The facts about...

Rabies

What is rabies?

Rabies is a viral infection to which all mammals, including man, are susceptible. The disease occurs when there is direct contact to infectious saliva and occasionally other bodily fluids through bites, scratches, broken skin. The incubation period ranges in general between 2 and 3 month (2 weeks to 6 years are reported) depending on the site of infliction, the amount of virus and the virus strain. It causes an acute and almost invariably fatal infection of the brain both in animals and humans.

The history of rabies infection

It is one of the oldest known animal diseases transmissible to humans. It has been present throughout the recorded history, and literature, and very likely predates the evolution of humans. The first description of the disease dates from the 23rd Century BC in the Eshuma Code of Babylon. Democritus provided a clear description of animal rabies in about 500 BC. Antiquity, did know rabies as well as the link between human disease and animals, especially dogs. But, it is a famous Italian scholar, Girolamo Fracastoro, born in Verona, who described the human disease, which obviously he had seen in many patients, and its routes of transmission in 1530, i.e. 350 years before Louis Pasteur.
According to records, wound cauterization (burning) was the preferred treatment in the 1st Century AD, and this had remained the only real therapy for a long time. Another ‘remedy’ that remains part of popular culture (with little recognition of its origin) was the hair of the dog that bit the patient.
In medieval villages, people bitten by wolves were tied to poles, and given only solid food (using long sticks, to avoid contact). Those who started begging for water were considered uninfected, since fear of water is one of the symptoms of rabies in humans. Hence the alternate name "hydrophobia".
In the 19th Century, canine or street rabies was a scourge everywhere, especially in Europe. Fear of rabies, related to the mode of transmission, the absence of any efficacious treatment, was almost irrational. Patients killed themselves or were killed when bitten by a dog believed to be rabid.
In this world of irrational terror the first post-exposure treatment in 1885 gave Louis Pasteur an international aura that his previous major scientific works had not been able to provide. A 9-year-old boy was the first person to have received an effective shot for rabies. In 1885, Joseph Meister was bitten by a rabid dog. His parents went to the famous French microbiologist Louis Pasteur. They begged him to help their son. Pasteur thought that if he injected a weak form of virus from one rabid animal into another, the second animal might be able to fight off the disease. He tried this hypothesis out on Joseph. The boy survived and lived a long life. That was how people starting giving shots for rabies. After this success other rabies vaccines were made.

How is rabies infection transmitted?

In animals, during the course of the illness there are disturbances of behaviour, which in some affected species, such as dogs, cause them to attack and bite other animals and occasionally humans. In many parts of the world (but not in the British Isles, some other parts of Western Europe and Australasia) rabies is found in terrestrial wildlife species. In Asia, Africa and Central and South America it also commonly infects domesticated animals such as dogs and cats, but rabies in domestic animals is very rare in Europe and North America. Bites from any of these animals can transmit the infection to other domestic and farm animals, and man. However, more than 99.9 % of human deaths from rabies reported worldwide result from the bite of a rabid dog. Bats can also transmit rabies and rabies-like viruses by bite or scratching. A rare route of infection is the inhalation of infected bat secretions in caves inhabited by bats.
Except through corneal transplantation, transmission of the virus from a human case to another human is unknown and there is no evidence that infected patients are a hazard to their families or health care workers looking after them, though vaccine should be offered to these close contacts.

What is human rabies infection like?

The incubation period in man is usually three to eight weeks, but may be as long as several years. The range of non-descript symptoms in patients, are numerous. Early symptoms may include itching, pain or numbness around the site of the bite (which has usually been healed by the time the first symptoms appear), fever, headache, and general malaise. General symptoms may then develop such as fever, tiredness, headache, muscle ache, anxiety, depression. The patient then develops either furious or paralytic rabies. However, such clinical symptoms are not always observed.
Furious rabies is the most common presentation in humans. The patient develops periods of generalised arousal characterised by agitation, aggression and hallucinations. These periods alternate with clear intervals. The patient is often unable to swallow the saliva, which is usually abundant and viscid, so that it hangs about the mouth and is expelled with difficulty. Vomiting is common.
In “dumb” rabies, the initial symptoms are followed by paralysis of the muscles and eventual paralysis of the respiratory muscles. Hydrophobia is not usually a feature of dumb rabies. In either form, the symptomatic course usually runs 2 to 14 days before coma supervenes. Once symptoms have developed rabies is almost invariably fatal. Death occurs an average of 18 days after the onset of symptoms, but the range is broad. Only two patients have been reported who recovered and a further four patients survived with severe neurological defects.

How is rabies infection diagnosed?

During the incubation period, no diagnostic studies in the patient are useful. Once symptoms develop, a number of diagnostic tests are available, including testing serum or cerebral spinal fluid, skin (nape of neck) or brain biopsies. Due to the nature of the disease progression it is necessary to test multiple samples over a period of days and weeks.

Epidemiology of rabies infection

Rabies is endemic in most countries of the world. However, several countries are currently classified as rabies-free including the UK, Iceland, mainland Norway, Sweden, Finland, mainland Spain, Portugal, Cyprus and other Mediterranean islands, New Guinea, Bali, New Zealand, Antarctica, Oceania, peninsular Malaysia, Singapore, Japan, Taiwan and Hong Kong islands. Although the UK is classified as rabies-free, in 2003 it was recognised that UK bats may carry a rabies-like virus; European Bat Lyssavirus type 2 after a Scottish bat handler had died from infection with this virus. The European Bat Lyssavirus type 2 is present in around 4% of Britain’s Daubenton’s bats.
The World Health Organization has estimated the annual number of human rabies deaths to be between 40,000 and as high as 70,000. Most of these deaths take place in developing countries, particularly in South and South East Asia.
In the UK the last human death from indigenous classical rabies occurred in 1902, and the last case of indigenous terrestrial animal rabies was in 1922. Of the few cases that have been reported in the UK almost all have occurred within quarantined animals, or in people infected abroad. Since 1946 there have been 22 UK deaths in people infected with rabies abroad.

Prevention of human rabies

There is no risk of classical rabies from terrestrial animal bites in the British Isles. For those who through occupation and/or travel risk close contact with rabid animals, e.g. workers in quarantine facilities and veterinarians working abroad, pre-exposure rabies immunisation provides protection against classical rabies and has shown to provide protection against EBLV-1 and 2. Pre-exposure rabies prophylactic treatment (rabies vaccination) is not a general requirement for travel to any country. Based on the local incidence of rabies in the country to be visited, the availability of appropriate anti-rabies biologicals, and the intended activity and duration of stay rabies vaccination may be recommended for international travellers. Travellers with extensive unprotected outdoor exposure in rural areas, such as people engaging in certain occupational activities (veterinarians, animal workers, etc) or tourists with activities like trekking, bicycling, camping, hiking, etc might be at high risk in rabies-endemic regions even if their trip is brief. Therefore travellers visiting rabies-endemic countries are advised to seek advice on the need for pre-exposure rabies vaccination.
The standard pre-exposure vaccination schedule is administered on days 0, 7 and 28, but more rapid courses can be given if necessary. Regardless of whether a traveller receives pre-exposure vaccination (s)he should be advised to seek medical attention if bitten by a mammal. Anyone who regularly handles bats in the UK should also be vaccinated against rabies (due to the risk of European Bat Lyssavirus Type 2infection).
Pre-exposure vaccination does not eliminate the need for additional medical attention after a rabies exposure but simplifies post-exposure prophylaxis in populations at risk by eliminating the need for rabies immune globulin and by decreasing the number of doses of vaccine required.
The cornerstone of rabies prevention is wound care, potentially reducing the risk of rabies by 90%. Therefore, any animal bite or scratch should receive prompt local treatment by thorough cleansing of the wound with soap and water; this local treatment will substantially reduce the risk of rabies as the virus. Travellers who might have been exposed to rabies should always contact local health authorities of the respective country immediately for advice about post-exposure prophylaxis and vaccine availability and should also contact their general practitioner at home as soon as possible thereafter. Those who have received pre-exposure vaccination will receive a further two doses following a potential rabies exposure. Such persons do not require rabies-specific immune globulin.
Those who have not received pre-exposure vaccination require a five dose, post-exposure vaccination schedule plus rabies immune globulin. If necessary the assistance of local officials should be sought to ensure that the animal is observed. Thirdly, advice should be taken from a local doctor about further wound treatment and rabies immunisation.

So what should you do if bitten by an animal?

Any person who has been bitten by a dog or other animal in a rabies-endemic country should be considered to be at risk of developing rabies. After the incident it is very important that the wound is flushed and thoroughly washed with soap and water or alcohol. Secondly, the name and address of the owner of the animal should be obtained and where possible the animal should be observed for 10 days to see if it begins to behave abnormally. If the animal is wild or a stray and observation is impossible, and if the doctor knows that rabies occurs in the locality, you may require immunisation. The rabies vaccines manufactured in Europe and North America are now widely available and safe, but expensive. If you are advised that the risk of rabies in the implicated animal is high and vaccine is unobtainable it will be necessary to travel to a larger centre or return to the UK for proper treatment.
If you are worried about an animal bite that you have sustained abroad, your general practitioner will advise you whether treatment is needed. His or her local Health Protection Unit can provide doctors with current information on rabies throughout the world and suggest the circumstances in which immunisation is appropriate. If you are bitten by a bat, whether in the UK or abroad, you should also seek your general practitioner’s advice.
Handling sick or grounded bats in the UK may represent a risk of contracting the European Bat Lyssavirus Type 2 (EBLV 2). If you find a bat do not handle it. If it seems to be injured or sick, or needs to be moved, seek advice from a local bat conservation group or the Bat Conservation Trust Helpline on 0845 130 0228 (http://www.bats.org.uk/). In Scotland you can also contact the Scottish SPCA on 0870 7377722.

Prevention of animal rabies

Although control of animal rabies is central to prevention of human disease, few nations have managed to eliminate it. Certain areas within the European Union are regarded as rabies free and there is a tremendous effort in to control and eradicate the disease. Therefore, pet travel has been subject to strict laws and regulations. This is meant to avoid spreading or reintroducing the disease. Basically these rules applying to the movement of dogs, cats and ferrets depend on the rabies epidemic situation both in the country of origin and in the country of destination.
In those parts of Western Europe where rabies is present in wildlife, it mainly infects foxes. Strenuous measures to control European fox rabies, for instance by vaccine left in baits, has reversed the movement of fox rabies towards the Channel ports, while strictly enforced quarantine regulations continue to prevent the introduction of other infected animals into Britain. In spite of its endemicity in foxes, rabies is rare in other animals in Western Europe. If you would like to take your pet abroad or if you want to import pet animals from abroad you should seek advice from your vet.

A final word of warning

An animal bite can spoil a trip abroad and give rise to needless anxiety. When you are abroad avoid unfamiliar animals and if you are travelling in a high risk area, e.g. trekking in Asia, keep your arms and legs covered. If you are bitten, promptly wash the wound, identify the animal and seek advice. If you are planning a walking or back-packing holiday in Asia, Africa or Central or South America, consider paying for immunisation against rabies before you go. It will not remove the need for treatment if you are bitten, but it will give you a large preliminary measure of protection.
Human rabies is a fatal but avoidable disease: learn how to avoid it.