WHO considers adding ‘noma,’ a rare childhood disease, to its list of neglected conditions

It’s a disease of children living in extreme poverty. Its cause is uncertain. So is the number of children it affects.

But soon, the devastating condition known as noma may be a bit less neglected.

The World Health Organization is currently considering a request to make noma the 21st neglected tropical disease on its list. Elevating noma from “neglected-neglected,” as it’s been called in the medical literature, into just plain neglected will take some doing, but it could also make a difference — mobilizing resources for research on prevention, diagnosis, and treatment, and increasing funding for clinical care and information campaigns.

The name “noma” comes from an ancient Greek word for the process of devouring, and the disease has been around for a long time. Hippocrates mentioned it in the 5th century B.C., and cases in Europe were described in 1649 in the first book known to have been written about neglected diseases, “Observationes Medicae de Affectibus Omissis.” As living conditions improved around the world, noma retreated into isolated areas of extreme poverty in Asia and Africa; it made a brief reappearance in the brutal conditions of the Auschwitz-Birkenau concentration camps during World War II.

The disease is unmistakably horrific. It starts as bleeding and ulcerated gums, generally in children between the ages of 2 and 6. Immune systems weakened by malnutrition or other infections or by poor living conditions and oral hygiene appear to trigger it. Within weeks it can kill enough bone and soft tissue around the face to make eating, breathing, speaking, even seeing difficult, and cause severe facial disfigurement.

Noma is not contagious, and no unique infectious organism has been found. But studies have identified several commonly occurring bacteria that normally don’t cause problems. The disease is preventable with adequate nutrition and oral hygiene, and it can be cured within the first few weeks of onset with antibiotics. Surgery later on can reduce pain, help people eat and breathe, and sometimes lessen disfigurement.

Incidence numbers are squishy. Estimates run from 35,000 to 140,000 people in Asia and Africa, mostly children. (WHO uses the higher estimate.) There’s less debate about the death rate. About 85%-90% of children with untreated cases die of blood poisoning, dehydration, or malnutrition.

The face of neglect

In 2021, a reporter at The Guardian popped “noma” into Google and found page after page of references to Noma, an immensely popular restaurant in Copenhagen scheduled to close next year; the situation isn’t much different today.

“It’s a vicious circle,” said Mark Sherlock, a health adviser to Doctors Without Borders, one of several NGOs trying to bring attention to the disease. It’s hard to get research funding and support for noma without it being on the WHO’s list, but it’s hard to get it on the list without knowing more about it.

The disease even has a low profile in many of the impoverished areas where it strikes. Parents may not recognize it, or have access to adequate treatment, or be able to afford antibiotics, or be able to get their child to a health center in time. Few health workers are trained to recognize it. And noma can masquerade as other conditions, such as leprosy, cancer, or a tooth abscess.

What it means for funding

The biggest funders in the global health world have so far not shown much interest in noma.

The Gates Foundation currently has no projects related to the disease. According to a spokesperson, the foundation focuses mainly on eight diseases identified by the London Declaration on NTDs issued in 2012, a list endorsed by a public-private partnership that includes the foundation, the Drugs for Neglected Diseases Initiative, USAID, the World Bank, UKaid, and 13 major drug companies. A representative for Wellcome said the giant U.K. charity, which plans to spend $16 billion in the next 10 years on mental health, infectious disease, climate change, and discovery research has not received any applications for funding noma research.

Interest in noma on the ground has been growing, however. Some countries “but not enough,” have programs, said Claire Jeantet, a former campaign manager for Doctors Without Borders and a filmmaker who co-produced a powerful documentary about the disease. In addition to Doctors Without Borders, NGOs such as Winds of Hope and Hilfsaktion Noma are deeply involved.

The WHO has a program as well that many would like to see expanded. In Senegal, efforts by the Ministry of Health and Social Action to inform people about noma have increased the number of reported cases from four or five a year to 19 cases so far this year. Codou Badiane Mané, a dentist and head of the oral health division of the health ministry, said the cases are often identified in time to avoid disfigurement or death.

She remembers seeing her first patient with noma. “I found it hard to believe that it could be possible to survive this mutilation and suffering,” she said via email. “My heart was broken by the suffering of patients and parents.”

The push begins

In February 2021, several organizations held an online international conference for policymakers, government representatives, physicians, and health care providers to go over what had been done, and what needed to be done.

At the WHO’s World Health Assembly the following year, Nigeria’s minister of health gave the country’s support to the campaign, and in January 2023, Nigeria submitted its request to the WHO.

There has been little public pushback to adding noma to the list, but some NTD researchers are concerned that, in the face of diminished funding for NTDs, the more diseases that go on the list, the fewer resources there will be for any one in particular. And some people suggest that because noma is a result of poverty, eradicating the disease will require eradicating poverty. “Poverty is a driving factor,” said Sherlock, “but so is lack of access to health care and childhood vaccinations, and malnutrition that harms the immune system” — which, with some effort, can be readily addressed.

Other concerns are that WHO is already overstretched, given its limited finances, and there are other “neglected-neglected” diseases that also need attention. Nilanthi de Silva, a former head of the WHO advisory group on neglected diseases, said she thought about those factors when another neglected disease, mycetoma, came up before the committee. The committee and WHO did add mycetoma to the list in 2016, as well as snake bite in 2017, and snake bite only had half the number of countries supporting it compared to noma.

Getting the attention of WHO

About 10 years ago, some NGOs lobbied the WHO to include noma on its NTD list, to no avail. But seven years ago, the WHO adopted a “systematic technically driven process” designed by its Strategic and Technical Advisory Group (STAG) for Neglected Tropical Diseases, and the current attempt is using the new rules.

The process starts with a WHO member country submitting a request to its local division of the WHO with evidence that includes peer-reviewed scientific publications and a description of the disease’s impact. That then goes to a regional division, and then finally STAG considers it and makes a recommendation to the WHO’s director-general, who ultimately decides. The criteria: The disease should disproportionately affect the poor, causing illness, death, and stigma; affect people in tropical and subtropical areas; be treatable, preventable, or even eliminable; and of course, be neglected. Noma fits all the criteria.

But committee members have a lot to think about when considering a “neglected” designation. De Silva, now a professor of parasitology at the University of Kelaniya in Sri Lanka, was head of STAG at the time it came up with its general recommendations — recommendations she felt were very needed. “You can’t just have random scientists push something because that’s the work they’ve been doing for 20 years,” she said.

Nigeria submitted what is said to be a thorough, well-researched dossier (the WHO has not released it publicly) that was discussed at a closed STAG meeting earlier this month. De Silva is no longer on the committee, but she has confidence that it will make the right recommendation, in part because of the committee’s makeup — it is impressively representative of WHO’s membership, not its funders. “A lot of the time final conclusions and decisions about global health are made by people who have the funds. But the needs in the NTDs arise not from those countries, but from the developing world,” she told STAT.

Where things stand

The WHO should recognize noma, said Codou. “It needs more visibility and more interest on the part of the powers that be and partners in order to carry out a more effective fight,” she said.

Since the failed attempt to get noma on the list 10 years ago, there have been several more peer-reviewed scientific papers published, and WHO now has a clear path forward for applications. “My sense is this one will be successful,” said Sherlock of Doctors Without Borders.

But recognition by the WHO won’t be the end of noma. “It’s not a silver bullet,” he noted. The possible addition of noma “does not automatically translate into an increased resource allocation,” according to a WHO spokesperson.

If the WHO says no or not this time, said Codou, “Senegal will continue to work to raise awareness.”

So will Doctors Without Borders. “We’d need to regroup and rethink,” said Sherlock. “Hundreds of thousands of people are being affected and we do not want them to be forgotten. We will continue the fight that we started.”

Correction: An earlier version of this story misstated the timing of the World Health Assembly where Nigeria’s minister of health gave the country’s support to the campaign to add noma to WHO’s list of neglected diseases.