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!)

Dr. J- Rant Of The Day

I work with a rather, uh, charismatic doctor here. He likes going off on rants about various subjects from time to time, that range from the incompetence of some staff to the living conditions of Chinese workers in Gabon. His chosen subject the other day was the state of medicine in Gabon. Which is: not good. The government has been unable (or unwilling) to pay its bills to the hospitals for quite some time, and the result is that all of the hospitals have been on strike since January. (Not this one, it’s funded mostly by international foundations.) So the result of the strike is that sick people are not getting medical care, then when they’re gravely ill, they come here (to the HAS: Hôpital Albert Schweitzer), where often it’s too late (Dr. J-: "Do they think we're magicians?!"). The HAS has also been relatively overwhelmed with patients since the strike started: the maternity service has patients on mats on the floor, because they’re out of both beds and mattresses (“Patients are on the floor, four to a room! That is no way to treat people”). The medicine service sometimes keeps patients in maternity and surgery because there are no medicine beds left. It is frustrating that the government is unwilling to pay for medical care for its citizens, and it’s easy to place blame on any number of factors for the fact that people are suffering and dying of treatable diseases simply because there are no resources available to them, so his next statement caught me off guard: “And who is responsible for this?? We all are.”

Medical pictures

Since I did, after all, come down here to particiate in some medically-related activities, I decided to post some pictures of that part. Central American trips can't be all about tortillas and rum, after all (or can they??). The first four pictures are from the Hospital Nacional de Amatitlan, and the last one is outside a health center in a small town a couple hours north of here (the city).

pre-op and post-op area outside the OR (yup, those are patients on stretchers in the hall)

the ER

EKG

Pica

A quick google search turned up that pica stands for: 1. The Portland Institute of Contemporary Art, 2. the Podiatry Insurance Company of America, and 3. the Printing Industry of the Carolinas. However, the Pica that I am endlessly fascinated with is the "persistent eating of nonnutritive substances." According to emedicine, people with pica eat substances, "including, but not limited to, clay, dirt, sand, stones, pebbles, hair, feces, lead, laundry starch, vinyl gloves, plastic, pencil erasers, ice, fingernails, paper, paint chips, coal, chalk, wood, plaster, light bulbs, needles, string, cigarette butts, wire, and burnt matches." Um, ew. Though pica has always held a morbid fascination for me, this post is inspired by a patient today (not mine, hipaa) who was found by the nurse eating baby powder, and subsequently found to have an earring in her colon on xray. And I had a patient a couple weeks ago who admitted to eating cigarette butts (seriously), but flat out denied eating coins (her exact words: "I save money! I don't eat it!"), though clearly there was a quarter in her cecum on xray. Crazy. Factoids about pica: eating ice chips can be a sign of iron deficiency; and eating clay or dirt is acceptable and a learned behavior in some cultures. Not helping Worcester's infant mortality problem: pregnant Ghanaian women eat clay (though I've heard that this problem gets more press than it's really worth in terms of actual harm to infants). Anyhoo, I got off track. Pica is weirdly cool. Except for the people that eat, you know, razorblades and whatnot.

Slow Medicine

This has been on my mind for a while, but I read a cool article on nytimes.com that really brought the issue forward again. Seriously, why do we treat death like it's something we can avoid??? We're all going to die. Deal with it. I am, you are, everybody is. You can't put it off forever. Why do people think that good medicine is just curing everything?? Shouldn't the goal at the end of life be maintaining a good quality of life for as long as possible, not merely delaying death?? So this article: it's about a movement at Dartmouth Medical School called Slow Medicine that emphazises comfort rather than cure at the end of life, and you can read it here. Think about it: if you were diagnosed at age 85 with cancer, would you choose surgery, chemo, and radiation, possibly ending up bedridden with DVT's and PE's and urosepsis and godknowswhat else, just for the possibility of another year or two, and not a healthy year or two at that, or would you rather say, "no thanks, I think I'd rather die on my own terms." I feel like I might go for option B (though in reality hopefully I'm quite a few years removed from a decision like that...maybe I would think differently if I were actually 85). It's certainly understandable to be afraid of death and to want to live as long as possible. I mean, life's short and pretty damn fun. But it ends for everyone eventually. When can we make the shift that the goal of good healthcare is helping people live good, quality years at the end of their life and then dying with dignity and with as little pain and suffering as possible? When will our collective mind stop treating death like something that can be avoided forever? Ok, please read that article, it's short and so interesting. And don't pay too much mind to my ramblings. Inpatient medicine is making me bitter and cynical about drastic end-of-life care, and incredibly sad about the state of some unfortunate people's lives. But that's a depressing story for another post. Please go outside and enjoy your working lungs, healthy muscles, the fact that you aren't paraplegic from a freak accident, or have crippling rheumatoid arthritis, or have had 3 strokes, or live in a nursing home at the age of 37. (This has been a fun week.)