Repost: originally appearing at endtheneglect.org April 29, 2011…
Imagine for a moment you don’t live where you live. Let’s say you live in Benin, or Togo, or Côte D’Ivoire, or perhaps Ghana (we can even add Australia to the list). Perhaps one day you notice on your ankle a small, somewhat pointed elevation of the skin. A nodule. What you have is so painless you consider it inconsequential – it will clear up in a few days. You awake one morning with a slight fever. For this particular ailment it would be rare to have a fever, but not unheard of. Where you normally reside “man flu” is the worst-case scenario you can think of. In Contonou, Benin, you can name at least three diseases that start with fever-like symptoms and could end with your death. Your niece died from one of them – before her fifth birthday, and the other caused blindness in your uncle. But this isn’t any of those.
After a few days this nodule is now the size of a boil. In Washington DC, a boil can be excised. In Togo this is worse than a mere boil. Your foot has swollen – so much that its hard to stand upright. Even more disconcerting is that this all comes without any pain. What follows can only be described as horrific. A painless necrotizing skin lesion, followed by ulcer and scar formation. Before you know it, your foot is being eaten away from the inside. The lesion grows and grows, oozing fluid and puss. A wide painless ulcer now covers a large area of your lower limb.
With this disease early diagnosis makes all the difference – but it is often rare. Surgical excision of the infected tissue has long been the first port of call. Today, along with surgery, you are given the antibiotics streptomycin and rifampin for 8 full weeks. That is if it was caught at an early stage. Your doctor did not know what it is until it was too late, as there are four other diseases that cause skin lesions and ulcers.
With treatment lesions heal but with permanent scarring and contractures which limit movement in your limb. You have no outer skin on the right side of your foot. A skin graft, taking skin from another part of your body, to patch up the infected area is needed. Imagine a small child of less than 15 years of age needing a skin graft. The lesions are sometimes so large that finding enough healthy skin to graft on is impossible. Eventually it will invade your bones, leading to severe disabilities and deformities. Maybe they will have to amputate. Any pain you experience will be due to secondary infections. Secondary infections that might also kill you.
What you have is the second most common bacterial infection we know of — Mycobacterium ulcerans. The bacteria, produces a toxin — mycolactone. The toxin is necrotizing and immunosuppressive, permeabilising soft tissue — eating away until there’s nothing left. In Ghana, Togo, Benin, Washington DC, Côte D’Ivoire, or France, Buruli ulcer is not on the list of diseases you know about. It wouldn’t matter anyway as you would have no idea how you caught it. Was it a mosquito? Infected water? Aquatic bugs? In humans, transmission and infection of the bacteria is unknown. On the shores of Lake Volta, Ghana, some call the disease vengeance while others call it witchcraft. Social stigma is a phrase pregnant with so many definitions – but largely meaningless until it happens to you.
For lack of a better metaphor – if there was a popularity contest for neglected tropical diseases, Buruli ulcer is probably the least popular of neglected diseases. Being unpopular at being neglected might sound like a contradiction in terms but Buruli ulcer hasn’t got a famous person or celebrity face attached to it. We’re still talking metaphors. George Clooney has bigger fish, or rather mosquitoes, to fry. Even in some parts of Europe chikungunya and Dengue fever get more column inches than Buruli ulcer.
“Significant progress has been made in the past 10 years in knowledge of Buruli ulcer, investments in related research, control of the disease, and improvement of tools for case diagnosis and development of treatment protocols. Substantial achievements have been made in diagnosis, treatment, immunology and epidemiology.”
This could have been the opening paragraph to any report on any neglected tropical disease. In fact, that was the second paragraph to the Contonou Declaration in March 2009. What sets Buruli ulcer apart from most other neglected diseases is the next line:
“Despite these achievements, little is known about the exact mode of transmission of the disease, and there is no simple diagnostic test usable in the field.”
All neglected tropical diseases suffer from lack of interest, but Buruli ulcer is a special case. At all levels not much is known. A mysterious disease indeed. The point is not that its a tiny little disease that we know almost nothing about – the point is that we know almost nothing about. It is an emerging disease, named after Buruli county in Uganda, which saw many cases during the 1960s – and only now given prominence after a former Director-General of WHO encountered the debilitating tropical disease. Rolling back the scourge of the diseases that are already endemic is as much a priority as combating those yet to emerge.
Significant first steps have been made in drawing the attention of the world to the suffering caused by this “mysterious” neglected disease. The Global Buruli Ulcer Initiative (GBUI) was established in 1998, dedicated to doing all those things international agencies do that is necessary to combat disease; raising awareness, improve access to early diagnosis and treatment, and promotion of research to develop better tools for treatment and prevention. The World Health Organisation has recently extended its agreement with the pharmaceutical company Sanofi-Aventis to provide free drugs to treat some of the most neglected tropical diseases as well as support for control programmes for Buruli ulcer.
Buruli ulcer is not a disease measured in the human death toll — rather the personal burden to the individual. Morbidity and disability.
“It may not kill but it destroys lives.”
Stienstra Y, van der Graaf WT, Asamoa K, & van der Werf TS (2002). Beliefs and attitudes toward Buruli ulcer in Ghana. The American journal of tropical medicine and hygiene, 67 (2), 207-13 PMID: 12389949
Walsh DS, Portaels F, & Meyers WM (2011). Buruli ulcer: Advances in understanding Mycobacterium ulcerans infection. Dermatologic clinics, 29 (1), 1-8 PMID: 21095521