Disease out on a limb

A disease in which limbs swell tends to affect the poorest farmers in remote regions of China, preventing them from working, …

A disease in which limbs swell tends to affect the poorest farmers in remote regions of China, preventing them from working, writes CLIFFORD COONANin Beijing

IT’S A strange, terrible and puzzling disease that tends to affect the poorest farmers in the most desolate parts of China, including the Tibetan plateau and Shaanxi province, causing swelling of the elbows, knees, wrists and ankles, slowing the growth of limbs and causing dwarfism in the worst cases.

As their cartilage dies and their joints swell, victims are unable to lift the tools they need to farm to survive. The disease is particularly hard on the young, and there is no known cure, only relief.

The images from these regions of those suffering from Kashin-Beck disease make for gruesome viewing – gnarled fingers and knee joints where the cartilage seems to have simply died away.

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Many are forced to take to crutches to get around, and the absence of any support network beyond that offered by your family means that many sufferers have to try to get by on their own.

The illness was first identified by Russian doctors and medics serving with Cossack troops near Lake Baikal, and has been recorded in Siberia and North Korea. In most areas where it is prevalent it is often referred to as “big bone disease”.

The disease now chiefly occurs in 15 Chinese provinces. Advances in nutrition and storage techniques mean it has largely disappeared elsewhere, but it has proven dogged in remote valleys around the Tibetan capital Lhasa and elsewhere on the Tibetan plateau.

In China, the health ministry reckons up to three million people are affected by Kashin-Beck disease, and 30 million are living in endemic areas.

The disease was at its worst in the late 1950s, when in many severely hit villages up to 90 per cent of children got sick. There are stories of apparently healthy children suddenly developing swollen joints before their growth was stunted.

In adults, similar symptoms can cripple once healthy farmers, who are left unable to make a fist.

However, the Chinese health system is under-funded and has more pressing illnesses to worry about, such as tuberculosis, so “big bone disease” is not a priority.

Little is known about Kashin-Beck disease, and the cause is still a mystery, although a poor water supply, fungal toxins in grain and iodine deficiencies have all been mooted as possible causes.

A lack of selenium and other nutrients in the diet and farming in mineral-poor soil are also contributing factors.

“The other students and I are really happy to come to a new school. It means we won’t get this weird disease,” says Huadan Jiamucuo (14), a Tibetan student who comes from Sichuan’s Aba county.

His hometown is Hezhi, which has been badly affected by Kashin-Beck disease and is one of the towns where the government follows a policy of “education in other places” – he has been sent to boarding school in a non-affected area. His father died young and his mother has Kashin-Beck and is unable to work.

“Teachers often give me remedial teaching. My classmates are also eager to help me. My grades have improved. The most important thing is that we never have to worry about the Kashin-Beck disease,” says Huadan.

Huadan is one of thousands of students from Aba county who have been moved out of the affected areas. It’s a difficult disease to combat as it is still under-researched and there is little documentation of the illness.

The remoteness of the areas and the lack of funding until now means that there has been little opportunity to carry out measures such as randomised controlled trials.

Some 312,000 farmers and herdsmen from nearly 60,000 families have been moved from shanty homes into new solid brick houses in Tibet this year under a government-subsidised housing project aimed at improving living conditions.

The Chinese government has introduced a plan with a total investment of €25 million to allow 26,000 students from Aba to study in boarding schools in non-disease areas.

“This means children can get away from the disease area during an important growth period. The disease chain could be cut off and the education they receive means they can provide a local back-up, high-quality labour force. This policy has a multiplier effect,” says Fu Ming from the Civil Affairs Office of the education department in Sichuan province.

“We will expand and build from scratch 70 schools for this project.”

The majority of schools are already completed and all children from Kashin-Beck disease areas will receive compulsory education free.

“Students from poor families can also enjoy a boarding living allowance, with the subsidy of 100 yuan [about €10] per person per month,” says Fu.

The policy of beating the disease is not only focused on education in other areas, but also on people giving birth in other non-affected parts and even by huge relocation projects. Tens of thousands of people have been moved out of the worst-affected areas in Sichuan and Shaanxi provinces.

Other ways of combating the disease seem to have to do with changing the way that the grain is stored, which has proven successful in Heilongjang province and in North Korea. A clean environment can help to stop fungal growth in the grain.

Also changing water sources to deep well water has been reported to decrease the contamination rate.

One of the most active groups in combating the illness has been the Belgian wing of Médecins Sans Frontières, which runs a physical therapy programme which aims to alleviate the symptoms of KBD patients with advanced joint impairment and pain in Lhasa prefecture. After running the MSF programme for 10 years, Dr Francoise Mathieu continued the programme in 2002 under the auspices of a new organisation, the Kashin-Beck Disease Fund (KBDF). Even though there is a fair degree of mystery surrounding the disease and its origins, and indeed ways to cure it, the incidence of dwarfism and other serious effects of the disease are slowly lessening, even in the worst-affected areas.

Speaking to Sciencemagazine recently, Liu Yunqi, a professor at Harbin Medical University's Kashin-Beck Disease Institute, predicted that by 2020, there will be no new KBD cases.

“The disease will be totally under control,” he says.