FAQs About Lymphedema

FAQs About Lymphedema
  • Lymphedema FAQS
  • Lipedema FAQs
  • The Lymphatic System FAQs

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What is lymphedema?
Lymphedema is a chronic lymphatic disease that results in disfiguring swelling in one or more parts of the body. It can be hereditary (Primary Lymphedema) or it can occur after a surgical procedure, infection, radiation or other physical trauma (Secondary Lymphedema). In breast cancer, for example, it can appear in the arm on the same side as the cancer, after lymph nodes are removed from the armpit region for cancer staging. Primary Lymphedema often occurs in the lower extremities. Lymph is the protein-rich body fluid that accumulates when the lymphatic system for fluid transport is damaged.
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Why is the lymphatic system important?
Fluid transport is one very important component, but many do not realize that the lymphatic system is part of the immune system. It fulfills the function of ‘immune trafficking,’ the process whereby infection-fighting cells can be mobilized to the tissues that require assistance. When the lymphatic system is compromised by surgery, trauma, or improper development, the affected part of the body is prone to recurrent infection because of the faulty surveillance mechanism.
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Why is it that I was not warned about the risk of lymphedema prior to my surgery?
Regrettably, many doctors are still very under-educated about the lymphatic system in general and, specifically, about the problem of lymphedema. Here at Stanford we’re trying to rectify ignorance about lymphedema through specific educational initiative and I believe that nationally awareness is also increasing. Patients can also help their doctors to learn. Physicians must learn from their patients about their lymphedema experiences so that they will be better prepared to deal with their future patients.
Download 10 Things I Want My Physician to Know About Lymphedema

As a breast cancer survivor, what is my risk of getting lymphedema?
Estimates vary and, fortunately, the risk has been progressively declining. In the beginning, breast cancer surgery carried a 50% risk of lymphedema development. Today, patients who have axillary lymph node dissection have a lifetime risk of 15-25%. For individuals whose surgery is limited to sentinel node techniques, without adjuvant radiation, the risk is about 6%.
Download 10 Things to Know About Cancer and Lymphedema
Download 10 Things To Know About Ovarian And Other Gynecologic Cancers And Lymphedema

How does surgery affect my risk of lymphedema?
The risk is chiefly aligned with lymph node removal. Individuals that have just lumpectomy, or other surgical interventions, are at such low risk that it is impossible to estimate. Sentinel node techniques, alone, are used in about 40% of current breast cancer patients. The sentinel node technique is defined as the removal of no more than four lymph nodes, where the risk is about 6%. If you have more than four lymph nodes removed, the risk rises to 15% to 25%.

Does radiation increase the risk of lymphedema?
Yes, radiation can traumatize the lymphatic system equivalently to surgery, even if surgery is not done. However, just as all surgery is not alike, not all radiation therapy is the same. In general, radiation therapy confers risk that is roughly equivalent to axillary lymph node dissection and increases the risk associated with surgery if both treatments are performed.

If I have had lymph nodes removed under one arm, do I have to worry about the lymph nodes in other areas of my body?
No. The lymphatic system is present throughout the body, but lymphedema is a regional disease that affects only the part of the body that is subjected to surgery or radiation.

Do I have to worry about this for my whole life?
The proper term to use is ‘vigilance.' Stated positively, the primary risk for lymphedema development is in the first year following surgery and radiation therapy, when 90% of the cases occur. By the end of 3 years, 95% of the cases will have appeared. If you don’t have lymphedema after 3 years of vigilance, the risk remains, but it is quite small. If you take the proper precautions and use the proper surveillance, the risk can be maintained as small as possible.

What about exercise after cancer surgery?
While, in the long run, exercise is very beneficial, it is very important to give your body adequate time for healing and to not push the exercise early on.  This is not to say that patients shouldn’t be very active at mobilizing the shoulder, because women are also prone to ‘frozen shoulder’ after a mastectomy. However, do not push the physical activity until the body is ready to do it. For the first three to four weeks, it is important to remain relatively inactive and, thereafter, to try to become progressively more active. 

And what about exercise during radiation therapy?
There are similar concerns. One can expect a certain amount of inflammation related to the radiation, and it would be important to avoid over-taxing the lymphatic system. Accordingly, it is reasonable to ‘take it easy’ during radiation therapy.

Should I be using my arm (or leg) for everyday activities?
Yes, absolutely. Bear in mind, however, that physical activity increases blood flow, which increases the formation of lymph in the arm or leg at risk.  You don’t want to push the lymphatic system ‘over the edge’. Once you recognize your threshold for activity, you can gently extend your limits over time, perhaps, in addition, by using a Class I compression garment during activity that is perceived to be physically stressful.
Download 10 Things To Know About Compression Garments for Lymphedema

After surviving breast cancer surgery and chemotherapy, I’ve developed lymphedema. I’m angry that I have to go through the rest of my life with swelling, wearing a sleeve and restricting my activities. Is there anything you can suggest to help with that?
The anger and the frustration are very understandable and very common. It seems like a second punishment after enduring so much to get your life back. Unfortunately, at this time, with current treatment techniques, the incidence of lymphedema breast cancer survivors remains at 15-25. There are no simple answers to this question. It is important to strive to keep life as normal as possible in the face of the fixed demands related to the presence of lymphedema. Seeking support from your fellow patients is also often a wonderful way to collectively share the experience and help to mitigate the anger. The ability to talk about the problem is an important step toward acceptance.
Video Living With Lymphedema, Sheila H. Ridner, PhD, RN, FAAN

Prior to my cancer diagnosis, I led a very active lifestyle. Now I am nervous that my former activities could cause lymphedema to emerge. What do you advise?
My earnest advice is that once you are a cancer survivor, you must enjoy the gift of life. This means not placing boundaries on what your definition of being alive. It is true that there is a small, finite risk of lymphedema appearing for the first time in somebody at risk, or becoming worse in someone who already has it. However, with the appropriate precautions, those risks are quite small. It is very important, activity by activity, to weigh the risk-to-benefit ratio. There is some finite risk of lymphedema with activities like surfing rock climbing, to choose two random examples. But you have to weigh the importance to your life of those continuing experiences. With or without ‘risky’ activities appropriate treatment for lymphedema is essential when the lymphedema appears. It is very important to faithfully use the garment that is provided to you, particularly during the activity poses theoretical risk. It is very important to maintain surveillance for changes in the limb-at-risk. Take precautions against infection when there are breaks in the skin and seek medical attention early if any changes are observed. When you go outside, put on a sun block, because sunburn can aggravate lymphedema. When you are working in the kitchen, be careful. When you’re in the garden, be careful, and wear gloves if there is the possibility of skin trauma. But what you want to lift, lift. What you want to do, do. And where you want to go, go. Just be careful.
Video Debunking the Myths of Exercise and Lymphedema, Nicole Stout, DPT, CLT-LANA

 

Falk Cardiovascular Research Center
Stanford University School of Medicine

Stanley G. Rockson, M.D., F.A.C.P., F.A.C.C.
Allan and Tina Neill Professor of Lymphatic Research and Medicine
Professor of Medicine
Chief of Consultative Cardiology

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What exactly is lipedema?
Lipedema is one of many forms of edema. In medical terminology, ‘edema’ means ‘swelling’. For people who have lipedema, the swelling is caused by an overgrowth of the fat storage cells (adipocytes) that are normally found in a layer of the skin. These cells grow abnormally in size and number when someone has lipedema. Because body fat has a liquid consistency at normal body temperatures, the fat accumulation can resemble other causes of fluid retention in the tissues.

What are the characteristics of someone who has lipedema?
Lipedema is one of many chronic swelling conditions. However, it is somewhat unique, in that it affects women almost exclusively, which leads us to believe that is driven, at least in part, by hormonal status.

In fact, most women who develop lipedema do so at the time of major changes in female hormonal status (puberty, pregnancy, menopause, among others). There are other common attributes of lipedema. Affected individuals often experience easy bruising and exquisite tenderness of the affected tissues. Lipedema almost always affects the legs, less commonly the arms, but typically spares the trunk. It is for this reason that a hallmark of lipedema is the disproportion between the size of the trunk and the size of the legs. When the legs are affected, the feet are normal in size, with the swelling arising from the level of the ankles. A family history of similarly affected individuals is very common, suggesting that there is a genetic predisposition to this condition.

What causes lipedema?
Simply stated, the answer is unknown. As stated, we suspect genetic and hormonal influences, but the disease is not predictable or preventable. There is a suggestion that it is a disease of the lymphatic microcirculation.

Why is the lymphatic system important in lipedema?
The lymphatics are responsible for clearing extra fluid from the tissues. It is important to note that people with lipedema have difficulty clearing a water load from the body, particularly when they remain upright. In addition, imaging of the microscopic lymphatics is abnormal in lipedema patients, and this abnormal pattern is distinct from what is seen in lipedema. One important theory is that poor function of the lymphatics leads to water retention that causes the fat cells to swell and, eventually to become abnormal.

If I am related to someone with lipedema, what is the chance that I will develop it?
The answer to this question is unknown.

Can lipedema be cured?
Unfortunately, no.

What are the stages of lipedema?
Throughout the natural history of lipedema, the tissues can undergo progressive change. In the first stage, the skin remains smooth but the fat layer beneath is increased in dimension. In the second stage, there are visible irregularitisin the skin surface and nodules can be felt within the fat tissue. Stage 3 is characterized by large exterior accumulations of tissue derived from the skin.

What is the difference between cellulite and lipedema?
Cellulite is also an alteration in the subcutaneous fat, but cellulite is primarily a cosmetic problem. In some individuals, the presence of cellulite could represent the earliest phase of a problem than can evolve into full-blown lipedema, but this is certainly not always the case.

Is lipedema a common problem?
The exact incidence of lipedema is not known because, at present, we lack any diagnostic or imaging tools that will reliably identify the presence of lipedema. Unfortunately, there are many common features between the medical diagnosis of lipedema and the normal pattern in which women tend to deposit excess body fat. Other edema causing conditions might be confused with lipedema, including lymphedema, vein disease, hypothyroidism, and systemic causes of edema, like heart failure.

How is lipedema diagnosed?
As already mentioned, there are no tests for lipedema. The diagnosis can only be established with reasonable certainty if a trained clinician can physically evaluate the patient.

What are the medical complications of lipedema?
Fortunately, the risk of infection (cellulitis) that is seen in lymphedema and chronic venous disease does not occur in lipedema. However, in later stages of lipedema, there is a distinct tendency to develop abnormalities in these other two circulations. Late stage lipedema with lymphedema development is called lipolipedema, and with vein abnormalities, phlebolipedema.

What investigations are useful for the diagnosis and monitoring of patients with lipedema?
Because there are no diagnostic tests, there is no investigation for monitoring the disease. If there is a concern for either phlebolipedema or lipolymphedema, the appropriate tests can be done for the associated conditions

What are the mainstays of therapy for patients with lipedema?
Unfortunately, the abnormal fat storage cell in lipedema does not shrink in response to caloric restriction, so diet will not change the outcome of the lipedema directly. However, it is commonly believed that, if there is truncal obesity, a calorically-restricted diet is desirable to optimize the size and weight of the trunk, which will indirectly reduce the likelihood of disease progression. As previously mentioned, there appears to be a defect in water excretion in lipedema. This water retention is intensified by dependent positions of the legs (sitting and standing). For this reason, we believe that low grade compression stockings can help to slow the progression of disease, provided that they can be tolerated in the face of bruising and tenderness. In cases of lipolymphedema, CDT (Complex/Complete Decongestive Therapy) may address the lymphedema component. There are no medications for lipedema.

What about surgery?
In recent years, there has been growing emphasis on surgical techniques to remove the abnormal fatty tissue. Liposuction, either tumescent or water-assisted is considered to be a suitable surgical therapy. This should not be considered to be curative, since the problem can recur and, even when successful, rarely will result in a complete return to a normal shape of the involved limb(s). Nevertheless, in selected cases, these surgical approaches can be very helpful in reducing the total disease burden, often resulting in increased mobility and decreased discomfort in the affected limbs. The surgery will often require multiple procedures to achieve a complete treatment effect.

Is there research on lipedema and can we hope for better treatments?
Most certainly. Scientists are trying to understand the mechanisms that produce the clinical problem of lipedema and there are active attempts to identify the gene(s) that may cause the disease. One important way to assist in that effort is to participate in LE&RN’s International Lymphatic Disease Registry and Biorepository.

 

Falk Cardiovascular Research Center
Stanford University School of Medicine

Stanley G. Rockson, M.D., F.A.C.P., F.A.C.C.
Allan and Tina Neill Professor of Lymphatic Research and Medicine
Professor of Medicine
Chief of Consultative Cardiology


The earlier lymphedema is treated, the more that can be done to prevent its progression

The lymphatic system is vital to the health of every individual. Scientific investigation of the lymphatic system will provide preventive and therapeutic benefits for a broad array of diseases. As an integral part of the immune system, it is both critical to the body's surveillance against cancer, and it can provide pathways for cancer to metastasize.

Unraveling the mysteries of the lymphatic system is vital to understanding many other disease processes. It plays a role in inflammatory and auto-immune disorders such as arthritis and systemic lupus, and is key to understanding the spread of AIDS and many infections. The Lymphatic Education & Research Network fosters and supports research to discover the links between lymphatic function, diseases, and levels of wellness.

 

What is the Lymphatic System

The lymphatic system is part of the immune system. It helps the body fight infections. It also helps maintain fluid balance in different parts of the body. It is made of a series of thin tubes, called lymphatic vessels. These vessels collect fluid, called lymph, from different parts of the body. Similar to blood vessels, these vessels then carry the lymph back to the bloodstream. Lymph is a colorless, watery fluid that is rich in white blood cells called lymphocytes. These cells play an important role in the immune system by protecting your body against infection. Lymphocytes form and are stored in lymph nodes, which are pea-sized collections of cells located throughout the body, such as in the armpit, groin, and neck area.

 

Disorders & Diseases

Lymphatic disease is a malfunction of the lymphatic system in which fluid, or lymph, does not pass properly through the lymph nodes and lymphatic vessels. The most common lymphatic disease is lymphedema. The most prevalent lymphatic disorder is lymphatic insufficiency, or lymphedema. This is an accumulation of lymphatic fluid in the interstitial tissue causing swelling, most often in the arm(s) and/or leg(s), and occasionally in other parts of the body. The severity of this disease varies from very mild complications to a disfiguring, painful and disabling condition. In addition, patients are often susceptible to serious life-threatening cellulite infections (deep skin), and if untreated, can spread systemically or require surgical intervention. It remains a lifelong functional problem requiring daily treatment for maintenance. Eventually the skin becomes fibrotic (thickening of the skin and subcutaneous tissues) with loss of normal architecture, function and mobility.

There are two main types of chronic lymphedema: Primary and Secondary

Primary Lymphedema is an inherited disease that affects approximately 0.6% of live births and in which there is a congenital defect of the lymph-transporting system. It can be present at birth, develop at the onset of puberty or present in adulthood, with no apparent causes.The lymphatic system does not develop properly. Those affected with Primary lymphedema will have swelling of various parts of the body due to the accumulation of lymph.

Nonne- Milroy’s lymphedema is characterized by swelling of one or both legs, arms, and/or face with gradual fibrotic changes.

Meige’s Syndrome-develops after the onset of puberty

Lymphedema Tarda- usually develops after the age of 35

Lymphedema-distichiasis may also present after puberty and patients have an auxiliary ( extra) set of eyelashes.

A number of the hereditary lymphedema conditions can be linked to a gene mutation  ( flt4 ;FOXC2)

There are several chromosomal disorders in addition that have been associated with lymphatic conditions including lymphedema.  Gorham’s Disease, Turner Syndrome, Klippel-Trenaunay Syndrome, Yellow Nail Syndrome, Noonan Syndrome are just some of these associated conditions.

Lymphangioma- a congenital lymphatic malformation that arises during embryonic development is another lymphatic condition. Lymphangiomatosis is the presence of multiple or widespread lymphatic vascular malformations.

Protein-Losing Enteropathy may occur in the presence of Intestinal lymphangiectasia. Those affected have excessive protein loss into the gastrointestinal lumen leading to hypoproteinemia. This leads to severe edema, in addition to ascites (excess abdominal fluid in the abdomen) and pleural effusion (excess fluid in the cavity lining the lung)

Congenital Pulmonary Lymphangiectasia- abnormally dilated ( widened) lymphatic vessels within the lungs leads to lymph collecting in the lungs and leads to respiratory distress.

Secondary Lymphedema (acquired regional lymphatic insufficiency) is a disease that is common among adults and children in the United States. It can occur following any trauma, infection or surgery that disrupts the lymphatic channels or results in the loss of lymph nodes. Among the more than 3 million breast cancer survivors alone, acquired or secondary lymphedema is believed to be present in approximately 30% of these individuals, predisposing them to the same long-term problems as described above. Lymphedema also results from prostate, uterine, cervical, abdominal, orthopedic cosmetic (liposuction) and other surgeries, malignant melanoma, and treatments used for both Hodgkin's and non-Hodgkin's lymphoma. Radiation, sports injuries, tattooing, and any physical insult to the lymphatic pathways can also cause lymphedema. Even though lymphatic insufficiency may not immediately present at the time any of the events occur, these individuals are at life-long risk for the onset of lymphedema.

Lymphatic Filariasis is a world health problem resulting from a parasitic-caused infection causing lymphatic insufficiency, and in some cases predisposes elephantiasis. The World Health Organization's recent efforts to eradicate the spread of infection do not address or eliminate the resulting lymphedema.

Lymphoma is a type cancer that begins in infection-fighting cells of the immune system called lymphocytes. The lymphatic system includes the lymph nodes, spleen, thymus, bone marrow, and other parts of the body. When you have lymphoma, these areas are affected as well as other organs in the body. There are two main types of lymphoma:  Non-Hodgkin (most people with lymphoma have this type) and Hodgkin.

Non-Hodgkin and Hodgkin lymphoma each affect a different kind of lymphocyte. Every type of lymphoma grows at a different rate and responds differently to treatment.