Since 1950, every year, 7th April is observed as World Health Day – an opportunity for drawing worldwide attention to the important issue of global health. The day is acknowledged by various governments and the NGOs interested in public health issues wherein, several international, regional and local events are organized on a particular theme, under the World Health Organization (WHO) sponsorship. Each year, officials select a theme for the World Health Day and some of the past themes included Healthy heart beat, Healthy blood pressure, Vector-borne diseases: small bite, big threat, Food safety and Halt the rise: Beat diabetes. This year’s focus is on “Lymphedema Awareness and Cures”. Lymphedema is a condition faced by many but still lacks basic awareness.

LET’S UNDERSTAND LYMPHEDEMA

Lymphedema is swelling in one or more extremities that results from impaired flow of the lymphatic system. The lymphatic system is a network of specialized vessels (lymph vessels) throughout the body whose purpose is to collect excess lymph fluid with proteins, lipids, and waste products from the tissues. This fluid is then carried to the lymph nodes, which filter waste products and contain infection-fighting cells called lymphocytes. The excess fluid in the lymph vessels is eventually returned to the bloodstream. When the lymph vessels are blocked or unable to carry lymph fluid away from the tissues, localized swelling (lymphedema) is the result. Lymphedema most often affects a single arm or leg, but in uncommon situations both limbs are affected. There are two types of Lymphedema conditions. These include

• Primary lymphedema is the result of an anatomical abnormality of the lymph vessels and is a rare, inherited condition.
• Secondary lymphedema results from an identifiable damage to or obstruction of normally-functioning lymph vessels and nodes.

Worldwide, lymphedema is most commonly caused by filariasis (a parasite infection), but in many countries, lymphedema most commonly occurs in women who have had breast cancer surgery, particularly when followed by radiation treatment. Mild lymphedema first may be noticed as a feeling of heaviness, tingling, tightness, warmth, or shooting pains in the affected extremity. These symptoms may be present before there is obvious swelling of an arm or leg. Other signs and symptoms of early or mild lymphedema include:

• A decreased ability to see or feel the veins or tendons in the extremities,
• Tightness of jewelry or clothing,
• Redness of the skin,
• Asymmetrical appearance of the extremities,
• Tightness or reduced flexibility in the joints, and
• Slight puffiness of the skin.

The swelling caused by lymphedema ranges from mild hardly noticeable changes in the size of your arm or leg to extreme changes that make the limb hard to use. Lymphedema caused by cancer treatment may not occur until months or years after treatment.

PRIMARY LYMPHEDEMA CAUSES

Primary lymphedema is an abnormality of an individual’s lymphatic system and is generally present at birth, although symptoms may not become apparent until later in life. Depending upon the age at which symptoms develop, three forms of primary lymphedema have been described. Most primary lymphedema occurs without any known family history of the condition.

• Congenital lymphedema is evident at birth, is more common in females, and accounts for about 20% of all cases of primary lymphedema. A subgroup of people with congenital lymphedema has a genetic inheritance which is termed Milroy disease.
• Lymphedema praecox is the most common form of primary lymphedema. It is defined as lymphedema that becomes apparent after birth and before age 35 years and symptoms most often develop during puberty. Lymphedema praecox is four times more common in females than in males.
• Primary lymphedema that becomes evident after 35 years of age is known as Meige disease or lymphedema tarda.

SECONDARY LYMPHEDEMA CAUSES

Secondary lymphedema develops when a normally-functioning lymphatic system is blocked or damaged. In many places, breast cancer surgery, particularly when combined with radiation treatment, is the most common cause. This results in one-sided (unilateral) lymphedema of the arm. Any type of surgical procedure that requires removal of regional lymph nodes or lymph vessels can potentially cause lymphedema. Surgical procedures that have been associated with lymphedema include vein stripping, lipectomy, burn scar excision, and peripheral vascular surgery. Damage to lymph nodes and lymph vessels, leading to lymphedema, can also occur due to trauma, burns, radiation, infections, or compression or invasion of lymph nodes by tumors.

Worldwide, however, filariasis is the most common cause of lymphedema. Filariasis is the direct infestation of lymph nodes by the parasite Wuchereria bancrofti. The disease is spread among persons by mosquitoes, and affects millions of people in the tropics and subtropics of Asia, Africa,Western Pacific, and parts of Central and South America. Infestation by the parasite damages the lymph system, leading to swelling in the arms, breasts, legs, and, for men, the genital area. The entire leg, arm, or genital area may swell to several times its normal size. Also, the swelling and the decreased function of the lymph system make it difficult for the body to fight infections. Lymphatic filariasis is a leading cause of permanent disability in the world. The swelling of lymphedema usually occurs in one or both arms or legs, depending upon the extent and localization of damage. Primary lymphedema can occur on one or both sides of the body as well. Lymphedema may be only mildly apparent or debilitating and severe, as in the case of lymphatic filariasis (see above), in which an extremity may swell to several times its normal size. It may first be noticed by the affected individual as an asymmetry between both arms or legs or difficulty fitting into clothing or jewellery. If the swelling becomes pronounced, fatigue due to added weight may occur, along with embarrassment and restriction of daily activities.

The long-term accumulation of fluid and proteins in the tissues leads to inflammation and eventual scarring of tissues, leading to a firm, taut swelling that does not retain its displacement when indented with a fingertip (nonpittingedema). The skin in the affected area thickens and may take on a lumpy appearance described as an orange-peel (peaud’ orange) effect. The overlying skin can also become scaly and cracked, and secondary bacterial or fungal infections of the skin may develop. Affected areas may feel tender and sore, and loss of mobility or flexibility can occur.

Other symptoms can accompany the swelling of lymphedema including:

• Warmth, redness, or itching
• Tingling or burning pains
• Fever and chills
• Decreased flexibility in the joints
• Aching, pain, fullness of the involved area
• Skin rash

The immune system function is also suppressed in the scarred and swollen areas affected by lymphedema, leading to frequent infections and even a malignant tumor of lymph vessels known as lymphangiosarcoma. After understanding the causes and symptoms lets understand the possible treatments of the condition. There is no cure for lymphedema. Treatments are designed to reduce the swelling and control discomfort and other symptoms. Compression treatments can help reduce swelling and prevent scarring and other complications. Examples of compression treatments are:

• Elastic sleeves or stockings: These must fit properly and provide gradual compression from the end of the extremity toward the trunk.
• Bandages: Bandages that are wrapped more tightly around the end of the extremity and wrapped more loosely toward the trunk, to encourage lymph flow out of the extremity toward the center of the body.
• Pneumatic compression devices: These are sleeves or stockings connected to a pump that provides sequential compression from the end of the extremity toward the body. These may be used in the clinic or in the home and are useful in preventing longterm scarring, but they cannot be used in all individuals, such as those with congestive heart failure, deep venous thrombosis, or certain infections.
• Manual compression: Massage techniques, known as manual lymph drainage, can be useful for some people with lymphedema.
• Exercises: Exercises that lightly contract and stimulate arm or leg muscles may be prescribed by the doctor or physical therapist to help stimulate lymph flow.

Surgical treatments for lymphedema are used to remove excess fluid and tissue in severe cases, but no surgical treatment is able to cure lymphedema. Infections of skin and tissues associated with lymphedema must be promptly and effectively treated with appropriate antibiotics to avoid spread to the bloodstream (sepsis). Patients affected by lymphedema must constantly monitor for infection of the affected area. In affected areas of the world, the drug diethylcarbamazine is used to treat filariasis. Lymphedema as a condition never gained much momentum despite many suffering from this issue. It was in 2017 that many signed petitions asking WHO to make Lymphedema the theme of this year’s World Health Day. The goal by this is to lymphedema (LE) and lymphatic diseases a global priority, to engage governments and researchers around the world and to create awareness.