A 47-year-old business executive came to me with fatigue and swelling in her legs. She said that for the past six months she had felt more tired than usual, but attributed it to putting in longer hours at work. She became more concerned when she developed swelling in her legs and her doctor told her she had an enlarged spleen. Her doctor thought she might have a liver problem and referred her to me.
She did in fact have cirrhosis. Her blood work pointed to autoimmune hepatitis, a disease in which the body attacks the liver and causes scarring. I performed a liver biopsy to confirm the diagnosis so she could start treatment.
She was very surprised to learn that she had cirrhosis, considering that she rarely drank and that, besides her fatigue, she felt well. I explained that alcohol abuse is not the only culprit in the disease, and that it causes a wide spectrum of symptoms.
Cirrhosis is irreversible end-stage liver disease, and is the eleventh leading cause of death in the United States. In a cirrhotic liver, the normal liver cells transform into nonfunctioning cells, and the overall shape and structure of the liver is changed, leaving it bumpy and scarred.
Imagine that the normal liver is a brand-new sponge that is soft and flexible when wet. Now imagine an old used sponge that is hard and crumbles when squeezed. Water easily flows through the new sponge, but has a harder time passing through the old sponge. A cirrhotic liver is like an old sponge.
Many patients are often surprised to learn that excessive alcohol consumption is not the only cause of cirrhosis. In fact, an increasing number of cirrhosis cases in the U.S. are being linked to non-alcoholic steatohepatitis, or NASH, in which fatty deposits build up in the liver, causing inflammation. This condition is related to obesity and diabetes, both of which are on the rise in the U.S. Other potential causes of cirrhosis include viral hepatitis, some metabolic disorders (such as hemochromatosis, a genetic disorder in which excess iron damages the liver and other organs) and autoimmune diseases. Sometimes a cause of cirrhosis is not even found.
There are a number of ways to diagnosis cirrhosis, including liver biopsy, patient history and symptoms, blood tests and x-rays. There are also different degrees of cirrhosis, ranging from mild to severe, and they can be graded using the Child-Turcotte-Pugh scoring system. Doctors can determine the degree of cirrhosis with blood tests that measure substances released by the liver, including certain proteins and bilirubin, a pigment that is produced when the liver breaks down old red blood cells.
The most certain method of diagnosing cirrhosis is with liver biopsy. This involves extracting cells from the liver itself for testing. However, in some patients liver biopsy may not be possible.
If it isn't possible, then there may be certain findings on your history and physical exam that suggest cirrhosis and that are supported by the blood work. In some cases it is unsafe to perform a liver biopsy; if you have a low platelet count, for instance, you are at risk of excessive bleeding from the procedure. In addition, a biopsy is probably unnecessary in cases where there is a history of heavy alcohol use, an enlarged spleen, a low platelet count and structural signs of cirrhosis on an ultrasound test.
If, however, there is doubt about the cause of the cirrhosis or the diagnosis itself, a liver biopsy is very useful.
Patient History and Symptoms
Clues provided by the patient can be a helpful first step in the diagnosis, but cirrhosis is a complex disease that can be difficult to confirm. There are individuals with the disease who have no symptoms and no obvious signs in their medical histories that might point to cirrhosis.
However, there are clues doctors can look for, like heavy drinking and chronic hepatitis, both of which can result in liver damage.
Other signs are fluid in the legs (edema) or swelling (distension) of the abdomen. Some patients may have disabling symptoms such as serious fatigue or confusion. But again, others may have no symptoms at all. Sometimes signs of cirrhosis are evident only in blood work.
Blood tests that point to possible cirrhosis include those that measure liver proteins, blood cells called platelets or hemoglobin, a protein in red blood cells. Among the proteins produced by the liver are prothrombin, a key blood-clotting substance, and albumin, another important blood protein. When the liver is damaged, as in cirrhosis, its production of these proteins declines, and low blood levels of the substances can signal liver damage. Cirrhosis is also associated with an enlarged spleen that may entrap platelets, the blood cells involved in clotting; so measuring a person's platelet count can be useful in diagnosing the liver disease. Also, it is not uncommon for people with cirrhosis to have a low red blood cell count, or anemia, which can be detected by measuring hemoglobin levels.
In addition to blood work, x-rays can help in forming a diagnosis, as well as detect any complications of cirrhosis. An ultrasound or CT scan will allow a doctor to see whether the liver is shrunken and has a bumpy surface - structural signs of cirrhosis.
Bleeding may occur from the stomach or esophagus. The low production of clotting proteins and the low platelet count render patients with cirrhosis particularly susceptible to bleeding.
Fluid retention may occur in the abdomen or legs. Fluid retention in the abdomen is called ascites.
One of the jobs of the liver is to clear toxins from the blood. In a cirrhotic liver this is not possible and toxins may build up in the body. These toxins may pass to the brain and cause confusion, mood changes and other symptoms. This brain dysfunction, called encephalopathy, may be very mild and unnoticeable or be so severe that it results in coma.
Individuals with cirrhosis may experience none or all of these symptoms, and patients with longstanding cirrhosis are at risk of developing liver cancer.
Treatment of Complications
Patients often say, "I've heard that liver cells can regenerate. If the liver can regenerate then why can't it recover from cirrhosis?" Liver cells in a cirrhotic liver do regenerate, but in a haphazard fashion with scarring. The combination causes a distorted shape and abnormal blood flow in the liver.
Once cirrhosis has occurred therapy is supportive, meaning only the complications can be treated. This includes diet and fluid pills (diuretics) for patients with fluid retention. Individuals with bleeding may be treated with oral medication or an endoscopy, a procedure in which a long tube with a camera is inserted through mouth and into the stomach to locate bleeding areas. Once these areas are found the bleeding can be stopped by burning the affected area or by injecting a substance that causes the blood vessels to close. Bleeding may also be prevented by a medication called propranolol, which lowers the pressure in the veins around the stomach and esophagus. Altered brain function (encephalopathy) associated with cirrhosis can be treated with a lactulose, a liquid medication that causes a diarrhea, which lowers the level of toxins in the body. In severe cases patients with cirrhosis may require liver transplantation.
If a diagnosis of cirrhosis has been made, then it's best to stop drinking all alcohol or to drink infrequently. Also patients should avoid taking iron pills unless needed for other medical conditions. Excess iron may be harmful to the liver. Otherwise, eating a balanced healthy diet is the best approach.
The liver is an important organ involved in many critical functions. It is fairly forgiving and can repair itself but patients need to be proactive about seeking treatment when they exhibit any symptoms. Remember cirrhosis is not just a "drinkers" disease, and diet/weight, autoimmune diseases and hepatitis are all factors. For some, lifestyle changes, like maintaining a healthy weight with a balanced/low-fat diet and proper diabetes management can help stop cirrhosis, or at the very least slow the onset and reduce the progression and severity of the symptoms. Therefore, it is important to take care of yourself and love your liver!