahepatic manifestations of the HBV
SYMPTOMS:
The symptoms appear about one to four months after the exposure to the virus but in most cases the patient remains asymptomatic. Symptoms may include:
- Abdominal pain
- dark urine
- fever
- joint pain
- loss of appetite
- nausea and vomiting
- weakness and fatigue
- yellowing of skin and the whites of eyes (jaundice).
TRANSMISSION:
The hepatitis B virus is transmitted through blood and body fluids. The virus can survive outside the body for at least 7 days. The incubation period of the hepatitis B virus is 75 days, but can vary from 30 to 180 days. Hepatitis B is most commonly spread from mother to child at birth or through exposure to infected blood, but the ways of transmission also include sexual contact and sharing of needles.
TREATMENT:
Most adults infected with acute hepatitis B recover fully within the 4 to 8 weeks. However, those who develop the chronic hepatitis B infection can be treated with medicines. Present treatments decrease the risk of developing cirrhosis or HCC and improve prognosis but the HBV cannot be totally cured due to long-lasting cccHBV-DNA, which persists inside the hepatocytes nucleus.
VACCINATION:
Hepatitis B infection can be prevented vaccinating newborns and infants. Most countries have anti HBV vaccine in the vaccines calendar for children.
Vaccine, which is known as the “anti-cancer” vaccine because it prevents hepatitis B, the leading cause of liver cancer worldwide, is also recommended for adults in high-risk groups.
If you are, or a person that you know is, suffering from the symptoms described above and are/is considered to belong to the populations at increased risk of HBV infection, contact your doctor or a liver patient organization in your country. European Liver Patients’ Association is an umbrella organization with the national patient organizations as members. List of our members can be found here.
[1] World Health Organization, Hepatitis B, www.who.int
Hepatitis C virus (HCV) is a blood born RNA virus that causes hepatitis (liver inflammation) and in its progression is one of the leading causes of cirrhosis/ liver cancer in the world, especially in patients with other risk factor of liver cirrhosis (co-infection, obesity, alcohol etc.). We do not have a preventive vaccine for HCV but it is a curable liver disease. The natural history of the HCV infection, occurs as follows: Acute hepatitis C within the first 6 months after being exposed to the virus, usually it is asymptomatic. It usually leads to chronic infection, but it can also be a short-term illness. If untreated, chronic hepatitis C can cause progressive liver damage, cirrhosis (scarring of the liver), liver cancer, and even death. Between 75%–85% of people who become infected with hepatitis C virus will develop a chronic infection, while 15%–25% of those infected can clear it from their bodies without treatment and do not develop chronic infection. [1]
Untreated HCV infection can cause significant complications, such as: scarring of the liver (cirrhosis), liver cancer and liver failure due to advanced cirrhosis.
POSSIBLE SYMPTOMS:
The incubation period for hepatitis C is 2 weeks to 6 months. Newly affected usually do not have symptoms or have mild symptoms. However, people affected may have “hepatitis” unspecific symptoms that may occur in hepatitis of different cause, such as: fever, fatigue, decreased appetite, nausea, vomiting, abdominal pain, dark urine, grey-colored faeces, joint pain, jaundice (yellowing of skin and the whites of the eyes).
TRANSMISSION:
The HCV is usually transmitted through: injecting drug use through the sharing of injection equipment; the reuse or inadequate sterilization of medical equipment like syringes and needles in healthcare settings as well as the transfusion of unscreened blood and blood products. The virus can be transmitted sexually and can be passed from an HCV infected mother to her baby.
Hepatitis C is not spread through breast milk, food, and water, personal contact such as hugging or by sharing food or drinks with an infected person.
TREATMENT:
Hepatitis C is a curable disease and nowadays the therapy consists in the administration of direct-acting antivirals (DAA), an oral treatment safe and well tolerated which can cure more than 95% of persons with HCV infection within usually 8-12 weeks.
Although the treatment exists, the gap between those who are diagnosed and those who are directed to the anti-HCV treatment still remains. All adults and children with chronic HCV infection should be assessed for antiviral treatment. In addition, due to the chronic HCV or even cirrhosis could be asymptomatic some people are not aware of their liver disease, for this reason population screening is recommended to achieve HCV elimination in 2030.
POPULATIONS AT INCREASED RISK OF HCV INFECTION[2]:
Hepatitis B infection can be prevented vaccinating newborns and infants. Most countries have anti HBV vaccine in the vaccines calendar for children.
Vaccine, which is known as the “anti-cancer” vaccine because it prevents hepatitis B, the leading cause of liver cancer worldwide, is also recommended for adults in high-risk groups.
If you are, or a person that you know is, suffering from the symptoms described above and are/is considered to belong to the populations at increased risk of HBV infection, contact your doctor or a liver patient organization in your country. European Liver Patients’ Association is an umbrella organization with the national patient organizations as members. List of our members can be found here.
If you are, or a person that you know is, may have features described above and are/is considered to belong to the populations at increased risk of HCV infection, contact your doctor or a liver patient organization in your country. European Liver Patients’ Association is an umbrella organization with the national patient organizations as members. List of our members can be found here.
[1] Center for Disease Control and Prevention, Hepatitis C Questions and Answers for the Public, https://www.cdc.gov/hepatitis/
[2] World Health Organization, Hepatitis C, www.who.int
[3] European Centre for Disease Prevention and Control, Hepatitis C Annual Epidemiological Report for 2017, https://ecdc.europa.eu/