Lymphedema is an abnormal accumulation of protein-rich fluid in the interstitium, which causes chronic inflammation and reactive fibrosis of the affected tissues. Primary lymphedema is congenital and is the result of lymphatic dysplasia that may present at birth but more often develops later in life (peri-pubertal onset). Secondary lymphedema is the result of lymphatic system failure secondary to a trauma or injury to the system. In this country, it most often occurs after surgery or radiation therapy to treat cancer and results in a mechanical insufficiency. The lymphatic system itself is damaged and cannot keep up with the demands of the additional fluid and proteins in the interstitium. Secondary lymphedema can also occur due to obesity, chronic venous insufficiency, cardiac edema, cardiac bypass, orthopedic surgery, liposuction, etc. and begins initially with a dynamic insufficiency (fluid overload but intact lymphatics). Over time, this progresses to a combined dynamic and mechanical insufficiency when the lymphatic system has been overtaxed to the point where permanent damage begins to occur.
Dynamic insufficiencies are low protein edemas consisting mostly of water and typically reverse with elevation. Mechanical insufficiencies are high protein edemas and will not respond as well, or at all, to elevation or pneumatic pumps because neither causes the proteins in the interstitium to be reabsorbed. This is when complete decongestive therapy (CDT) is needed, which consists of manual lymph drainage (MLD), multi-layer compression bandaging, long term use of compression garments, remedial exercise, skin/nail care, and patient education for prevention and maintenance since lymphedema is a chronic condition and there is no cure.
We can help people with mechanical, dynamic, or mechanical/dynamic insufficiency but the course of treatment will be different and we will expect different outcomes based on the patient’s type of edema. Those who have a dynamic insufficiency (fluid overload) with no damage to the lymphatic system will respond very well to compression garments, exercise, and education and may be able to skip the MLD and bandaging. However, for those with a high protein or mixed edema, the gold standard of treatment is CDT.
Early Treatment is Important
Lymphedema progresses at different unpredictable rates. Some limbs may stay in the congested state without developing fibrosis for quite a while and others develop fibrosis more quickly. Once fibrosis develops, it becomes more difficult to achieve good outcomes, so early treatment is crucial.
Patient education: patients are instructed to contact a healthcare provider immediately if they begin to experience feelings of heaviness, tightness, notice that clothing or jewelry are fitting more tightly, or if the at-risk area becomes hot or red. A patient who has no visible edema may still be in the initial stage 0 of lymphedema and referral for treatment is important even in early stages.
Signs and Symptoms of Various Edemas
Chronic venous insufficiency: Without appropriate therapy, venous edema triggers a lymphatic failure (mechanical insufficiency) and results in a combined phlebolymphostatic edema.
Traits: Gaiter distribution, non-pitting (indentation occurs but quickly refills), brawny (leathery or woody texture), hemosiderin staining (dark blue, brown or purple discoloration of the gaiter distribution), fibrosis of the subcutaneous tissue, ulcers are common.
Cardiac Edema: (Caused by Right Heart Failure) CHF creates hypertension in the venous limb of the system, continued circulation increases blood capillary pressure at the microcirculation level resulting in back pressure and edema.
Traits: Most fluid builds up distally, always bilateral, pitting (but will refill quickly), complete resolution with elevation, no pain, jugular venous distention, orthopnea, paroxysmal nocturnal dyspnea, or dyspnea on exertion.
Acute Deep Vein Thrombosis: Sudden onset, usually unilateral, pain, cyanosis, swelling, distention of surface veins and pulmonary embolism.
Malignant lymphedema: May be an indication of new onset or recurrent cancer.
Traits: Pain, paresthesia, paralysis, proximal onset, rapid development and continuous progression, shiny tight skin, swelling in nodules in supraclavicular fossa, hematoma-like discoloration, ulcers, non-healing open wounds.
Stages of Lymphedema:
Stage 0: the latency stage, no visible or palpable edema, subjective complaints possible.
Stage 1: accumulation of protein-rich edema fluid, pitting edema, reduces with elevation, no fibrosis.
Stage 2: accumulation of protein-rich edema fluid, pitting becomes progressively more difficult, connective tissue proliferation (fibrosis).
Stage 3: accumulation of protein-rich edema fluid, non-pitting, fibrosis and sclerosis, skin changes (papillomas, hyperkeratosis etc.).
How does CDT reduce and manage chronic edema?
Manual lymphatic drainage (MLD) decongests the limb. Stretching of skin causes anchoring filaments attached to lymph capillaries to open the endothelial junctions and absorb proteins in interstitial fluid. The slow wave-like rhythm speeds the contractions and transport of the lymphatic fluid while also promoting relaxation and an increase in parasympathetic activity.
MLD is followed by multi layer compression bandaging, which provides an external force increasing interstitial pressure and promoting reabsorption to cause further volume reduction and maintain progress.
Remedial exercise with compression bandages in place further decreases fluid through muscle pumping action, while the limb is surrounded by short stretch bandages that offer a high working pressure during activity and low resting pressure when the muscles are not being used.
When the patient’s decongestive phase reaches a plateau, they are fit for long-term compression garments to maintain progress made during the intensive phase. At this time, the patient is educated in self MLD, exercises, prevention and management as well as meticulous skin/nail care to prevent complications such as cellulitis.
Efficacy: CDT will render a 50-70% reduction in limb volume during the intensive phase and is capable of reducing recurrent cellulitis by one-half.
Contraindications to treatment:
Uncontrolled CHF, renal failure, cardiac edema, acute DVT and active infection (cellulitis, erysipelas) are all contraindications. If the patient does not currently have cellulitis but fungus is present, they may need an anti fungal regime prescribed by the doctor prior to treatment.
There is no evidence of CDT expediting the spread of cancer but the patient must be monitored by physician. For those with active cancer, MLD is highly recommended as an analgesic modality.
Meeting the Needs of Our Patients:
The first consideration should always be: who is qualified to offer the best care? There is very little lymphedema education given in schools. Valerie Anderson, DPT, CLT has undergone 135 hours of additional training to become a certified lymphedema therapist. She will soon take the Lymphology Association of North America (LANA) exam and become nationally recognized as a LANA certified therapist.
CDT is the gold standard of care but people may not be able to commit to the time, expense and full-time compression garments required. Currently, most insurances do not cover bandages and compression garments, which can present a severe financial burden to the patient. We will thoroughly evaluate the patient and discuss with them the options of treatment. Together, we will formulate a plan to give the best course of treatment while still remaining in their budget and time constraints.