I am very grateful to Carol Doeringer, lymphedema patient and advocate, who submitted this interesting and very insightful contribution on the risk factors contributing to breast cancer related lymphedema. The material is excerpted from a self-study course Carol has developed with the support of friends and experts in the lymphedema and nursing communities. . . . → Read More: The Effect of Post-Surgical Exercise and Therapy on Breast Cancer Related Lymphedema Risk]]> Tweet 

I am very grateful to Carol Doeringer, lymphedema patient and advocate, who submitted this interesting and very insightful contribution on the risk factors contributing to breast cancer related lymphedema. The material is excerpted from a self-study course Carol has developed with the support of friends and experts in the lymphedema and nursing communities. The course is called Breast Cancer-Related Lymphedema: The Nurse’s Role in Care and Prevention, the program will soon be available at no charge to any interested nurse.  Those interested can learn more by visiting the Lymphedema Speaks website

The effect of post-surgical exercise and therapy on breast cancer related lymphedema (BCRL) risk

Immediate post-surgical range-of-motion exercises are shown to increase BCRL risk

Surgeons commonly prescribe shoulder and arm exercises to restore upper limb and shoulder function after mastectomy, radiation therapy, and/or axillary node dissection. Many studies demonstrate the effectiveness of immediate exercise intervention (1) to avoid painful conditions such as the common adhesive capsulitis of the shoulder (‘frozen shoulder’) after mastectomy.

However, immediate shoulder mobilization has also been shown to increase the incidence of BCRL among women with early breast cancer whose surgery included axillary node dissection. Todd et al (2008) conducted a two-group (delayed vs. early full range shoulder mobilization), single-blind, randomized controlled trial with 116 women. Both groups engaged in immediate post-operative exercise, with one group using a full range of motion, and the other group restricting arm movements to below 90° in all planes of movement for the first week, followed by introduction of full range-of-motion in the second week. Each group continued the prescribed exercise program until full shoulder motion was restored and then once a day for the first post-operative year.

These researchers found that women in the early full-shoulder mobilization group had 2.7 times the incidence of lymphedema, and that limb volume differences (affected compared to unaffected arm) were significantly higher compared to the delayed-exercise group. There were no statistically significant differences in shoulder movement, grip strength, or self-evaluated outcomes between the groups at one year post-surgery (2). Earlier, Shamley et al (2005) found in a systematic review of random controlled trials of early vs. delayed arm exercises that delaying exercises significantly decreases seroma formation (3) (which you will recall from the discussion above, Fu et al have found to be an important risk factor for BCRL).

Post-surgical physical therapy shown to reduce BCRL risk

Torres et al (2010) tested early postsurgical intervention using physical therapy that included manual lymph drainage, massage of scar tissue, and shoulder exercises, beginning the therapy not earlier than three to five days after hospital discharge, and continuing three times per week for three weeks. At one year post surgery, 7% of women in the intervention group had developed lymphedema, compared to 25% of women in the control (education only) group (4).

The Torres study report is not clear on whether any participants began the therapy within the seven-day post-surgical period Todd et al found to be risky for future BCRL development, stating that study subjects were assigned three to five days after discharge to either an education-only group or an education-plus-intervention therapy group. It seems likely that exercise was delayed at least five days.

Key Points

• Early post-surgical arm and/or shoulder exercises seem to increase the likelihood breast cancer patients will develop BCRL and/or seromas, which are associated with increased BCRL risk.
• Delaying shoulder-mobilization exercises by seven days seems to reduce the incidence of BCRL without adding risk of deficit in shoulder movement and related outcomes.

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1. McNeely ML, Campbell K, Ospina M, Rowe BH, et al. (2010) Exercise interventions for upper-limb dysfunction due to breast cancer treatment, Cochrane Database of Systematic Reviews, Published online Jun 16, 2010; http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD005211.pub2/abstract.

2. Todd J, Scally A, et al. (2008) A randomized controlled trial of two programmes of shoulder exercise following axillary node dissection for invasive breast cancer, Physiotherapy, 94: 265-273.

3. Shamley DR, Barker K, Simonite V, Beardshaw A. (2005) Delayed versus immediate exercises following surgery for breast cancer: a systematic review, Breast Cancer Research and Treatment, 90(3): 263-71.

4. Torres Lacompa, M, Yuse Sanches, MJ, et al.(2010) Effectiveness of early physiotherapy to prevent lymphedema after surgery for breast cancer: randomized, single blinded, clinical trial, BMJ, 340:b5397.

As outlined in a  previous entry, lipedema is a chronically progressive, symmetrical accumulation of fat in the subcutaneous tissue occurring almost exclusively in women. Primarily the lower extremities are affected, but lipedema may occur in combination with the upper extremities as well. Lipedema is characterized by symmetric enlargement of the limbs, combined with tenderness . . . → Read More: Differences between Lipedema and Lymphedema]]> Tweet 

As outlined in a  previous entry, lipedema is a chronically progressive, symmetrical accumulation of fat in the subcutaneous tissue occurring almost exclusively in women. Primarily the lower extremities are affected, but lipedema may occur in combination with the upper extremities as well. Lipedema is characterized by symmetric enlargement of the limbs, combined with tenderness and easy bruising.

Lipedema is not caused by a disorder of the lymphatic system; however, it is commonly misdiagnosed as bilateral primary lymphedema.

Several marked differences between lipedema and primary lymphedema can be distinguished; these differences are highlighted in the table below.

Lipedema

While lipedema always affects both legs symmetrically (bilateral appearance), primary lymphedema usually affects one leg only. If both legs are involved in primary lymphedema the swelling appears asymmetric (see image on bottom of this article). The feet are not involved in lipedema; the symmetrical distribution of fat is located between the hips and the ankles.
In contrast, the feet in lymphedema are involved in the swelling, and a diagnostic indicator known as the Stemmer sign is positive.
The Stemmer sign is a diagnostic test that involves pinching the skin on the upper surface of the toe (usually second toe) or fingers. If a fold of skin can be pinched and lifted up at the base of the second toe or middle finger, the Stemmer sign is negative. The Stemmer sign is positive and indicative of lymphedema when a skin fold cannot be lifted, but can only be grasped as a lump of tissue. This sign  will become positive if lipedema develops into lipo-lymphedema. Circumstances that can lead to lipedema developing into lymphedema are explained in a previous post.
Tissue in lipedema has a soft rubber-like feel in early stages and may include small fatty lumps (nodules) within the tissues in

Fatty nodules in lipedema

later stages. Pressure with the thumb does not leave an indentation (no pitting) in lipedema. Lymphedema is pitting and the tissue feels firmer that the one in lipedema, especially with fibrotic tissue typically being present starting in stage 2. The cause for the onset of lymphedema are malformations of the lymphatic system, while he underlying cause for the development of lipedema remains unknown; it is thought to be associated with hormonal disorders.

Differences at a Glance

Stemmer sign; positive on left

Primary bilateral lymphedema

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Additional Reading:

http://www.hanse-klinik.com/englisch/Lipoedema.pdf

http://www.lymphedemapeople.com/wiki/doku.php?id=lipedema

http://www.lymphedema-therapy.com/Lipedema.htm

The results reported in a recent study published in the Journal of Lymphology1 emphasized the significant effect of Manual Lymph Drainage (MLD) in preventing the onset of secondary lymphedema of the upper extremity on the operated side following breast cancer surgery. The study showed that prophylactic application of MLD administered immediately following breast cancer . . . → Read More: Efficacy of Manual Lymph Drainage in preventing Secondary Lymphedema following Breast Cancer Surgery]]> Tweet 

The results reported in a recent study published in the Journal of Lymphology¹ emphasized the significant effect of Manual Lymph Drainage (MLD) in preventing the onset of secondary lymphedema of the upper extremity on the operated side following breast cancer surgery. The study showed that prophylactic application of MLD administered immediately following breast cancer surgery helped to prevent or considerably alleviate secondary lymphedema of the arm irrespective of the method of breast cancer treatment.

The study included 67 women with an age range of 34-81 years, who underwent breast cancer surgery. 40 women received breast conserving therapy and 27 women underwent modified mastectomy. A total of 32 women received sentinel lymph node dissection (SLND) with an average of 2 lymph nodes removed (1-10), and 35 women underwent axillary lymph node dissection (ALND) with an average of 17 (8-29) lymph nodes removed during the procedure. In addition, 47 individuals underwent post-operative external radiation therapy, and 28 women received chemo, or endocrine therapy.

In 33 randomly chosen women (mean age 60.3 years) MLD was administered beginning on the second day, and for a duration of 6 months following surgery.
34 women (mean age 58.6 years) represented the control group and did not receive MLD, but were instructed in the application of self-Manual Lymph Drainage.
The individuals in both groups were of similar age and had no statistically significant differences in body mass index (BMI) and fat distribution prior to the surgery and 6 months after the surgical procedure.

Chemotherapy, endocrine therapy and radiation were applied to 39, 42, and 67% of the women who received MLD, respectively, and to 44, 56, and 73% of the women in the control group.

Starting on the second post-operative day individuals of both groups received a standard protocol of physical therapy exercises, and among the 33 randomly chosen women MLD was administered five times a week for the first 2 post-operative weeks, and twice a week from day 14 to 6 months following the surgery.
The MLD treatments were administered by the same therapist following the standard treatment protocol for secondary lymphedema of the upper extremity.

The volumes of both arms of all women participating in the study were measured using the water displacement method and taken before surgery and on days 2, 7, 14, and at 3 and 6 months following the surgery.

Compared with the arm volume before surgery, a significant increase in the arm volume (10%) on the operated side was observed among the women who did not receive MLD treatment at 6 month following the breast cancer surgery. In this control group the mean arm volume values on the operated side showed a continual increase beginning on the second post-operative day; at 3 months following surgery a 6% increase in volume was demonstrated, which increased to 10% in volume difference after 6 months; 70.6% of the individuals in the this group suffered from lymphedema at that time.

In the group of women who received MLD, mean arm volumes on the operated side increased on the second post-operative day and resolved by day 7 after surgery. At 6 months following breast cancer surgery, no increase in volume was evident and lymphedema of the arm on the operated side did not occur.

This study demonstrates the effectiveness of MLD in preventing the onset of secondary lymphedema irrespective of the type of surgery performed, the number of lymph nodes removed (ALND/SLND), and if radiation was applied. Furthermore, even though further studies are needed, this study shows that MLD applied directly following surgery for breast cancer and over a certain time, should be considered for the prevention of the onset of secondary lymphedema.

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1. Zimmermann A, Wozniewski M, Szklarska A, Lipowicz A, Szuba A: Efficacy of Manual Lymph Drainage in preventing Secondary Lymphedema after Breast Cancer Surgery. Lymphology 45 (2012) 103-112

The duration of the intensive phase varies with the severity of the condition and . . . → Read More: Self Manual Lymph Drainage for Lymphedema Affecting the Leg]]> Tweet 
Complete decongestive therapy (CDT) is performed in two phases; in the first phase, also known as the intensive or decongestive phase, treatments are administered by trained lymphedema therapists on a daily basis until the affected body part is decongested.

The duration of the intensive phase varies with the severity of the condition and averages two-three weeks for patients with lymphedema affecting the lower extremity. However, in extreme cases the decongestive phase may last longer and may have to be repeated several times.

The end of the first phase of CDT is determined by the results of measurements taken by the therapist on the affected body part. Once measurements approach a plateau, the end of phase one is reached and the patient progresses seamlessly into phase two of CDT, which is also known as the self-management phase.

Phase two is an ongoing and individualized part of CDT, in which the patient assumes responsibility for maintaining and improving the treatment results achieved in the intensive phase (phase one).

During the intensive phase patients are instructed by the therapist in the individual components of self-management, which include self-manual lymph drainage (self MLD, or simple MLD), abdominal breathing techniques, a skin care regimen, home exercises, and the application of compression garments (and bandages).

The self-manual lymph drainage techniques are relatively easy to perform and therapists generally teach one or two MLD strokes that can be learned and safely performed by the patient. It may help if the patient’s spouse, a relative or a friend is present during the therapist’s demonstration of these strokes to observe, take notes, or record the techniques with a camera.

The strokes are based on the same principles as those performed by the therapist, and it is very important that the patient clearly understands the hand movements, specifically the principles of skin elasticity, the pressures used during the working and resting phases of the strokes, and in which direction the pressure should be applied.

To help understand the techniques of MLD it is advisable to read the following articles published on this blog: 

1. Manual lymph drainage and its role in the treatment of lymphedema
2. The Science behind Manual Lymph Drainage in the Treatment of Lymphedema
3. Skin and Nail Care in Lymphedema Management
4. Primary Lymphedema
5. Secondary Lymphedema

The following techniques can be used for lymphedema affecting one leg; the illustrations and techniques describe the sequence used for lymphedema affecting the left leg. In this case the lymph nodes located in the opposite groin (right) and those in the axilla of the same side (left) are unaffected and working properly.

This sequence should not be used in lymphedema affecting both legs, or if the axillary lymph nodes on the affected, or the inguinal (groin) lymph nodes on the unaffected side are removed, or non-functioning due to other reasons.

Ideally, self MLD should be applied at least once daily for 15-20 minutes, directly preceding the exercise program, and should be followed by appropriate skin care and compression therapy. Each stroke should be repeated 5-7 times.

The techniques and sequences below are standard examples and may vary from those demonstrated by the therapist. Therapists may have different preferences, or the techniques may have to be adjusted to accommodate specific requirements or physical limitations of the individual patient.

Preparation and Abdominal Breathing:

1. Stationary circles with flat fingers above the collarbone on both sides. The fingers of the right hand manipulate the skin above the collarbone on the left and the fingers of the left hand manipulate the skin above the collarbone on the right. The pressure is applied with the flat phalanges of the fingers (generally the index, middle and ring fingers) and the pressure is directed toward the neck. This technique can be applied simultaneously on both sides or on each side individually.
   

   Step 1

2. Stationary circles with the flat hand in the center of the axilla (armpit, underarm) on the same (affected) side. The pressure is directed downward (deep) into the axilla and applied with the flat fingers and palm.
   

   Step 2

3. Stationary circles with the flat hand in several placements from the waist on the affected side to the axillary lymph nodes on the same side covering the entire surface of the lateral trunk (flank). The pressure is directed toward the axillary lymph nodes (same side).
   

   Step 3

4. Stationary circles with the flat hand in the area of the groin lymph nodes (inguinal lymph nodes) on the opposite side. The hand is placed just below the inguinal ligament (in the green area depicted on the illustration on the very bottom of this page) and the pressure is directed toward the belly.
   

   Step 4

5. Stationary circles with the flat hand in several placements from the groin (inguinal) area on the affected side to the groin (inguinal) area on the opposite side. The pressure is directed toward the groin (inguinal) area on the opposite side.
   

   Step 5

6. Diaphragmatic (abdominal) breathing:
   It is important to discuss any possible contraindications for this technique with the therapist!
   Abdominal breathing is done by contracting the diaphragm, a muscle located horizontally between the chest cavity and stomach cavity. As air enters the lungs this deep breathing is marked by expansion of the abdomen rather than the chest when inhaling. During the inhalation both hands that are placed flat on the belly provide resistance to the expanding abdomen.
   

   Step 6 – Inhalation

   During the exhalation the hands follow the belly and at the end of the exhalation, the hands press gently downward and upward toward the chest.

   

   Step 6 – Exhalation

   This technique should be repeated five times.

Leg

7. Soft effleurage over the skin of the entire leg from the ankles (or knees) to the waist.
   

   Step 7

8. Stationary circles with the flat hand and fingers in several placements on the lateral (outside) thigh and hip. The pressure is directed toward the waist.
   

   Step 8

9. Stationary circles with the flat hand and fingers in several placements from the medial (inside) portion to the lateral portion of the thigh. With the pressure directed toward the lateral aspect of the thigh, the entire thigh from the top (just below the groin) down to the knee should be covered.
   

   Step 9

10. Stationary circles with the flat fingers of both hands behind the knee. The pressure is directed upward toward the thigh.
    

    Step 10

11. Stationary circles with the flat fingers of both hands on the medial (inside) lower leg. With the pressure directed toward the thigh, the entire area between the knee and the medial ankle bone should be covered.
    

    Step 11

12. Stationary circles with the flat hand and fingers of both hands on the inner and outer surface of the lower leg. With the pressure directed toward the thigh, the entire area between the area below the knee and the ankle bones should be covered.
    

    Step 12

13. Repeat as many of the steps on the leg as you wish.
14. Repeat steps 2, 4, and 6
    

    Inguinal Ligament, Groin Lymph Nodes – Step 4

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Complete decongestive therapy (CDT) is performed in two phases; in the first phase, also known as the intensive or decongestive phase, treatments are administered by trained lymphedema therapists on a daily basis until the affected body part is decongested.

The duration of the intensive phase varies with the severity of the condition and . . . → Read More: Self Manual Lymph Drainage for Lymphedema Affecting the Arm]]> Tweet 

Complete decongestive therapy (CDT) is performed in two phases; in the first phase, also known as the intensive or decongestive phase, treatments are administered by trained lymphedema therapists on a daily basis until the affected body part is decongested.

The duration of the intensive phase varies with the severity of the condition and averages two-three weeks for patients with lymphedema affecting the arm. However, in extreme cases the decongestive phase may last longer and may have to be repeated several times.

The end of the first phase of CDT is determined by the results of measurements taken by the therapist on the affected body part. Once measurements approach a plateau, the end of phase one is reached and the patient progresses seamlessly into phase two of CDT, which is also known as the self-management phase.
Phase two is an ongoing and individualized part of CDT, in which the patient assumes responsibility for maintaining and improving the treatment results achieved in the intensive phase (phase one).
During the intensive phase patients are instructed by the therapist in the individual components of self-management, which include self-manual lymph drainage (self MLD, or simple MLD), a skin care regimen, home exercises, and the application of compression garments (and bandages).

The self-manual lymph drainage techniques are relatively easy to perform and therapists generally teach one or two MLD strokes that can be learned and safely performed by the patient. It may help if the patient’s spouse, a relative or a friend is present during the therapist’s demonstration of these strokes to observe, take notes, or record the techniques with a camera.
The strokes are based on the same principles as those performed by the therapist, and it is very important that the patient clearly understands the hand movements, specifically the principles of skin elasticity, the pressures used during the working and resting phases of the strokes, and in which direction the pressure should be applied.

To help understand the techniques of MLD it is advisable to read the following articles published on this blog:

1. Manual lymph drainage and its role in the treatment of lymphedema

2. The Science behind Manual Lymph Drainage in the Treatment of Lymphedema

3. The Role of Complete Decongestive Therapy in Breast Cancer Related Lymphedema

4. Skin and Nail Care in Lymphedema Management

The following techniques can be used for lymphedema affecting one arm; the illustrations and techniques describe the sequence used for lymphedema affecting the left arm. In this case the lymph nodes located in the opposite axilla (right armpit, underarm) and those in the groin of the same side (left) are unaffected and working properly.
This sequence should not be used in lymphedema affecting both arms, or if the axillary lymph nodes on the unaffected, or the inguinal (groin) lymph nodes on the affected side are removed, or non-functioning due to other reasons.

The techniques described are performed in the sitting position; ideally, self MLD should be applied at least once daily for 10-15 minutes, directly preceding the exercise program, and should be followed by appropriate skin care and compression therapy.
Each stroke should be repeated 5-7 times, and, if not noted otherwise, the hand of the unaffected side (in this case the right hand) should be used to perform the strokes.

The techniques and sequences below represent standard examples and may vary from those demonstrated by the therapist. Therapists may have different preferences, or the techniques may have to be adjusted to accommodate specific requirements or physical limitations of the individual patient.

Neck

1. Stationary circles with flat fingers above the collarbone on both sides. The fingers of the right hand manipulate the skin above the collarbone on the left and the fingers of the left hand manipulate the skin above the collarbone on the right. The pressure is applied with the flat phalanges of the fingers (generally the index, middle and ring fingers) and the pressure is directed toward the neck. This technique can be applied simultaneously on both sides or on each side individually.
   

   Step 1

2. Stationary circles with the flat hand in the center of the opposite axilla (armpit, underarm). The pressure is directed downward (deep) into the axilla and applied with the flat fingers and palm of the affected arm.
   

   Step 2

3. Soft effleurage over the skin from the affected axilla to the axilla of the opposite side.
   

   Step 3

4. Stationary circles with the flat hand in several placements from the axilla on the affected side to the axilla on the opposite side. The pressure is directed toward the axilla on the opposite side.
   

   Step 4

5. Stationary circles with the flat hand (use hand of affected side) in the area of the groin lymph nodes (inguinal lymph nodes) on the same side. The hand is placed just below the inguinal ligament (in the green area depicted on the illustration on the very bottom of the page) and the pressure is directed toward the belly.
   

   Step 5

6. Stationary circles with the flat hand in several placements from the axilla on the affected side to the inguinal lymph nodes on the same side covering the entire surface of the lateral trunk (flank). The pressure is directed toward the inguinal lymph nodes (same side).

Affected Arm

7. Soft effleurage over the skin of the arm from the hand to the top of the shoulder.
8. Stationary circles with the flat hand and fingers in several placements on the upper portion of the lateral upper arm, from the shoulder muscle (deltoid muscle area) of the affected arm to the top of the shoulder. The pressure is directed toward the neck.
   

   Step 8

9. Stationary circles with the flat hand and fingers in several placements from the medial (inside) portion to the lateral (outside) portion of the upper arm. With the pressure directed toward the lateral aspect of the arm, the entire upper arm from the top (just below the axilla) down to the elbow should be covered.
   

   Step 9

10. Stationary circles with the flat hand and fingers in several placements on the lateral upper arm. The entire lateral surface of the upper arm, from the elbow to the shoulder should be covered, with the pressure directed toward the shoulder muscle.
    

    Step 10

11. Stationary circles with the flat hand and fingers in several placements covering the entire frontal (anterior) aspect of the lower arm, from the elbow crease to the hand. In order to reach all aspects of the forearm, the arm should be held in supination with the palm of the hand pointing to the front. The pressure is directed toward the upper arm.
    

    Step 11

12. Repeat step number 10.
13. Stationary circles with the flat hand and fingers in several placements on the posterior aspect of the lower arm, from the elbow to the back of the hand. In order to reach all aspects of the forearm, the arm should be held in pronation with the palm of the hand resting on the thigh. The pressure is directed toward the upper arm.
    

    Step 13

14. Repeat steps 1, 2, and 5. Other steps may be repeated as well.
    

    Inguinal Ligament Step 5

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It is with great pleasure to announce that the third edition of the textbook “Lymphedema Management” is now published and available.

The first two editions authored by Joachim Zuther have enjoyed wide distribution, aiding thousands of practitioners and patients worldwide, and this new edition represents an exciting step forward for . . . → Read More: New Publication on Lymphedema]]> Tweet 

It is with great pleasure to announce that the third edition of the textbook “Lymphedema Management” is now published and available.

The first two editions authored by Joachim Zuther have enjoyed wide distribution, aiding thousands of practitioners and patients worldwide, and this new edition represents an exciting step forward for the field of lymphedema therapy.

In addition to all features presented in the previous editions, which have been updated, some topics have been extensively revised.

Updated topics include:

Anatomy, physiology, and pathology of the lymphatic system, venous insufficiencies, lipedema, axillary web syndrome, Klippel-Trenaunay and Parkes-Weber syndromes, and wounds.

Expanded and revised features include:

Filariasis, surgical and pharmaceutical treatment options, edema versus lymphedema, lymphedema risk reduction, obesity as it relates to lymphedema, radiation induced brachial plexopathy, nutritional aspects of lymphedema, low level laser therapy, intermittent compression therapy, care for compression bandages and garments, truncal lymphedema and diagnosis. Treatment sequences for primary and secondary lymphedema affecting the extremities, trunk, genitalia and head/neck area have been extensively updated as well.

Various highly experienced and respected contributors have been added to the existing list of  exceptional contributors for this book; new contributors include Jane Armer, who also wrote the foreword, Judith Nudelman, Marga Massey, Michael Bernas, Janice Cormier, Maureen Mc Beth, and many more.

Steve Norton, a well respected lymphedema educator in the U.S., collaborated with Joachim Zuther as co-author on this third (and any forthcoming) edition. With this statement the two authors hope to strongly reflect their unity and shared vision in regard to Complete Decongestive Therapy as the gold standard in lymphedema therapy. Their two respective schools, the Academy of Lymphatic Studies and the Norton School of Lymphatic Therapy, will align in using this textbook as their respective course guides. The authors are confident that this collaboration and alliance will contribute to the commitment to advancement in the field of lymphedema therapy.

The new contributors, many of whom are internationally recognized practicing clinicians have added the following features:

Expanded diagnosis and evaluation, lymphedema taping, expanded treatment of the head and neck, trunk and external genitalia (male and female), treatment and compression strategies for patients with wounds, paralyzed limbs, morbid obesity, and advanced-stage involvement, to include a multitude of treatment adaptations and nuances (foam, step-by-step instructions, garment selection and adaptation). New sections also include, lymphatic imaging, quality of life issues, surgical options (reconstruction, liposuction) and lymphatic microsurgery.

This new edition is published in a larger format (11” x 8”) than the previous two editions and presents a great contribution to the literature with the chief objective of providing thorough information on lymphedema, and ensuring the future of safe and effective therapy for patients affected by this condition.

Click here for more information

Click here to view the foreword and the list of contributors

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Lipedema is characterized by symmetric enlargement of the limbs, generally affecting the lower extremities extending from the hips to the ankles secondary to the deposition of fat; upper extremities are affected in 30% (1) of the cases.  

Lipedema is not rare and not caused by a disorder of the lymphatic system, but . . . → Read More: A Closer Look at Lipedema and the Effects on the Lymphatic System]]> Tweet 

Lipedema is characterized by symmetric enlargement of the limbs, generally affecting the lower extremities extending from the hips to the ankles secondary to the deposition of fat; upper extremities are affected in 30% (1) of the cases.  

Lipedema is not rare and not caused by a disorder of the lymphatic system, but is commonly misdiagnosed as bilateral lymphedema, extreme cellulitis, or morbid obesity.

Most commonly used synonyms for lipedema include:

• Adiposalgia/Adipoalgesia
• Adiposis dolorosa
• Lipalgia
• Lipomatosis dolorosa of the legs
• Lipodystrophia dolorosa
• Painful column leg

This condition almost exclusively affects women; according to an epidemiologic study by Földi E. and Földi M.(2), lipedema affects 11% of the female population, and literature suggests that lipedema is associated with extensive hormonal disorders or liver dysfunctions if present in males.

Lipedema is a painful fat disorder and if left untreated can cause multiple secondary health problems, to include mobility issues and lymphedema. The quality of life, emotionally and physically, for individuals affected by lipedema is often decreased due to the fact that the condition is typically dismissed as simple obesity by clinicians unfamiliar with the symptoms.

The underlying cause for the development of lipedema remains unknown; it is thought to be associated with hormonal disorders and can be hereditary with 14% of affected individuals having a family history of lipedema (3). Lipedema can develop early in puberty; however, the mean age of diagnosis is approximately 35.

Lipedema can be diagnosed based on clinical criteria (history, typical clinical features) and by physical examination rather than with diagnostic tests.

Clinical Features:

• Symmetrical distribution of fat between the hips and ankles, the feet are not involved
• Ring of fatty tissue overlapping the tops of the feet
• Tissue has a soft rubber-like feel in early stages
• Initially, the skin color is normal
• Typical bulges of fatty tissue on the medial thigh (above the knee and close to the groin) are seen in later stages
• Small fatty lumps (nodules) within the tissues start to form in later stages
• In the early stages of lipedema the upper part of the body may be slender
• Weight loss does not have an effect on the areas affected by lipedema
• Swelling (edema) is common in the second half of the day and includes the feet, but decreases in the early stage with elevation and night-time rest
• Pain, tenderness, sensitivity to pressure
• Easy bruising

For the purpose of this forum, the additional swelling that develops in the later hours of the day in lipedema is of particular interest and is indicative for the involvement of the lymphatic system if lipedema remains without proper management.

Corkscrew-like appearance of lymph collectors in lipedema

The excessive amount of fatty tissue present in lipedema compresses the lymph collectors of the superficial lymphatic system, which are embedded in the fatty subcutaneous tissue. Lymphangiographic imaging shows that the lymph collectors within the proliferated fatty tissue have a coiled or corkscrew-like appearance rather than passing fairly straight towards the lymph nodes as is the case in healthy tissue. This can result in a reduced transport capacity of the lymphatic system in the affected area.

If the capacity of the lymphatic system is reduced to such an extent that it becomes unable to perform one of its basic functions, the removal of water from the tissues, fluid will accumulate and “real” edema develops in addition to lipedema.
In the initial stages the swelling may recede with elevation and rest, but over time and without adequate treatment (compression, elevation, exercise), the constant strain on the lymphatic system may cause damage to the lymphatic vessels, leading to further reduction of its transport capacity, and swelling may be constantly present.

Lipo-lymphedema

As a result of prolonged overstrain of the lymphatic system, lymphedema may develop secondary to lipedema (lipo-lymphedema), thereby increasing the complexity of treatment. If lipo-lymphedema remains without treatment, it will progress through the same stages as primary or secondary lymphedema.

Treatment

Active treatment for lipedema is necessary because early diagnosis and treatment can determine the individual’s long-term prognosis. Therapy for lipedema can be largely divided into conservative treatments to reduce edema, and surgical treatments such as liposuction.
Main goals in the conservative treatment of lipedema are to decrease pain and hypersensitivity, increase mobility and to prevent, or if already present, to address the edematous component associated with lipedema. If lipedema, or lipo-lymphedema is associated with obesity, nutritional guidance must be provided to reduce weight and avoid further weight gain.

Conservative approaches include complete decongestive therapy (CDT); CDT does not address proliferated fatty tissue, but contributes to the reduction of edema and the prevention of the manifestation of lipo-lymphedema. The various components of CDT also contribute to reducing pain and hypersensitivity to pressure.
In most cases it is necessary to apply a lower level of compression (bandages and compression garments) due to pain and hypersensitivity in the affected areas. Compression garments generally have to be custom-made to the individuals’ measurements. If the use of compression garments is discontinued, edema will return.

Surgical treatment may be considered for patients with lipedema who do not respond to conservative treatment. Liposuction is currently the standard surgical treatment method; however, this procedure may cause bleeding and secondary damage to lymph vessels resulting in persistent swelling. New and more advanced techniques may reduce these risks; however, individuals considering this approach should ensure that the performing physician is experienced and follows internationally established guidelines.

Postoperatively, there is generally an increased tendency for swelling, thus CDT should be initiated or continued within a few days of the procedure.

In the presence of additional lymphedema (lipo-lymphedema) the treatment protocol for complete decongestive therapy corresponds with that for primary lymphedema. CDT shows good long-term results in lipo-lymphedema; however affected individuals need to understand that, although the lymphedemateous component responds well and generally relatively fast to CDT, the lipedema itself, i.e. the reduction of fatty tissue responds more slowly, and sometimes not at all. According to several authors, reduction of the excessive fatty tissue in lipedema is possible if compression garments are worn constantly.

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(1) Herpertz, U. (1995) Das Lipödem. Lymphologie 19, 1-11

(2) Földi, E., and Földi, M. (2006) Lipedema. In Földi’s Textbook of Lymphology (Földi, M., and Földi, E., eds) pp. 417-427, Elsevier GmbH,Munich,Germany

(3) Child AH, Gordon KD, Sharpe P, Brice G, Ostergaard P, Jeffery S, Mortimer PS. Lipedema: an inherited condition. Am J Med Genet A. 2010;152A:970–976. [PubMed]

Additional Reading:

http://www.hanse-klinik.com/englisch/Lipoedema.pdf

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3309375/

Fibromyalgia, also known as fibromyalgia syndrome, fibromyositis and fibrositis, is one of the most common chronic pain conditions, affecting millions of individuals in the United States and worldwide. While numbers on the prevalence of fibromyalgia in the literature vary considerably, the American College of Rheumatology (2008) estimates the number of individuals affected in . . . → Read More: The Role of Manual Lymphatic Drainage in Fibromyalgia]]> Tweet 

Fibromyalgia, also known as fibromyalgia syndrome, fibromyositis and fibrositis, is one of the most common chronic pain conditions, affecting millions of individuals in the United States and worldwide. While numbers on the prevalence of fibromyalgia in the literature vary considerably, the American College of Rheumatology (2008) estimates the number of individuals affected in the U.S.to be 5 million¹. The National Fibromyalgia Association (NFA) estimates the number to be 10 million in the U.S., and 3-6% of the world population².

While fibromyalgia can occur in women and men of all ethnic groups and ages, the condition is more common in middle-aged women (80%) and those women who have a family member affected by fibromyalgia are more likely to develop the condition themselves (American College of Rheumatology, 2004).

These numbers clearly indicate that fibromyalgia is a common condition; it is a syndrome rather than a disease, which includes a number of signs, symptoms, and medical problems that tend to occur together but are not related to a specific, identifiable cause.

The most prevalent symptom is chronic widespread pain and tenderness (sensitivity to touch), in muscles, joints and soft tissue, fatigue and sleep disturbances.
These main symptoms of fibromyalgia are identical with those of many other conditions, explaining why fibromyalgia is difficult to diagnose. Physicians often have to rule out other potential causes of these symptoms before making the diagnosis of fibromyalgia. The absence of diagnostic laboratory tests and no generally accepted, objective test for fibromyalgia present additional stepping stones in making the correct diagnosis.
Some physicians unfortunately may not be able to proper diagnose this condition and tell the patient there is little that can be done.

Specific causes for fibromyalgia are unknown, but it is thought that a number of factors may be involved that could trigger fibromyalgia, which may include

• Physical or emotional trauma
• Abnormal pain response, i.e. problems with how the central nervous system (brain and spinal cord) processes pain – areas in the brain that are responsible for pain may react differently in fibromyalgia patients
• Infection, such as a virus – however, none has yet been identified

Diagnosis of Fibromyalgia

Physicians have to rely on the patient’s self-reported symptoms, the history and a physical examination, which includes checking of specific manual tender points.
This examination is based on criteria set by the American College of Rheumatology (ACR) for the diagnosis and classification of fibromyalgia³.

To receive a diagnosis of fibromyalgia, the patient must meet the following diagnostic criteria:

• Widespread pain in all four quadrants of the body for a minimum duration of three months. The four quadrants include both sides of the body, above and below the waist line
• Tenderness or pain in at least 11 of the 18 specified tender points when pressure is applied (see illustration)
  

  Fibromyalgia tender points

Individuals affected by fibromyalgia may feel pain at other sites as well, however, those 18 standard possible sites on the body are the criteria used for classification.

Treatment

Due to the difficult nature of fibromyalgia, treatment requires a team approach including the patient, physicians familiar with the condition (rheumatologists, internists), physical-, massage- and occupational therapists, and other health care professionals.
The main goal is the relief of pain and other symptoms associated with fibromyalgia and helping the affected individual to cope with the condition.
Treatment may include physical therapy, stress-relief methods, including light massage and manual lymph drainage, and medication for pain and sleep management.
More information on these various treatment approaches is available on various online resources^{4, 5}.

Manual Lymph Drainage

Several studies on the effectiveness of manual lymph drainage (MLD) in the treatment of fibromyalgia indicate that MLD yields positive results in terms of pain relief, stiffness, sleep and general health status^{6, 7, 8}.

The result of a randomized controlled trial comparing manual lymph drainage with connective tissue massage in the treatment of women affected by fibromyalgia suggests MLD to be more effective in the treatment of fibromyalgia than massage⁶.

The Difference between MLD and Massage

As readers of this blog know, massage and manual lymph drainage are two very different manual treatment modalities. It is unfortunate that the term massage is often wrongly used to describe MLD. The origin of the word massage is derived from the Greek massain (to knead) and is used to describe such techniques as effleurage, petrissage, vibration, etc.
Compared to traditional massage, the pressure applied with manual lymph drainage is much lower in intensity. The goal of these techniques is to manipulate the lymphatic structures located in the subcutaneous tissues. In order to achieve the desired effect, the pressure should be sufficient enough to stretch the subcutaneous tissues against the fascia (a structure separating the skin from the muscle layer) located underneath, but not to manipulate the underlying muscle tissue. The amount of pressure needed in MLD is sometimes described as the pressure applied stroking a newborn’s head.

More information on MLD is available here:

The Science Behind Manual Lymph Drainage
The Role of Manual Lymph Drainage in the Treatment of Lymphedema 

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References:

1. http://www.rheumatology.org/about/newsroom/prevalence/prevalence-two.pdf
2. http://fmaware.org/site/PageServera6cc.html?pagename=fibromyalgia_affected
3. http://www.nfra.net/Diagnost.htm
4. http://fmaware.org/PageServerf195.html?pagename=fibromyalgia_treated,
5. http://www.niams.nih.gov/Health_Info/Fibromyalgia/default.asp#e
6. Comparison of manual lymph drainage therapy and connective tissue massage in women with fibromyalgia: a randomized controlled trial: http://www.ncbi.nlm.nih.gov/pubmed/19243724
7. Manual lymph drainage therapy using light massage for fibromyalgia sufferers: a pilot study: http://www.orthopaedic-nursing.com/article/S1361-3111(03)00084-0/abstract
8. Systematic Review of Efficacy for Manual Lymphatic Drainage Techniques in Sports Medicine and Rehabilitation: An Evidence-Based Practice Approach: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2755111/

 FurtherReading

 http://www.approvedarticles.com/Article/Manual-Lymphatic-Drainage–the-Autonomic-Nervous-System–CFS-and-Fibromyalgia–Part-1/749

Dear Readers!

Today an article appeared in the New York Times outlining the proposed settlement of a lawsuit that challenged the government’s practice of denying some coverage to patients whose condition was not improving. This settlement will certainly have an effect on current procedures in terms of Medicare coverage for patients affected by . . . → Read More: Proposed Settlement to Broaden Medicare Coverage for Chronic Conditions]]> Tweet 

Dear Readers!

Today an article appeared in the New York Times outlining the proposed settlement of a lawsuit that challenged the government’s practice of denying some coverage to patients whose condition was not improving. This settlement will certainly have an effect on current procedures in terms of Medicare coverage for patients affected by lymphedema.

Under the terms of the settlement, which is expected to be approved by judge Christina Reiss, the chief judge of the Federal District Court in Vermont in coming months, Medicare would not deny skilled nursing care and various forms of therapy for beneficiaries, regardless of their prognosis.

Medicare is required by law to cover healthcare services that are “reasonable and necessary for the diagnosis or treatment of illness or injury.” In practice however, Medicare has at times denied coverage for skilled care for beneficiaries whose condition was not considered likely to improve under what came to be known as the “improvement standard.” Under this standard, individuals on Medicare suffering from disabilities or chronic conditions that were not expected to improve, might have been denied coverage for physical therapy that could help keep them stable or prevent a further decline in their health.

The proposed settlement would allow Medicare beneficiaries with disabilities or chronic conditions to qualify for Medicare benefits for physical, speech and occupational therapy, and skilled nursing services that the program would not pay for previously.

If the proposed settlement is accepted by the court, the Centers for Medicare and Medicaid Services (CMS) will re-write portions of its Medicare Benefit Policy Manual and include rules to “maintain the patient’s current condition or prevent or slow further deterioration” for skilled nursing and home health services.

Here some excerpts on revisions from the settlement document:

Manual Revisions

1. The agency will revise the relevant portions of Chapters 7, 8, and 15 of the Medicare Benefit Policy Manual (MBPM) to clarify the coverage standards for the skilled nursing facility (SNF), home health (HH), and outpatient therapy (OPT) benefits when a patient has no restoration or improvement potential but when that patient needs skilled SNF, HH, or OPT services (SNF, HH, OPT “maintenance coverage standard”).

The agency will also revise the relevant portions of Chapter 1, Section 110 of the MBPM to clarify the coverage standards for services performed in an inpatient rehabilitation facility (IRF).

2. The manual revisions to be made pursuant to this Settlement Agreement will clarify the SNF, HH, and OPT maintenance coverage standards and IRF coverage standard only as set forth below in Sections IX.6 through IX.8.

Existing Medicare eligibility requirements for coverage remain in effect.

Nothing in this Settlement Agreement modifies, contracts, or expands the existing eligibility requirements for receiving Medicare coverage, including such requirements found in: a. Post hospital SNF Care, as set forth in 42 C.F.R. Part 409, Subparts C and D, and related sub regulatory guidance;

b. Home Health Services, as set forth in 42 C.F.R. Part 409, Subpart E, 42 C.F.R. Part 410, Subpart C, and related sub regulatory guidance;

c. Outpatient Therapy Services, as set forth in 42 C.F.R. Part 410, Subpart B, and related sub regulatory guidance; and

d. Inpatient Rehabilitation Facility services, as set forth in 42 C.F.R. Part 412, Subpart P, and related sub regulatory guidance.

3. CMS will revise or eliminate any manual provisions in Chapters 7, 8, and 15 and Chapter 1, Section 110 of the MBPM that CMS determines are in conflict with the standards set forth below in Sections IX.6 through IX.8.

Maintenance Coverage Standard for Therapy Services under the SNF, HH, and OPT Benefits

6. Manual revisions will clarify that SNF, HH, and OPT coverage of therapy to perform a maintenance program does not turn on the presence or absence of a beneficiary’s potential for improvement from the therapy, but rather on the beneficiary’s need for skilled care.
a. The manual revisions will clarify that, under the SNF, HH, and OPT maintenance coverage standards, skilled therapy services are covered when an individualized assessment of the patient’s clinical condition demonstrates that the specialized judgment, knowledge, and skills of a qualified therapist (“skilled care”) are necessary for the performance of a safe and effective maintenance program. Such a maintenance program to maintain the patient’s current condition or to prevent or slow further deterioration is covered so long as the beneficiary requires skilled care for the safe and effective performance of the program. When, however, the individualized assessment does not demonstrate such a necessity for skilled care, including when the performance of a maintenance program does not require the skills of a therapist because it could safely and effectively be accomplished by the patient or with the assistance of non-therapists, including unskilled caregivers, such maintenance services will not be covered under the SNF, HH, or OPT benefits.

b. The manual revisions will further clarify that, under the standard set forth in the previous paragraph (Section IX.6.a.), skilled care is necessary for the performance of a safe and effective maintenance program only when (a) the particular patient’s special medical complications require the skills of a qualified therapist to perform a therapy service that would otherwise be considered non-skilled; or (b) the needed therapy procedures are of such complexity that the skills of a qualified therapist are required to perform the procedure.

c. The manual revisions will further clarify that, to the extent provided by regulation, the establishment or design of a maintenance program by a qualified therapist, the instruction of the beneficiary or appropriate caregiver by a qualified therapist regarding a maintenance program, and the necessary periodic reevaluations by a qualified therapist of the beneficiary and maintenance program are covered to the degree that the specialized knowledge and judgment of a qualified therapist are required.

d. The maintenance coverage standard for therapy as outlined in this section does not apply to therapy services provided in an inpatient rehabilitation facility (IRF) or a comprehensive outpatient rehabilitation facility (CORF).

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Here the link to the complete settlement agreement: http://www.medicareadvocacy.org/wp-content/uploads/2012/10/Proposed-Settlement-Agreement.101612.pdf

New York Times article: http://www.nytimes.com/2012/10/23/us/politics/settlement-eases-rules-for-some-medicare-patients.html?ref=us&_r=1

Chronic lymphedema is considered to be a progressive condition regardless if it is classified as primary or secondary and can not simply be described as an accumulation of protein-rich fluid. It is a chronic degenerative and inflammatory process affecting the soft tissues, skin, lymph vessels and nodes and may result in severe and . . . → Read More: Stages of Lymphedema]]> Tweet 

Chronic lymphedema is considered to be a progressive condition regardless if it is classified as primary or secondary and can not simply be described as an accumulation of protein-rich fluid. It is a chronic degenerative and inflammatory process affecting the soft tissues, skin, lymph vessels and nodes and may result in severe and often disabling swelling.
Lymphedema may present in the extremities, trunk, abdomen, head and neck and external genitalia and can develop anytime during the course of a lifetime in primary cases; secondary cases may occur­ immediately following the surgical procedure or trauma, within a few months, a couple of years, or twenty years or more after treatment.

Lymphedema progresses through stages, and treatment intervention in early stages (stage 0 and stage I) has been shown to result in very good treatment outcomes if managed appropriately (1).

There are four stages of lymphedema

Stage 0, also known as latent stage or subclinical stage of lymphedema

In this stage the transport capacity of the lymphatic system is reduced, but the remaining lymph vessels are sufficient to manage the flow of lymph, and swelling is not visibly present.

Examples include individuals who underwent surgeries for malignancies, such as breast cancer, cancer affecting the genitourinary and gynecologic systems, cancers in the head and neck region, melanoma or soft tissue malignancies. These procedures generally include the removal of lymph nodes with subsequent disruption of lymphatic pathways.
A condition known as lymphangiopathy is present if the reduction in the transport capacity of the lymphatic system is caused by pathology affecting the lymphatic system directly in form of a developmental abnormality (malformations, as in primary lymphedema). In this case lymphedema is not clinically present as long as the lymphatic system is able to cope. 

In stage 0 patients may experience early symptoms, such as the feeling of numbness, tingling or fullness in a limb, which is often accompanied by low-grade discomfort. It may be difficult to fit into clothing, and watches, rings or bracelets may feel tight. This subclinical stage can exist for months, or years, before any more serious signs appear. The onset of lymphedema correlates to the ability of the lymphatic system to compensate for the reduced transport capacity and any added stress to the system that may cause an increase in the volume of lymphatic fluid.

Perometer

Early diagnosis and appropriate treatment of lymphedema is of paramount importance to limit progression of the swelling and to avoid complications often associated with untreated or incorrectly treated lymphedema; several studies have shown that patients’ self-reported symptoms are very accurate indicators of early lymphedema. While subclinical lymphedema can be detected using methods such as bioimpedance (2) and perometry (3), these technologies are not yet widely available.

Treatment intervention in this early and easily manageable stage has been shown to result in very good treatment outcomes using simple, non-custom compression garments (4).

Stage I, also known as pitting or reversible stage

Body parts such as the arms or legs are visibly swollen as protein-rich fluid starts to accumulate in the tissues. In many cases, the swelling subsides with elevation and the limb may appear normal in the morning; however, as soon as the limb is in a dependent position, the swelling returns. Pitting is easily induced by pressing with the thumb, and the indentation produced by this pressure is retained for some time.

While an increase in proliferating cells (increase in fibrous connective tissue) may be present, this early stage lymphedema is considered reversible because the skin and tissues have not yet been permanently damaged. With proper management it is possible for the patient to expect reduction of the extremity to a normal size (compared with the uninvolved limb). Without proper treatment, progression to the next stage is unavoidable in the vast majority of the cases.

Stage II, also known as spontaneously-irreversible stage

It is important to point out that the stage of lymphedema is not defined by size, but rather by the consistency of the tissues. This stage is primarily identified by tissue proliferation with subsequent thickening and hardening of the soft tissues. In many cases the swelling increases and elevation of the limb rarely reduces the swelling; pitting is evident. Over time, the tissue continues to harden and excess fatty tissue begins to form and pitting becomes difficult to induce.

A reduction in volume can be expected if proper treatment is initiated in this stage. In most cases, the excess fibrotic tissue typical in this stage will not recede during the intensive phase of complete decongestive therapy (CDT). Reduction in tissue fibrosis is mainly achieved in the second phase of CDT with proper compression and good patient compliance.

Stage III, also known as lymphostatic elephantiasis

Lymphedema often stabilizes in stage II. However, if lymphedema remains untreated, protein-rich fluid continues to accumulate, leading to further increase of swelling, sometimes resulting in extreme proportions. Hardening of the tissue continues and further deposition of fat it present. In this state, pitting is absent and the swollen body part becomes a perfect culture medium for bacteria and subsequent recurrent infections (lymphangitis) are frequent. Moreover, untreated lymphedema can lead into a decrease or loss of functioning of the affected extremity, skin breakdown and sometimes irreversible complications.

Reduction can still be expected if treatment starts in this stage. In most cases the duration of the intensive phase of complete decongestive therapy has to be extended and repeated several times. In extreme cases the surgical removal of excess skin following the conservative therapy may be indicated (5).

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1. Torres Lacompa, M, Yuse Sanches, MJ, et al.(2010) Effectiveness of early physiotherapy to prevent lymphedema after surgery for breast cancer: randomized, single blinded, clinical trial, BMJ, 340:b5397.

2. The use of bioimpedance analysis to evaluate lymphedema

3. Perometry

4. Stout Gergich NL, Pfalzer LA, McGarvey C, Springer B, Gerber LH, Soballe P. (2008) Preoperative assessment enables the early diagnosis and successful treatment of lymphedema. Cancer, 112:2809-2819.

5. Chronic Lymphedema – Treatment and Surgical Options