Noma, also called water cancer or buccal gangrene, is a serious infectious disease of the buccal mucosa that originates from bacteria in the oral mucosa and, if left untreated, spreads to the surrounding soft tissue and bone. Noma primarily affects children in developing countries because of environmental factors such as malnutrition, poor sanitation, and inadequately treated infectious diseases that favor the development of noma.
What is noma?
Noma is the name given to a severe form of progressive (advancing) buccal mucositis caused by bacterial infection by Borrelia and Fusobacteria. Noma manifests itself when there are insufficient hygienic conditions as well as a generally poor state of health of the affected person, which is why the disease usually occurs in children with impaired immune defenses as a result of infectious diseases or malnutrition in developing countries. In this case, noma is initially accompanied by ulcers of the oral mucosa, which spread as the disease progresses, decomposing the body’s own tissues and damaging the facial bones, thus causing the symptoms characteristic of noma, such as foul-smelling bad breath, necrotic areas in the facial and mucous membranes, pain, and fever. In addition, as a result of the increasing size of necrotic areas in advanced stages, noma entails impairment of the sensory organs and the speech apparatus.
Causes
Noma is caused by bacteria (Borrelia, Fusobacteria) that are normally harmless to the human organism and are typically found in the human mouth area. If the immune defense is weakened by poor hygienic conditions, infectious diseases such as measles, scarlet fever, rubella or meningitis, and malnutrition (especially lack of proteins, vitamins, electrolytes), the bacteria, especially in children up to six years, can multiply in the oral mucosa, from where they spread to the surrounding tissues (skin, mucosa and facial bones) and cause noma.
Symptoms, complaints, and signs
Noma (water cancer) is a serious disease of malnourished children in developing countries that causes death if left untreated or facial disfigurement despite treatment. The disease starts from a small wound in the oral mucosa and leads to extensive death of tissues and bone parts of the face. Due to malnutrition, the children’s immune system is very weakened. In addition, there are the catastrophic hygienic conditions in these countries. This can lead to this catastrophic infection with often otherwise quite harmless bacteria. The disease often begins with bleeding gums and foul breath. A reddish-bluish lump initially forms on a small sore in the oral mucosa, which quickly spreads to the cheeks and lips. Swelling occurs in the inflamed areas, and the affected area becomes hard and thick. Furthermore, pus is increasingly formed with an increasingly unbearable odor. Patients also suffer from severe pain and fever. At the site of the swelling, necrosis of the tissue then occurs in a further stage. The dead tissue turns black. This area is surrounded by a white line, which acts as a borderline to indicate the further progress of tissue decomposition. The general condition deteriorates drastically and is accompanied by diarrhea and fever. In the final stage of the disease, destruction of almost all parts of the face may occur. Death occurs in untreated individuals from pneumonia, blood poisoning, or the bloody diarrhea.
Diagnosis and course
Noma is usually diagnosed on the basis of characteristic symptoms and associated circumstances, such as a weakened immune system due to a previous infectious disease or malnutrition, and inadequate sanitation. The symptoms typical of noma are ulcers in the mucous membrane of the mouth, which cause a foul mouth odor and successively spread to the soft and bony parts of the face, thus permanently affecting the sensory organs and the speech apparatus. In advanced stages, parts of the facial bones may be exposed and sepsis (blood poisoning) or pneumonia (aspiration pneumonia) may assume life-threatening proportions.In developing countries, noma often takes a severe course, as there are often insufficient treatment options in these areas. About 90 percent of children affected by noma die in these areas, although the prognosis is good if treatment is started early. In contrast, survivors exhibit severe facial disfigurement as a result of contracting noma.
Complications
Noma (water cancer) invariably leads to fatal complications if left untreated. In the early stages of the disease, there is still a very good chance of cure. However, if there is no change in diet with adequate supply of proteins and vitamins at the beginning of the disease, the number of bacteria in the mouth area increases to such an extent that decomposition of the tissue there occurs. The final condition is characterized by fatal sepsis, further tissue decay in the mouth area, pneumonia or severe bloody diarrhea. More than 90 percent of children affected by noma do not survive the disease. Even after medical treatment, long-term consequences and complications can still occur. These consequences include a severely scarred face. Sometimes even the tissue of the lips, cheeks or eye sockets completely disintegrates. The face is then disfigured forever. The consequences of the disfigurement are particularly bad for those affected, because the scars and mutilations often lead to lifelong burdens and disabilities for the person. Those affected then suffer most from the psychological problems. They are often discriminated against because of their disfigurements and thus fall into loneliness and social isolation. In the affected families, the children with the disease are often abandoned, which makes them completely neglected. They do not show themselves publicly, but live hidden. Thus, they are excluded from normal development.
When should you go to the doctor?
When infections of the mouth and face, ulcers and other signs of severe disease appear, a doctor must be consulted. The medical professional can diagnose noma and help speed recovery through prompt treatment. Therefore, initial symptoms should already be clarified. People who live in poor hygienic conditions or suffer from malnutrition are particularly susceptible to infection. HIV, AIDS and typhoid patients as well as people with an immune system disease are also among the risk groups and should go to the family doctor with the complaints mentioned. If the symptoms occur after a stay in one of the risk countries, a doctor must be consulted immediately. The same applies to advanced diseases that may have already spread to the internal organs. For example, skin bleeding, coughing up blood and severe gastrointestinal complaints must be taken to the nearest hospital immediately. In addition to the family doctor, an internist or dermatologist may be involved in the treatment of noma. Children should be presented to a pediatrician immediately if they show signs of noma infection.
Treatment and therapy
Therapeutic measures for noma are conditioned by the stage of disease present and focus on containing and eliminating the infection, preventing recurrence (reoccurrence of the disease), and treating associated symptoms. Thus, in the early stage (stage I) of disease with noma, therapeutic measures aimed at eliminating malnutrition, mainly through an additional supply of proteins, vitamins and electrolytes, and containment of the focus of infection by antiseptic mouth rinses with chlorhexidine and metronidazole are sufficient. In the second stage of the disease, a swab is taken from the affected area to determine the specific strain of bacteria present as well as the mix of antibiotics to be used for treatment, while mouth rinses continue to be used. In the later course of noma (stage III), artificial nutrition is required in addition to antibiotic therapy to compensate for fluid and electrolyte deficiencies. If the noma progresses further so that there is already detachment of the necrotic (dead) tissue (stage IV), plastic reconstruction is indicated to restore the damaged areas of the face through surgery.Such surgical procedures are often beyond the scope of medical care in developing countries, and affected children must live with facial scarring and disfigurement for the rest of their lives, necessitating additional psychological care for children affected by noma.
Outlook and prognosis
The prognosis of noma is unfavorable. Without comprehensive medical care, the affected person faces premature death. The high-risk group for the disease includes children with malnutrition, most of whom live in developing countries. If treating physicians can save the child’s survival with drug therapy and special mouth rinses, long-term damage is nevertheless almost inevitable. The more advanced the disease, the more difficult the outlook. The children suffer visual changes and adjustments in the facial area. Despite all efforts and early therapy, it is not yet possible to exclude the possibility that these are beyond the scope of care in developing countries. If it is possible to bring the patient to western regions, cosmetic surgical procedures can provide relief from the optical abnormalities. The interventions are associated with a high expenditure as well as the possibility of complications. Therefore, they are granted to very few sufferers. Due to the discomfort and optical peculiarities, the affected person is threatened with emotional and mental conflicts. States of stress can lead to psychological sequelae. These have a further negative impact on the quality of life and general well-being of the affected person. If the course of the disease is very favorable, not only can the survival of the child be ensured, but there is also the prospect that exclusive facial scars will remain.
Prevention
Noma can be prevented by adequate hygienic measures and comprehensive medical care. Accordingly, the disease could be prevented in developing countries by improving children’s living conditions and quality of life. In particular, minimizing malnutrition or undernutrition, improving hygienic conditions, and providing early and comprehensive medical treatment for infectious diseases, as well as appropriate vaccination, can contribute to lower rates of contracting noma in developing countries.
Follow-up
The disease of water cancer actually requires minor medical therapy. However, this is not available in the areas where it spreads. The same is true for medical aftercare. Therefore, two groups must be distinguished: those who die and the others who survive. The former include up to 90 percent of patients. The survivors of cheek burn need follow-up care, but do not receive it due to the desolate medical care system. For them, only a life of disfigurement and disadvantages remains. According to European standards, the water cancer leads to a permanent treatment in which the patient receives any support. Especially the mutilated and disfigured face requires medical attention. It can be restored by plastic reconstruction. This requires several procedures and numerous visits to the doctor. Until a satisfactory result is obtained, routine check-ups take place. In addition to physical examinations, blood tests are also ordered. The disease noma may recur. Close follow-up attempts to prevent this in the areas where the disease is prevalent. Doctors combat malnutrition, administer the missing vaccinations and teach basic hygiene standards. Compliance with the latter is the responsibility of the patient or his parents.
What you can do yourself
To be able to treat noma (water cancer) well, the general condition of the patients – they are mostly children in developing countries – must stabilize. This includes regular meals with increased protein, vitamins and electrolytes, as well as drinking plenty of fluids, getting enough rest and protection from sunlight and/or even insects. At the same time, the patient’s immune system must be built up so that it can cope with the Borrelia and bacteria that have triggered the disease. Hygiene in the environment is also extremely important. It must be possible for the patient to wash daily and also to clean and wound-technically care for the affected areas.At the beginning, antiseptic mouth rinses are sufficient, but if the disease is already advanced, antibiotics must be taken additionally. The prescribed medication should be taken regularly and should not be discontinued prematurely. Once the infection is overcome, scars may still disfigure the face. Depending on the culture, corrective surgery is not always possible. Nevertheless, ways must be found for patients to cope with their sometimes significant disfigurements. The best options are accompanying psychotherapeutic treatments or further assistance, as offered by various aid organizations on site (see also http://www.nonoma.org/).
