September 20th, 2014

A funny thing happened on the way to adding rheumatic fever to the list of disappearing diseases – not funny as in “ha-ha,” but funny as in strange and troubling.

rmfThe hospital wards once set up to care for children with this dangerous disease (it can cripple the heart) had closed. To most young people, the image of the sad-eyed rheumatic fever victim watching from a window while friends romped and played outside was just a scene from some old melodrama, an artistic idea from the past. A dramatic 90 percent drop in the annual rate of new cases since the 1960s seemed to show rheumatic fever was going out with bell bottoms and love beads. Then–like Jason and Freddie and those other indestructible horror movie villains–it was back.

Unwelcome and unexplainable outbreaks of rheumatic fever in places as far apart as Salt Lake City, Pittsburgh, and Boston began in the mid-1980s and caused medical experts to take a new look at an old disease. This disease, that usually strikes first among young people between the ages of 5 and 15, can lead to a lifetime with a damaged heart.

In the Begiining — Bacteria

If rheumatic fever is an unfamiliar term to you, how about group A hemolytic streptoccocci? You probably know that better as the bacteria that cause strep throat — and in the beginning, there is strep throat. Though rheumatic fever is not contagious (you cannot “catch” the disease), its potential parents, the strep germs, are. Because these bacteria are easily spread in coughs and sneezes, many young people are all too familiar with the classic strep symptoms: a suddenly sore throat that makes swallowing painful; fever; swolen lymph glands under the jaw, and often headache, stomachache, nausea, and vomiting.

Sometimes a red rash with goose-pimple-size dots will break out on the chest and back. The rush turns white when pressed and later peels. This form of strep complication is known as scarlet fver, and can be treated with antibiotics to kill the bacteria.

Antibodies: Defense and Deception

If there is no tell-tale rash, the presence of streptococci can be confirmed by a throat culture. A cotton swab is rubber over the back of the throat and the throat mucus is analyzed. If th result is positive, 10 days of antibiotic therapy will usually eliminate the strep.

Yet even without tretment, most strep throats last only a few days. If you’re going to get well anyway, why bother with tests and treatments?

The answer to that lies in American Heart Association statistics that show that more than 2 million Americans are victims of rheumatic fever and rheumatic heart disease. In about 1 percent to 3 percent of children with untreated strep infection, the antibodies created by the immune system to fight off the invading bacteria mistakenly begin to attack healthy body tissues, especially in the joints and the heart muscle. Exactly what triggers this immune system reaction is still not known; the results of it are — rheumatic fever with its potential for permanent heart damage.

The strep throat goes away; the immune system reaction goes silently on. Several weeks after the sore throat, the high fever and joint pains for which rheumatic fever is named appear. The pains move around, lasting a day or so in one joint (usually a large one such as the knee, ankle, elbow, or wrist), then migrating to another. A child may have chest pains or be short of breath. Less common symptoms include hard nodules under the skin or a lacy red skin rash that comes and goes. Sometimes a nervous system disorder (chorea or St. Vitus’ dance) will develop with aimless, uncontrolled movements of the arms and legs.

Aiming for the Heart

The main target of the illness, however, is the heart, particularly its valves, which regulate the flow of blood. Though any part can be affected, it is usually the mitral valve between the left atrium and left ventricle that becomes inflamed and, over time, scarred and distorted. The damaged valve may not open completely (stenosis), so less blood than normal flows to the ventricle. Or it may not close completely (insufficiency), so blood will leak back into the atrium. Either way, the heart is continually overworked in its pumping efforts, and rheumatic heart disease, a condition of serious permanent damage, may result.

Most people recover from a case of rheumatic fever in a few weeks. There is no cure for the disease, but bed rest and medication to control the fever and inflammation help make recovery easier and keep the heart from more strain. If a valve is damaged, the normally soundless flow of blood through a valve becomes a disturbed murmur that can be picked up by a stethoscope during examination. A doctor can further determine heart damage through echocardiography, which uses sound waves bounced off the heart walls and valves to show the movement of the valves and size of the heart chambers.

Having a strep throat does not lead automatically to rheumatic fever (the majority of cases do not develop into the disease), and having rheumatic fever does not lead automatically to lifelong heart damage. The key in both cases is prevention. Prompt diagnosis of strep followed by tretment with antibiotics can cancel both the bacteria and the risk of an attack of rheumatic fever.

Prevent and Protect

Equally important is protection from another strep infection by anyone who has had rheumatic fever. Having the disease once does not bring immunity. Instead, it seems to make the risk of future attacks after a strep infection even greater. This in turn increases the risk of further heart damage. Long-term, sometimes lifelong, protection through antibiotics is needed, with special precautions necessary before and after dental work or surgery.

Knowing the importance of prevention has meant an added worry for doctors studying the new reports of rheumatic fever outbreaks. In many cases, the strep infection that set off the immune system reaction was so mild that children never suffered the painful sore throat or other common clues that would have alerted parents and physicians. Researchers, therefore, are looking at the possibility of an unfamiliar group A bacterial strain, and experts are urging a check for strep bacteria in any case of a sore throat. A new rapid strep test that uses antibodies from the immune system to identify the bacteria can be done in the doctor’s office with almost immediate results.

But why not just give penicillin or some other antibiotic for all sore throats? Most throat infections are caused by viruses that are not affected by antibiotics; unfortunately, the person receiving the antibiotic may be. Drugs usually have side effects, and the risk of developing an allergy to the antibiotic increases with frequent, unnecessary use.

The search for a vaccine to keep the strep bacteria from triggering the self-destructive immune system attack goes on. Until such a solution is found, however, the need to identify the bacteria remains vital. “Out of sight, out of mind” can be a dangerous concept, say medical experts charting the comeback trail of rheumatic fever, only recently a rare disease, almost forgotten . . . but not gone.