June 1, 2018

What is Lymphedema?

Lymphedema or Lymphoedema (as it is known in the European community) is the collection of fluid, cellular matter and fat products from tissues that results in swelling of one or more limbs, neck, face or abdomen area due to a blockage in the lymphatic system.  Unlike Lipedema that presents symmetrically on both sides of the body, Lymphedema usually affects just one limb or area.  The most common form of Lymphedema is in the arm of women recovering from breast cancer.

Lymphedema is also present in those suffering with Lipedema in Stage 4, which is why they call it Lipo-Lymphedema.  Lymphedema can be slightly noticeable to disfiguring in those with the condition. While there is no cure for Lymphedema there are garments and special compression devices to assist in managing the swelling and movement of fluid out of the system.

Types of Lymphedema

Lower limb lymphedema Lymphedema is classified as either Primary or Secondary.

Primary Lymphedema

Primary Lymphedema is present at birth usually the result of a genetic defect that causes abnormal formation of the lymphatic vessels.  While present at birth symptoms may not develop until later in life.  Primary Lymphedema has been classified into three forms and depends on the age when symptoms appear.  It is not known whether there is a genetic link to this condition at this time.

  • Congenital Lymphedema – symptomatically affects mostly females at birth with a 20% prevalence rate.  A small group of people in this group show a genetic connection, which is known as Milroy disease.
  • Lymphedema Praecox – demonstrates between birth and 35 years of age and is the most common form of primary lymphedema.  Symptoms usually occur in females during puberty.
  • Meige disease or Lymphedema Tarda – symptoms appears after 35 years of age and is less common than the other two forms.

It is not evident at this time if Primary Lymphedema can develop in conjunction with Lipedema.  (I cannot find any supporting material to link Primary Lymphedema with Lipedema.)

Secondary Lymphedema

Secondary Lymphedema is caused by an unavoidable obstruction of lymphatic vessels and nodes usually brought about by surgery or a traumatic event to the body, such as deep cut, burns, radiation, scarring, vein stripping, or removal of a body part.   The most common Secondary Lymphedema in the United States is from breast cancer.  The combination of invasive surgery, coupled with chemotherapy, radiation therapy and removal of surrounding lymph nodes, damage surrounding lymph vessels and nodes causing swelling in the corresponding arm.

Those with Lipedema usually suffer with Secondary Lymphedema when fat clusters grow large enough to block existing lymph vessels.

Understanding the Lymphatic System (Basics)

The lymphatic system is one of least studied but most important parts of the human body.  It is a major part of your cardiovascular system and responsible for half of what keeps your body in homeostasis, or in balance. It is responsible for recycling used fluid (much like a garbage collection system), inspecting all objects flowing through its borders for invaders, like germs or cancer cells, and calls out the cavalry, your immune system, when invaders are detected.

Whether you are suffering from Lymphedema or Lipedema induced Lymphedema you need to have a cursory understanding what is happening in your lymph system.  Your body is a remarkable machine.  It has always fascinated me how all these systems work together behind the scenes to keep you healthy, moving, digesting, breathing, healing and feeling everything around you.  Let’s see if I can do a high level overview so that you can appreciate what is (and in some cases isn’t) happening inside your body. You can also watch this great Patreon video for a quick overview of the Lymphatic System too.

In school you probably remember lots of talks and movies about your heart and how it pumps blood throughout your body, carrying food, oxygen and water through arteries and capillaries and then returns it to your heart to repeat the process.  What you probably didn’t cover was what happens when food and fluid is dropped off to the cellular areas and how the waste is processed.  This is where the lymph system comes in.

Blood carrying oxygen, food (proteins) and water travels from the heart to large arterial vessels all the way down to  smaller vessels, capillaries, by way of a powerful high pressure system. This high pressure keeps the contents of the vessel from leaking out until it reaches an area of lower pressure, which is down in the thousands of capillaries in your legs, feet, arms, hands and other body parts. Your capillaries, which are semi-porous in nature then leak out oxygen, plasma, small proteins and water providing nourishment to surrounding tissue cells.  The fluid builds up is the area between the cells, called extracellular fluid, until the pressure becomes too great and the fluid is forced into a surrounding lymph capillaries.

Lymphatic Capillary Diagram
Lymphatic Capillary Diagram

Lymph capillaries are intertwined with blood delivering and returning capillaries ready to pick up the waste products and return them into the bloodstream via larger lymph vessels.  Lymph capillaries are unique in that interstitial fluid enters the capillary by a change in osmotic pressure via a one-way valve (cannot leak back out into the interstitial, or the between cellular, space) where it begins its journey back to the bloodstream.  Once the collected fluid and cellular matter is in the lymph capillary it is now called lymph fluid.

Lymph capillaries dump their contents into surrounding larger lymph vessels, which then are filtered through a lymph node.  Lymph nodes are checkpoints where the fluid is inspected for infectious material.  If found the offending material is quarantined in the node and reinforcements are called to try and eradicate the material.  This is why you sometimes feel a large bump in your neck or under your arm.  It’s your lymph nodes trapping identified bad material that cannot be allowed to re-enter your bloodstream.

Clean lymph fluid continues its journey through other lymph vessels, by means of muscle and body motion to propel it, until it reaches one of the two major lymphatic ducts – the right lymphatic duct and the thoracic duct (the left lymphatic duct).  These ducts then drain into the right subclavian vein and left subclavian vein respectively, which are two separate and unequal drainage areas.  The right subclavian vein receives its cleaned lymph fluid from the right side of the head and right arm area while the left subclavian vein receives its cleaned lymph fluid from the left side of the head and remaining body area. Now the process begins again.

Lymphatic System Basics
Lymphatic System Basics

As you can see from the above explanation the circulatory system is a closed system meaning the process of producing nutrient rich blood ends in the recycling of the spent blood at the same point with the process repeating numerous times per day.  The heart beats powerfully strong and hard to move blood to the extremities of your system and back.  The lymphatic system on the other hand is not a closed system nor does it have an equivocal heart to pump its fluid to the two drainage areas.  Thus it relies on muscle, body and internal pressures to move lymph fluid to its destination.

Why is all this important to know?  Well, any damage to the lymph system affects flow.  When lymph tissues, vessels or nodes are damaged, destroyed, removed or impaired, lymph cannot flow properly!  In fact excess lymph fluid will pool in areas causing swelling and pain.  This is the beginning of Lymphedema.  Depending on the nature, size of the affected area and the time left untreated Lymphedema can spread to affect a whole limb, neck, face or trunk.

While there is no cure for Lymphedema there are manual methods that can be employed to manage the condition and from it getting worse.  We will cover that in our next document on treatment options.

Lymphedema Signs and Symptoms

Lymphedema can affect virtually any part of the body.  I have personally seen people suffering from Lymphedema of the face, neck, upper chest, arm and legs during my physical therapy sessions. It usually affects only one limb but in some cases, especially with Lipo-Lymphedema, may affect both matching limbs.   The extent of the Lymphedema will depend on the nature of impacted area meaning how extensive the damage, node removal or tissue may be.

At first it may not be apparent but it could rapidly expand to encase most the limb, sometime causing swelling that doubles the size of the original limb.  Sometimes Lymphedema does not appear until months after damage has been done, as in the case of breast cancer.

What are the signs and symptoms in the three stages of Lymphedema?

Stages of Lymphedema
Stages of Lymphedema

Pre-Stage:

Some have added a pre-stage phase for Lymphedema recognition and treatment.  In this pre-stage an overload to the lymphatic system is identified and immediate treatment is started.  People respond very well and the Lymphedema is completely reversed.

Stage 1:

  • recognition that one limb is larger than the other (tighter fitting clothes, shoes or jewelry)
  • feelings of heaviness or tightness
  • range of motion difficulty and flexibility
  • feelings of tenderness or soreness
  • may be warm or red to the touch
  • presence of a rash
  • pitting edema may occur (pressing on the affected leaves an indentation that slowly returns to normal)
  • Stemmer’s sign may be positive or negative
  • feelings of embarrassment for others to see a deformation in body
  • elevating affected part provides relief and limb should return to normal
  • this stage of Lymphedema is reversible

Stage 2:

  • feelings of heaviness or tightness
  • range of motion difficulty and flexibility (walking impairment if foot is swollen)
  • feelings of pain or soreness
  • may be warm or red to the touch
  • presence of hyperpigmentation (skin discoloration. Mine turned light reddish-purplish in the lower leg area.)
  • non-pitting edema occurs (pressing on the affected leaves does not leave an indentation)
  • connective tissue fibrosis occurs causing hardened patches of skin and underlying tissues
  • Stemmer’s sign may be positive
  • feelings of embarrassment for others to see a deformation in body
  • feelings of guilt you made this happen (this not your fault! It is the result of an impairment in your body’s function that you did not create.)
  • elevating affected area does not bring much relief
  • this stage of Lymphedema is irreversible

Stage 3:

  • feelings of heaviness in affected area
  • fatigue from carrying extra weight in affected area
  • restriction in daily activities due to reduced range of motion up to and including immobility
  • increased inflammation in tissue resulting in pain
  • presence of hyperpigmentation (skin discoloration. Mine turned light reddish-purplish in the lower leg area.)
  • pitting edema
  • Stemmer’s sign is positive
  • skin thickens and becomes lumpy in appearance with an orange peel effect
  • lymphostatic elephantiasis occurs ( appearance and touch looks like an elephant’s leg with thick, discolored and large dimpled skin)
  • skin flaps or folds may occur
  • may develop separate large Lymphedema lobules on limbs
  • skin becomes drier and may flakes
  • skin cracks or splits open with weeping of lymph fluid (may take time to close and heal)
  • significant increase in cellulitis infections which may take weeks, months or years to heal (bacteria infection that settles in affected area causing open sores or wounds. I got mine from untreated strep throat bacteria.)
  • increase in fungal infections in the cracks or crevices of skin (proper hygiene is paramount to reduce infections)
  • feelings of guilt you made this happen (this not your fault! It is the result of an impairment in your body’s function that you did not create.)
  • elevating affected area does not bring relief
  • this stage of Lymphedema is irreversible

Note: Some add an additional stage(s) to describe terribly disfigured and diseased limbs affected by Lymphedema.  However, most medical organizations consider there to be three stages of Lymphedema.

Here is a great recap on the lymphatic system and the signs and symptoms in the three stages of Lymphedema:

 

Stay tuned for more information on treatment options.

To your improved health!

 

Resources:

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