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Newsletter Article Reprint Volume 11, No. 2 April–June 1999 Treatment of Lymphedema with Complete Decongestive Physiotherapy Joachim E. Zuther, Certified Instructor MLD/CDP SUMMARY Lymphedema, caused by a low output failure, i.e., a reduced transport capacity (TC) of the lymphatic system, which in the case of lymphedema has fallen below the physiological level of the protein and water load, is a common and serious condition worldwide. Complete Decongestive Physiotherapy (CDP) is the treatment of choice, even in the most advanced stages of lymphostatic edema — provided that both a physician with broad experience in clinical lymphology and a specially trained therapist are available. A sufficient lymphatic system is able to return the physiological amount of protein and water load back to the venous system. The lymphatic protein load consists of plasma proteins continuously leaving the blood capillaries. The fraction of water ultra-filtrated in the area of the blood capillaries which is not reabsorbed, is called the lymphatic load of water. In the event of an increase of water and protein, a healthy lymphatic system is, for some time, able to prevent the onset of edema by increasing its lymph time volume (LTV), i.e.: lymph angions will increase their pulsation frequency and amplitude. This is called the lymphatic safety factor or safety valve function of the lymphatic system. The highest lymph time volume is known as the transport capacity of the lymphatic system which is approximately ten times higher than the lymph time volume under physiological conditions10. Lymphedema arises due to an imbalance between the normal amount of protein load and the reduced transport capacity of the lymph vascular system. This condition, known as “mechanical insufficiency,” results in an accumulation of proteins in the interstitial tissue with subsequent fibrosclerotic changes. Since, in such cases, the lymphatic system is not able to activate its lymphatic safety factor, other pathologic (inflammation, chronic venous insufficiency) can lead to even more serious complications such as ulcerations. Common causes for mechanical insufficiencies in the case of secondary lymphedema are surgery, radiation, trauma or inflammation. The reason for an insufficient transport capacity in primary lymphedema is congenital malformations of the lymphatic system. Primary lymphedema can be present at birth or develop at some time during the course of life5,12. Stages of lymphedema If the reduced transport capacity is still sufficient in managing the lymphatic load there is no clinical lymphedema present. The time preceding the onset of lymphedema is called the “latency stage5.” The first stage of lymphedema (reversible stage) is characterized by a smooth texture of the tissue. The area affected with lymphedema is pitting and may vanish more or less over night. If the protein-rich swelling LYMPHEDEMA ~ TABLE 2 Stages Characteristics Latency stage no swelling reduced transport capacity “normal” consistency Stage I (reversible) edema is soft (pitting) no secondary tissue changes elevation reduces swelling Stage II (spontaneously irreversible) fibrosclerotic changes Hardening of the tissue (no pitting) frequent infections Stage III (lymphostatic elephantiasis) extreme increase in volume and texture with typical skin changes (papillomas, deep skin folds) ETIOLOGY ~ TABLE 1 Primary Lymphedema Secondary Lymphedema aplasia hypoplasia hyperplasia dissection of lymph nodes radiation post-traumatic post-inflammatory malignancies self-induced (artificial) fibrosis of lymph nodes agenesis of lymph nodes congenital <35 years of age (lymphedema precox) >35 years of age (lymphedema tardum) factors that produce an increased level of lymphatic load persists, fibrosclerotic tissue changes will result in increased hardening of the tissues (stage II); elevation has no effect and, –1– National Lymphedema Network • Latham Square • 1611 Telegraph Avenue • Suite 1111 • Oakland, CA 94612-2138 • Tel: 800-541-3259 • Fax: 510-208-3110 in addition, patients are prone to developing frequent infections which worsen the condition4. Typical for the third stage of lymphedema (lymphostatic elephantiasis), is an extreme increase of the swelling combined with skin changes, loss of function and other complications. Lymphedema, if left untreated, may lead to invalidity or even the development of angiosarcoma (Stewart-TrevesSyndrome)8. Therapy Since there is no cure for lymphedema10, the goal of therapy is to reduce the swelling and maintain the reduction, i.e., to bring the lymphedema back to a stage of latency. For a majority of patients, this can be achieved by the skillful application of Complete Decongestive Physiotherapy — a non-invasive, safe and reliable method that shows good, long-term results in both primary and secondary lymphedema. CDP is also cost-effective: y it transfers the care from the doctor to the patient/family; y it significantly reduces the risk factors of developing cellulitis attacks, described by Olszewski as “Dermatolymphangioadenitis” (DLA), by improving lymph cysts, lymphocutaneous fistulas, varicose lymphatics or fungal infections 7. Even though the basic steps of CDP had already been described by Winiwarter at the end of the last century, this therapy became widely accepted only during the past two and a half decades1,2,13. Numerous studies have proven the effectiveness of this therapy which is well established in European countries and is now becoming widely recognized in the United States1,2,8,10. CDP consists of four basic steps : 1. Skin and nail care, that may also include topical and systemic antimycotic drug treatment (the skin must be free of infections before treatment can be started) 2. Manual Lymph Drainage 3. Compression therapy 4. Decongestive exercises The treatment itself is done in two phases8. In phase one, the goal is to mobilize the accumulated protein-rich fluid and to initiate the reduction of fibrosclerotic tissues (if present). The average duration of this intensive phase is four weeks. The treatment is done twice a day, five days a week. Another important goal in this first phase is to instruct the patient in techniques designed to maintain and improve the success of the treatment (proper skin care, correct application of bandages, wearing of compression garments, etc.). The first phase of the therapy is immediately followed by phase two, aimed to preserve and also to improve the success achieved in phase one. This phase is, for the most part, continued at the patient’s home. With good patient compliance, the volume reduction can not only be maintained, but also improved by progressive reduction of fibrosclerotic tissues. In more severe cases, it is sometimes necessary to repeat phase one and if lymphedema is associated with other conditions, the individual steps of CDP will be modified accordingly. STAGES OF LYMPHEDEMA AND THERAPEUTIC APPROACH ~ TABLE 3 Stages Duration Latency Phase I (decongestive) Phase II (preserve and maintain) patient instruction Stage I 2-3 weeks MLD 1-2x/day short-stretch bandages skin care remedial exercises patient instruction MLD if necessary compression garments skin care remedial exercises Stage II 3-4 weeks MLD 2x/day short-stretch bandages skin care remedial exercises patient instruction repeat Phase I (1-2x) MLD as needed (1-2x/wk) compression garments bandages at night skin care remedial exercises Stage III 4-6 weeks MLD 2-3x/day short-stretch bandages skin care remedial exercises patient instruction MLD 1-2x/week) compression garments (in combination with bandages) bandages at night skin care remedial exercises repeat Phase I (3-4x) if indicated, plastic surgery Manual Lymph Drainage is a gentle manual treatment technique which improves the activity of intact lymph vessels by mild mechanical stretches on the wall of lymph collectors10. A better filling of lymph capillaries, achieved by a mild increase in tissue pressure during MLD, also results in a higher lymphangiomotoricity. In most of the post-mastectomy patients, lymphedema not only includes the arm but also the ipsilateral trunk quadrant, since the collecting area of the axillary lymph nodes are the upper extremity and the homolateral upper trunk quadrant. In cases of secondary lymphedema of the lower extremities, the lower trunk quadrant and/or the genitalia may be involved in the lymphostasis because the inguinal lymph nodes receive lymph fluid from the leg, the ipsilateral lower quadrant of he trunk and the exterior genitals10. MLD is therefore performed in steps: the first step is to stimulate the lymph vessels in the non-affected contralateral trunk quadrant which results in a suction effect6 on the lymphatics of the affected trunk quadrant. In the second step, –2– National Lymphedema Network • 2211 Post Street • Suite 404 • San Francisco, California 94115 • Tel: 800-541-3259 • Fax: 415-921-4284 edema fluid is cautiously pushed from the congested quadrant into the quadrant free of edema via tissue channels, initial lymphatics and lymph vessels bridging the watersheds, thus creating a connection between regional lymph nodes on the contralateral and ipsilateral sides. After the trunk is decongested, the upper part of the extremity is treated and, later on, the distal part and the hand/foot – always making sure not to overwhelm the drainage areas previously stimulated. Many patients we see report that even though they received many treatments in “Manual Lymph Drainage,” the lymphedema didn’t improve and sometimes the limb size even increased. Asking the patient how the treatment was performed, in many cases we hear that the therapist performed an effleurage beginning at the fingers or toes or used massage techniques on the swollen extremity. As mentioned before, MLD is a very gentle manual technique consisting of four basic strokes and any combination of same. MLD has nothing to do with “classical” or “Swedish” massage and shouldn’t be called massage. The word “massage” means “to knead” (Greek: massain) – Manual Lymph Drainage does not have kneading elements and generally is applied suprafascially, whereas massage usually is applied to subfascial tissues. Compression Therapy Since the elastic fibers of the skin are damaged in lymphedema, it is mandatory to apply sufficient compression to the affected area in order to prevent re-accumulation of fluid. Compression therapy increases the tissue pressure (TP) which results in lower effective ultra-filtration and better reabsorption on the venous end of the blood capillaries. It also promotes the filling of initial lymph vessels, improves the function of the muscle pumps and helps to reduce fibrosclerotic tissue. In phase I of the therapy compression is applied via shortstretch bandages. Short-stretch bandages have a high working pressure (pressure the bandage exerts on the musculature working underneath) and a low resting pressure (pressure exerted on the tissue while resting). Long-stretch bandages have exactly opposite characteristics and, therefore, are not indicated in the treatment of lymphostatic edema since they tend to cut into the tissue while resting, causing a tourniquet effect and thus impeding sufficient lymph and blood flow. Long-stretch bandages also fail to produce an effective counterforce to the working muscles. In order to avoid irritation on bony prominences and tendons, padding with cotton bandages or foam is applied underneath the bandages. To enhance the reduction of fibrosclerosis, high density foam is frequently used in combination with short-stretch bandages. Low pH-lotion is used to keep the skin moist and tubular bandages to avoid allergic reactions and to protect the bandage materials are also applied. During phase I of CDP, compression therapy during day and night is achieved by short-stretch bandages. In phase II, the patient wears compression garments during the day and applies bandages for the night. Measurements for these elastic support garments should be taken at the end of phase I by the therapist or the supervising physician. An incorrectly fitted sleeve or stocking will have negative effects on the lymphedema itself and on the patients compliance. To achieve the best results with CDP, good compliance of the patient is absolutely necessary. The compression class and the type of garment (round or flat-knit style) depends on the severity of the swelling, the patients age and any other relevant factors. In general the pressure of the garment should be as high as the patient can tolerate14. For lower extremity lymphedema, compression classes III (30-40 mm/Hg) or IV (>50 mm/Hg); for lymphedema of the upper extremities, compression classes I (10-20 mm/Hg) or II (20-30 mm/Hg), sometimes compression class III, are suitable. In some cases, it might be necessary to apply even a greater compression than class IV which can be achieved by wearing two stockings on top of each other, or by the application of bandages on top of a stocking. To have the maximum effect, garments must be worn every day and replaced after six months. Remedial exercises aid the lymphokinetic effects of joint and muscle pumps and should be performed by the patient wearing the compression bandage or the garment. The exercise program should be customized for each patient depending on the individual capacities. Exercises should be performed slowly and with both the affected and non-affected extremity. Vigorous movements or exercises causing pain must be avoided. When does CDP fail? Phase I : malignant lymphedema artificial lymphedema improper treatment (MLD as the only treatment, no MLD or improper bandage) associated illnesses lack of compliance Phase II : lack of compliance lack of hygiene reoccurrence of cancer associated illnesses Conclusion Lymphedema can be treated successfully by a skilled therapist with extensive training in all components of Complete Decongestive Physiotherapy and good patient compliance. The treatment success must be monitored by circumferential and/or volumetric measurements. If phase I of CDP is performed in the early “pitting” stage of lymphedema, total remission of the swelling is possible. In later stages of lymphedema, phase I only reduces the swelling but fibrosclerotic tissue changes will still be present. For these –3– National Lymphedema Network • 2211 Post Street • Suite 404 • San Francisco, California 94115 • Tel: 800-541-3259 • Fax: 415-921-4284 more advanced stages, phase II of Complete Decongestive Physiotherapy not only preserves the treatment success achieved in the intensive phase but also improves the edema over time, restoring the limb to a normal or near normal size. REFERENCES: 1 2 3 4 5 6 7 8 9 10 11 12 13 14 Marvin Boris, et al. Lymphedema Reduction By Noninvasive Complex Lymphedema Therapy Cornell University School of Medicine and Lymphedema Therapy. Woodbury, NY, Sept. 1994 G.Bringezu, O.Schreiner Die Therapieform Manuelle Lymphdrainage Otto Haase Verlag, Germany 1987 J.R. Casley-Smith Lymphedema, The Poor and Benzo-Pyrones: Proposed Amendments To The Consensus Document Lymphology 29, 1996, 137-140 J.Casley-Smith, R.Morgan, N.Piller Treatment of Lymphedema of the arms and legs with 5,6-Benzopyrone New England Journal of Medicine Vol. 329 No 16, Oct. 14, 1993 L.Clodius, M.Foeldi Therapy For Lymphedema Today Inter. Angio., 3 1984 E.Földi, M.Földi, L.Clodius The Lymphedema Chaos: A Lancet Ann Plast Surg 22:505-515, 1989 E.Földi Preventions of Dermatolymphangioadenitis By Combined Physiotherapy Of The Swollen Arm After Treatment For Breast Cancer Lymphology 29, 1996, 48-49 M.Földi Treatment of Lymphedema (Editorial) Lymphology 27,1994 1-5 M.Földi, E.Földi Das Lymphoedem Gustav Fischer Verlag, Germany 1991 M.Földi, S.Kubik Lehrbuch der Lymphologie Gustav Fischer Verlag, Germany 1992 S.Kubik The Lymphatic System Springer, NY 1985 R.Lerner, J.Petrek Lymphedema Diseases Of The Breast, Lippincott, pgs. 896-902 – Raven, 1996 J.Zuther Understanding Lymphedema, Pathophysiology and Treatment PT&OT Today Vol.5, No. 39 The Diagnosis and Treatment of Peripheral Lymphedema Consensus Document of the International Society of Lymphology Executive Committee Lymphology 28 (1995) 113-117 Joachim E. Zuther is a Certified Instructor for MLD/CDP and the founder of the Academy of Lymphatic Studies based in Florida. He is also the Director and primary Instructor at the ulmkolleg, Dept. of Lymphology in Ulm, Germany and the Director of Quality Control at Lerner Lymphedema Services. –4– National Lymphedema Network • 2211 Post Street • Suite 404 • San Francisco, California 94115 • Tel: 800-541-3259 • Fax: 415-921-4284