Marianne Lynnworth, 66, a writer and former geographer, isn’t sure why she got lymphedema, though she thinks a case of frostbite when she was a teenager probably touched off a hereditary tendency to the disease.
But she sure does know what a struggle it’s been for the last 52 years.
Sometimes, the swelling makes her legs so heavy it “zaps my energy,” says Lynnworth, who lives in Waltham. She can’t go shopping for more than an hour because standing in line becomes so difficult. “And I can’t wear dresses anymore because my legs look so swollen.”
For years, doctors had little to offer patients with lymphedema, a condition in which damage to the lymphatic system causes the arms or legs to swell to several times their normal size. It also sets the stage for massive infections from even the tiniest cuts in the skin.
In fact, lymphedema has until recently been such an orphan condition — it falls through the cracks of medical specialties — that even today no one is quite sure how many people have it.
Some put the figure at 1 to 2 million Americans, plus another 250 million worldwide, many in tropical countries where parasites cause a severe form of the disease called elephantiasis.
For some, lymphedema stems from genetic bad luck. Others acquire it after treatment for other diseases, usually cancer. Roughly half a million breast cancer survivors have it from surgery or radiation that destroys lymph nodes in the armpit; others get it in the legs after lymph nodes in the groin are destroyed by surgery or radiation for melanoma and prostate cancer.
But thanks to strenuous efforts by patient advocates and a growing understanding among doctors, lymphedema is finally moving out of the closet — and into the clinic.
In the last year and a half, five lymphedema programs have gotten going in the Boston area. Dozens more have sprouted nationwide, driven by the growing popularity of a low-tech treatment from Europe that, proponents say, combats many of the complications of lymphedema.
The lymphatic system, specialized vessels through which a clear, protein-rich fluid flows, is an under-recognized but crucial part of our circulatory and disease-fighting machinery.
It is “the garbage-hauling system of the body,” says Dr. Robert Lerner, a New York surgeon who has pioneered American lymphedema care and who, with Dr. A. Benedict Cosimi, now runs a new Boston clinic that opened in January, backed by Massachusetts General and Brigham and Women’s hospitals and the Dana Farber Cancer Institute.
In the bloodstream, much of what we call blood is actually plasma, a clear fluid. When you sprain your ankle, for instance, much of the swelling is plasma that seeps out of tiny capillaries into tissues around the injury. Most of this fluid is then reabsorbed into the bloodstream through small veins.
But a small part of it, called lymph, is picked up by lymphatic vessels instead, which carry it to a duct in the chest, from where it is dumped back into the primary circulation. The lymph often contains bacteria, dead white blood cells used to fight infection, proteins, fats, and, sometimes, cancer cells that have been shed from a tumor.
As lymph flows slowly back toward the primary circulation — there’s no pump like the heart to push it — the lymph nodes filter out the debris.
But if these nodes or lymphatic vessels are damaged, there is no place for the lymph to go, notes Dr. Sumner Slavin, a plastic surgeon and lymphedema researcher at the Beth Israel Deaconess Medical Center.
And as the fluid builds up, any cut in the skin can trigger a raging infection called cellulitis (not to be confused with the skin-puckering condition called cellulite). In fact, lymphedema provides a veritable feast for invading bacteria.
Each infection can lead to scar tissue that further damages lymph vessels, setting the stage for yet more lymphedema and cellulitis.
So the trick is getting the fluid that is stuck in a limb past the damaged lymph vessels and back into circulation.
Elevating the swollen arm or leg can help, but that’s often impractical on a sustained basis. Pneumatic pumps help, too, but they work best in the earliest stages of the disease. Once lymphedema has progressed to fibrosis, a hardening of the tissue, pumps may actually damage still-healthy lymph vessels.
Surgery to remove skin and fat and squeeze out fluid can help in extreme cases, but swelling almost always returns.
Diuretics — pills that reduce fluid retention — may get rid of excess lymph, but they leave behind a concentrated solution of lymph proteins that acts as a chemical magnet for more fluid. Another drug, coumarin, has been tried in some countries, though it’s not available not here. It has been linked to several deaths.
That leaves the low-tech stuff, typically a combination treatment called complete decongestive physiotherapy. This includes manually draining the lymph fluid, a technique in which a physical therapist lightly massages lymph vessels to stimulate lymph flow; scrupulous skin care to guard against infections; using non-elastic bandages that squeeze the limb tightly at the wrist or ankle, and exercises done while the bandages are in place.
Finding therapists trained to do lymph drainage can be tough.
The Spence Center for Women’s Health in Cambridge, believed to be the first local clinic to offer it, sent its lymph therapist, Cindy Stewart, to Canada for training.
At St. Elizabeth’s Medical Center, a lymphedema service, including a therapist, opened two weeks ago, says Dr. Kathleen Hogan, medical director for women’s health.
The Lahey-Hitchcock Medical Center in Burlington will open a lymphedema service as soon as its therapist finishes training, probably in May or June, says Dr. Donald Breslin, a peripheral vascular disease specialist. Mt. Auburn Hospital in Cambridge hopes to follow suit soon thereafter.
But promising as it sounds, hard data on the effectiveness of lymph drainage massage are scarce, says Slavin of Beth Israel.
Though apparently safe, the technique can be both “onerous and expensive,” he adds. Sessions cost roughly $100 each — insurance coverage is spotty at best — and many patients need one to two sessions a day for up to four weeks.
“That’s why I haven’t tried it,” says Allison Stieber, a 46-year-old Somerville copy editor who has had lymphedema for 17 years.
But Lerner says he has treated more than 4,000 people and has followed 2,000. Most of them, he says, experience a 60 percent reduction in swelling, progress that persists with an at-home maintenance program.
A study published in January in the journal Oncology suggests that in many patients, lymphedema can be reduced by at least two-thirds with the combined treatment.
Still, the ultimate solution may be a different approach to surgery — removing fewer lymph nodes in the first place — and perhaps new ways to get damaged lymphatic vessels to regrow.
Surgeons are already cutting back on the number of lymph glands they remove. At some hospitals, they are experimenting with removing only a few “sentinel” nodes in the armpits of women having breast surgery, says Lerner, especially if the women have small tumors and plan to have chemotherapy.
Most surgeons, though, are awaiting the results of a major study before changing their practice, says Dr. Susan Pories , a breast surgeon at Mt. Auburn and Beth Israel.
Slavin, meanwhile, is trying to coax damaged lymphatic vessels to regrow.
The bottom line, he says, is that “we should never underestimate the capacity of the human body to repair itself.”
Tips to prevent and help control lymphedema:
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Keep the skin on the affected limb very clean.
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Don’t cut your cuticles, because bacteria can enter the body through cracks in the skin. Use a cuticle cream instead.
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Try to avoid having blood drawn from the affected arm.
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Try to avoid having your blood pressure taken in that arm.
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Seek treatment promptly for an injury or infection.
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Avoid lifting heavy objects with the affected arm.
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Wear gloves while gardening.
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Wear a pressure sleeve during airplane travel.
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Try to avoid scratches or bites from animals or insects; if they occur, seek treatment right away.
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Avoid tight clothing.
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Try to maintain optimum weight because obesity may make lymphedma worse.
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Exercise may help.
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Wear sunblock on the affected limb — it’s more susceptible to sunburn.