Lymphedema, as its name suggests, is an illness of the lymphatic system. Other than that, most would be hard-pressed to define it, as it is a condition that is relatively unknown, certainly underpublicized, and—for those reasons—all the more dangerous. Little-known but not uncommon—especially in our cancer-ridden society, of which it is often a direct result—and especially insidious because those who contract the illness may have no idea what is afflicting them. And until as recently as 20 years ago, neither did the medical community, especially in the United States.
Often associated with obesity, lymphedema can be of the primary, genetic variety, which can appear at any stage of life, or the much more common secondary type caused by damage to the lymphatic system resulting from surgery, accident, or, more rarely, pathogenic infection. But whatever the cause, there is no cure for the often unsightly and dangerous swelling of the limbs and other areas of the body that typify lymphedema. The disease can, however, be managed and mitigated, but requires unflagging care and attention on the part of both the committed therapist and patient.
There are no primary medications for lymphedema. Occasionally, surgery is performed to reduce bulky swelling—particularly if it has become fibrotic and hard—but this is not recommended because it can cause further damage to the lymph nodes and eventual worsening of the condition.
Currently, the way to treat lymphedema safely and effectively is through comprehensive (complex) decongestive therapy, which includes gentle massage of the affected areas to assist lymph fluid circulation, appropriate exercises, and pressured bandaging and garments. Thus, the task of treating this illness falls largely to rehab therapists, rather than physicians, and the therapists must receive specialized training and certification in lymphedema treatment.
At the Good Shepherd Rehab Center, Allentown, Pa, Cynthia Bauer, director of musculoskeletal outpatient services, heads up a lymphedema program that has four certified specialists on staff. All of the patients are referrals from outside physicians, and almost all (95%) are ambulatory outpatients. Some are in the “super obese” category—more than 350 pounds and as high as 500 plus—and these patients often have difficulty walking because of the lymphedema in the area of their inner thighs. One of the first objectives in treating these patients is to reduce that tissue pooling so that they can get moving again, since movement and exercise are essential to improving lymphatic circulation.
Bauer describes the program as “comprehensive management,” and it consists of several approaches beginning with an evaluation by the therapist and measurement of the volume of the affected limb. The therapist then begins a program of massage, exercise, and compression bandaging, and teaches the patient how to do all of these for himself or herself at home. The at-home program is essential to the success of the therapy, as lymphedema management is a daily and lifelong task.
Once the swelling in the affected limb has been reduced as much as possible through initial treatment, the therapist will determine what type of pressurized garment the patient will need to wear over the affected area. If it’s a hand or arm problem, the garment will be a glove or sleeve, or stockings for the feet and legs and cylindrical garments for the trunk. A garment can be provided for any part of the body, including the face and neck, and it is of significant value in helping to reduce the swelling. Sometimes ready-made garments are available, and other times they need to be custom-made to fit the patient and to maintain the required amount of pressure. They can be worn anytime and are generally used overnight.
Another type of therapy is an external pump, which, Bauer says, “are more sophisticated than they used to be, and can have big sleeves to wrap around large areas of the body.” The pumps aid in the crucial task of restoring the movement of fluid throughout the lymphatic system.
The original reason for the program, Bauer explains, was to deal with the lymphedema that could result from mastectomy surgery and/or radiation, which would often involve incision or damage to the lymph nodes. ‘’Today,” however, “the problem is more widespread, and we have many patients referred to us whose lymphedema is not cancer-related.”
Many determining factors contribute to the treatment’s outcome, including the length of time the patient has had the illness and how much fibrous tissue has formed in the affected areas. “However,” Bauer reports gladly, “there is improvement in every case.”
The spotlight in lymphedema treatment is on therapists, who are often challenged by the excessive weight of patients. Licensed PTs must complete an additional course in lymphedema treatment, and must be prepared to bring compassion and enthusiasm to their job. “What’s important,” Bauer concludes, “is that the therapists like helping patients with lymphedema, and here at Good Shepherd they definitely do.”
At War Memorial Hospital in Sault Saint Marie, Mich, something new is going on in lymphedema treatment. Heidi Janz, MS, occupational and lymphedema therapist, directs a program that teaches total hip- and knee-replacement patients about lymphedema prevention presurgery and shows them techniques to use on themselves that not only help to prevent the onset of lymphedema but also assist considerably in recovery, cutting hospital recuperation time by 2 days. Patients are shown how to clear their lymph nodes with self-massage, exercise, and compression bandaging. “What happens after joint-replacement surgery is you have so much congestion of lymph fluid that it can’t get out. So before the surgery we teach them how to be responsible for themselves after the surgery.” The program began in May of 2007, and already, Janz reports, “The doctors are thrilled—their patients have better range of motion faster, better function faster. They’re getting better faster!”
This approach is also being tried with breast cancer patients. Says Janz, “We don’t treat while they’re receiving radiation or chemotherapy, so again we teach them before their surgery, what they can expect, what to look for. We tell them, ‘Hey, you don’t have to come in and see us if everything goes well and you don’t have swelling. But here’s how to measure yourself, here’s what you need to do, and if you see a change, don’t wait until it gets out of hand. Come and see us right away.’ As with the joint-replacement patients, we teach them exercises to do right after surgery to help the lymph flow, how to massage themselves in the hope that they won’t need to see us again after surgery.”
Good training is essential. Janz recommends that those wishing to be lymphedema therapists should consider the Cedarhaven Rehab program at the University of Wisconsin. The 8-day morning-to-night program is one of the longest-running, most successful programs in the country, attracting both European and American students. Advanced courses are also available. All lymphedema therapists must be certified in lymphedema therapy and also hold a degree in either occupational or physical therapy. “Just being a massage therapist is not enough,” Janz cautions. “A lymphedema therapist has to be trained in contraindications like congestive heart failure and renal failure—you have to have a medical background. Nearly all of our therapists have an MA or PhD in occupational or physical therapy.”
Janz notes that lymphedema therapy is considered “skilled” and is billable to Medicare, which designates it as “complex decongestive physiotherapy,” and recognizes the four-step program consisting of manual lymph drainage, exercise, skin care, and compression wraps. Decompression pumps can be useful, she says, but only in conjunction with manual drainage. “I’m not a big fan of the pumps. We have three pumps at our hospital, and I’ve only used it one time, to soften indurated fibrotic tissue—there’s a danger that pumps can move the fluid to other parts of the body where you don’t want it to go. First, the lymphatic pathways have to be opened with manual drainage so the fluid will be able to travel safely through the body.”
For the future, Janz would like to see the use of more manual therapies and fewer pills, such as diuretics that are used to remove the water from lymphedema patients while leaving behind the protein solids and all the cellular waste material. “If I remove the water out of your legs and leave behind the protein solids, you’re going to start getting infections, cellulitis, all kinds of problems.” Diuretics, such as lasix, are not the answer, she says. “Someone who’s been long-term on lasix has legs that feel like tree trunks, really hard, and with a typical reddish stain in the front. You take them off the diuretic, let that leg swell up a little, then treat it with manual drainage massage, and soon it gets better and even the stain goes away. If you have fluid around your heart or lungs, then yes, you need a diuretic for that, but if it’s just lymphedema, diuretics will hurt you by leaving the solids behind in your legs.”
As positive as she is, Janz does not envision a cure for lymphedema. “Once you have it, it’s something that has to be controlled throughout your life span.” And the best way to do that is through hands-on treatment by both the therapist and patient.
At Lymphedema Therapy, Woodbury, NY, head therapist Bonnie Lasinski, MA, PT, CLT-LANA, is pleased to report that she and the other five therapists on staff—as well as resident physicians Marvin Boris, MD, and Stanley Weindorf, MD—received training from two of the world experts: Judith Casley-Smith, MD, and J.R. Casley-Smith, MD, of Australia.
Although there are currently several million cases of lymphedema in the United States, knowledge and treatment of this serious and widespread disease have lagged far behind Europe, Australia, and elsewhere. “It’s not a specialty in this country; there aren’t any lymphologists here like there are in Europe. They don’t even cover it in medical schools,” Lasinski points out, “but we’re working hard to increase awareness.”
Lasinski has been a rehab therapist for 31 years and has worked in such areas of therapy as neuro, orthopedic, pediatric, diabetic, and amputee. She believes that her work as a lymphedema therapist has been especially satisfying because, “I get the opportunity to really problem-solve with each patient, and it’s one of the few treatment programs that gives significant results in a very short period of time—improvements in quality of life, pain, function—usually after just 1 month of daily treatments.” The clinic has treated about 2,700 patients since its founding in 1992.
Although there are many possible causes of secondary lymphedema, breast cancer surgery and/or radiation remain the leading contributor. “Estimates vary,” Lasinski says, “but approximately one out of every three women treated for breast cancer will develop lymphedema.” And that, she adds, is with the much less invasive sentinel lymph node biopsy, which, as a first line of defense, has by and large replaced the older lymph node dissection method that involved the removal of 10 to 15 nodes from the axilla.
Lasinski insists that the best way to counter this disfiguring and often painful illness is through early detection and diagnosis. “Feeling that your clothes, shoes, rings, wristwatches, or bracelets are too tight is a warning sign. Also, a feeling of fullness in your arms or legs, and less flexibility in your wrists, hands, and ankles.” The patient and physician should address these indications immediately.
Regarding the specially prescribed exercises, which the therapist teaches the patient how to do at home every day, the objective is to enhance muscle contraction in the affected areas to increase fluid circulation. “We start with the trunk, so we have patients do deep breathing exercises to stimulate the entire lymphatic system. Then, if it’s a limb lymphedema, we work on proximal (limb girdle) areas such as the whole shoulder complex, or if it’s lower body, in the pelvic girdle and abdominal muscles. We’d then work our way down the whole limb, to, for example, the foot and then back up again to the center of the body. We want to bring the fluids from the distal end of the swollen part back to the center of the body. In this way the exercises follow the same program as the manual drainage, bringing the fluid out of the limbs back into the trunk of the body where it can be processed by the newly stimulated lymphatic system.”
Lasinski’s clinic is currently testing the effects of low-level light laser treatments on its clients. Recently approved by the FDA, this treatment—when combined with complex decongestive therapy—helps to accelerate healing, particular where fibrosis has set in, by penetrating the hardened skin to stimulate tissue repair through a process of biostimulation. The laser light penetrates into tissue, where it is absorbed by cells and converted into energy that positively influences the metabolic process. Reduced volume in the affected area and halting of tissue hardening are proven results.
Although the laser may well prove effective and the pump and even surgery are sometimes still used, Lasinski—like all lymphedema therapists—believes that there is no substitute for the four-step complex decongestive program involving manual drainage, exercise, skin care, and compression garments. In treating a disease where the therapist plays such a central role—including teaching the patient crucial at-home care—there is also no substitute for the skill, compassion, and enthusiasm that are so amply displayed by these dedicated caregivers.
CREATING A CIRCLE OF HOPE
Lymphedema patient Jeanne Tassis created a foundation to increase awareness of the condition.
If there is anyone who has firsthand knowledge of the battle against lymphedema, it is Jeanne Tassis, founder of the Circle of Hope Lymphedema Foundation in Prospect, Conn. Tassis had breast cancer surgery in 1983, then, 2 years later, began to have swelling in her arm. It was not until 1993, a full 10 years after her surgery, that she finally was able to get a correct diagnosis when she visited Marvin Boris, MD, in Long Island, NY. Boris’ niece had developed lymphedema in her leg after a bicycle accident, and this drove him to study it with experts in other countries where research and treatment was well ahead of the United States. Judith Casley-Smith, MD, of Australia, and Michael Földi, MD, in Germany, were two of these pioneers, both of whom Tassis had become aware of but was unable to visit.
By this time, Tassis’ arm had become so large that she was unable to wear normal clothing. Her career as a teacher was seriously affected, particularly in her preschool, which she had to give up out of fear of infection—a very serious problem for lymphedema sufferers for whom rampaging infections in the affected areas are a constant danger. At one point, Tassis had to undergo intravenous antibiotic treatment every 3 weeks to keep infections down.
The reason she was getting the infections is that there was so much scar tissue in the upper part of her arm from surgery and radiation that lymph fluid could no longer get out of the arm, leaving a protein-rich environment in which bacteria can flourish. “The antibiotics would stop the infection for a week or so, and then it would come back again because the lymph fluid couldn’t get out. It was like a dam in my arm.” However, as Tassis explains, the medication is just to control the outbreak of infection while the therapy is progressing, because if infection occurs then the therapy has to stop. “There’s no magic pill for lymphedema. You have to go through the treatment with the manual lymphatic drainage.”
Her therapy included having the lymph nodes on the unaffected side of her body stimulated to get them working to their fullest, then working on the affected side with gentle massage to channel the fluids across her chest to the good side where they could be effectively processed by the functional lymphatic vessels. With daily treatment for a month, the swelling in her arm was reduced by 60%.
Although her arm improved substantially, because Tassis had undergone so much radiation treatment in 1983, including in her neck, axilla, and chest—the kind of extensive radiation that is no longer performed —the lymphedema spread years later to her trunk, neck, and face, and today she is battling as hard as ever to control and improve her condition.
Tassis is hopeful about a newly approved treatment, the low-level light laser, that was first developed in Australia and received FDA certification in 2006. When combined with complex decongestive therapy, it helps to accelerate healing—particularly where fibrosis has set in—by penetrating the hardened skin to stimulate tissue repair through a process of biostimulation. The laser light penetrates into tissue, where it is absorbed by cells and converted into energy that positively influences the metabolic process. Reduced volume in the affected area and halting of tissue hardening are proven results.
But perhaps the greatest thing that has emerged out of Tassis’ struggle is the Circle of Hope Lymphedema Foundation, which she started in 1999 to help increase awareness of the disease for all and provide hope for those suffering from it. “About 30% to 40% of all patients who have had a dissection of the lymph nodes with breast cancer will go on to develop lymphedema at some point in their lives. It can also come from prostate cancer surgery and melanoma surgery, as well as other kinds of surgeries. And that is just the surgical causes, not to mention the trauma-connected cases from accidents and so on.”
Tassis says that while undergoing treatment, she made up her mind that, “I was going to go home and not stop talking about lymphedema until people listened. I started a support group, and then after a few years my husband and I took a little money and started a foundation.”
Today, the Circle of Hope Lymphedema Foundation puts out a comprehensive newsletter and other publications, runs various programs, and has an extensive Web site at www.lymphedemacircleofhope.org. Issues of obesity and other contributing causes, remedies, case histories, etc, are addressed for both the layperson and professional. A beautifully designed butterfly pin is the newly created symbol of the foundation, and it is available for purchase to assist in the foundation’s mission of “awareness and education of patients and medical professionals, increasing the number of treatment centers, and legislation for insurance coverage and supporting research.”
Alan Ruskin is a staff writer for Rehab Management. For more information, contact .