A highly complex condition that can impact people of both genders and all ages, lymphedema presents a number of challenges to healthcare professionals.
As a chronic condition, lymphedema has no cure, but there are ongoing treatments involving massage, drainage and compression — the laborintensive nature of which can wreak havoc with patient compliance. By its nature — much of which seems to be random, or at least not fully understood yet — lymphedema can also pose challenges to seating & mobility clinicians and providers, who may be called upon to create systems that can accommodate asymmetries and high weight capacities, while remaining compact enough to maneuver within homes, schools, work environments and motor vehicles.
Defining & Understanding Lymphedema
The National Lymphedema Network (lymphnet.org) defines lymphedema as “an accumulation of lymphatic fluid in the interstitial tissue that causes swelling, most often in the arm(s) and/or leg(s), and occasionally in other parts of the body. Lymphedema can develop when lymphatic vessels are missing or impaired or when lymph vessels are damaged or lymph nodes removed.
“When the impairment becomes so great that the lymphatic fluid exceeds the lymphatic transport capacity, an abnormal amount of proteinrich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid not only causes tissue channels to increase in size and number, but also reduces oxygen availability in the transport system, interferes with wound healing, and provides a culture medium for bacteria that can result in lymphangitis (infection).”
Saskia R.J. Thiedens, RN, founded the National Lymphedema Network in 1988, at a time when the healthcare field was paying relatively little attention to the condition or its treatment. She notes there are “great variations” in lymphedema types, including “very early lymphedema patients with very mild swelling. That’s when you want to catch them and start treating immediately, and teach them how to treat themselves.”
Lymphedema can be present at birth — known as primary lymphedema — or can appear at puberty (known as praecox) or in adulthood. While in the past lymphedema was most commonly correlated with women who have had breast cancer, Thiedens says that’s no longer the case.
“Slowly,” she says, “(the medical community) realized there were many other edemas as well, such as with the morbidly obese and with traumatic injuries. There’s a huge population of primary lymphedema patients, meaning it’s idiopathic. People are born with a compromised lymphatic system, and basically we see babies who have one or two or three swollen limbs — sometimes four. You often see it (emerge) at puberty time in young girls. You see one limb that all of sudden starts to swell up, maybe because of a little injury or a twisted ankle. You see it a lot at puberty, when hormones are changing.”
Such patients, Thiedens explains, have compromised lymphatic systems, but symptoms may take years to emerge, and it’s not always known what causes lymphedema symptoms to be appearing at a certain time.
“If the person has a compromised lymphatic system, it will progress,” she says. “And then you see, mostly in women in their late 30s to early 40s, all of a sudden their legs start puffing up. It’s idiopathic. That’s the age where the body is not able to compensate, to move the fl uid naturally through the body, because it’s compromised. And little by little, the symptoms occur.”
ADLs & Other Challenges
Thiedens says the majority of lymphedema patients “are pretty independent and are able to take care of themselves.” But there is also a significant subset of lymphedema patients who — often because of extreme swelling and weight — have difficulty carrying out their activities of daily living (ADLs).
“Some of these patients,” Thiedens says, “are 400, 500, 600 lbs. The treatment of lymphedema is very labor intensive, even if it’s for a very mild case. It’s an hour of manual lymphatic drainage, then you need to bandage the limb, step by step — you need teach the patient how to bandage him or herself — and then of course, eventually get a garment on it.”
For these patients, whose lymphedema-affected limbs may account for much or most of their total body weight, performing the treatment regimen can be very time consuming and labor intensive. And then there’s the rest of their day.
“It really depends upon the severity (of the lymphedema),” Thiedens says, “but let’s visualize it. Let’s say I’m 450 lbs., and I have a full-time job. In the morning, I get up. I need to take a shower, I need to put on my (compression) garments. When you have these huge limbs, it’s a chore. It’s a huge undertaking. So just getting dressed is a lot of work.”
Mobility can be another challenge.
“If I’m trying to get to work, I need to make sure I have the correct shoes,” Thiedens says. “I don’t want to accidentally slip or fall, because patients with lymphedema are at very high risk of infections. You need to protect the limbs and the skin really, really carefully, and avoid breaking the skin, because that could lead to infections. And once patients develop infections, they often multiply, and these infections become recurrent. Every time there is an infection, there is inflammation, there is more obstruction, and there’s more swelling.”
Basic ADLs can also be difficult to perform, Thiedens says: “Eventually, they have to go to the restroom. A lot of these patients wear pantyhose, because they also have swelling in the genital area or the abdomen, so they have to pull these garments down. It’s really important that, specifically with heavier people, you need to dry and keep the folds really clean. Make sure you don’t get any bacteria in those areas, again leading to infections.”
While lymphedema can be a patient’s primary medical issue, it also can impact patients with other mobility-related diagnoses.
“You have patients who have other mobility problems,” Thiedens says. “For example, you have the patient who has spina bifida and a lot of swelling in the leg. You have the patient with polio, who’s sitting in a chair all day long. Our focus is to teach patients their own self-care, but often that’s not an option. We bring in a family member — a husband or a daughter or a sister — to be part of the treatment and really make sure they can aid in the treatment, that they can help with putting on the garments and make sure that everything fits correctly. For patients with lymphedema, there’s a lot of work involved because they need to do their own self-care, they need to make sure their garments fit correctly, that they are clean. There’s constant paying attention to the possibility of injury.”
For obvious reasons, fatigue can be a major issue for lymphedema patients, particularly those in the bariatric categories. Thiedens says patients living with lymphedema of any kind can also experience emotional difficulties.
“There’s the whole psychological aspect,” she points out, adding that while lymphedema among seniors can be emotionally trying, “I think in the younger population, it’s even more difficult. (There are) 12-, 13-, 14-yearold girls with large legs and ugly-looking garments. It’s tough, it’s really tough. For breast cancer patients, it’s ‘What do I wear to look feminine?’ That’s often very difficult as well.”
A World of Progress
Just a couple of decades ago, Thiedens says, a physician who encountered lymphedema typically assumed nothing could be done to help the patient — thereby allowing the condition to continue untreated and unchecked.
Today, Thiedens says, “We’ve made tremendous strides and tremendous progress. Initially, it started out with breast cancer, because breast cancer really gets the majority of the attention, and there are lots of advocates out there. Today, we have more and more doctors who have become involved in the field and have become lymphologists and have added it to their existing practice. Thousands of physical therapists and occupational therapists and nurses have been trained in the field, and we have many affiliate clinics and other therapists who are treating this condition. We now have numerous position papers in place, widely used, mostly in the oncological world.”
The National Lymphedema Network Web site, for instance, features a position paper focusing on recommended practices for lymphedema patients who travel by air and for air travelers who may be at risk for lymphedema. For instance, the paper says: “Air travel is sedentary in nature, which causes blood and lymphatic circulation to slow. This may precipitate an exacerbation of swelling as fluid pools in a dependent extremity. All individuals traveling by plane are advised to move their limbs frequently to help prevent swelling. Standing and moving around the cabin frequently will encourage improved uptake of extracellular fluid and help prevent further accumulation of fluid in the tissues.”
Of course, more education is still needed, not only for healthcare professionals, but for the general public. “When (a lymphedema patient) goes on a flight, we encourage them to wear not only their garment, but an extra garment over their arm or leg so it’s kind of a padded garment,” Thiedens says. “But when they go through (airport) security, people say, ‘What is this?’ People have been told to take it off. That’s what’s been so frustrating to many patients.”
Still, Thiedens is proud of the progress that has been made, and hopeful about more being made in the future. “We see more and more patient advocates who have gotten out there and educated their local communities,” she says. “It’s really, really changing.”
As for her peers in healthcare, they’re getting the message, too. “We’re sitting at the table with all the oncological disciplines,” Thiedens says. “In the early years, I would go to conferences, and no one would even talk to me. It would be ‘Why are you here?’ Today, there’s a tremendous amount of research being done, and a lot of people have become involved in the field.”
A Show of Its Own
More healthcare professionals than ever before are becoming aware of lymphedema, its challenges and treatments in great part due to advocacy efforts among those who’ve already dedicated their lives to helping affected patients.
And a great part of that advocacy effort is education.
Every other year, the National Lymphedema Network hosts an international educational symposium for clinicians and other lymphedema specialists. The next event has been dubbed “Research Roundup” and takes place in Dallas, Sept. 5-9, 2012.
At press time, scheduled sessions included:
- Skin & Wound Management Bootcamp for Patients with Lymphedema
- Self Management Strategies for Lymphedema In the Physically Challenged
- Managing the Patient with Morbid Obesity and Lymphedema
- Congenital Lymphedema & Peripheral Vascular Malformations: Treating the Pediatric Patient & Beyond
- Integrative Approaches To Lymphedema Management
The 2010 event in Orlando, Fla., hosted participants from more than a dozen different countries. Registration for next year’s event opens this October, with abstracts due in January. For additional details on registration and course content, visit lymphnet.org, and click on the Research Roundup button.