Surviving Rabies: A Medical First Takes Place at Children's Hospital

Rabies is a nightmare disease. It can hide in the body for months before it invades the brain, causing encephalitis and a rapid, fearful death. There is no cure, and even with treatment, only 6 people are known to have survived it. The virus is carried in the saliva of infected animals, and spreads to humans who are bitten by the animals. In theory, it's even possible to acquire the virus from licks or scratches. An estimated 50,000 people die from rabies every year, the vast majority of them in developing countries without good rabies control measures - especially parts of Asia, Africa, and Central and South America.

Although any mammal can carry the rabies virus, worldwide it's most commonly spread by dogs. In the US, though, where rabies is uncommon and rabies control measures are the norm, rabies is found mostly in wild animals like raccoons, skunks, coyotes, bats, woodchucks and foxes. Although raccoons are the most common wild animal with rabies today in the US, most cases of rabies in humans have actually been caused by bat variants of the rabies virus.

This was true of the most recent incident, where, last October, 15-year-old Jeanna Giese of Fond du Lac, Wisconsin picked up a bat she found in church and carried it outside. The bat bit her on the finger, which was misinterpreted as a cut, starting a chain reaction that has been - until now - uniformly fatal without early treatment. Jeanna's care, provided by Medical College of Wisconsin doctors practicing at Children's Hospital in Milwaukee, led her to become the first person ever known to recover from rabies without the medicines used to prevent the disease.

The rabies vaccine can be given to people who are at risk of wild animal bites (for instance, those who work with wild animals or those who are visiting countries where rabies is common); this is called pre-exposure prophylaxis. The vaccine can also be given after a bite has occurred but before the virus has spread to the nervous system (called post-exposure prophylaxis). Pre- and post-exposure prophylaxis are both commonly referred to as PEP.

Virus Incubation
Once a bite has occurred, the rabies virus begins to infect local tissues at the site of the injury. At this point, the person who's been bitten doesn't even have symptoms. It's during this time that post-exposure prophylaxis can be given to stop the spread of the rabies virus.

The ultimate goal of the virus is to enter the central nervous system - the spinal cord and brain. Depending on the location of the bite and other factors, it typically takes from 1 to 3 months for the virus to make this journey. When it does reach the central nervous system, it wreaks havoc. Symptoms begin. At first, the patient may feel fatigued and have a fever or headache; the sickness might be mistaken for the flu at this point. Some people experience aching or tingling at the site of the bite.

Within a week of her symptoms beginning - about a month after her bite - Jeanna Giese was suffering from weakness, double vision, and a 102° F fever. She was admitted to the hospital with tremors and trouble walking, and was given a tentative diagnosis of post-infectious encephalitis. Her symptoms continued to progress and she was referred to Children's Hospital of Wisconsin (CHW) in Milwaukee and into the care of Kelly Tieves, DO, and other critical care physicians. By this time, says infectious disease specialist Rodney Willoughby, MD, who had been contacted by the CHW transport team, "she was unable to stand or keep her eyes open."

When her local pediatrician went over her story a second time and heard about Jeanna's exposure to a bat, rabies became a real possibility. Samples of her blood, spinal fluid, skin tissue and saliva were sent to the Centers for Disease Control and Prevention (CDC) in Atlanta. Although it's never been documented, it's theoretically possible to spread rabies from person to person, and Dr. Willoughby ordered the medical team to wear protective gear.

Deadly Diagnosis
As the rabies virus multiplies in the brain, signs of cerebral dysfunction begin - anxiety, confusion, and agitation progress to delirium, abnormal behavior, hallucinations, insomnia and seizures. Throughout this process, the brain remains undamaged and the rabies patient is intermittently conscious until very near the end.

The acute period of disease typically ends after 2 to 10 days. Once clinical signs of rabies appear the disease is considered fatal, and treatment consists of supportive measures until death occurs. The medical team's worst fears were confirmed when a positive identification of the rabies virus came back from the CDC.

Jeanna's doctors did an in-depth search for any new or hopeful data on treatment of rabies, but unfortunately, said Dr. Willoughby, "it was evident on review of the literature that there was no treatment available." However, there were tantalizing hints that death was caused by brain dysfunction rather than permanent damage, and that rabid animals could eradicate the infection if they had enough time for their immune systems to do so. But the disease generally moves so quickly, and brings death so soon, that survival by humans is impossible.

An Improvised Plan
In Jeanna's case, her doctors offered her parents first palliative care and then a never-tested alternative: use drugs to induce a coma state and let Jeanna's body fight the virus. Her parents agreed to the improvised plan.

The medical team and staff provided painstaking care. "There were eight key physicians* who designed her care plan. Everyone pitched in totally unselfishly," says Dr. Willoughby.

Amazingly, Jeanna survived. Tests of her spinal fluid showed a full immune response and she was brought out of the coma after 7 days.

Jeanna Giese left Children's Hospital in January and is working hard to regain her lost physical functions. She's had six months of rehabilitation, says Dr. Willoughby, and has 12 more to go. She is walking with a companion and attending school part-time.

Dr. Willoughby and the team that worked with him to save Jeanna's life have been kept busy fielding calls and answering questions; they'll be publishing a paper on their technique as soon as possible. It's nothing technologically new or earth-shattering, says Dr. Willoughby, just meticulous care and old tools used in new ways. But around the world, particularly in the less-developed regions where rabies tends to thrive, there's a ray of hope where none has existed before.

Eileen Early, BA, BSN, RN
HealthLink Editor

*Co-authors from the soon-to-be-released paper on the case are, from the Medical College and Children's Hospital, Rodney Willoughby, MD, Associate Professor of Pediatrics (Infectious Disease); Kelly S. Tieves, DO, MS, Assistant Professor, Pediatric Critical Care; George M. Hoffman, MD, Professor of Anesthesiology and Clinical Care; Nancy S. Ghanayem, MD, Assistant Professor of Pediatric Critical Care; Catherine M. Amlie-Lefond, MD, Assistant Professor of Neurology; Michael J. Schwabe, MD, Assistant Professor of Neurology; and Michael J. Chusid, MD, Professor and Chief of Pediatric Infectious Disease; Charles E. Rupprecht, VMD, PhD, is from the Centers for Disease Control and Prevention (CDC), Atlanta, Georgia.

This article includes information from the Centers for Disease Control and Prevention and the Morbidity and Mortality Weekly Report.

For more information on this topic, see the HealthLink article Rabies Team Leader Urges Study of "Milwaukee Protocol"

MCW Health News presents up-to-date information on patient care and medical research by the physicians of the Medical College of Wisconsin.