NOMA: THE LATEST NEGLECTED TROPICAL DISEASE SPOTLIGHTED BY THE WHO

Noma disease, also known as noma cancrum oris or noma gangrene, is a devastating gangrenous disease of the mouth and face that primarily affects malnourished children in developing countries, although cases have been reported in other regions.

Noma's history stems from the 16th and 17th centuries in classical and medieval European societies. The condition was known as "water canker" or "water cancer" until Dutch surgeons differentiated it from "cancer."^[1]

The hallmark of noma is rapid tissue destruction in the face, particularly in the oral cavity. This “face-eating disease” typically starts as a small ulcer in the mouth that can quickly progress to necrotizing gingivitis, a condition in which the gums become inflamed and begin to die.

If it stays untreated, the infection can spread to surrounding soft tissues such as the cheeks, lips, and nose, leading to extensive facial destruction and loss of teeth.

Noma has a high fatality rate of almost 90 percent, with an annual worldwide prevalence of 140,000 cases. Serious complications can plague untreated survivors, such as severe facial disfigurement and speech impairments.^[2]

As a bacterial disease, noma is not contagious. It emerges from an intricate interplay of factors like malnutrition, poor oral hygiene, and depleted immunity caused by infections like malaria and HIV. Effective medications exist that can combat the disease, yet those affected are often too impoverished to receive adequate healthcare.

Towards the beginning of the 20th century, noma largely vanished from the Western world due to economic improvement and lower rates of child malnourishment.

The disease currently has the highest prevalence in sub-Saharan Africa. Tragically, thousands of lives are lost annually, and even survivors face long-term challenges due to extreme facial disfigurement.

In December 2023, the World Health Organization (WHO) added noma disease to its list of neglected tropical diseases, bringing much-needed attention to this misunderstood condition. According to the United Nations, noma indicates a fundamental human rights violation in which affected children are underfed, are without basic sanitation, and lack access to medical treatment.

It is just one of many neglected tropical diseases affecting millions worldwide. These diseases often receive little attention and funding despite their devastating impact on individuals and communities.

This article examines the causes, signs, and treatment options for those with noma disease, alongside its impact on affected individuals and communities.

Noma Disease Symptoms

The symptoms of noma disease can vary depending on the stage of the infection.

In the early stages, a small ulcer may appear in the mouth, often on the inside of the cheek or lip. This ulcer may be painful and make it difficult for the child to eat or speak. Bad breath is common.

The ulcer may become larger and more painful as the infection progresses, and the surrounding tissues may become swollen and inflamed. The odor intensifies at this stage. The child may also develop a fever, enlarged glands, and difficulty opening their mouth due to the swelling.

The disease can disseminate to the nearby soft tissues, leading to extensive tissue destruction and loss of teeth. In severe cases, the illness can also spread to the bones of the face, causing disfigurement and even death. If sufferers survive, pain and difficulty swallowing can significantly impact their quality of life.

Prompt treatment and adequate nutrition are required to prevent lifelong complications or mortality.

Complications

Beyond the physical damage, complications of noma include:

• Speech problems
• Trismus (extreme jaw joint tightness)
• Trouble eating or swallowing
• Complete loss of taste or smell
• Breathing difficulties
• Constant saliva leakage (oral incontinence)

The psychological and social impact on children affected by noma is profound. Facial disfigurement can lead to isolation, stigma, and difficulty accessing education and opportunities. These effects further increase the risk of mental disorders.

Causes

Researchers are still debating the precise cause of noma disease.^[3] It falls into a broader group of diseases known as necrotizing periodontal diseases (NPDs). These consist of necrotizing gingivitis (NG), necrotizing periodontitis (NP), and necrotizing stomatitis (NS). In the worst cases, all of these NPDs can lead to noma disease (cancrum oris).

The main contributing factors are severe immune suppression and poor oral hygiene, which infections, diseases, and severe malnourishment can bring about. Common infectious illnesses associated with noma include Malaria, HIV, and measles. Prolonged infection causes reduced blood flow and damage to the gums, leading to gangrenous tissue destruction.^[4]

Risk Factors

Noma disease primarily affects children between the ages of 2 and 6 who live in poverty and have limited access to healthcare.

Other risk factors pertain to extreme poverty. These include:

• Malnutrition
• Poor oral hygiene
• Living in unsanitary conditions
• Exposure to infectious diseases such as malaria, measles, and HIV

These factors weaken the immune system and make individuals more susceptible to infections.

Prognosis

According to the WHO, there are five official stages of noma progression^[5]:

• Stage 0: Simple gingivitis or gum inflammation (not considered noma yet).
• Stage 1: Acute necrotizing gingivitis, characterized by severe gum inflammation and tissue death.
• Stage 2: Edema, where the gums become swollen and may bleed easily.
• Stage 3: Gangrene, a more severe stage where the gum tissue begins to decay. This stage is also known as necrotizing periodontitis.
• Stage 4: Scarring, as the body attempts to repair the damaged gum tissue.
• Stage 5: Sequelae, which refers to the long-term effects or complications that may arise from gum disease. When other facial tissues begin to decay, this stage is called necrotizing stomatitis and may involve bone deformation (osteonecrosis).

Early intervention is crucial to prevent irreversible damage. If the child receives swift treatment, the condition is reversible, and the prognosis is excellent.

During stages 0-2, the disease is still easily reversible. From stage three, tissue destruction may not be fully correctable, and the patient may require surgery to overcome part of their disfigurement.

Noma disease not only has a devastating impact on the affected individuals but also on their families and communities. Severe facial disfigurement can cause survivors to require constant care and can lead to social stigma and isolation. Complications can have a considerable impact on their mental health and overall well-being.

In addition, the cost of treating noma disease can result in a consequential financial burden for impoverished families. The cost of surgery and ongoing medical care can leave children to live with the destructive outcome of the disease.

Diagnosis

Noma is diagnosable with a physical examination. The doctor will examine the gums, facial features, and glands to check for oral and systemic illness. If bone deformation is present, they may conduct a radiographic test, such as an X-ray or CT scan.

Blood tests can confirm potential diseases like leukemia, neutropenia, and agranulocytosis. These may also help to diagnose any nutritional deficiencies.

Treatment Options and Management

Early awareness and treatment are critical for successfully managing noma disease.

The first line of noma disease treatment usually includes antibiotics to control the infection and prevent it from spreading. Nutritional interventions help to improve the child's immunity, limit the damage, and prevent severe infectious outbreaks in the future.

In some cases, surgery may be necessary to remove the dead tissue and prevent further damage.

In patients where bacterial infection has caused noteworthy tissue destruction, reconstructive surgery may be necessary to restore the child's appearance and function. Surgical approaches may involve plastic surgery to repair the damaged tissues and restore the child's ability to eat, speak, and breathe properly.

While noma can be life-threatening, early intervention offers a good chance of remission. Recovery time differs depending on disease severity and the extent of surgery required.

Preventing Noma Disease

Prevention is pivotal when it comes to noma disease. Simple measures such as improving oral hygiene, promoting good nutrition, and providing access to healthcare can go a long way in preventing the disease.^[6] Regular dental checkups can combat frequent gum infections or tooth decay.

Success requires increased awareness and education among healthcare providers and communities in high-risk areas. A higher level of understanding will promote early intervention when symptoms manifest.

All of these factors demand the absence of extreme poverty. Those unable to provide for their families need to seek the help of local initiatives that offer food, water, hygiene products, and access to rudimentary medical treatment.

Conclusion

Noma disease is a devastating condition that primarily affects malnourished children in developing countries. A combination of factors causes it, including poor oral hygiene, malnutrition, and a weakened immune system. Successful management demands early detection and treatment. The WHO's recent designation of noma disease as a neglected tropical disease is a step in the right direction towards addressing this often overlooked condition.

By promoting good oral hygiene, improving access to healthcare, and increasing awareness and education, we can work towards preventing noma disease and improving the lives of those affected by this neglected disease.

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