Lymphedema

Lymphedema is either primary or secondary. Primary lymphedema is congenital or inherited and is caused by genetic factors that disrupt the functioning of lymphatic vessels. Secondary lymphedema is far more common and is usually the result of cancer treatment. Radiation, and surgery that involves the lymph nodes or vessels is the most common direct cause. Injuries and infections can also lead to secondary lymphedema.

Here is a quick look at the various stages and associated symptoms of lymphedema: 

• Stage 1: Abnormal flow caused by blockage in the lymphatic vessels. The patient will not have any symptoms or signs at this stage.
• Stage 2: Swelling will develop in the area, but may not be too noticeable. Elevating or moving the limb will reduce the swelling; skin will feel soft and will dent when pressed.
• Stage 3: The skin will begin to scar and you will notice that it feels firmer and heavier. Mobility may be affected as well. Swelling will not decrease in elevation or movement. (For more information, read our article on scars.) 
• Stage 4: By this stage, the swelling will be substantial and obvious. The limbs will look asymmetrical and there will be scarring on the skin. Infections are more likely at this stage and you have to be careful with the affected area. Further, more drastic interventions will be required to bring the disease under control now.

Lymphedema, in its earlier stages, manifests in subtle ways and may be hard to detect. Symptoms become more severe and much more obvious as the disease progresses, and include: 

• Sensory changes such as tingling, discomfort or dull aches in the affected areas.
• The skin feels warmer than normal around the affected areas.
• Swelling that slowly becomes conspicuous. Rings, clothes and other accessories will start to feel tighter or not fit right.
• Affected area feels heavy and range of motion and utility diminishes.
• Hardening and thickening of skin.
• Increased infections, and a longer recovery time for injuries in the area.

As mentioned above, lymphedema is caused by the blockage of lymph nodes which prevent excess fluids and toxins from being drained by the body. Secondary lymphedema is more common and is caused when another condition puts a strain on the lymphatic system, such as: 

• Cancer: Cancerous growths can block lymphatic vessels or grow around lymph nodes which diminishes their ability to drain excess fluid. Underlying cancer or growths like this need to be addressed to treat this type of lymphedema.
• Surgery: This is one of the leading causes of lymphedema. Lymph vessels or nodes can be damaged during surgery that involves removing malignant growths, or for biopsies to check for suspicious lesions. This impairs the ability of the lymphatic system to drain fluids optimally and leads to swelling and other complications. 
• Radiation therapy: Radiation therapy can cause damage to lymphatic vessels, impairing their abilities to work properly.
• Infections: Various infections that are able to penetrate the lymphatic system can cause circulation issues and lead to lymphedema. 

Primary lymphedema is rare and inherited. Certain genetic mutations cause deformities in structure and function of lymphatic vessels and lead to various types of lymphatic dysfunction. Some of these disorders include:

• Milroy’s disease: This is a congenital disease that causes abnormal formation of lymphatic vessels.
• Meige disease: This disease leads to lymphedema usually during pregnancy or before the age of 35.
• Late onset lymphedema: This a rare condition in which lymphedema develops later on in life due to issues in the lymphatic system.

Apart from these, certain characteristics increase the likelihood of lymphedema as well, including age, being overweight or obesity, and rheumatoid or psoriatic arthritis.

Unfortunately, there is not much you can do to prevent lymphedema. In some cases it is congenital or inherited, so there is no way to prevent it. Before going in for cancer treatment, or interventions that will affect lymphatic vessels, consult with your doctor about minimizing risks or opting for alternative therapies. This is all the more necessary if there is a history of lymphatic dysfunction in your family. 

If you are at a high risk of lymphedema, you must take extra precautions, especially with your limbs. You may be more vulnerable to cuts and scrapes transforming into more serious infections, so take adequate steps to reduce the likelihood of injuries. Further, healthy lifestyle choices will improve your overall health and reduce the chances of developing chronic conditions such as lymphedema. 

(Read our article on healthy diets for more information.)

As mentioned above, a person is at a higher risk of contracting lymphedema if they have had cancer surgery involving the lymph nodes, as the doctor may be able to diagnose it based on the visible, physical signs and other symptoms.

Other imaging tests are also recommended in case the symptoms aren't on a surface level. High-resolution images from an MRI scan, CT scans, colour doppler ultrasounds, or a more specific imaging of the lymphatic system called lymphoscintigraphy, where the patient is injected with a dye can detect blockages in the lymph nodes.

There is currently no treatment available for lymphedema. Management of the condition, however, is aimed at reducing swelling, minimizing pain and retaining function in the affected area. Remember, that even more comprehensive approaches will only minimize symptoms; there will be a recurrence of swelling and discomfort that will require constant care. 

Treatment depends on the underlying condition and the stage of the disease. For stages 1 and 2, these practical interventions help:

• Physiotherapy and light exercise: Lightly exercising the affected area could improve circulation and encourage excess fluid to drain or move towards another node. Exercises should be simple and not cause you to exert yourself; the idea is to just get the blood and lymph fluids flowing. A physiotherapist can assist you in planning exercises that fit these requirements. 
• Compression garments: These can be fit snugly on the affected limb to encourage the movement of excess fluid. The garment will be chosen depending on the severity of the swelling - the more severe the situation, the tighter the garment. Usually, gradient compression garments are used that are tighter around the toes or finger and looser towards the center of the limb. Wrapping the affected limb in such a way can ease symptoms as well - your doctor will show you how to do this properly. 
• Pneumatic compression: This involves a sleeve wrapped around the affected limb that is regularly inflated and deflated by a machine connected to it. The regular compression and decompression will improve blood flow and may also have a soothing effect and reduce pain. 
• Massage: A special type of massage called manual lymph drainage may help some people as well. Similar to the previously mentioned approaches, the technique will encourage excess fluid to move and drain in other nodes and reduce swelling. Massage is not recommended if there is a skin infection or blood clots from active diseases in the lymph nodes.
• Complete decongestive therapy (CDT): This strategy involves using all or some of the above mentioned approaches, along with lifestyle and dietary changes. Obesity and lack of exercise also increases the likelihood and severity of lymphedema, so this holistic approach can help those with these problems as well. 

For more serious cases, these interventions may not be enough. If symptoms do not improve, surgery may be required. Some options are available:

• Liposuction: Excess lymph fluid that is not being drained by lymph nodes will cause fat cells to proliferate and cause the affected area to swell up. Liposuction of the affected area is a simple procedure wherein the excess fat and fluid is drained to reduce swelling.
• Lymphovenous bypass: This involves rerouting the lymphatic system such that the affected node is bypassed and/or connecting the affected part of the system directly to blood vessels to reduce the flow to the affected area and reducing swelling.
• Lymphovenous transplant: In this procedure, functional nodes from other parts of the body are transplanted to the affected area to improve drainage and reduce swelling.

If lymphedema is caused by a cancerous growth impeding the functioning of the lymph node, then the underlying cancer will need to be treated. Similarly, if an infection is responsible for the condition, this will have to be appropriately addressed as well.

Lymphedema increases the likelihood of infections to the affected area since the immune system is compromised. Wounds take longer to heal as well. Lymphangiosarcoma, a rare type of cancer, can also be precipitated by severe forms of lymphedema. 

(Read more: Open wounds)