Information for Families
What is acute liver failure?
Why is the liver important?
Finding a cause: first step of treatment
Information about the patient to share with the doctor
Will the ALF patient recover?
What to expect in the hospital
The intensive care unit
The Pediatric Acute Liver Failure Study Group
What is acute liver failure?Acute liver failure occurs when many of the cells in the liver suddenly die or stop functioning. When liver cells stop functioning, the liver fails to perform its duties to keep the body functioning. Acute liver failure is the medical term used to describe the condition in which the liver fails to perform its critical functions. Acute liver failure, sometimes called ALF, develops over a period of days or weeks and demands immediate care. There are many causes of ALF and treatment depends upon what caused the liver to stop functioning properly. It is difficult to predict what will happen to a child with ALF; some will recover completely, others will require liver transplantation, and, unfortunately, a few will die from this condition.
In the early stages, ALF is difficult to diagnose because it resembles other illnesses and is quite rare. The ALF patient may initially have non-specific symptoms such as tiredness, nausea, vomiting, and fever. These are common symptoms that would not suggest a liver problem. The majority of children will eventually show signs of a liver problem if they develop jaundice (yellow eyes and skin). As the disease progresses, most patients develop signs of brain dysfunction that can range from increased sleepiness or confusion to severe agitation and even coma. They can also bleed from the gums or stomach or bruise easily due to poor blood clotting.
Since ALF progresses so quickly, time is of the essence. It is important that patients with ALF be seen at a center with experience in treating ALF and in liver transplantation. ALF should not be confused with other types of liver failure due to cirrhosis or chronic hepatitis, which normally take years to develop. The various causes of ALF include reactions to prescription drugs, acetaminophen (such as TylenolŪ) overdoses, viruses, and genetic, immunologic and metabolic disorders. Unfortunately, many times no cause can be found.
Why is the liver important?The liver is located in the upper right corner of the abdomen and performs over 500 vital functions. For example, the liver:
Finding a cause: first step of treatmentUpon the patient's arrival at the hospital, the doctor will want to be sure that the illness is acute liver failure (ALF) and then determine its cause. There are a variety of categories of diseases that will be investigated. They include infection, medication or drug reaction, toxins, heart or blood vessel problems, and genetic abnormalities. Unfortunately, a specific cause is not determined in almost 50 percent of patients despite many blood and urine tests. Certain diagnoses are more common depending upon the child's age. There are conditions, such as an overdose of acetaminophen (TylenolŪ, for example), which can respond to specific treatment. Other conditions, such as some viruses or genetic disorders, may not have a specific treatment even if the disease is identified. However, identification of the disease that is causing your child's illness will help your doctor to determine the best treatment plan.
Information about the patient to share with the doctor
Will the ALF patient recover?The liver has a remarkable ability to recover despite serious injury. Although ALF is very serious and sometimes ends in death over a matter of a few days, full recovery is possible. Except for those therapies that stop the effects of certain drugs or treat specific infections or metabolic conditions, there are no sure treatments for acute liver failure. Patients get sick very quickly and can recover just as fast, but sometimes remain sick for days. Some do not recover and need a liver transplant. When a child has ALF, other organs such as the kidneys, brain, heart, and lungs can also begin to fail. To date, we do not have an absolute way to predict whether the child will recover completely, require a liver transplant, or die. Decisions are made based on the clinical progress of the individual patient as well as the cause of ALF.
What to expect in the hospitalALF patients may require the level of care provided in an intensive care unit, or ICU. Once the initial testing is done, patients are put to bed in a quiet setting with their head slightly elevated. Antidotes such as N-acetylcysteine (NAC) are given to patients who have suffered from an acetaminophen overdose. Most of the care is focused on monitoring and supporting the functions that a healthy liver usually provides, such as clearing ammonia from our blood and maintaining proper blood sugar levels. It is hoped that this support will provide a "bridge" to the time that the liver can heal itself. Unfortunately, it is impossible to predict when or if the liver will recover.
The intensive care unitNot all children with ALF require ICU care. When intensive care is required, ICUs have the best resources to manage and monitor all bodily functions and are the safest place for the patient to be.
In the past 30 years, improved intensive care has increased survival for patients with ALF. However, the ICU can sometimes appear to be a "cold" place to outsiders. There are often several tubes inserted into the patient, which help to keep track of kidney and heart functions. Brain function is closely monitored, because swelling of the brain is one of the most frequent complications of ALF. Sometimes a pressure monitor is inserted into the skull to keep track of the brain swelling. Although monitoring the swelling is not a treatment in and of itself, it does allow the medical staff to make informed decisions about other therapies.
The doctors and nursing staff in the ICU watch for changes and sometimes move in to prevent or to treat serious complications, such as swelling of the brain, bleeding, infection, and kidney failure. When patients have acute liver failure, pain is usually not a problem. They are often drowsy and may appear to be asleep. Remember to stay positive at your child's bedside!
Considering transplantationIf the liver is so badly damaged that there appears to be little or no chance of recovery, liver transplantation will be considered. The doctors will use blood tests, response to treatments, clinical measures of brain function, and, in some cases, a sample of the liver tissue to help determine if a liver transplant would help your child. There are standard guidelines to determine whether a patient is sick enough or, in fact, too sick for liver transplant surgery. A number of factors are weighed in making the decision to perform a transplant, including how bad the liver injury appears to be, the patient's age and previous health conditions, the presence of complications such as infection, and the availability of a suitable organ.
Transplantation cannot be taken lightly because it means that the patient will have a life-long requirement to take potent drugs to keep the new liver functioning. Patients who receive transplants still require intensive care but, if the new liver works well, their recovery is quite rapid. Even without liver transplantation, about 1/3 of ALF patients, where the cause of ALF is not determined, will recover on their own.
Experimental therapiesThe outlook is often very poor for ALF patients, and transplantation may not always be a suitable option. However, experimental treatments are sometimes used. If experimental therapies are available and appropriate, the physicians will discuss these options with you.
The Pediatric Acute Liver Failure Study GroupBecause ALF is such a rare disease, a group of university hospitals is collecting information and blood samples to improve understanding and to discover better ways of caring for ALF patients. The Pediatric Acute Liver Failure Study Group consists of 12 pediatric centers, with a headquarters at the University of Pittsburgh.
Information from the patient's hospital record as well as blood and tissue samples, when available, are used to study this condition. All information obtained is held in strict confidence, and no participation in the study is possible without informed consent from the child's parents or legal guardian. No names are used at any time in the study, so the information is truly confidential. If information about your child's illness is sent to this group, doctors and scientists will have a more complete picture of the disease and its causes so that new treatments for this rare and very serious condition can be found.
This program is funded by the National Institutes of Health in Bethesda, MD, and the Food and Drug Administration in Rockville, MD.
Back to top