Leprosy’s Legacy : Disease Is Rare, So Center Turns to Aiding Others
In March, retired aluminum worker James Casteel faced a grim prognosis. An ulcer about the diameter of a tennis ball refused to heal on the bottom of his foot. Two skin grafts didn’t help. Neither did a host of other treatments, including therapy with pressurized oxygen. The only solution, it seemed, was amputation.
“I thought for sure I was going to lose it,” said Casteel, one of the 200,000 diabetics nationwide who suffer serious limb problems as a result of their illness.
But then his doctor referred him to the country’s 95-year-old leprosy sanitarium in Louisiana, now called the National Hansen’s Disease Center and run by the U.S. Public Health Service. Here a team of podiatrists, physical therapists and physicians began treating Casteel’s wound. A week later, the persistent ulcer finally began to heal. His still-swollen and deformed foot won’t win any beauty contests, but he no longer faces amputation.
“Once I get all fixed up, I’m going to get on my boat and sail around the world,” said Casteel, 60.
As Hansen’s disease becomes increasingly rare in the United States, the federal facility dedicated to leprosy care is turning its attention to other diseases where disfigurement and loss of limbs are major problems.
Hansen’s disease--also known as leprosy--afflicts about 15 million people worldwide. In underdeveloped countries, the illness is at epidemic proportions, afflicting as many as five individuals of every thousand. But in the United States, just 6,000 patients have the disease. Improved drug therapy means that all but a few hundred are treated as outpatients.
Other Chronic Illnesses
Now, the lessons doctors have learned in treating Hansen’s disease are helping health professionals treat other chronic illnesses, such as diabetes and rheumatoid arthritis. The techniques could also have applications for other diseases, researchers said, including spina bifida, the chronic nerve damage seen in alcoholics, and even a few rare genetic diseases that render victims incapable of experiencing pain, and thus leave them vulnerable to frequent injury.
At the forefront of this research is a special foot-care program that has treated about 300 diabetics. Most, like Casteel, came to Carville as the last stop before likely amputation. All but one has successfully avoided surgery.
“It’s exciting to see patients who have had ulcers for years get healed,” said Charles Patout Jr., a physician and chief of the Rehabilitative Branch at the center.
About 15% of all diabetics develop foot ulcers, and one in every five patients hospitalized with diabetes is admitted because of a foot problem. About 200,000 diabetics undergo amputation of legs and feet each year, according to the National Center for Health Statistics. The cost: About $1.2 billion annually for hospitalization alone, not to mention the additional cost of chronic disability, welfare and days away from work.
Simple Strategy
“Yet probably 50% of the amputations of diabetics could be avoided if we could transfer the technology from the leprosy lab to the diabetic bedside,” U.S. Surgeon General C. Everett Koop said.
The strategy to save limbs is surprisingly simple and was developed by surgeon Paul Brand, a former missionary who treated leprosy patients in India for 19 years before going to Carville in 1966.
Brand noticed that leprosy patients repeatedly injured themselves because they lost the ability to feel pain in their limbs. The injuries produced oozing ulcers that refused to heal because the patients, lacking the sense of pain, continued to re-injure themselves.
The ulcers often became gangrenous and forced the amputation of limbs. But when the injured feet were immobilized in a cast, Brand found that “the ulcers healed as quickly as a similar wound in a person without leprosy.”
The patients, he said, simply “needed to be forced to behave as though they had pain.” Once healed, the cast was removed and the patients were given specially made shoes. “We called them rocker shoes,” said Brand, who is now retired and lives in Seattle.
Special Shoes
“They had a thick, rigid sole, much like an ordinary Swedish clog, and don’t allow the foot to bend.” Injuries decreased dramatically. So did amputations. Within two years of Brand’s arrival, the rate of foot amputations--which had been the most common operation performed at the center--dropped to zero.
In 1970, Brand read a paper in a scientific journal on diabetic feet. The author of the article, a physician from Texas, reported that “ ‘This is what diabetes does to the bones of the foot,’ and showed an X-ray,” Brand recalled recently. “And I looked at the picture and said to myself, ‘My goodness--those are leprosy feet.’ ”
Brand and his colleagues wrote to the physician and proposed a meeting. They suggested that many foot ulcers, whether a result of Hansen’s disease, diabetes or any other condition, could be traced to the same problem: insensitive feet caused by nerve damage. Brand’s prescription: cast, bed rest when needed and then special shoes. But convincing other doctors that the strategy worked for diseases besides Hansen’s was difficult.
It wasn’t until Brand carted a load of foot X-rays from Hansen’s disease patients to a medical meeting that same year and placed them alongside X-rays from diabetics that other physicians outside the Hansen’s disease center began to listen. “The (X-rays) matched so closely,” he said, “that they became convinced.”
In the early 1980s, John Davidson, physician and director of the Grady Hospital diabetic clinic in Atlanta, began referring a few difficult diabetic foot ulcer cases to the Hansen’s disease center for treatment. Since then the facility has treated 375 diabetic patients.
Less Costly Techniques
“Today,” said Surgeon General Koop, “the rehabilitation staff here has the expertise to educate health professionals in preventing most if not all of these unnecessary amputations.”
What’s more, the limb-saving techniques are relatively inexpensive. “What they are doing here, you can do in any hospital in America without adding another piece of equipment,” said psychiatrist John Duffy, director of the center.
The program is also likely to be helped by a Department of Health and Human Services demonstration project begun last October to test the cost-effectiveness of furnishing therapeutic shoes, such as the ones designed at the center, to Medicare patients with foot problems.
Within a year or two, the center will open an outpatient satellite clinic in more centrally located Baton Rouge to expand the diabetic foot care program. Hansen’s disease patients receive free care as part of an act passed by Congress years ago. Diabetics, however, pay a nominal fee, about $25 per visit.
In addition to the foot program, researchers are making inroads into treating insensitive hands, often another casualty of Hansen’s disease. Although far fewer diabetics suffer from hand problems, other illnesses, such as rheumatoid arthritis, take a big toll on hands. So do work-related injuries, ranging from industrial accidents to carpal tunnel syndrome, found among those engaged in repetitive hand activities such as word-processing and some assembly-line jobs.
Hand Program
At the center, a cast program for healing hand ulcers has been started, similar to the foot program. So far about 200 patients have been treated in the hand program. Most have peripheral nerve damage from injuries. Others have suffered from diabetes or rheumatoid arthritis. The center also has developed and standardized a set of five simple filaments or plastic fibers used to test hand sensitivity.
Each filament is a single plastic strand inbedded in a small Lucite handle. The filaments, which range in size from about the diameter of a human hair to a brush bristle, are used to lightly touch fingertips and other areas of the hand.
People with normal feeling can detect each of the filaments. Those who are losing sensitivity can’t. “Our hope is that a set of filaments will one day be in every physician’s office that takes care of diabetic patients,” Duffy said. “It would be simple to train a nurse or receptionist to do the screen, which takes just a minute or two to do, and it would alert the physician to the fact that a diabetic patient was showing signs of loss of nervous sensation.”
The most sophisticated technology to emerge from the center is a computerized surgical work station that can assess all parts and functions of the hand and simulate movement. Designed by the rehabilitative research department in conjunction with mechanical engineers at Louisiana State University, the surgical station uses a CT scan and portrays the 44 separate muscle tendons, 27 major bones, 18 joints and more than two dozen degrees of freedom in a human hand.
Experimental Work Stations
Long before a scalpel is raised to move tendons and restore some function to a diseased hand, the still-experimental work station allows a surgeon to ask what happens to the different elements of the hand when the thumb moves this way, the index finger moves that way and a tendon is transferred from one finger to another.
Researchers at the center have only evaluated one patient using the computer work station, but more are planned. And in the next 18 months, the station will be used to help design implants to replaced damaged joints, said William Buford, a biomedical engineer who heads the center’s Rehabilitative Research Department. “It’s real 21st Century stuff,” Duffy said.
Another important avenue of research at the Hansen’s center involves the drug thalidomide, which is banned for general prescription use in the U.S. because it causes birth defects. Children of women who took thalidomide during pregnancy were often born missing arms and legs.
But thalidomide is one of the best treatments for a complication of Hansen’s known as ENL, or erythema nodosom leprosum. ENL produces fever, nerve inflammation, eye problems and a rash of red bumps, which can cause scarring and other damage. Thalidomide prevents permanent damage. “It works like magic for ENL,” said Robert C. Hastings, the physician who heads the center’s laboratory research branch.
The Hansen’s center is one of just a handful of manufacturers of thalidomide in the world. Housed in a refurbished first-floor wing, the laboratory not only produces thalidomide but also searches for improved versions of the drug that would not have the side effect of causing birth defects.
Some studies suggest that thalidomide could also play a role in the treatment of cancer. Studies of cancer patients at Johns Hopkins Medical Institutions in Baltimore suggest that the drug can prevent many of the unwanted side effects of radiation therapy given to bone marrow transplant recipients. Thalidomide is able to stop the graft versus host reaction that kills some patients. The caution, of course, is that thalidomide could never be used in fertile women.
For researchers at the Hansen’s center, the range of new studies based on this ancient disease represents an enduring legacy of the shrinking group of older patients who still live at the government facility. Said Duffy: “What a wonderful thing that out of a disease that is such a scourge, there is this evolution of something to give back to society.”
