Lymphedema finally getting some attention
By:
Judy Foreman
03/10/1997
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:
-
Keep the skin on the affected limb very clean.
-
Don't cut your cuticles, because bacteria can
enter the body through cracks in the skin. Use a
cuticle cream instead.
-
Try to avoid having blood drawn from the
affected arm.
-
Try to avoid having your blood pressure taken in
that arm.
-
Seek treatment promptly for an injury or
infection.
-
Avoid lifting heavy objects with the affected
arm.
-
Wear gloves while gardening.
-
Wear a pressure sleeve during airplane travel.
-
Try to avoid scratches or bites from animals or
insects; if they occur, seek treatment right
away.
-
Avoid tight clothing.
-
Try to maintain optimum weight because obesity
may make lymphedma worse.
-
Exercise may help.
-
Wear sunblock on the affected limb -- it's more
susceptible to sunburn.
