'Strangling angel' of diptheria caught Spain off guard – here's how

The return of diphtheria in Spain after nearly three decades highlights the challenges posed by infectious diseases that had been mostly eliminated from Europe.

Falling vaccination rates, complex population movements, and the disappearance of international health practices perceived as redundant, all contribute to the emergence and spread of infectious diseases that were thought forgotten. At the same time, such public health crises throw light on the delicate relationship between state and citizens, and competing concepts of responsibility for health.

A six-year-old boy from the Catalan town of Olot was admitted to hospital suffering from diphtheria last week. This was the first case of the disease recorded in Spain for 28 years. The boy, who is in a critical but stable condition in a Barcelona hospital, had not been vaccinated due to his parents’ concerns about the safety of the vaccine. They now say they feel tricked by the anti-vaccination groups which had originally stoked their fears.

Diphtheria, now a rare disease in Europe, is a serious, potentially fatal disease caused by bacteria that can cause heart failure, pneumonia and paralysis of the muscles used for swallowing. Up until the 1920s it was one of the leading cause for death in children, sometimes referred to as “strangling angel”, because the bacteria can create a pseudo-membrane in the airways, causing death by choking.

It was widespread in Spain during the first half of the 20th century, particularly following the physical and economic devastation caused by the Spanish Civil War. Over 27,000 cases of diphtheria were recorded in 1940 alone. Improving infant health was officially a priority for the new Franco regime, and cases did reduce significantly in the post-war era. However, Spain’s impoverished and fragmented public health system failed to make the rapid progress towards eradication achieved by its western European neighbours, with almost 250 new cases a year still reported in the late 1960s. A comprehensive vaccination programme was finally implemented in 1966 and until last week, there had been no diphtheria cases recorded since 1987.

Franco meets nurses in 1939.Teresa Avellanosa, CC BY

Russia to the rescue

As diphtheria had been eliminated in Spain for the last three decades, stocks of the anti-toxin needed to treat the disease are no longer available in the country. This anti-toxin, listed as an “essential medicine” by the World Health Organisation, is increasingly unavailable due to the disease’s rarity and because, as a blood-derived product, its production is highly regulated.

For instance, in the US there are no licensed anti-toxin products available in the whole country, and in case of diphtheria an unlicensed Brazilian product is used by the CDC under investigational new drug status, which basically provides an exemption from federal regulation. In Europe, some public health experts have called for the establishment of a central EU stockpile that all countries can access in times of emergency.

This time, Spanish health authorities were forced to look abroad for supplies, which were personally flown in from Moscow by the Russian ambassador to Madrid. The difficulties in locating and supplying the relevant anti-toxins highlight how quickly international structures can break down once a disease has been eliminated in a particular country and has disappeared from the authorities’ radar.

The recourse to diplomatic channels and the ad hoc supply of medication echoes the chaotic situation more usually associated with times of war and international emergency. A look into the Spanish government archives reveals that during the World War II, for example, Spain’s small West-African colony in what is now Equatorial Guinea faced a dangerous outbreak of Yellow Fever. Facing the collapse of its economy and wartime disruption to international supply, the Spanish government struggled to secure basic medication and to distribute the relevant vaccines both at home and in its overseas colonies.

Help eventually arrived from the local British Consul who agreed to fly to Lagos and bring back supplies of the vaccine in thermos flasks. While these kind of ad hoc solutions may have been a necessary during a time of international conflict, they seem profoundly out of place in today’s apparently more orderly international system.

A lingering presence

It was no coincidence that the anti-toxins were available in Russia. Diphtheria cases had begun to fall in the Soviet Union following the introduction of universal childhood immunisation in 1958. By the mid-1970s they had plummeted to an all-time low, approximately the same level as the US. However, changing immunisation schedules contributed to a rise in diphtheria in the 1980s, partly because of reduced levels of public support stoked by a strong anti-vaccination movement that channelled distrust in the state during the period of perestroika. With the collapse of the Soviet Union and much of its public health services, especially in the newly independent states, a severe epidemic broke out in 1993.

Shortages in vaccine supply, economic hardship and mass population movements all contributed to the outbreak and the difficulties authorities faced in controlling it. Eventually diphtheria in Russia and the former Soviet states was brought under control through international cooperation between governments, NGOs and UN agencies. Since then, the former Soviet republics and Russia have remained the only area of Europe where diphtheria is still a public health concern. Although no longer in epidemic proportions, the lingering presence of the disease led Russian authorities to keep stocks of diphtheria anti-toxin readily available.

Spain’s fascist past complicates the debate

Long-forgotten diseases that make a comeback also bring to light problematic relationships between citizens and the state. Recent American anti-vaccination movements highlight conflicting ideas of individual and public health, while Spain’s authoritarian legacy has coloured the vaccination debate in the country. This week Luis Garciano, economic spokesman for Spain’s new centrist party Ciudadanos (Citizens), said that unvaccinated children should be withdrawn from school and their parents fined and stripped of benefits.

Garciano’s comments have been denounced as “neofascist”, and anti-vaccination campaigners have frequently drawn parallels between the idea of obligatory vaccination and Spain’s fascist past. For them, the right to reject vaccination is a freedom that should be protected in the era of democracy.

The return of diphtheria to Spain mirrors the measles, TB and other infectious diseases in Europe and the US. When diseases disappear due to high vaccination coverage, the national and international public health infrastructures needed to deal with them often also whither away.

When distrust in public health organisations and practices or the break-down of relationship between state and citizens give way to faltering vaccination rates (whether through anti-vaccination movements or through lack of access to vaccines), forgotten diseases can make a quick comeback. The case of diphtheria reminds us of the very real stakes at hand when infectious diseases re-emerge and throw light on the personal, national and international consequences of declaring the end of a disease.

Dora Vargha receives funding from the Wellcome Trust

David Bryan receives funding from the Wellcome Trust