Noma – the face of poverty

The following two tabs change content below.
Jack Milln
Jack is a doctor from the UK working in Sub Saharan Africa after recently completing a diploma in tropical medicine at The London School of Hygiene and Tropical Medicine (LSHTM).
Jack Milln

Latest posts by Jack Milln (see all)

 

NOMA – from the Greek nomein ‘to devour’.

Noma, or cancrum oris, is a disease that exclusively affects the poor. It disappeared from Europe around 100 years ago although there were sporadic cases during the two world wars. The disease usually starts as an ulcerative gingivitis in malnourished children. Infection then spreads opportunistically into the soft tissues of the mouth and face in the context of malnutrition-related immunodeficiency. The microbiota of a typical lesion includes Borrelia vincentii, Fusobacterium necrophorum, and other mixed anaerobes. The infection causes necrosis of the soft tissues, and the highly fibrotic healing process leaves a host of facial disfigurements, such as oro-cutaneous fistulae and ankylosis of the tempero-mandibular joint. The term ‘anarchie dentaire’ is sometimes used to describe the disordered anatomy. Around 90% of children die either from overwhelming sepsis or subsequent feeding difficulties. Those that survive are left with disorders of jaw function and severely stigmatising facial injuries.

acute noma

An example of acute cancrum oris  

FACING AFRICA

The treatment for Noma is complex and expensive maxillo-facial reconstructive surgery. There is no condition that poses a greater challenge to the surgeon and anaesthetist. Intubation is often nasal, using fibre optic guidance. The condition occurs in countries with limited medical resources, and affects those with no money themselves for private care. As such the condition would remain untreated if it weren’t for niche NGOs. I’m a junior doctor in Ethiopia with ‘Facing Africa’, an NGO based in the UK which has been working here for around 6 years. They do two missions every year, treating about 30 patients per mission. The cost is about $3000 per operation. Complex cases may need around four operations. I  find myself working at the opposite end of the care spectrum from where I am comfortable. My interests lie in widespread cheap measures which prevent disease, rather than expensive specialised measures which treat the condition in a few. However the argument supporting NGOs like Facing Africa is strong. As long as there are people suffering with the condition, there is a need, and the charity make all their own money for this particular condition. This argument is strengthened when you meet those with the condition, those who are suffering at the extreme end of the human condition. We will see how my opinions regarding NGOs like Facing Africa mature of the course of the mission.

See www.facingafrica.org

A TYPICAL CASE

Derrn, an 15 year old girl from Somalia, is one of those that survived the infection as a child. The wound she was left with affects the majority of the left side of her face. As I removed her head scarf to examine her the first thing to hit me was the smell. Instead of a cheek I found a disordered mass of gum and teeth protruding, covered by opportunistic supporative bacterial and fungal infection. The lower half of the left eye socket has been destroyed and the globe of the eye was falling forward. The cornea of the eye is scarred and she is blind from that side. The jaw is clenched shut. I asked how she eats, and I am shown where teeth have been removed to allow her to squeeze in what she can, something she’s been doing since she was four. As doctors we see many gruesome things, defects of many different parts of the body, but when a face is affected to this extent, it affects you in a different way. I’m not ashamed to admit that I made an excuse to sit down for a minute, write some notes, and compose myself, before continuing with the examination. There is no other word to describe how you immediately feel looking into a face like that – you feel revulsion. The doctor’s office is often a stage, and I acted as best I could, but I think Derrn could sense how I had been affected, because she has sensed that revulsion in every person that has ever looked at her face, as long as she can remember. She has never been allowed to leave the house and she’s painfully shy.

DSC_1732  DernAnon

SURGICAL SELECTION DAY

This weekend we will welcome the surgical team to Ethiopia. They are comprised of the UK’s most skilled and experienced maxillo-facial/plastics/ENT surgeons, anaesthetists and theatre nurses. They flew overnight and will drive straight to Menagesha, 20km west of Addis Ababa, where we will present the medical and surgical details of the adults and children to whom we have grown so close over the last two weeks. The question on everyone’s lips is ‘who gets surgery, and who doesn’t.’ The anticipation of the patients is immense. Most have been living with this stigmatising condition all their life, and here was a dream opportunity to have it fixed by the best surgeons in the world, and for free. Some have travelled across borders, having never left their home village and surroundings. They have spent two weeks meeting others who have seen the benefits of surgery, talking about the new life that awaits them after an operation. Unfortunately not all cases are amenable to surgery, and not all the cases have been caused by Noma. Some of our patients are inevitably going to be let down.

2 CASES FOR SELECTION

Deshio is from South Sudan. She doesn’t know her age but from the profile of the unaffected side of her face she looks about 25. The damage to the left side of her face is extensive. The whole upper jaw has been destroyed and I can see deep into her oral cavity and the long tongue extending to the back of the throat. The roof of her mouth has also been destroyed and clearly in sight are the moist spiral conchae of the inner nasal cavity against which the tongue rests. It reminds me of the faces that have seen dissected for display in the anatomy demonstration lab, except the parts of Deshio’s inner face move as she talks. I am fascinated from a scientific point of view, but the face is unattractive. One would think that this woman was outcast from her village but in her arms is an 18 month old baby, and by her side is her husband, a striking tall man with a face covered in beautiful tribal scarifications. I take the history through two translators. I want to find out about both the functional impact of her injury, and the social impact it has on her life; she can eat everything normally, including solids; she has no trouble speaking or breathing; she is accepted in her village and does not cover her face; she is married and this is her third child; her primary concern is the aesthetics of her defect. She is likely to need a series of four major operations to repair the damage. We must consider that with four major operations there is a risk of leaving her three children orphaned in rural South Sudan. It seems like she is achieving a lot of her life goals, but the people have donated money to treat Noma, and here is a classic case.

NadgioAnonJPEG  DSC_1792

Mascaram is a 17 year old girl from Northern Ethiopia. She has had a disorder of the jaw since birth. Her mandible has not grown in the normal way and remains very small in comparison to the rest of her face. The jaw does not function properly and is clamped shut but for 3mm of movement. She’s only ever been able to eat a soft diet, hence a remarkably low BMI of 14.0. She is not married and has no children. When asked if she suffers bullying she becomes visibly upset. This disorder has plagued her childhood, and now it’s stopping her in all the social discourse that make up a normal transition into adulthood. An operation could successfully open her jaw, but previous experience shows that without quality post-operative jaw physiotherapy the jaw is likely to clamp shut again. Do we know this girl well enough to ensure she performs daily physio in the community with no professional follow up? An operation could change this girl’s life, giving her all the opportunities of marriage and children that may otherwise be out of her reach. However it was not Noma that caused this jaw defect, and the money we are spending was given to us on good faith for the treatment of Noma. The typical face of Noma seems to generate a lot more money than a picture of a small chin.

MascAnon  Masc1Anon

MAKING THE DECISION

The decision will be made by the surgical and anaesthetic team based on their experience and wisdom. There are no set criteria to decide who will make the cut and who will not. This condition lies at the frontier of current practice, and the body of evidence outlining surgical outcomes from the condition is sparse. The opinions of the maxillo-facial surgeons, the ENT surgeons, the plastics surgeons and the anaesthetists will vary. They will take the following into consideration; the chances of a safe anaethetic; chances of a successful surgical outcome; whether the defects are functional or cosmetic; the patient’s priorities and wishes; chances of the patient continuing future oral physiotherapy for ankylosis; the social history of the patient; and options for treatment with another organisation.

The surgeons have to be very careful working in a context that they are not used to. The doctor-patient relationship in Ethiopia is more paternalistic than in the UK, and patients are more likely to trust whatever decision the doctor makes. This is especially true of patients coming from rural areas who may have a lower level of education. This is further amplified by the inherent intimidating nature of white faces and imported high technology. There is a power imbalance which the surgeons must be careful not to abuse. Taking the time to talk to the patients about their wishes is especially important. Unfortunately one is not always completely clear after translation through two or three languages from say English to a rural South Sudanese dialect. It’s tempting to think that the team here are taking advantage of a situation to undertake surgical procedures that they would not be able to do in the UK. However talking to the team I realise that they use all the codes of ethics that are used in patient selection back in England. If anything they are more risk averse due to the limited facilities for intensive care and long term follow up. It is clear that a major complication would not only affect the patient, but may affect the organisation’s ability to deliver treatment and surgery to those suffering this debiltating condition in the future. It seems that at Facing Africa there is no place for the cavalier surgeon taking advantage of the poor. But how appropriate is an imported code of ethics in Ethiopia? I will report in a few weeks time with my thoughts as to whether the right decisions were made on the day.

123

The view from our home in the highlands outside Addis Ababa

 

FURTHER READING

Noma (cancrum oris) – Lancet. 2006 Jul 8;368(9530):147-56.

Noma – the ulcer of extreme poverty – N Engl J Med. 2006 Jan 19;354(3):221-4.

Noma: life cycle of a devastating sore – case report and literature review – J Can Dent Assoc. 2005 Nov;71(10):757.

 

MY PERSONAL BLOG – feel free to follow

www.jackmilln.wordpress.com

 

 

One Comment

  1. Posted Feb. 06, 2014 at 17:42 | Permalink

    Great blog Jack