Hospice of the Comforter

A healthy liver helps fight infections and filters toxins from the blood. It also helps to digest food, store nutrients for future needs, manufactures protein, bile and blood-clotting factors and metabolizes medications. A healthy liver has the ability to grow back, or regenerate, when it is damaged. Anything that prevents the liver from performing these functions – or from growing back after injury – can severely impact health and very possibly length of life.

Liver disease affects one in ten Americans, and End-Stage Liver Disease (ESLD) is the 7th leading cause of death among adults, ages 25-64. It arises primarily from chronic liver disease that eventually leads to liver failure. In the early stages, symptoms are often vague and liver disease is often undiagnosed. By the time significant symptoms appear, the disease is usually quite progressed.

The most common causes of ESLD are chronic alcohol abuse, infection with the viruses, Hepatitis A, B or C, fatty liver disease, damage to the bile ducts, genetic disorders, a build-up of drugs or other toxins or auto-immune hepatitis.

Eventually, if not addressed and stopped in the earlier stages, the damage resulting from these multiple causes leads to scarring of the liver, known as cirrhosis, where large portions of the organ begin to lose their capacity to function or regenerate. Signs of liver disease include: weakness, fatigue, nausea, yellowing of skin or eyes (jaundice), loss of appetite, weight loss, fever, abdominal pain, itching, and abnormal blood vessels on the skin (spider angiomas).

With End-Stage Liver Disease, there are many symptoms and conditions which may occur. The following are some of the most common:

• Ascites: This is an increase in abdominal girth, due to fluid build-up in the abdomen as the liver fails. This can cause difficulty breathing as it limits the movement of the diaphragm and crowds the lungs. It can be painful due to pressure on the internal organs, and this fluid can develop bacterial infections leading to peritonitis, infection of the lining of the intestinal tract. The pressure in the abdominal cavity can lead to swelling in the lower extremities as well.

• Hepatic encephalopathy: As the liver fails, it’s ability to metabolize and rid the body of toxins becomes compromised, and these begin to build up in the bloodstream. This toxin buildup, particularly high ammonia levels, affects the central nervous system and results in fatigue, short-term memory loss, day-night sleep reversal, mental slowness and slurred speech. Patients with increased levels of these toxins may exhibit restless movement and asterixis, a hand-flapping tremor. If the encephalopathy becomes severe, the patient is likely to become unresponsive and comatose.

• Gastroesophageal varices/Portal Hypertension: As the liver fails, the circulation through it becomes more restricted, the pressure inside the blood vessels increases, and leads to a “ballooning” of blood vessels in other parts of the intestinal tract, especially around the esophagus and leading to the spleen. A serious complication is increased risk for esophageal hemorrhage, which can be life-threatening.

• Hepatocellular carcinoma (HCC): Primary liver cancer is diagnosed in approximately 500,000 people per year, with approximately 80% of cases occurring in patients with cirrhosis. It is a common result of chronic viral hepatitis.

• Hepatorenal syndrome: There can be a decrease in kidney function in a person with a liver disorder. Because less urine is removed from the body, nitrogen-containing waste products build up in the bloodstream (azotemia). The disorder occurs in up to 10% of patients hospitalized with liver failure and is a life-threatening complication, often leading to a hastened death.

• Laboratory findings: Abnormal laboratory findings are common in patients with ESLD and include low levels of the blood protein, albumin (hypoalbuminemia), a lowering of important blood-clotting factors, and a low platelet and red blood cell count (anemia). These can lead to a tendency to bleed and bruise easily.

Treatment of patients with liver failure is specific to the unique symptoms and conditions experienced by each individual. Any patient with liver damage will be asked to abstain from alcohol. For patients with cirrhosis and end-stage liver disease, medications may be required to control the amount of protein absorbed in the diet. If there has been a build-up of toxins, particularly high ammonia levels, medication will be offered which lowers these levels. Low sodium diet and water pills (diuretics) may be required to minimize water retention. In those with large amounts of ascites fluid, the excess fluid may have to be occasionally removed with a needle and syringe (paracentesis). Using local anesthetic, a needle is inserted through the abdominal wall and the fluid withdrawn. Sometimes surgery is performed to minimize portal hypertension and lower the risk of gastroesophageal bleeding.

At this point, a person may become a candidate for liver transplant of part or all of the liver. Transplant success has improved in recent years with 1-year patient survival rates of up to 87%, but more than 17,000 candidates now await liver transplantation in the United States. Due to the severe organ shortages, patients who are listed for liver transplantation have an estimated wait time of 1 to 3 years, depending on blood type and illness severity. Many patients are never able to be considered for transplants due to severity of their disease, other medical problems, or social considerations such as ongoing alcohol use or non-compliance with treatment recommendations. Others die while waiting for a transplant as their disease continues to progress.

The impact of these various symptoms and conditions on suffering and quality of life are profound, and ESLD patients can benefit greatly from hospice and palliative care. Even, when an ESLD patient is on a transplant list, this does not automatically prevent them from being on hospice services.

According to Medicare guidelines, patients will be considered to be in the terminal stage of liver disease (life expectancy of six months or less) and eligible for hospice care, if they meet the following criteria (1 and 2 must be present; factors from 3 will lend supporting documentation):

1. The patient should show both a and b:
a. Prothrombin time prolonged more than 5 seconds over control, or International Normalized Ratio (INR)> 1.5
b. Serum albumin <2.5 gm/d1

2. End stage liver disease is present and the patient shows at least one of the following:
a. ascites, refractory to treatment or patient non-compliant
b. spontaneous bacterial peritonitis
c. hepatorenal syndrome (elevated creatinine and BUN with oliguria (<400ml/day) and urine sodium concentration <10 mEq/l)
d. hepatic encephalopathy, refractory to treatment, or patient non-complaint
e. recurrent variceal bleeding, despite intensive therapy

3. Documentation of the following factors will support eligibility for hospice care:
a. progressive malnutrition
b. muscle wasting with reduced strength and endurance
c. continued active alcoholism (> 80 gm ethanol/day)
d. hepatocellular carcinoma
e. HBsAg (Hepatitis B) positivity
f. hepatitis C refractory to interferon treatment

The hospice nurse and medical director will address issues such as pain, shortness of breath, poor nutrition and confusion, while trying to prevent some of the more traumatic complications of ESLD. The hospice social worker will address caregiving needs, advance directives and help both the patient and family deal with the emotional roller coaster associated with lost abilities, independence, identity/roles and the impending death. The hospice chaplain will provide support for the many spiritual issues that may be raised by terminal illness. The hospice aides continue to provide the daily care that allows the patient to maintain cleanliness and dignity, and the hospice volunteers provide services ranging from companionship and caregiver respite to legacy building as the patient evaluates their life.

References

_{http://www.nlm.nih.gov/medlineplus/ency/article/000489.htm}

_{http://www.nlm.nih.gov/medlineplus/ency/article/000255.htm}

_{David C. Wolf. Encephalopathy, Hepatic: http://emedicine.medscape.com/article/186101-overview} 

_{Andres Cardenas. (2005). Hepatorenal Syndrome: A Dreaded Complication of End Stage Liver Disease. Am J Gastroenterol: 100, 460-467.}

_{Valentina Medici, Lorenzo Rossaro, Jacob A. Wegelin, Amit Kamboj, Junko Nakai, Kelli Fisher, and Frederick J. Meyers. (2008). The Utility of the Model for End-Stage Liver Disease Score: A Reliable Guide for Liver Transplant Candidacy and, for Select Patients, Simultaneous Hospice Referral. Liver transplantation 14:1100-1106.}

_{Michael D. Leise, W. Ray Kim, Walter K. Kremers, Joseph J. Larson, Joanne T. Benson, Terry M. Therneau. (2011). A Revised Model for End-Stage Liver Disease Optimizes Prediction of Mortality Among Patients Awaiting Liver Transplantation. Gastroenterology: Volume 140, Issue 7, 1952-1960.}

_{Lori Rosenthal. (2006). End Stage Liver Disease. Advance for NP’s and PA’s: Vol. 14, Issue 2, 46.}