Less than 15% of patients seek out care for this vicious form of gangrene.
A colourful drawing flutters from Kasimu Yahashe’s lap on to the hospital bed. It is of a cheerful-looking bird, maybe a hen, a bright splash on the dark sheet.
The five-year-old tugs at her mother’s robes.
She is hungry, Kasimu indicates with urgent gestures and low, throaty sounds. Yagana Mustapha gently tilts her daughter’s head back, trickling runny maize porridge from a cup into Kasimu’s throat.
Kasimu can’t chew.
Her upper lip has been eaten away by a deadly, ancient illness that has also ravaged her right cheek, nose and eye. Kasimu has been mutilated by noma, or cancrum oris, a disease known in the Hausa language in Nigeria as ciwon iska, the disease of the wind. Because it’s a mysterious illness with a sudden onset, many people here believe it is caused by spirits or witchcraft — that it is a curse or a spell cast on a person or family.
Noma gets its name from the Greek word nomein, “to devour”. It is a severe type of gangrene that starts as a small lesion on the gums or cheeks before rapidly destroying the soft and hard tissues of the face and mouth, according to the World Health Organization’s (WHO) website. The illness mostly affects children under the age of six and, without treatment, it kills most of its victims. Survivors are disfigured for the rest of their lives.
Yagana has travelled over 1 000 km from Maiduguri to the Noma Children’s Hospital in Sokoto, a city in the arid, hot northwestern region of Nigeria, to seek help for her daughter. This is the world’s only dedicated noma hospital.
In December Kasimu’s father was killed in a bomb attack by Boko Haram insurgents. The militant Islamist group’s drawn-out campaign has devastated northeastern Nigeria. The region has also been hit by drought and Unicef warns that the number of children with severe acute malnutrition is expected to reach 450 000 this year in three states.
Yagana, who was left destitute and struggling to feed her children while mourning her husband’s death, did not notice anything amiss until Kasimu’s cheek started discolouring.
“Her right cheek became very dark and swollen,” Yagana says. “After 16 days the cheek burst open.”
At first Kasimu’s gums were partially exposed, but the disease continued to destroy the tissues of her face and left the girl disfigured.
“It scared me and everyone else in our community. I was really down,” says Yagana.
It is hard for parents to see their children defaced, says Manighandan Sivaramakrishnan, project co-ordinator of the international aid organisation Doctors Without Borders (MSF) at the hospital.
“Sometimes children have stones thrown at them because some people consider it to be the result of witchcraft,” says Sivaramakrishnan.
Despite being described by ancient physicians such as Hippocrates in the fifth century BCE, noma is a poorly studied disease and the cause remains unknown, a 2013 study published in The Lancet medical journal explains.
Often described as “the face of poverty”, it has always been a disease of extreme poverty and malnourishment, according to a 2010 review in the open-access online journal PLoS ONE, and has been reported throughout history in Asia, Europe, South America and Africa. The last reports of noma in Europe were of Nazi concentration camp and prison camp inmates.
In high-income countries, noma is now extremely rare. But it persists in the poorest developing countries and mostly in Africa, although cases have also been reported from Latin America and the Asia-Pacific region.
It is not fully known how widespread noma is or how many new cases there are, researchers write in PLoS ONE, and it is believed that less than 15% of patients seek medical care.
“Noma is a disease of shame, and the condition often results in forced isolation from the community and family; many children are sent to live in isolation rather than being taken into medical care,” states the PLoS ONE review.
The WHO estimates there are 140 000 new cases each year, with the highest numbers in the so-called noma belt that stretches from Senegal to Ethiopia and includes Mali, Niger and Nigeria.
Several aid organisations have called on the WHO to recognise noma officially as a neglected tropical disease, which would transfer it from the medical field into the political domain. In a joint letter by MSF and the aid organisations Winds of Hope and Sentinelles published in January, the authors say the victims of noma “are so neglected that their deaths are not recorded in mortality statistics or in the Global Burden of Disease”. The Global Burden of Disease study captures premature death and disability from more than 300 diseases and injuries in 195 countries and is produced by the Institute for Health Metrics and Evaluation in the United States.
The noma hospital in Sokoto has seen a recent surge in patients from all over northern Nigeria because of the food crisis and increasing poverty in the area, health workers say, with up to 17 cases arriving in one week.
“We find noma where there is malnutrition, poor oral hygiene, bad sanitation and possibly close proximity to cattle,” says Sivaramakrishnan, who heads the MSF noma project’s outreach in the area. They are using radio jingles and flyers to inform people in four northern states of the warning signs of the disease, urging them to seek urgent medical help.
The messages urge parents to inspect their children’s gums regularly for lesions or red or purplish red spots.
This is crucial, explains Sivaramakrishnan. If left untreated, noma is fatal in 90% of cases.
“There are many people who don’t have access to care. We need to take the message to them and we need to sensitise healthcare workers,” he says.
Parents leave their livelihoods behind
The WHO makes it clear that the disease is unlikely to be caused by a single “infectious agent” (virus or bacteria). Factors contributing to malnutrition would be poor oral hygiene and weakened immune systems, such as when a victim is HIV positive or has had malaria or measles.
Noma starts as a sore on the gums or inside the mouth. If detected early, the disease can be rapidly halted, either by following basic hygiene rules or with antibiotics. But without rapid treatment, the patient’s condition becomes life-threatening within days, the WHO warns.
For the survivors, prospects are often bleak. Like Kasimu, many can’t talk and are severely malnourished and dehydrated. Parents of children infected with noma often don’t know how to look after them. Sometimes they stop feeding them, MSF reports on its website. Others take them to traditional healers, with reported cases of these healers using caustic herbs or battery acid in an attempt to treat the children.
Parents who do know about the services at the Noma Children Hospital have to leave behind their livelihoods to trek there. Hulking low and dusty in the sweltering heat, the hospital opened in 1999 with international surgical teams visiting to do reconstructive surgery.
With the worsening security situation many of the international teams pulled out, Sivaramakrishnan says, and MSF, partnered with the ministry of health, offered its services in 2014.
Although there are no accurate official statistics, he estimates at least 250 noma patients are treated each year.
This includes four major “surgical interventions” a year, during which 35 patients at a time undergo reconstructive surgery. MSF brings specialist expertise teams, including plastic surgeons and maxillofacial surgeons, from different countries for each of these interventions.
“Without surgery, survivors of noma disease face not only social isolation but also recurrent opportunistic infections,” Sivaramakrishnan explains. Deaths occur as a result of sepsis or because of difficulty in eating and swallowing, which leads to dehydration and malnourishment.
“Surgeries also help them restore dignity, self-confidence and acceptance from society. This helps to integrate them socially.”
Many patients need multiple operations to restore the lost facial tissue and the healing process may take time — up to years in severe cases.
Psychosocial support sessions
Hauwau Abdullahu, 16, was one of the first patients to be admitted to the hospital in 1999 and also one of the first to be operated on by MSF surgeons in 2014.
The teenager was only three when she contracted the disease. It affected her nasal cavity and upper lip, forcing her to breathe through a small opening.
“I’m no longer a patient, but I come here all the time,” Hauwau explains, speaking slowly but clearly.
She is learning to weave cloth, listening intently to instructions from an experienced teacher on the verandah outside the women’s ward.
These psychosocial support sessions are as important as the surgery, says Sivaramakrishnan. Although patients are put on strong antibiotics and nutritional support on arrival, they are also counselled.
Adults and teenagers are told what to expect from society and given advice on how to respond. Parents are given guidance on nutrition and good oral hygiene.
“Some patients, especially the children, are very withdrawn. They don’t interact with friends or they are very aggressive.
“We reduce the anxiety level by giving them mental healthcare. This includes play therapy, early stimulation, individual and group counselling and physiotherapy services,” says Sivaramakrishnan.
The long process is worth it, Sivaramakrishnan says.
“The community generally accepts them well after surgery. There are noma patients who have been employed by the government and are leading a normal life.
“Some of the patients, especially the adults, are employed in the hospital and now they are productive members of society. Some have even married, they have their confidence restored,” Sivaramakrishnan explains.
Hauwau insists she has no plans to marry yet. But the surgery has given her back a life and she feels comfortable at the hospital.
“I’m not ashamed of my face, but I would really like an upper lip,” she says, running a bright red thread through her fingers.
— Additional reporting by Adri Kotze
Festus Iyorah is a Nigerian freelance journalist and photographer covering global health, conflict, social innovation, gender equality, technology, and development. His work has been published by Al Jazeera, Guardian (UK), Mail & Guardian, Newsdeeply, Ozy, Stanford Social Innovation Review, World Politics Review and more.
