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A young patient fights to survive acute liver failure.

One day a healthy 19-year-old woman was enjoying her normal, active life; the next day she fighting to stay alive. Jenny* came to the emergency room at Rush University Medical Center after suddenly feeling severely ill and seeing her skin turn into a deep shade of yellow. Physicians at Rush diagnosed her with acute liver failure. She was immediately taken to the intensive care unit, where a team of hepatologists (liver specialists), transplant surgeons and critical care physicians joined forces to save her life.

"She did not have a known liver disease, but she suddenly got very sick and her liver was failing," says Nikunj Shah, MD, a hepatologist at Rush. "She had significant damage to her liver and needed a transplant within seven days to survive."

With acute liver failure, the liver rapidly loses its ability to function — often developing in just a matter of days. This can then lead to excessive bleeding issues, pressure on the brain and coma. It is an often deadly condition that requires swift intervention and treatment. People with acute liver failure are treated in an intensive care unit and can typically only survive five to seven days prior to transplant.

A Rare Diagnosis

The team found that Jenny had Wilson's disease, a rare inherited condition in which the body accumulates too much copper, which damages the liver. "Usually when patients are young like her, they don't know they have Wilson's disease because there are no signs or symptoms. Liver failure happens very suddenly," says Shah.

The liver of a person with Wilson’s disease does not release copper into bile as it should. As copper builds up it begins damaging the liver. Eventually, the liver releases copper directly into the bloodstream, leading to copper build up in the kidneys, brain and eyes. If left untreated, Wilson's disease can cause severe brain damage, liver failure and death.

Jenny received a new liver within 48 hours and went home seven days later. "The good thing about Wilson's disease is that a transplant will cure the disease because the new liver is able to eliminate copper properly," says Shah.

Now, nearly five years after her transplant, Jenny is thriving. She completed college and is working, and aside from not drinking alcohol, there are no restrictions on her life.

Shah screens all patients for Wilson's disease when they come in with elevated liver enzyme tests. If detected early, Wilson's disease can be managed with medications that prevent liver damage and failure.

*Not the patient's real name.

December 2012
 

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