This is some "refresher" information, but I thought the detailed information about self care was very good. It's very discouraging that there is no cure for lymphadema, but one of the best things you can do is to remain hopeful and do your best to take care of yourself - both physically and mentally. I know it can be difficult at times, especially if you don't have people around you that understand what it's like to live with lymphadema - lucky for us we have the internet and we know that we aren't really alone.
Here's to a brighter New Year - hoping that you find a treatment that helps you, and that you find some peace within yourself while coping with this disease!
Lymphedema is a notoriously debilitating progressive condition with no known cure. The unfortunate patient faces a lifelong struggle of medical, and sometimes surgical, treatment fraught with potentially lethal complications.
The underlying problem is lymphatic dysfunction, resulting in an abnormal accumulation of interstitial fluid containing high molecular weight proteins. This condition underscores the tremendous importance of a normally functioning lymphatic system, which returns proteins, lipids, and accompanying water from the interstitium to the venous circulation near the subclavian vein–internal jugular vein junction, bilaterally.
- In the United States, the highest incidence of lymphedema is observed following breast cancer surgery, particularly among those who undergo radiation therapy following axillary lymphadenectomy. Among this population, 10-40% develop some degree of ipsilateral upper extremity lymphedema.
- Worldwide, 140-250 million cases of lymphedema are estimated to exist, with filariasis being the most common cause
At present no cure for lymphedema exists. Surgery is palliative at best, and it may be a part of the lifelong therapy patients must endure to manage this disease.
Medical therapy: The goal of conservative therapy is to eliminate protein stagnation and restore normal lymphatic circulation. Initiate therapy as early as possible before extensive irreversible fibrosclerotic changes occur in the interstitium. These techniques are often cumbersome, uncomfortable, inconvenient, and time-consuming. Strict compliance is essential, and treatment lasts throughout the lifetime of the individual. The majority of compliant patients can be treated successfully with conservative measures.
Meticulous hygiene is necessary to remove keratinaceous debris and bacteria. Cleanse the skin regularly and dry thoroughly. Regular inspection is necessary to identify any open wounds or developing cellulitis. Bland skin moisturizers applied conservatively may ameliorate cracking and furrowing. Even with excellent skin care, chronic cellulitis may occur. At the earliest signs of infection, institute topical or systemic antifungal or antimicrobial therapy to prevent sepsis from developing. Long-term prophylactic antimicrobial treatment with agents such as penicillin, cephalexin, or erythromycin may be required in 15-25% of patients experiencing recurrent lymphangitis or cellulitis. Although relatively uncommon in this country, filariasis is treated with diethylcarbamazine.
Encourage patients to lose weight, avoid even minor trauma, and avoid constrictive clothing that might have a tourniquet effect. Encourage elevation of the affected extremity whenever possible, particularly at night. For lower extremity lymphedema, this may be accomplished by elevating the foot of the bed to an appropriate level.
Patients should use compression garments continuously during the day. They may be removed at night when the extremity is elevated in bed, but they should be replaced promptly each morning. To encourage compliance, the elastic compression garments must fit appropriately. Garments should be custom fit when the extremity is decompressed, they should be comfortable, and they should not have a tourniquet effect. They should also have graduated compression, increasing from distal to proximal, on the affected extremity.
Intermittent pneumatic pump compression therapy may also be instituted on an outpatient basis or in the home. These manual lymphatic devices are most appropriate prior to fibrosclerotic evolution, and they assist in preventing fibrosclerotic evolution of the condition. These devices provide sequential active compression from distal to proximal, effectively milking the lymph from the extremity. In Europe, the best available nonsurgical therapy is manual lymphatic drainage according to the Vodder and/or Leduc techniques. Compression garments are essential between treatments. Contraindications to this therapy include congestive heart failure, deep vein thrombosis, and active infection. Similarly, other authors advocate manual massage of the affected extremity to recruit collateral vessels so that the accumulated lymph can be drained into neighboring regions with normally functioning lymphatics.
Benzopyrenes, including flavonoids and coumarin, have become a useful adjuvant in other countries but are currently not available for clinical use in the United States. These drugs bind to accumulated interstitial proteins, inducing macrophage phagocytosis and proteolysis. The resulting protein fragments pass more readily into the venous capillaries and are removed by the vascular system.
Surgical therapy: Surgical treatment is palliative, not curative, and it does not obviate the need for continued medical therapy. Moreover, it is rarely indicated as the primary treatment modality. Rather, reserve surgical treatment for those who do not improve with conservative measures or in cases where the extremity is so large that it impairs daily activities and prevents successful conservative management. The goals of surgical therapy are volume reduction to improve function, facilitation of conservative therapy, and prevention of complications. A myriad of surgical procedures have been advocated, reflecting a lack of clear superiority of one procedure over the others. In general, surgical procedures are classified as physiologic or excisional.
Physiologic procedures attempt to improve lymphatic drainage. Multiple techniques have been described, including omental transposition, buried dermal flaps, enteromesenteric bridging, lymphangioplasty, and microvascular lympholymphatic or lymphovenous anastomoses. None of these techniques has clearly documented favorable long-term results. Further evaluation is necessary. Moreover, many of theses physiologic techniques also include an excisional component, making it difficult to distinguish the two approaches.
Excisional techniques remove the affected tissues, thus reducing the lymphedema load. Some authors advocate suction-assisted removal of subcutaneous tissues, but this technique is difficult because of the extensive subcutaneous fibrosis that is present. Additionally, this approach does not reduce the skin envelope, and the lymphedema often rapidly recurs. Suction-assisted removal of subcutaneous tissue followed by excision of the excess skin envelope has no clear advantage over direct excisional techniques alone.
The Charles procedure is another quite radical excisional technique. This procedure involves the total excision of all skin and subcutaneous tissue from the affected extremity. The underlying fascia is then grafted, using the skin that has been excised. This technique is extreme and is reserved for only the most severe cases. Complications include ulceration, hyperkeratosis, keloid formation, hyperpigmentation, weeping dermatitis, and severe cosmetic deformity.
Staged excision has become the option of choice for many authors and is described in greater detail. This procedure involves removing only a portion of skin and subcutaneous tissue, followed by primary closure. After approximately 3 months, the procedure is repeated on a different area of the extremity. This procedure is safe, reliable, and demonstrates the most consistent improvement with the lowest incidence of complications.