In prior posts, I’ve talked about the difference between edema and lymphedema (click here & here to see those posts). Today, let’s take a look at conditions that begin as edema & can become lymphedema as the swelling persists.  How can that happen?

To understand how edema can become lymphedema, we need to revisit what lymphedema is.  By definition, true lymphedema is an accumulation of protein-rich fluid (tissue which has excess protein in it).  How does protein get into fluid?  You eat protein (like meat, eggs, etc.), but your body also makes proteins that play an essential role in bodily function.  I won’t get into the bio-physiology of proteins except to comment on one: a plasma (blood) protein called albumin.

Albumin helps regulates which way fluid goes, into the blood vessels or into the tissue to potentially cause swelling (known as “colloid osmotic pressure”). Albumin, which is made by the liver, accounts for 70% of the colloid osmotic pressure.^1,2  That’s huge!

Second, by definition, true lymphedema is the result of damage to the lymphatic system. How does the system get damaged? Genetic causes (called primary lymphedema) or trauma (called secondary lymphedema).  Trauma is the most common. Chronic edema can be considered trauma because it increases the amount of fluid the lymphatic system must transport out of your tissue & back to the heart over time (sort of like abusing the lymphatic system).

Your lymphatic system has a pump, little “hearts” called lymph angions. Just as your heart pumps blood, these angions pump lymph fluid. But while the heart is a closed, circulatory system, the lymphatic system carries fluid one way, from the tissue spaces (throughout the entire body) to the heart. Just as blood enters the heart, stretching the wall of the right atrium & causing it to contract, fluid enters a lymph angion, stretching its wall & causing it to contract. At rest, contractions are 6-10 times per minutes. When necessary, this pumping can increase up to 20 times per minute.⁴ However, just like with the heart, such endurance cannot be sustained indefinitely.  The lymph angion will fail & stop working. ⁵ When that happens, the lymphatic load remains in the tissue causing inflammation & scarring. This leads to an important point: 

Lymphedema is not reversible once damage has occurred because of the mechanical, structural changes that have taken place. Progression of the condition can be stopped & symptoms can be improved, but the anatomy cannot be like it was before the damage.

In acute injury or initial trauma, edema is present & the lymphatic system is pumping as hard as it can to remove the excess fluid.  The body usually recovers, the swelling inundation subsides & the lymphatics return to normal. But over time (such as with chronic edema), the lymph system becomes overwhelmed & eventually stops working.  When that happens, inflammation & scarring occur.  The system becomes mechanically & structurally damaged in the area of congestion.

Now that we’ve reviewed what lymphedema is, what are some causes of chronic edema & why can they cause secondary lymphedema?

Obesity, lipedema & other fat disorders

These conditions slow down the flow of lymphatic fluid. I often use the analogy of driving around a mountain. Because of the curves & narrow roads, you have to slow your speed. When there are several cars, congestion results. When lymphatic vessels must weave through fat tissue, lymph fluid can become sluggish & congestion results, creating a backup of fluid which can lead to scarring of the lymphatic vessels.

Additionally, fat (or adipose) causes inflammation itself & inflammation can cause more fat development. Inflammation is the body’s response to trauma which increases blood flow to the affected (which increases swelling in that area). The cycle continues.

Venous pressure

Venous pressure causes an increase in the lymphatic load.  Conditions such as chronic venous insufficiency or varicose veins are conditions which cause blood flow to regurgitate in the lower legs instead of efficiently making its way northward to the heart. 

Another source of increased venous pressure is positioning.  Feet remaining in a dependent position (hanging down) for extended periods such as jobs requiring long episodes of standing or sitting create significant pressure in the lower legs that must be overcome.  Both the amount and speed of blood flow decrease in the legs after 15 minutes of static, dependent positioning.⁶

Another source of venous pressure is inactivity or not contracting muscles (even if in a gravity neutral position) as is seen in paralysis after spinal cord injury or a stroke. The lymph system is not able to overcome the increased tissue pressure in these cases & eventually fails, leading to scarring.

Organ dysfunction

Conditions such as heart failure, kidney failure, thyroid impairment & liver disease all can create an excessive amount of fluid⁷ & an inability of the lymph system to keep up with the demands, resulting in fatigue & eventual scarring of lymphatic vessels.

Infection

Localized infection such as cellulitis (particularly when repeated infections occur) result in scarring of lymphatic vessels.

Diabetes

Diabetes causes changes in the smallest parts of your blood vessels (making up the capillary beds).  The capillary bed wall thickens & enlarges, allowing the exit of blood matter (water, proteins & other molecules) that would not otherwise exit. That creates an added burden for the lymphatic vessels to manage. ⁸ Additionally, in my own thinking, because this results in loss of blood flow to other areas, it may likely cause disease of lymphatic vessels themselves. Either case would eventually cause scarring.

One of roles of the lymphatic system includes maintaining homeostasis (preventing swelling). Anything that causes edema creates more work for the lymphatic system. And when you work, work, & work more without rest, you get tired. So does your lymphatic system. When the lymphatic system gets tired, the fluid, protein & other molecules it’s responsible for get left in the tissue. That causes an inflammatory response which creates a cascade of events that create a cycle of chronic inflammation & scarring called lymphedema. As the cycle continues, you begin to see the symptoms of lymphedema.  In all the cases mentioned above, lymphedema occurs as a result of structural, mechanical damage to the lymphatic vessels, rendering it ineffective as a result of being overtaxed.

Note: Check out this link to read more on what is being done to increase awareness of edema induced lymphedema (added 01/12/21)

What can you do to prevent lymphedema resulting from chronic edema? Manage your health condition &, if your doctor approves, get compression. Check out past blogs for posts on compression!

¹ https://www.ncbi.nlm.nih.gov/books/NBK531504/, https://en.wikipedia.org/wiki/Oncotic_pressure
² https://www.ncbi.nlm.nih.gov/books/NBK204/
³ https://www.mayoclinic.org/diseases-conditions/cirrhosis/symptoms-causes/syc-20351487
⁴ Weissleder, H., Schuchhardt, C. (2008).  Lymphedema Diagnosis and Therapy (4^th ed.), p.36.  Germany: Viavital Verlag GmbH.
⁵ Foldi, M, Foldi, E. (2006). Foldi’s Textbook of Lymphology (2^nd ed.), p. 201. Germany: Urban & Fisher.
⁶ Foldi, M, Foldi, E. (2006). Foldi’s Textbook of Lymphology (2^nd ed.), p. 434. Germany: Urban & Fisher.
7 Foldi, M, Foldi, E. (2006). Foldi’s Textbook of Lymphology (2^nd ed.), p. 242-259, . Germany: Urban & Fisher.

Lymphedema Therapy Source, PLLC opened its doors in 2011 to provide lymphedema services to those in the Dallas-Fort Worth Metroplex. In 2018, we narrowed the geography of our therapy services, but we’re expanding in other ways in 2020!

Changes will be coming to our website beginning this month & continuing in the months to follow including the addition of ebooks, video content, an interactive blog & more. Stay tuned!

Most of the time, therapy is understood to be restorative (rehabilitative). For example, a woman may have lymphedema (protein-rich swelling) in her arm.  Because of this swelling, she is at risk for progression of her lymphedema (including infection & skin changes).  Normal activities such as combing her hair may be more difficult.  Therapy is administered & successful in reducing limb size, providing training, etc.  She would be measured for a custom compression garment to maintain the state attained &, ideally, be independent at the time of discharge.  She would return to see her therapist only if needed.  (In the case of lymphedema, a periodic checkup is recommended to monitor tissue, volume & garment state.)

On a few occasions, a person will decline if care is not ongoing.  Therapy in this case would be maintenance.  For example, an elderly gentleman lives alone & has swelling in both legs.  He is at risk for infection & wound development.  He also has functional challenges (such as getting into a car).  To complicate matters, arthritis makes applying his compression impossible.  He may be a candidate for maintenance care by his therapist through Medicare.  However, obtaining coverage isn’t that easy.  Medicare won’t cover the cost just because a doctor orders ongoing therapy or a therapist provides the care.  Several requirements must be met.

Background
In the past, Medicare only covered restorative therapy. They have a long history of denying coverage in cases where a person doesn’t have potential to improve or isn’t showing measurable progress (referred to as the “improvement standard”).  This changed in 2013 due to a class-action lawsuit (titled “Jimmo vs Sebelius”).  As a result, Medicare released Transmittal 175 which they purported didn’t change their coverage (they stated they have always provided maintenance care).  Instead, it was released to clarify existing standards & applies to home health, outpatient & skilled nursing facilities.  It states:

• therapy services are covered when a therapist’s assessment of the patient’s condition demonstrates that the therapist’s specialized judgment, knowledge, and skills are necessary to maintain, prevent or slow further deterioration of a person’s condition or functional status
• coverage isn’t dependent upon a patient’s potential for improvement but rather on the beneficiary’s need for skilled care

Requirements
Assuming a person meets Medicare’s general qualifications for service, for maintenance care to be covered, several items are required (this list is not exhaustive):

• therapist’s assessment must show the skilled need for their specialized services; the services are not able to be safely and effectively carried out by the patient or another person**
• services are shown to be reasonable & necessary for the patient’s illness or injury
• the assessment (which includes the plan of care) must be certified (i.e. signed) by the physician within 30 days (delayed signature is possible with certain criteria)
• therapist must complete a progress note each 10^th visit (or 30^th calendar day – whichever is less) assessing the patient’s status & appropriateness of continued care
• recertification every 90 days (or before if a plan of care expires)
• additional documentation to justify care may be required (such as visit notes)
• treatment must be performed by a therapist (not an assistant such as an OTA or PTA) in home health or outpatient
• further requirements (such as no stamped signatures, appropriate billing which includes modifier codes when applicable, objective testing, etc.)

Coverage
Once a patient meets the 2017 therapy cap of $1,980 (for OT or PT/ST combined), if services are still medically reasonable & necessary (& shown to be in the therapist’s documentation & billing), Medicare will continue to provide coverage in threshold amounts of $3,700 (for OT or PT/ST combined). However, they will likely request proof of medical necessity.  A therapist may request a patient to sign an Advanced Beneficiary Notice if services are not reasonable & necessary but a patient requests to continue or the therapist has reason to believe coverage may be denied.

Denials
If a claim is denied, a person can file an appeal. The process for an appeal is outlined here:   https://www.medicare.gov/claims-and-appeals/file-an-appeal/appeals.html

Disclaimer
According to the Local Coverage Determination rules for Texas, “Medicare does not expect to be routinely billed for lymphedema treatments.”  Additionally, rules state treatment is only covered when:

1. there is a physician-documented diagnosis of lymphedema (primary or secondary)
2. the patient has documented signs or symptoms of lymphedema
3. the patient or patient caregiver has the ability to understand and comply with the continuation of the treatment regimen at home.

“Documentation must clearly state the need for continued manual therapy beyond 12-18 visits.  When the patient or caregiver has been instructed in the performance of specific techniques, the performance of these same techniques should not be continued in the clinic setting and counted as minutes of skilled THERAPY.”

What can you do to ensure you have needed coverage?
First, contact Congress.  Yes – your input will make a difference! Currently, Medicare does not cover compression for lymphedema (despite the Women’s Health & Cancer Rights Act of 1988).  Legislation has been introduced & continues to make progress in Congress.  You can use this link to easily contact your legislators: Lymphedema Treatment Act.

Second, consider contacting the Center for Medicare Advocacy for help.  They are a nonprofit, nonpartisan organization which led the legal action against Medicare in reform for maintenance care (see above “Jimmo vs Sebelius”).

** Per Medicare Transmittal 179, “A service is not considered as a skilled therapy service merely because it is furnished by a therapist…If a service can be self-administered or safely and effectively furnished by an unskilled person, without the direct supervision of a therapist, the service cannot be regarded as a skilled therapy service even when a therapist actually furnishes the service.  Similarly, the unavailability of a competent person to provide a non-skilled service, regardless of the importance of the service to the patient, does not make it a skilled service when a therapist furnishes the service.”

** Therapists need to be aware that manual lymph drainage (CPT code 97140) & multi-layer compression bandaging (CPT codes 29581-29584) cannot be billed together (according to Noridian – another Medicare contractor which doesn’t have jurisdiction over Texas but their interpretation likely still applies). They state, “Treatment of lymphedema with the application of high compression bandaging continues to be non-covered” except when it is used to teach a patient/caregiver (CPT 97575).” In the latter case, no more than 3 visits should be billed.

References
http://www.aota.org/advocacy-policy/federal-reg-affairs/news/2013/medicare-policy-improvement.aspx
https://www.cms.gov/Regulations-and-Guidance/Guidance/Transmittals/Downloads/R179BP.pdf
www.homehealthsection.org/resource/resmgr/CSM2015/HH_1977479.pdf
Medicare Benefit Policy Manual, Pub 100-02
MLN Matters: MM8458 Revised
Code of Federal Regulations (Title 42, Sections 410.59-410.61; 485.70; 486.150-163)
Medicare.gov: Exceeding Therapy Cap
CMS – Local Coverage Determination (Texas)
Noridian’s Interpretation of CDT

There are three primary methods for treating cancer.  These are surgery, chemotherapy (“chemo” for short) & radiation.  Sometimes these methods may be combined.  Depending on the type of cancer, additional treatment options might include bone marrow transplant, hormone therapy, molecular-targeted therapy, clinical trials & unconventional methods.

Surgery – Tumors may be removed or shrunk through surgery (often followed by chemo &/or radiation to treat any cells that may have spread).  This surgery is localized to the tumor site & might be done with the old-fashioned surgical knife.  However, there are also less extensive surgeries such as sentinel node biopsy, minimally invasive (like laparoscopy or thoracoscopy) & organ-preserving (e.g. lumpectomy). Newer approaches to destroy tumor tissue include focused sound waves (i.e. ultrasound), cold (cryotherapy), radiowaves (radiofrequency ablation) & light (phototherapy).

Chemotherapy – A drug or combination of drugs primarily administered orally, through injection or through a vein (via a catheter such as a PICC line or a port (i.e. a device placed under your skin on the chest).  Chemo is a systemic treatment, meaning it affects the whole body.  It targets fast-growing cells which include not only cancer cells but other fast-growing cells like skin, hair, bone marrow (including white or red blood cells & platelets) & cells that line your digestive tract (such as stomach & mouth).  Fortunately, the side effects from damage to healthy cells (such as nausea or diarrhea) can often be managed during treatment & other side effects (such as hair loss, dry skin or skin rash) usually go away.

Radiation – High-energy radiation is delivered to the tumor(s) by a machine via an external beam (i.e. X-Ray or gamma ray) or internally (via radioactive material placed in the body near the tumor or via systemic administration called brachytherapy).  The purpose of radiation is to shrink or kill the cancer cells by damaging their DNA. Unfortunately, normal cells can also be damaged leading to long-term effects such as fibrosis & lymphedema. However, doctors have an idea of how much radiation normal tissue can receive & take this into consideration when planning your treatment course.

 

References
www.cancer.org
Coleman, Norman, MD (2006). Understanding Cancer, p. 85-106. Baltimore: The John Hopkins University Press.

In March’s blog, we discussed what cancer is.  We said,

“When a cell is told to multiply, it begins a multi-step process which has “checkpoints.”  These checkpoints inspect the cell, ensure it is progressing normally & allow it to continue its development.  If a cell is found defective (i.e. several genes have changed or mutated), it will usually self-destruct or it will be removed by other cells (thanks to our immune system).  This process is programmed so there is a balance between the cells that multiply & those that die.   In cancer, however, this process is defective.  Instead of self-destructing or being removed, the defective cell passes through the checkpoints & multiplies at-will.”

This describes one reason people get cancer: Cells malfunction (usually as a result of age or genetic predisposition).  However, environmental factors may also contribute.  For example, chemicals found in pesticides or in cigarettes (or other forms of tobacco use).  UV radiation from too much sun exposure or sunburns has been linked to cancer development.  Behavioral factors may also increase risk.  For example, an unhealthy diet & sedentary lifestyle can lead to obesity* which increases the risk of developing cancer.  Smoking, heavy/regular alcohol consumption, chronic stress & hormones (such as menopausal therapy or oral contraceptives) do as well.

*Obesity & other conditions that cause chronic inflammation (such as chronic inflammatory bowel diseases – e.g. Crohn’s Disease – or even untreated lymphedema) has been linked to cancer.  The longer chronic inflammation persists, the greater the risk of cancer development.

References
http://www.cancer.gov/about-cancer/understanding/what-is-cancer
http://www.cancer.gov/about-cancer/causes-prevention
https://www.mdanderson.org/publications/focused-on-health/december-2014/how-stress-affects-cancer-risk.html.html

If you or someone you know has been given a diagnosis of breast cancer, you may have heard unfamiliar terms like “in situ,” “ductal,” “invasive,” “lobular,” “inflammatory” & “carcinoma.”  What do these words mean?

When the breast creates milk, it begins in a lobule.  Once the lobule develops milk, the liquid flows through the milk ducts & exits through the nipple.  When cancer cells develop in one of these areas, the cells may stay within that site or they may invade other parts of the breast tissue.  “In situ” & “invasive” describe what the cancer cells have done.  If the cancer has not spread to surrounding breast tissue, it is said to be “in situ” (i.e. in its original site).  If the cancer has spread beyond the borders of its original location & invaded surrounding breast tissue, it is said to be “invasive.”  The words describing the involved breast area & what the cancer cells have done are combined to form the name of a particular type of breast cancer:

• Ductal – refers to the milk ducts of the breast
  • ductal carcinoma in situ (or DCIS for short) means the tumor (or cancer mass) is contained within the milk ducts
  • invasive ductal carcinoma (or IDC for short) means the tumor growth has broken through the duct wall & spread to the surrounding breast tissue
• Lobular – refers to the lobule milk glands
  • lobular carcinoma in situ (or LCIS for short) means cells that look like cancer are growing in the milk glands; this type isn’t considered cancer yet – it is, however, a condition that needs to be watched
  • invasive lobular carcinoma (or ILC for short; also called “infiltrating lobular carcinoma”) means the tumor growth has broken through the lobule wall & spread to the surrounding breast tissue

For a diagram of the breast anatomy & above types of cancer, click: http://www.breastcancer.org/pictures/types

More terms

• carcinoma – refers to any cancer that begins in the skin or other tissue that cover organs
• inflammatory – refers to the description of inflammatory symptoms accompanying this type of cancer (tenderness, warmth, redness, swelling, &/or an orange-peel appearance to the skin).
  • inflammatory breast cancer (or IBC for short) means an inflammatory response within the breast tissue caused by cancer cells blocking the flow of lymph fluid.  Note: While an antibiotic should improve symptoms caused by an infection or mastitis, it will not help this condition. Be sure to notify your doctor if you’ve been prescribed an antibiotic but your symptoms haven’t gone away.

It’s worth noting there are other types of breast cancer not mentioned.  A comprehensive list includes:

ductal carcinoma in situ
invasive ductal carcinoma
lobular carcinoma in situ
invasive lobular carcinoma
inflammatory
medullary
mucinous or colloid
Paget’s disease

and even less common:
tubular
cribiform
papillary
micropapillary
apocrine
adenocystic
carcinosarcoma
squamous
sarcoma

References
Kneece, Judy, RN, OCN (2012). Breast Cancer Treatment Handbook (8^th ed), p 20-21, 66. South Carolina: EduCare
www.cancer.org
www.breastcancer.org

In our last blog, we described what cancer is.  In this blog, we’ll outline the different types of cancer (listed below).  A starting point to understanding the different types of cancer is to understand how a particular type of cancer gets its name.  Cancers are named based on their appearance & what part of the body they originate in.  For example, breast cancer is named “breast cancer” because the cancer is located in breast tissue.  Lung cancer is named “lung cancer” because the cancer is located in lung tissue & so on.  It’s important to note, however, that even if the cancer progresses (i.e. metastasizes) to other body parts, it is still named based on its original location.  For example, breast cancer which has metastases that go to the lung(s) isn’t called “lung cancer,” it’s called “metastatic breast cancer.”

 

Breast Cancer – the number one cause of lymphedema in the United States

• Ductal carcinoma in situ
• Lobular carcinoma in situ
• Invasive ductal carcinoma
• Invasive lobular carcinoma
• Inflammatory breast cancer

Lung Cancer

• Small-cell lung cancer
• Non-small-cell lung cancer

Skin Cancer

• Melanoma
• Basal cell carcinoma
• Squamous cell carcinoma
• Neuroendocrine carcinoma

Gastrointestinal Cancer

• Esophageal
• Stomach
• Liver
• Pancreas
• Colon & Rectum

Genitourinary Cancers (cancer of sexual organs & urinary organs)

• Prostate
• Cervix
• Bladder
• Kidney

Hematological Cancers (cancer of the blood & stem cells)

• Leukemia
• Lymphoma
• Multiple Myeloma

Head & Neck Cancers

• Oral cavity
• Nasal cavity
• Thyroid gland
• Pharynx

Sarcomas (cancerous tumors of soft tissue & bone anywhere in the body)

• Osteosarcomas (primary bone sarcomas)
• Soft tissue sarcoma (such as tumors arising from fat tissue, muscles, nerves, blood vessels, etc.)

Brain & Spinal Cord Tumors

• Glioma (tumors that start in glial cells)
• Meningioma (tumors that start in the outer lining of the brain)
• Acoustic Neuromas & Schwannomas (tumors that develop from Schwann cells – which line the cranial & peripheral nerves)
• Medulloblastoma (which arise from fetal cells in the cerebellum; more commonly found in children but they can be found in adults)

 

http://www.cancer.org/cancer/showallcancertypes/index
Smith-Gabai, Helene (2011).  Occupational Therapy in Acute Care, p. 416-426.  Maryland: The American Occupational Therapy Association, Inc.
Coleman, Norman, MD (2006). Understanding Cancer, p. 43-45. Baltimore: The John Hopkins University Press.

National Cancer Institute

American Cancer Society

National Comprehensive Cancer Network

PDQ system

p. 85 Understanding Cancer book

Science has come a long way in treating cancer, but cancer is a word that still causes anxiety for most who receive (or have loved ones who receive) the diagnosis.  That anxiety is complicated by several unknowns – starting with understanding what cancer is (in user-friendly terms). Cancer (specifically, breast cancer) is the number one cause of lymphedema in the United States & so finds its way on our blog.  This series will address cancer, what it is, how it may be developed, treatments & other related topics.

So, what exactly is cancer?  The simple answer is cells multiplying (i.e. dividing themselves) out of control.  For a deeper insight, let’s take a look at what happens on a biological level.

Our bodies are made up of cells, many different types of cells which have different functions.  For example, there are muscle cells, bone cells, blood cells, skin cells, defense cells, etc.  Some cells continue to multiply throughout their life (like blood & skin cells) while others stop multiplying when their type is complete (cells in our nervous system).  Normal cells only multiply when they are told to do so by

1. hormones
2. growth factors (usually a protein or hormone) &
3. cytokines (“messenger” proteins)

When a cell is told to multiply, it begins a multi-step process which has “checkpoints.”  These checkpoints inspect the cell, ensure it is progressing normally & allow it to continue its development.  If a cell is found defective (i.e. several genes have changed or mutated), it will usually self-destruct or it will be removed by other cells (thanks to our immune system).  This process is programmed so there is a balance between the cells that multiply & those that die.   In cancer, however, this process is defective.  Instead of self-destructing or being removed, the defective cell passes through the checkpoints & multiplies at-will.  Amazingly & thankfully, this breakdown doesn’t happen very often.

 

http://www.cancer.org/cancer/cancerbasics/what-is-cancer
Kumar, V., Abbas, A., Aster, J. (2013).  Robbins Basic Pathology (9^th ed.), location 1644ff.  Pennsylvania: Elsevier Saunders.
Coleman, Norman, MD (2006). Understanding Cancer, p. 28ff. Baltimore: The John Hopkins University Press.
Smith-Gabai, Helene (2011).  Occupational Therapy in Acute Care, p. 410.  Maryland: The American Occupational Therapy Association, Inc.