Most of the functions of the human body boil down to the basic plumbing. Your heart, kidneys and liver are no exception. All of these organs are used to move or filter fluids in the body. However, not all fluids are controlled by the pumping organs, as there is also fluid between tissues.
The fluid that flows through and between most tissues in the body is called lymph. It is mainly composed of chyle, an intestinal fluid that contains proteins and fats, and white blood cells called lymphocytes.
Lymph fluid collects from tissue spaces around the body and flows through at least one of about 600 lymph nodes. Lymph nodes filter potentially harmful or infectious particles from lymph fluid, which moves about 4 liters of this fluid around your body each day.
The lymphatic system is usually very efficient at moving fluids around the body and maintaining balance. But when the flow of this fluid is interrupted and builds up, it can cause a painful swelling called lymphedema, usually in the arms or legs. This article will explore why some people develop lymphedema and what can be done to treat it.
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Types of Lymphedema
Lymphedema is the term used to describe the swelling that occurs when lymph fluid builds up in your body tissues. This usually happens due to damage to the lymphatic system or some type of blockage. There are two main types of lymphedema: primary and secondary.
Primary lymphedema develops as a rare genetic disorder. It usually occurs in one of the following stages:
- Infancy: This is a form of congenital lymphedema, also known as Milroy’s disease.
- Adolescence, pregnancy, and before age 35: Lymphedema that develops during these life stages may be caused by a combination of genetic and environmental factors. It may also be called Meg’s disease.
- After age 35: This late-onset lymphedema is rare and usually only causes swelling in the legs.
What’s in the name?
Primary lymphedema, as its name might suggest, is the predominant form of the disease, but it is the rarest, affecting about 1 in 100,000 people in the United States. This type of lymphedema is called primary lymphedema because the condition develops on its own, not due to other medical conditions or injuries.
Secondary lymphedema is the most common form of the disease, affecting about 1 in 1,000 Americans. This condition occurs when the drainage of the lymphatic system is damaged by trauma, injury, or other types of damage. Secondary lymphedema is a common complication of cancer treatment.
Swelling is the main symptom associated with lymphedema, but you may also experience other sensations or sensations as this swelling puts pressure on your body.
Common symptoms of lymphedema include:
- swelling, mainly in the arms or legs
- Skin that feels tight, hot, firm, or has a change in texture
- pain or tingling
- numbness in the swollen area
- A feeling of heaviness in the limbs
- loss of mobility or flexibility
- Change the way your clothes or jewelry fit without gaining weight
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Any condition, injury, or treatment that affects your immune or lymphatic system can disrupt lymph flow. Some of the common causes associated with the development of secondary lymphedema are:
- Breast cancer surgery, especially if lymph nodes are involved
- cancerous tumor
- cancer treatment
- crush injury
- scar tissue or surgical incisions that disrupt the flow of lymph
- congestive heart failure or other heart disease
- Vascular diseases such as venous insufficiency
- kidney disease
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Lymphedema is usually diagnosed by reviewing your medical history and physical examination. For a physical exam, your doctor may take measurements and examine the skin of the affected limb or body area.
Your healthcare provider may also look for something called a Stemmer sign. If they try to pinch a little tissue on the back of your hand or instep and can’t, that’s a positive Stemmer sign. A positive Stemmer sign is usually consistent with a diagnosis of lymphedema.
Your medical history and details of any recent injuries or infections are also an important part of the diagnosis. Diagnosis may be easier if you have an injury, cancer, or other event that puts you at higher risk of developing lymphedema.
If there is any doubt about what’s causing the swelling, your doctor may perform additional tests.
The main test for lymphedema is called fluoroscopy. This is a computer-based test that helps measure the amount and displacement of fluids in your body. Other tests to help narrow down the cause of swelling include a tonometer that measures pressure or imaging studies such as computed tomography (CT).
There are also some more detailed studies commonly used for chronic, severe or advanced lymphedema. These include:
- Bioimpedance Spectroscopy (BIS)
- Near Infrared Fluorescence Imaging (NIRF)
- Magnetic resonance lymphangiography
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There are both surgical and non-surgical options for treating lymphedema.
Complete decongestant therapy (CDT) is a major non-surgical option that combines a variety of therapies, including:
- manual lymphatic drainage
- compression therapy
- compression device or garment
- skin care
Good self-care is important to manage lymphedema without surgery, and weight loss or weight management may also be part of your treatment plan.
The surgical option is often used in cases of lymphedema that have not responded to other, less invasive treatments. The main purpose of these surgeries is to clear the lymphatic system and/or remove parts of the tissue affected by lymphedema. Some common surgeries that can be used to treat lymphedema include:
- Lymphatic shunts alter lymphatic drainage pathways
- Lymph node metastases move healthy lymph nodes to poorly draining areas
- Liposuction to remove fat and other tissue from the affected area
- excision to remove a larger area of tissue and place a skin graft in its place
In some cases, lymphedema may be temporary, but for most people affected by this condition, it is a chronic, progressive disease. Early diagnosis and treatment can help control swelling and help you avoid serious swelling or infection.
Lymphedema is not usually life-threatening, but untreated or severe cases of lymphedema can lead to complications such as lymphangiosarcoma, a soft tissue cancer.
Living with any chronic disease can be difficult. Living with lymphedema means practicing good self-care and making lifestyle changes to help you avoid complications.
Steps you can take to help manage your lymphedema include:
- not strenuous exercise
- Keep your skin clean to avoid infection
- Handle even minor injuries with care
- Avoid tight clothing, shoes or jewelry
- Sit well and avoid crossing your legs
- Consider wearing tights regularly
- eat a healthy diet
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Lymphedema is a chronic condition that causes swelling that makes your skin tight and painful, and may even limit your movement. Some surgeries are available for advanced or severe cases, but most treatments for this condition focus on careful skin care, avoiding infection, and a healthy diet and exercise regimen.
Living with lymphedema can be frustrating because it restricts your movement and causes discomfort. There is no cure for lymphedema, a chronic condition that requires some major lifestyle changes and self-care. Talk to your doctor about management strategies that are right for you. With proper care, lymphedema is usually not fatal and can be managed to avoid serious complications.