A Tale of Rabies

From The Floor

A Tale of Rabies

The only hope was a radical experimental protocol

By Genevieve M. Clavreul, RN, Ph.D.
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We called him Peanut: a small, fragile boy who was admitted to our pediatric intensive care unit (PICU) at Columbus Medical Center in Columbus, Ga., in the late ‘70s with an infection we would later diagnosis as rabies.

Although rabies is no longer as common as it used to be in the U.S. — an aggressive public health campaign has reduced the number of confirmed cases from almost 6,000 a year in 1979 to fewer than three in 2010 — it is still a lethal disease that can test caregivers to their limits. When Peanut came to us three decades ago, it was an almost certain death sentence if not treated in time.

GRIM DIAGNOSIS
No one was ever quite sure how a little boy in one of Georgia’s larger cities had come to be bitten by a rabid animal. The most common carriers of rabies in western Georgia were raccoons, skunks, foxes and bats — not your typical urban fauna. Peanut’s parents had brought him to the emergency room with no idea of the nature of his illness and his symptoms had puzzled the ER physicians. When he was transferred to our PICU, the doctors were still unsure what was wrong.

Caring for Peanut presented some unique challenges. He was just a toddler and his age and condition limited his ability to communicate with the staff. We used a combination of sign language, pantomime and pictograms to quiz him on how he felt, where he had pain and so forth. His parents did their best to help, but they were as flummoxed by his condition as we were.

Rabies is a viral infection with three clinical stages. Peanut was admitted during the prodromal phase, which may include several days of flu-like symptoms. During the second stage, known as the excitation or “furious” phase, the patient may present with the symptoms commonly associated with rabies, such as dilated or rolling eyes, tachycardia, hyperventilation and hydrophobia. If the patient survives this phase, the disease moves into its third and final stage with the progressive onset of paralysis, stupor, coma and death.

Days passed as Peanut’s physician ordered test after test to whittle down the list of suspects. Then, Peanut presented with two of the unmistakable signs of rabies: a “locked jaw” and excessive salivating. The evening when I came on shift and saw the latter symptom made me and my team dread the impending diagnosis. Reviewing the likely outcomes left us on the brink of despair. Survival from rabies at that stage was so rare that it was not even included in the list of outcomes.

Our PICU team was by no means unaccustomed to death, but nothing in Peanut’s original symptoms had prepared us or his family for such a grim prognosis. How were we going to inform Peanut’s parents that their son’s most likely outcome was death?

THE EXPERIMENT
Later that day, our unit’s doctors and nurses held a meeting to discuss Peanut’s case. Since his condition was so advanced, neither rabies vaccine nor antiserum was an option. Instead, we decided to place him in an induced paralytic state, use supportive therapy to get him through the worst of the symptoms and hope for the best. (This was decades before the development of the Milwaukee protocol.)

After many telephone calls and much research on different paralytic drugs, our pediatric chief of staff settled on Pavulon, the brand name for Pancuronium bromide, a non-depolarizing curare-mimetic muscle relaxant. There was just one hurdle: Pavulon was not approved for pediatric patients and this off-label use required the approval of both the manufacturer and the FDA. Fortunately, after much finagling and promises to carefully document everything, our team got the necessary authorizations and set about developing a protocol for Peanut.

Since this was uncharted territory, we kept the protocol as simple as possible. We titrated the muscle relaxant until we discovered the minimum amount of Pavulon needed to paralyze Peanut. Doing so left him completely aware but unable to move, which was the only way to keep him from fighting the vent. (In those days, we had no inline suction and lacked the sophisticated equipment now available to anchor and stabilize an endotracheal tube.)

Our chief of staff then wrote a standing order to administer that same amount of Pavulon whenever Peanut exhibited any signs of movement. To help us monitor Peanut’s sedation level, we came up with an ingenious system of strings and mobiles that would move with his slightest motion. I can’t remember whose idea this was or where it originated, but it was wonderfully simple and very effective.

After many weeks, we were able to wean our patient off both the Pavulon and the vent. During this period, poor little Peanut could barely move his stiffened muscles, but every time he reached out for something or took a step, however stiffly or woodenly, we saw it as one more sign of a hard-won recovery from a devastating disease.
 
VITAL LESSONS
Peanut’s case was highly inspirational to those of us in the PICU, showing us that if we came together as a team, we could move mountains. He also taught me an important lesson: No matter what condition or state of mind patients may be in, they can still tell you a great deal about how the nurses interact with their patients.

As Peanut emerged from his induced paralysis, I noticed that there was one nurse on our team around whom he was visibly apprehensive. Later, when he was again able to move, he would run screaming from her into the arms of his parents or any other nurse who happened to be nearby.

At first, I couldn’t understand why Peanut would so be afraid of that particular nurse, who hadn’t subjected him to any procedure that hadn’t also been performed by every other nurse in our unit. It wasn’t until much later that I discovered Peanut had good reason to fear that nurse, who had a cruel streak that would eventually lead to her dismissal from our unit. She didn’t have the temperament for the PICU and Peanut was the first to catch on. I wish I had listened sooner.

HOPING FOR THE BEST
Eventually, Peanut was stable enough to be released and return home. I lost track of this spunky little patient as my life and career handed me other challenges. I’d like to think that he was one of the lucky few to survive his encounter with rabies, although the odds were against it.

As of 2008, there were only three known unvaccinated rabies survivors in the United States. Peanut might have been one of those three; I certainly hope so. I do know that I and the PICU staff at Columbus did everything in our power to make that outcome possible. 

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Rabies and Organ Transplants

by Aaron Severson

Most humans who contract rabies are infected via a bite from a rabid animal. However, there have been several reported incidents in the past decade of patients contracting the disease after receiving an organ transplant from an infected donor.

TRANSPLANT DEATHS
The first such incident occurred in June 2004, when four patients in Texas died of encephalitis within two months of receiving transplant organs from the same donor. The donor’s cause of death was a brain hemorrhage, but interviews with his friends later revealed that the 20-year-old Arkansas man had previous suffered a bat bite, which health officials believe was the original source of the infection. Postmortem investigation subsequently determined that the donor and all four transplant patients were infected with rabies.

A similar incident in Germany in early 2005 led to the deaths of three more transplant patients. Two others were vaccinated in time, while a third was found to have received a rabies vaccination before the transplant. In March of this year, a transplant patient in Maryland died of rabies more than year after receiving a kidney from an infected donor. In that case, three other patients who had received organs from the same donor were vaccinated prophylactically and did not develop symptoms.

SCREENING PROBLEMS
Although rabies in humans has become very rare in the U.S. and Europe, the disease can sometimes have an incubation period of three months or more after the original exposure. As a result, patients who have been bitten by an infected animal may not connect the bite with later symptoms, potentially leading healthcare providers to misdiagnose rabies as some other neurological condition.

That makes it more difficult for organ procurement organizations to properly screen for the disease. While organ donors are screened for certain infectious diseases such as HIV, the CDC notes that doctors have only a short window of time for testing before a potential transplant organ is no longer viable.  
 

This article is from workingnurse.com.

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