Biomedical Ephemera, or: A Frog for Your Boils

Ways to Die: Noma

Noma, also known as stomatitis gangrenosa or cancrum oris, is a rapidly-progressing polymicrobial ("multiple microbes") opportunistic infection that takes hold in children and infants (and occasionally adults) with compromised immune systems, due to autoimmune or nutritional deficiencies. It has historically also occurred in children recovering from infectious diseases, such as scarlet fever or measles, and has been known since the time of Galen.

The condition often begins with a mild sore throat and mild swelling of the oral mucosa. This stage often persists for days or weeks before progressing, and if the condition is caught in time, mild antibiotics and nutritional rehabilitation are all that is needed. 

However, it's often not caught until the obvious symptoms begin. These include oral ulceration, extreme swelling, and intense pain. Within a few days, a black furrow appears within the swelling, and the gangrenous process begins. The tissue on either side of the furrow dies and dries up, and when the scab falls away, a gaping hole is left in the face. If the condition is not caught until the later stages, the infection and tissue necrosis often overwhelms the body, leading to a mortality rate of over 90%.

It's much easier to prevent this condition with proper nutrition than it is to cure it. While modern treatment of any stage involves antibiotics, the condition largely disappeared from the Americas and Europe before antibiotics were even available, because of increased understanding of nutritional requirements for a healthy body, and programs to feed the under-privileged.

By the 20th century, it had all but disappeared. Its connection to poor nutrition and an unhealthy immune system was once again proven during WWII, when prisoners at Auschwitz and other Nazi work camps developed the condition while recovering from the infectious diseases that ran rampant.

------------------

As great as it is that most of the world doesn't have to deal with this horrible condition these days, it's nowhere near as rare as it should be. In many rural parts of Africa and Southwest Asia, noma still affects children, especially when there are famines.

The WHO estimates that there are nearly 140,000 cases of noma every year, with over 125,000 deaths from the condition. The vast majority of these cases are in sub-Saharan African children between 2 and 12 years old.

Of those that survive, the disfiguring condition can cause lifelong pain and suffering, including shunning, constant pain, and an inability to eat. Foundations such as Facing Africa, AWD Stiftung Kinderhilfe, and Dutch Noma Foundation work with regional hospitals in Africa to bring in reconstructive surgeons and dentists in order to help the survivors live a more normal life. 

In 2010, BBC Two produced a documentary with Facing Africa, called "Make Me A New Face: Hope For Africa's Hidden Children", which is available to watch on YouTube.

Images:

The Diseases of Infancy and Childhood. L. Emmett Holt, 1897.

Diseases of Children. Benjamin Knox Rachford, 1912.

Facing Africa: Images Of Noma

A case of cancrum oris following whooping cough. While whooping cough was resolving, a black sore was noted on the cheek. The sore remained there for 5 days, and on the fifth day spread to the gums and all the nearby teeth fell out. Cheek sloughed away soon after. Standard treatment for noma (gangrenous stomatitis) was given, but to no avail. Child died within 72 hours. Though noma was and is a serious disease, it was rarely so quickly fatal, especially when treated. On autopsy, the bronchi were inflamed and filled with mucous as happens with children who die of broncho-pneumonia.

The author of the book noted that noma and cancrum oris were two separate conditions. From what I can find in the modern literature, cancrum oris is now considered an antiquated term for noma, and noma is a disease of varying degrees. 

Somewhat frighteningly, the only successful method of arresting the advance of what was known as "cancrum oris" was noted to be destroying all gangrenous tissue with nitric acid. These days, very strong antibiotics and greatly improved nutrition are the typical cures, though gangrenous tissue does still have to be excised in the end. At least we don't have to burn it off with acid.

From The Surgical Diseases of Children. By J. Cooper Forster, 1860.

Noma, also known as cancrum oris or gangrenous stomatitis. Used to sometimes present itself after serious infections like scarlet fever, chicken pox, measles, or TB. More likely to occur if the patient is malnourished or immunodeficient, and was observed in the Nazi concentration camps (Josef Mengele is actually the one that found out a lot of what we know about the disease progression and mechanism). Still occurs in places like Cambodia, Ethiopia, and Nigeria, but is extremely rare in industrialized nations.

Usually found in kids 2-6 years old. Starts with a low fever and swelling in the cheek for one to two days, then suddenly progresses to the gangrenous phase. Gangrenous regions form from the oral cavity, extending to the lips, cheeks, and sometimes maxillary and mandibular bones. Progresses extremely quickly and tissue destruction in necrotic zones can result in the loss of the jaw and cheekbones. Most children die of septicemia during this phase. 

Disease can be halted with IV antibiotics and improved nutrition, but full recovery can take up to a year. Without treatment, mortality approaches 100%. Even with treatment, mortality is around 60%, due to the rapid progression of the disease.