Tuesday, February 16, 2010

FAQ: Buruli ulcer

Today's Global Health topic: Buruli ulcer. What’s that, you say? Never heard of it before? It’s OK, that’s why it’s one of the World Health Organization’s Neglected Tropical Diseases (more on that below). So consider this a public service announcement of sorts about Buruli ulcers.

Q: What is a Buruli ulcer? (Warning: google image at your own risk)

A: It’s an infection of skin, soft tissue, and bone that is caused by a bacteria called Mycobacterium Ulcerans. Mycobacteria are a unique kind of bacteria, and cause other serious infections (M. tuberculosis causes (surprise!) tuberculosis, and M. leprae causes leprosy). It’s called a Buruli ulcer because of a large study done on it in Buruli, Uganda in the 1960’s.

Q: Can I get it?

A: You can only get infected if you live in tropical wetlands. It’s most commonly found in Africa, but it can be found pretty much everywhere in the world if the climate’s right (It used to be called a Bairnsdale ulcer after Bairnsdale, Australia). No one’s sure how people get infected exactly, but at the moment it’s thought that you have to get bitten by a bug that carries the bacteria in its salivary glands. Yum.

Q: What’s it do to you?

A: The bacteria get into your skin and make toxins that cause cell death (How fun! No other mycobacteria do this). Because the toxins also suppress your immune system, you don’t get any “normal” signs of skin infection, like fever, pain, redness, or swelling, because all these symptoms of infection are actually caused by your body’s normal response to invading bacteria. Because people can have little more than a tiny bump or dark spot on their skin, they ignore it, and the infection is allowed to spread and cause serious problems. Untreated, it can cause severe deformity (again, the google image warning), resulting in permanent disability (the most common site of infection is the legs) and death.

Q: How do you treat it?

A: Antibiotics and surgery. Traditionally, surgery was the definitive treatment (to cut out all the dead tissue), but more recent studies show that if you diagnose the infection and start antibiotics early enough, they can cure nearly 100% of infections, with no need for surgery. But it’s a little tricky to diagnose it at an early stage, because there are no real symptoms that prompt people to seek medical attention, and there’s really no good diagnostic test for it, especially one that could practically be used in the rural, resource-poor areas where this infection is endemic.

Q: What’s a Neglected Tropical Disease (NTD)?

A: The World Health Organization has made a list of diseases that affect a large number of people worldwide, with high morbidity and mortality, yet receive relatively little funding and attention. The NTD’s affect one billion people, and together cause 534,000 deaths per year. From the WHO’s website: “Those most affected are the poorest populations often living in remote, rural areas, urban slums or in conflict zones. With little political voice, neglected tropical diseases have a low profile and status in public health priorities.” NTD’s affect 1 in 6 people worldwide. To compare that number to a disease that gets more press time, 1 person in 1,762 has Mutiple Sclerosis. (Ever heard of trachoma? Eight million people are blind because of it. Filariasis? Chagas' disease?) See their website for more info.

Such a pretty lake, but potentially so deadly: Lac Zilé, near Lambaréné, Gabon.

The story of Buruli ulcers in Lambaréné

Although I was doing adult medicine (and BU mainly affects children), I became interested in BU because of the several children who literally lived in the surgery ward, where they were under long-term treatment for the ulcers. All patients at the hospital were cared for by a family member, who was responsible for cleaning, buying and cooking food, giving medications, and many of the jobs that nurses in the US do for patients (even including taking and charting temperatures). Because these kids were there so long-term, they could not have a family member stay with them, so they were essentially on their own. We befriended three of the girls in particular (their picture is in another post), whom I would sometimes sneak away after rounds to visit, or would come over to our house on the weekends.

In the morning, one or two of the girls could be found sweeping the small stoop outside their rooms (all of the rooms opened into a main hallway inside, and had a door to the outside), or washing their sheets and spreading them out on the lawn to dry. I had difficulty picturing American tweens fending for themselves, cleaning their own hospital rooms, entertaining themselves, and finding their own food (even without one arm completely bandaged up). One evening after afternoon rounds, my attending and I passed the entire group of children, most of whom had Buruli ulcers, playing soccer on the large lawn between the surgery and ob/gyn wards. Most were running around, screeching and laughing and playing, enjoying the setting sun with their friends; only two boys sat watching from their wheelchairs, as neither had walked in several months due to surgical debridement for BU that spanned their legs from their hips to their toes. I was truly touched during the three months we knew the girls at how they created a normal childhood for themselves despite their disabling disease: constantly spending time together, choreographing dances to their favorite pop songs, and throwing birthday parties for their friends on the lawn outside the surgery ward.

One afternoon I left the three girls on our porch, and came home to find my camera filled with literally hundreds of pictures they had taken of each other, capturing their adolescence in a way no photographer never could. Looking at these pictures now (I couldn’t bring myself to delete even one) makes my heart break. They are only a few years younger than, but a lifetime removed from, the many young women that would come into clinic with the outlines of their skeletons visible and CD4 counts in the single digits, who would die overnight or the next day from AIDS. So what’s happened to these three girls? They were all discharged within one week of each other in late July, and I only hope they have stayed healthy since then.

Wednesday, February 3, 2010

Global Health

Hello everyone, loyal followers of the sporadic postings of PaleBlueDotBlog,

I'm currently taking a course in Global Health, and some of the topics are really really interesting. I didn't get a chance to post as often as I wanted to from Africa, so I'm going to try to post a few topics that come up in this course that I think are interesting. (Let's look at this as a chance to redeem myself for a dearth of postings from abroad about topics that would actually be fun [well, in my geeky opinion] to read about.) Hope everyone's good! :)


Q & A: Rheumatic Fever and Rheumatic Heart Disease

Q: What are rheumatic fever and rheumatic heart disease?

· A: Rheumatic fever is a disease that can develop as a complication of strep throat. It is rare in the US (for reasons discussed below), but is relatively common in the developing world. Rheumatic heart disease is a long-term result of rheumatic fever (which sometimes you have to get multiple times) which can cause valve deficiencies, heart failure, and death.

Q: How do I know if I have it?

· A: You don’t. But symptoms occur several weeks after strep throat and can include fever, joint pain (without swelling), a heart murmur or heart failure, a rash, and involuntary movements. Kids make up 80% of the cases, and adults 20%. [For people who have taken medical boards and theoretically should remember this stuff: the major (Jones) criteria for acute rheumatic fever are: 1. Migratory polyarthritis, 2. Carditis, 3. Subcutaneous nodules, 4. Erythema marginatum, and 5. Syndenham’s Chorea*.]

Q: What causes it?

· A: I’d like to just say, “strep throat,” but that’s oversimplifying things. The most common cause of a throat infection (pharyngitis, if you will) is a virus, but the reason doctors always stabbing kids’ throats with long Q-tips is that they are worried about “strep throat,” which is infection of the throat caused by the streptococci bacteria. There are very very few strains of strep which can actually lead to Rheumatic Fever (and these are known as the rheumatogenic strains).

Q: So everyone who gets strep throat with one of these rheumatogenic strains of Strep develops Rheumatic Fever?

· A: Nope, that would be too simple. Rheumatic Fever comes about when someone’s body reacts (for unknown reasons) to the bacteria, causing the body to attack its own tissues (kind of like an auto-immune disease like lupus).

Q: What is Rheumatic Fever vs Rheumatic Heart Disease?

· A: Nope. Rheumatic heart disease is the long-term results of several bouts of (or a bad or long-term case of) rheumatic fever. If you catch and treat rheumatic fever (with literally years of penicillin), it is possible to avoid the heart disease. Rheumatic heart disease is a huge problem in the developing world. It is estimated that 15,000,000 children and young adults have rheumatic heart disease, and 230,000 die of it each year. It is especially bad because once you develop the heart disease, the only treatment is a valve replacement. As you can imagine, the majority of people who actually have rheumatic heart disease don’t have access to a cardiothoracic surgeon to pop in a new valve, as would be the case in the US. Also, dying from heart failure is a drawn-out and painful process.

Q: Why don’t we have rheumatic heart disease in the US?

· A: One hundred years ago, rheumatic heart disease was the #1 killer of children and young adults in the US, and now the incidence is nearly 0%. It would be nice to pat ourselves on the back about our great medical care and widespread use of penicillin in eradicating this deadly disease, but that’s not why it practically doesn’t exist here anymore. For reasons that are unclear, the rheumatogenic strains of Strep have practically disappeared in the US, except when they recur sporadically in specific pockets of the country (like Western PA). The incidence of rheumatic fever was already decreasing rapidly before we even started diagnosing it and treating it with penicillin.

Q: Does this mean we don’t have to test every single kid who has a sore throat for Strep?

· A: Probably, but who wants to open that can of worms? Also, why stop doing something you probably don’t have to be doing when it costs only a half a billion (that’s right, that’s a B) dollars per year??

*The word chorea describes a disorder of abnormal, involuntary movements. Chorea is a kind of ancient Greek dance (and also gives us the word choreography). Syndenham’s chorea is a term only used to describe the chorea of rheumatic fever (cause there are other kinds, like in patients with Huntington’s Disease). The other term for it (cause why only have one term in medicine when you can have two or three for us to memorize) is St. Vitus’ Dance, in reference to the “manic dancing that historically took place in front of [St. Vitus’] statue during the feast of Saint Vitus in Germanic and Latvian cultures.” (Thanks, Wikipedia!)