Last week, news of an Australian 7-year-old diagnosed with a vaccine preventable illness made the rounds. The child had not received a recommended series of vaccines that would have reduced her risk after exposure by about 100%, but this is a common occurrence in New South Wales where childhood immunization rates are below 50% in some areas. Mullumbimby, the birthplace of “rapper” Iggy Azalea and a recent host to a talk by New Age meme generator David Wolfe, in particular is a home to many unprotected children.

The vaccine-preventable disease in this case is tetanus. You’ve undoubtedly heard of tetanus before, likely when getting one of the recommended booster shots every ten years, but never known anyone who has had it. Many people are at least familiar with the term lockjaw, known as trismus in medical jargon, but there are a variety of signs and symptoms, some of which can result in severe pain and difficulty breathing even to the point of death. Although details of her presentation are few and untrustworthy, this little girl has very likely suffered significant physical discomfort and emotional distress. Her parents, regardless of whether or not they are to blame for their child’s condition, certainly have as well.

Tetanus is extremely rare in the United States and other developed regions including Australia, thanks to the widespread availability of a safe and effective vaccine. The developing world isn’t so lucky, experiencing about a million cases annually with a few hundred thousand deaths. And anytime there is a natural disaster we see a spike of cases related to dirty wounds and a lack of adequate medical care.

When modern medical care is available, mortality is low except for the most severe cases. The very young (specifically when a neonate acquires tetanus after their umbilical cord is handled in a non-sterile manner) and those over 65 years of age tend to have the worst outcomes. Unfortunately, those in developing regions who are diagnosed with tetanus have a more grim prognosis. Overall, the mortality rate can be as high as 50% without treatment.

It appears that the child in question is now stable and will recover, although it will takes weeks. She is lucky to be alive, thanks to the availability of modern medical treatment. Meryl Dorey, ringleader of the notorious anti-vaccine organization known as the Australian Vaccine-skeptics Network, which is headquartered in New South Wales, has responded to the situation here and here. I’ll address her propaganda, but first a bit more on tetanus because it’s interesting and I really want to paint a picture of what this poor child likely experienced.

What is tetanus?

Tetanus occurs when the spores of a specific bacterium commonly found in soil and mammalian feces inoculate unhealthy human tissue, most often via a penetrating injury through the skin. When the conditions are right, such as in the setting of an infection, a foreign body, or tissue that is poorly perfused or dead, the Clostridium tetani spores “wake up” and begin to produce tetanus toxin (tetanospasmin). Tetanus is also known to occur in the setting of home births, septic abortions, necrotic bowel, non-sterile ritual circumcision, and when injecting with dirty needles to name a few other causes.

Rarely there will only be local effects, but in the vast majority of patients the toxin will eventually gain access to peripheral nerves and travel along their course in a retrograde fashion until reaching the brainstem and spinal cord. The toxin will then bind irreversibly to inhibitory neurons and begin to interrupt neurotransmission of GABA and glycine. This results in complete disinhibition of neurons responsible for keeping the motor cortex and the autonomic nervous system in check.

What are the signs and symptoms of tetanus?

The end result of exposure to tetanus toxin, a neurotoxin considered by some to be the runner up to Botulinum toxin when it comes to deadliness, in most cases is painful muscular spasms in the setting of a general increased muscle tone. As if that weren’t enough, disinhibition of the autonomic nervous system can cause varying degrees of hell to break lose, with an overproduction of catecholamines and subsequent profuse sweating, increased heart rate and blood pressure, fever, and agitation. It’s the fight of the “fight or flight” reflex in overdrive. It can even result in life threatening cardiac arrhythmias.

As demonstrated in the video linked to above, patients with generalized tetanus have diffuse tonic contraction of their muscles. This is interrupted by terribly painful spasms that can be triggered by a variety of stimuli, even just a noise or a light breeze, and can be powerful enough to cause fractures of the spine and long bones. Patients with tetanus are generally kept in dark and quiet rooms with a goal of reducing external stimuli as much as possible.

Trismus occurs when the muscles of the jaw go into spasm and can limit the ability to open the mouth, making it difficult to speak, eat, or breathe. Another classic symptom, known as risus sardonicus, is when spasm of facial and forehead muscles produce a sustained smile with raised eyebrows. Intense spasm of chest and/or throat muscles can cause an inability to breath that can be extremely distressing, and is the primary cause of death in tetanus. Other causes of death are pneumonia, blood clots in the lungs, and heart attacks.

How is tetanus treated?

If you are lucky enough to develop tetanus in a developed country, you’ll probably live. It won’t be a fun few weeks, but there are a variety of interventions that can ameliorate the symptoms and speed recovery a bit. First off, any wounds need to be cleaned and cleared of dead tissue that may be harboring spores and toxin-producing bacteria.

Antibiotics aimed at killing the tetanus causing bacteria, as well as any suspected co-infecting organisms, is standard as well. It isn’t entirely clear just how much of a role antimicrobial use plays in recovery, but limited data is supportive. More important is the use of human tetanus immune globulin (HTIG).

I previously described the attachment of tetanus toxin to inhibitory neurons as irreversible. This means that the neuron will not recover function. Therefore a focus on binding up and neutralizing toxin that has yet to find a neuronal home is key, and this is just what HTIG does. This is known as passive immunization and has been clearly shown to improve survival. Recovery from tetanus occurs as neurons regenerate, a process which can take weeks to months.

Other aspects of management focus on symptom control. This can involve the use of sedatives and paralyzing agents in order to reduce the severity of muscular spasms. Magnesium sulfate has been shown to reduce autonomic dysfunction in randomized trials. Other drugs are often used, such as beta blockers and morphine, but they have less of an evidence base to support them.

Patients with severe presentations generally require placement of a surgical airway, such as a tracheostomy, and mechanical ventilation. They may be immobilized and on a ventilator for weeks, which puts them at risk of hospital acquired infections, ulcers, and numerous other complications. Nutritional management is extremely important as well, with most patients admitted to an ICU requiring feeds via a surgically placed gastrostomy tube, or even IV nutrition, in order to meet metabolic needs. Physical therapy is also important in speeding recovery.

Patients diagnosed with tetanus are obviously not immune and require active immunization as well in order to protect them in the future. Both the vaccine as well as HTIG are often given in the ED as tetanus prophylaxis when people seek care for wounds. This depends on whether or not the patient has been fully vaccinated (at least three prior doses), the timing of the most recent dose, and the nature of the wound. For example, if you are fully vaccinated but it has been more than five years since the last dose, you would receive the vaccine if you shoved a dirty nail into your foot. If you weren’t fully vaccinated, the same wound would call for HTIG in addition to the vaccine.

About that vaccine

Developed in the 1920s, tetanus vaccine, known as tetanus toxoid, consists of formaldehyde-treated toxin. This renders it incapable of causing disease but able to coax the body into mounting an immune response. The vaccine does not result in permanent immunity and must be boosted every ten years, although some people begin to have a significant decrease in antibody levels after five years.

Tetanus vaccine was first truly put into action during World War II, resulting in a significant decrease in cases compared to the previous world war. In the late 1940s, the vaccine was introduced in the U.S. (1950s in Australia) as a routine childhood immunization and national reporting was initiated. Prior to the vaccine, the number of cases had been steadily falling with better recognition of high risk injuries and improved wound management, but it has plummeted since in developed regions. In the United States, we have gone from 500-600 cases per year as the vaccine was being rolled out to less than 30 today.

The tetanus vaccine is extremely effective, with virtually 100% of people who have received the full series achieving more than adequate levels of protective antibodies. For this reason, almost all reported cases of tetanus occur in individuals who did not receive the full series or who have gone longer than ten years since their last dose, typically after failing to seek medical care for a high-risk wound. Very rarely do people with evidence of immunity become ill, and there is a clear inverse relationship between antibody levels and severity of disease.

The anti-vaccine community responds

Earlier in the post I mentioned and linked to two blog posts from Australian vaccine denier Meryl Dorey. In her posts, she questions the safety and efficacy of the vaccine, implies negligence on the part of the medical community caring for the child, and asks what the big deal is since loads of kids in Australia are killed by cars every year. There is truly too much pure nonsense to discuss in one post but I’ll pick out a few highlights.
In Respecting our Families, Dorey laments the unfair treatment that the child’s parents have received from the public. This may be a fair concern. Medical professionals caring for the child cannot share private details regarding the reasons that she is not vaccinated and the family has not spoken to the press. We don’t know if this family is rabidly anti-vaccine or simply hesitant to allow their child to receive one or more vaccines. There may also be a true medical contraindication to the tetanus vaccine.

Dorey claims to have spoken with someone who knows the family, and proceeds to give numerous specific details regarding the events leading up to her diagnosis. I don’t know if these details are accurate, but they include an explanation for why the family has “rejected vaccines”:

I was told that this little girl had two members of her family who reacted badly to vaccines – one of whom nearly died from a tetanus shot. Another close relative had a serious reaction to a different vaccine which led to long-term physical problems.

If true, I can certainly understand why a caregiver would be hesitant to give consent for their child to be vaccinated although this would not be considered a true contraindication. The reality however, is that while there certainly are extremely rare severe reactions to vaccines, it is exceedingly more likely that vaccination was not to blame for any long-term problems or near death experiences. But without actual medical information we can’t be sure.

Dorey explains that the child suffered a crush injury to her toe and received what sounds like appropriate medical care. She then relates how three weeks after the injury, a time span that does make sense in the context of tetanus, she began to have what may have been trismus, convulsions (which were likely muscular spasms), and difficulty standing. The following day her condition worsened and she was referred to a hospital where tetanus was diagnosed and treatment initiated.

Dorey claims that several times during the period between her injury and ultimate diagnosis with tetanus, the child’s family asked medical professionals if she might have tetanus. Each time they were reassured. Maybe this is true, although it is more likely that their recollection has been altered by time, stress, new information, and the need to recall the memory over and over again while reducing cognitive dissonance.

Based on Dorey’s description of the events, there doesn’t appear to have been a reason to suspect tetanus during the three weeks prior to becoming symptomatic, nor a significant delay in obtaining appropriate medical care. Yet she implies that a delay in care occurred that prolonged the child’s suffering. If anything, she describes parents that waited until the afternoon to seek medical care for an extremely ill and uncomfortable child that had been up all night having painful muscle spasms and an inability to open her mouth.

She then butchers an attempt to discuss the medical facts about tetanus:

A hydrogen peroxide solution is often applied to deep puncture wounds, forcing oxygen into the site and if necessary, there have been recommendations to apply pressure to the wound, forcing blood to the surface since blood is highly oxygenated.

Hydrogen peroxide use is frequently discussed on anti-vaccine websites. In reality, it would likely increase the risk of tetanus because it injures tissue and is not recommended by medical experts. I would be hesitant to trust any medical information following such inaccurate statements. Like this for example:

Tetanus is very rare in Australia – mainly due to the fact that very few of us now live on the same land occupied by large animals such as cattle and horses who carry tetanus spores in their faeces. We are also much more likely to clean wounds thoroughly and properly when they occur, not allowing them to fester.

Tetanus is extremely rare in Australia because of the tetanus vaccine. Proper wound management is also important, but the implication that the vaccine isn’t the primary reason reveals her bias. She goes on to imply that the vaccine doesn’t really work very well anyway and is dangerous, and she links to a whale.to article.

In Both Doctors and the Media Misinform the Public About Tetanus, Dorey implies that the science behind vaccine safety and efficacy is mere “guesswork.” She also attempts to weasel out of her implication that the medical community failed to appropriately treat the child’s initial wound and to diagnose her tetanus in a timely fashion. This is immediately followed with the overt claim that that is exactly what they did.

It gets worse from there, including this gem:

Now, I will ask you all the question I have asked medicos for nearly two decades without any answer at all – if natural infection cannot convey immunity, how can vaccination do what the disease cannot?

No answer at all huh? Remember when I mentioned how deadly this toxin is? Only Botulinum toxin is more potent. The amount of toxin produced leading to most cases of tetanus is minuscule, yet it can kill the patient. This tiny amount isn’t enough to trigger an immune response, which is why the vaccine is necessary.

Conclusion: Tetanus sucks, get vaccinated

Tetanus is an awful disease and causes great suffering in those unlucky enough to acquire it, and it can be fatal. There is an extremely safe and virtually 100% effective means of preventing it that sadly some parents are denying their children. I can’t say with certainly what the motivation behind this child’s caregivers’ decision to refuse vaccines was, but it was almost certainly influenced by misinformation from people like Meryl Dorey.

Trivia: Tetanus is the only vaccine-preventable illness for which herd immunity does not apply as it is not spread from person to person. It lives in the soil and does not require a human reservoir to survive. Please make sure that you are up to date and seek appropriate medical attention for high-risk wounds.

Here is a description of another case of childhood tetanus recently published by the AAP.

Posted by Clay Jones

Clay Jones, M.D. is a pediatrician practicing at Newton-Wellesley Hospital in Newton, MA, and a regular contributor to the Science-Based Medicine blog. He primarily cares for healthy newborns and hospitalized children, and devotes his full time to educating pediatric residents and medical students. Dr. Jones first became aware of and interested in the incursion of pseudoscience into his chosen profession while completing his pediatric residency at Vanderbilt Children’s Hospital a decade ago. He has since focused his efforts on teaching the application of critical thinking and scientific skepticism to the practice of pediatric medicine. Dr. Jones has no conflicts of interest to disclose and no ties to the pharmaceutical industry. He can be found on Twitter as @skepticpedi and is the co-host of The Prism Podcast with fellow SBM contributor Grant Ritchey.