Pathology and treatment in cases of lymphedema

If lymphedema is detected early, it can be managed effectively. The biggest factor for success is the close interdisciplinary cooperation between the physician, physical therapist, medical supply retailer and patient.

The majority of those affected are women. The condition usually develops on the lower extremities and often looks like mild swelling at first: lymphedema, a chronic, inflammatory disease of the connective tissue accompanied by accumulations of fluid. It is caused by damage to lymphatic drainage system. This leads to an initial increase in the interstitial tissue fluid, later in the tissue itself. If lymphedema remains undetected for a long time, the condition will continue to develop until patients lose a large part of their mobility and therefore quality of life. Education and raising awareness among primary care physicians, care services, but also physical therapists can counteract the progression by making an early diagnosis. A specialist will then prescribe complex decongestive therapy (CPD). It consists of manual lymphatic drainage, compression therapy, kinesiotherapy, skin care and self-management. If the physician, physical therapist and medical supply retailer work together closely, involving the patient right from the start, this therapy can have great success. The three specialists Dr. Ulrich Eberlein, Peter Nolte and Dr. Michael Kraus report on the pathology of lymphedema and their experiences with treating edema patients.

Dr. Ulrich Eberlein:
Obesity makes the condition worse

life: Dr. Eberlein, what classifications of lymphedema are there in medicine?

Dr. Ulrich Eberlein: There are two types: a primary lymphedema is a genetic problem with the development of the lymph vessels or lymph nodes, a hereditary genetic defect or a spontaneous mutation. This impairment may not appear right after birth; usually it’s around the age of 17, sometimes later. Secondary lymphedema is triggered by external damage, for example, a tumor or metastases, radiation treatment, surgery, inflammation or traumas.

What impact does obesity have on the development of lymphedema?

Dr. Ulrich Eberlein: Obesity makes the condition much worse. If the BMI is over 40, it may even be the sole factor for lymphedema. The delicate and fine lymphatic vessels are constricted in the groin area, whereas tissue fibrosis develops in hanging bellies due to congestion. Add to that a lack of movement and the fact that patients can no longer put on their compression stockings by themselves. It’s a vicious cycle. In industrialized countries, obesity is now considered the most common reason for secondary lymphedema. Thanks to refined surgical techniques and gentler radiation, these causes have become more rare.
Globally, however, filariasis is the most common trigger.

What are the stages of lymphedema?

Dr. Ulrich Eberlein: Stage 0 is the latency stage. No abnormalities and no swellings are visible, the patient doesn’t feel any discomfort and therefore doesn’t consult a doctor. In stage 1, soft swelling can be seen, indentations can easily be pushed into the skin. Those return to normal after raising the body part or over night. This is no longer the case in stage 2, and the tissue becomes increasingly harder and firmer. In stage 3, patients suffer from permanent hard swelling, known as fibrosclerosis. The tissue is as hard as a rock and irreversibly damaged, the skin becomes thicker. This condition can be improved with therapy, but not reversed to stage 1 or 0.

When do patients usually come to see you?

Dr. Ulrich Eberlein: A lot of them leave it as late as stage 2. It would be better to start treatment in stage 1, of course – the earlier it starts, the better the outcome. There are no clear-cut transitions: if nothing is done, the condition will inevitably progress. A targeted awareness program and education for physicians, care staff and medical supply retailers, as well as patient mobilization, of course, are therefore very important to me.

What kind of complications can there be, and how can patients deal with them?

Dr. Ulrich Eberlein: Erysipelas can develop as early as stage 1. This is an acute inflammation of the skin caused by streptococci. Suddenly, the patient will suffer from a high fever and the chills, nausea, vomiting and will feel seriously ill. The inflamed area is usually red like fire and hot. Erysipelas can be easily treated with penicillin, yet with the same logic applying: the earlier, the better.

Peter Nolte:
Ignorance is the biggest problem

life: Mr. Nolte, what does a physician have to bear in mind when prescribing the treatment elements for patients suffering from lymphedema?

Peter Nolte: The most important prerequisite is being familiar with lymphology guidelines. Once a physician has absorbed these, appropriate prescriptions can be provided. Many patients have had 15 to 20 appointments with doctors before they come to me – often recommended by physical therapists or medical supply retail staff who have noticed the wrong prescription approach. Ignorance when treating lymphedema is the biggest problem!

What is the specific examination like, what questions are crucial?

Peter Nolte: In the beginning, a comprehensive patient history must be obtained. I ask patients when they first noticed the problems, and why they have chosen this time to consult a doctor. When I’m sure that the patient has lymphedema, the most important questions are: when you wake up in the morning, do you have indentations in your skin that you can push in? Are your legs swollen? If this is the case, the patient is suffering from a condition that must be treated. The patient has to get undressed for the diagnosis, by the way. I have to touch the patient’s tissue to understand the condition. What does the tissue look like, how does the swelling feel, are there any fibroses?

What must be taken into account for complex decongestive therapy?

Peter Nolte: If a leg is congested, a physical therapist has to perform manual lymphatic drainage for at least five days in a row for 60 minutes each, with subsequent lymphological compression bandaging. This performed ideally every 22 hours. One or two hours before this treatment, the patient must remove the bandaging, shower and apply cream to the skin. Afterwards, the bandaging is put on again. Using this method, a leg can be completely decongested within 10 to 14 days. When it’s slim, the care provider can take measurements. At this point, the supply of flat knit compression stockings must have already been applied for. As a physician, I have to prescribe at least 15 sessions of manual lymphatic drainage, the compression stocking as well as the compression material right at the beginning of CDT. This includes elastic short-stretch bandages, padded foam bandages, a cotton warp-knit stocking for the decongestion phase and a flat knit compression stocking for the time after the decongestion.

How important is exercise for this treatment?

Peter Nolte: The patient’s compliance is crucial for the overall progress of the treatment. Patients must be active, meaning going for a walk or cycling, or using home training equipment, for at least 30 minutes per day. Gymnastic exercises are just as much a part of this. The physician should prescribe rehabilitation exercise. Water aerobics, aqua jogging or swimming are best. This is because the water pressure exerts additional compression and excessive tissue water is excreted. The active engagement of the patient is crucial here, but also good coaching provided by the physician. I have to motivate the patient and regularly check the product, at least once every three months. I also have to stay in touch with the physical therapist. Furthermore, I will ask a cardiologist and a nephrologist to exclude cardiac or nephrological causes of the edema. That means I have to approach the treatment in a completely different way.

Dr. Michael Kraus:
The right compression is crucial

life: Dr. Kraus, in which medical specialist areas is lymphedema evident?

Dr. Michael Kraus: Lymphedema can occur in practically all specialist fields. But since lymphology is still quite a small area, the condition is often recognized late, and patients suffering from lymphedema frequently receive insufficient care. A higher life expectancy and more surgeries result in rising patient numbers.

What role does compression play in the treatment chain?

Dr. Michael Kraus: Compression is the central and crucial link when treating lymphedema. Primarily custom-made, flat knit compression stockings help with the removal of lymph from the swollen leg and improve lymph drainage overall. But there are also other useful methods, such as intermittent pneumatic compression. During this procedure, the affected extremities are surrounded by a kind of air cushion, with a compressor controlling the air pressure and therefore the level of compression.

Why do the compression stockings have to be flat knit?

Dr. Michael Kraus: Flat knit products have a high degree of stiffness and a sufficiently high working pressure. In this way, they can offer maximum resistance to the edema. Circular-knit stockings don’t have the same degree of resistance. They often constrict in the areas of the ankle and knee, they reduce drainage and cause the lymphatic fluid to pool. We often notice that lymphatic drainage actually gets worse in these cases. That is why nobody should wear constricting clothing. The advantage of flat knit stockings is that they are knitted as a surface area. Stitches can be added or removed. This means that the stockings can be adapted to the patient’s anatomy, and the required compression can be achieved for all body measurements.

Do you document the treatment progress with photos?

Dr. Michael Kraus: Documentation and especially measurement of the leg circumference are extremely important because they are ways of showing and proving how the patient is developing and how the condition is improving. We take pictures of each patient once every three months.

How do you motivate your patients to lead a healthier lifestyle and do more exercise?

Dr. Michael Kraus: Initially, the most effective motivation is a thorough conversation between the physician and the patient, discussing the problems and how to deal with the condition. Lymphedema is often connected with obesity. Nutrition counseling is therefore a must. We don’t recommend short-term diets or fasting, but permanent lifestyle changes including a healthy diet, exercise and activity. So the exercise doesn’t fall by the wayside, I recommend looking for a partner you enjoy meeting and chatting to. This will also turn regular walking into a pleasant activity. We also established an edema sports club in Fürth five years ago, which is led by a specifically trained coach. Exercise will play a vital role in the new lymphedema guidelines, which are currently revised. In the long term, however, the best motivation is always the close interdisciplinary cooperation between physician, physical therapist, medical supply retailer and patient.

Images: Bauerfeind, Foto Meffert/Eva Siebert, Ebbepraxis, Adobe Stock/vpardi, ARTARCO Fotostudio