Pathways Winter 2013/2014 (Volume 3, Issue 1) by Canadian Lymphedema Framework

Canada’s Lymphedema Magazine

Pathways

WINTER 2014

Empowering patients and professionals

First national lymphedema conference

Lymphedema distichiasis syndrome Exercise for and with lymphedema WH A T ’S INSIDE... Research update

Pneumatic pumps

A family’s journey

CLF initiatives

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Editor’s Message

Bright hope on the horizon

f there was ever a time to feel encouraged about the future of lymphedema diagnosis, treatment and a cure, it is now. My optimism comes directly from the recent Canadian Lymphedema Conference. Canada’s first national event was a success, attracting delegates, speakers and exhibitors from across the country. A variety of oral and poster presentations showcased the research, and clinical and community work being done in Canada and abroad. It was encouraging to see new students participating, interested in lymphology as an area of study. The conference highlight was Guest Speaker, Dr. Stanley Rockson’s exciting plenary session about recent research discoveries, inspiring hope for future diagnostics and drugs for lymphedema. We thank John Mulligan for his thorough review of the conference. The Pathways magazine was also featured at the conference, in a poster presentation entitled The collaboration to launch a unique Canadian lymphedema magazine. It outlined the work done by the Canadian Lymphedema Framework (CLF) and its provincial affiliates to launch and produce this national magazine with individualized local provincial inserts. The poster placed second in the People’s Choice Awards. To pump or not to pump was the topic

of a mini presentation during the Panel of Experts session at the conference. Guenter Klose was kind enough to repeat his presentation in our Ask the Expert column, explaining how sequential pumps may be beneficial, when used in conjunction with other daily self-care measures. An important component of daily selfcare for lymphedema patients is exercise. Dorit Tidhar and DeCourcy Squire discuss the role of exercise in lymphedema management and provide guidelines for a safe return to a more vigorous exercise program. What else do I feel optimistic about? Some landmark announcements were made recently regarding lymphedema research funding. A $5 million donation was awarded to the University of Calgary. Within the same month, a $2 million grant was announced at Georgia Tech in the USA for studying the lymphatic system through engineering. This increase in lymphedema research funding will help perpetuate the interest in lymphedema as an area of study. All in all, this is an exciting time for the lymphedema community with funding and priorities set aside to give bright hope for changes in the future. However, there are many lymphedema aspects we still know little about, such as the lymphedema distichiasis syndrome.

Dr. Vaughn Keeley shares current knowledge about this rare, congenital condition. For the families who are affected with this disease, it can be isolating and frustrating to find answers and medical help. Joanne Small-Hopkins shares her family’s journey of finding out every member of her family is carrying the gene responsible for this syndrome. The diagnosis can also bring considerable financial burden. Her family has spent close to $25,000 to travel to Toronto six times for medical care including consultations and surgeries. They received minimal government support.

All in all—this is an exciting time for the lymphedema community with funding and priorities set aside to give bright hope for changes in the future. This highlights the work that still needs to be done to ensure all Canadians receive access to lymphedema treatment, regardless of what type of lymphatic condition they may have. Advocacy efforts are growing across the country. The CLF supported these initiatives by organizing an Advocacy Workshop for all provincial lymphedema organization leaders, at the recent conference. United, we will be much stronger. This issue marks the first in our third cycle of publications. Launched in the spring of 2012 the magazine has had eighteen months of tremendous support from readers, sponsors, advertisers, contributors and subscribers. The Editorial Board and I hope you will agree: we can all be proud of Pathways. Your contributions, suggestions and input will make it even better.

Provincial lymphedema association leaders from across Canada. Wi n t e r 2 0 1 4

Anna Kennedy Ly m p h e d e m a p a t h w a y s . c a 3

Contents

First Canadian Lymphedema Conference Pathways to collaboration Winter 2014

Volume 3 Issue 1

Publisher Pathways is published four times per year by BCS Communications Ltd. on behalf of the Canadian Lymphedema Framework (CLF) and its provincial affiliates. Editorial Board David Keast MD FCFP Winkle Kwan MD FRCPC Roanne Thomas PhD Anna Towers MD FCFP Lucette Wesley CIM Janice Yurick PT Editor Anna Kennedy Advertising and Submissions We welcome your ideas, contributions, letters and ad copy. While all material is subject to Editorial Board approval, inclusion does not constitute an endorsement or reflection of the views and opinions of the CLF and its Editorial Board. Contents may not be reproduced without written permission of the Canadian Lymphedema Framework. Subscription Rates (1 year) Canada–$25. International–$50. Single copies and back issues–$7.

Canada’s first national event was a success, attracting delegates, speakers and exhibitors from across the country for topics focusing on research, education and partnerships.

................................................................... Lymphedema Distichiasis Syndrome Our understanding of this inherited condition

A double row of eyelashes is associated with this type of primary lymphedema. It is a rare, inherited condition due to mutation in the FOXC2 gene.

...................................................................

Canadian Publications Mail Agreement 40065546 ISSN 1929-1418 Printed in Canada

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Learning About Primary Lymphedema Our personal journey

A family experiences the maze of learning about a congenital condition and inherited lymphedema.

...................................................................

Exercise For and With Lymphedema Proceed with caution—but proceed Experts discuss the role of exercise in the treatment for lymphedema—exercise for lymphedema—and about guidelines for a safe return to more vigorous activity– exercise with lymphedema.

........................................... Research New Advances Funding sparks hope for change

Subscriptions/return mail to Canadian Lymphedema Framework 4161 Dundas Street West, 1st Floor Toronto, Ontario M8X 1Y2 Telephone: 416-410-2250 Email: canadalymph@live.ca www.canadalymph.ca Some photos in this issue are courtesy of K. Dobbin, D. Keast, G. Klose, LRF, L. Wesley and Tenovus.

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This is an exciting time for the lymphedema community with funding and priorities set aside to give bright hope for changes in the future.

........................................... Ask the Expert To pump or not to pump

When used in conjunction with other daily self-care measures, a pump may lead to further limb volume reduction, improved range of motion, a decrease in subjective complaints such as heaviness and achiness, and ultimately, an improved quality of life.

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Conference Review

Canadian Lymphedema Conference

A Success By John Mulligan

he Canadian Lymphedema Conference for patients and professionals was held at the Sheraton Toronto Airport Hotel and Conference Centre on Friday October 25th and Saturday October 26th. Pathways to Collaboration— research, education, partnership was the theme of the conference, which was jointly hosted by the Lymphedema Association of Ontario (LAO) and the Canadian Lymphedema Framework (CLF). The educational event offered a wide variety of plenary and concurrent sessions delivered by 25 different speakers. Topics were divided into three different levels of Learning Streams to help delegates choose the appropriate and most relevant sessions. Learning Stream I was targeted to patients and those at risk. Learning Stream II was designed for new therapists, general health care professionals, garment fitters and experienced patients. Learning Stream III was comprised of programs deemed appropriate for experienced CDT therapists and educators. There were also some new offerings at this conference. Leaders from all the provincial lymphedema associations were joined by members of the Partnership Working Group of the CLF for a National Lymphedema Advocacy Workshop. This was the first time that all of the provincial leaders were able to be in one place for a face-to-face meeting, rather than their usual conference calls. It was a great opportunity for them to catch

This first call for abstracts by the Canadian Lymphedema Framework resulted in 24 accepted submissions from Canada, the United States, Wales and India. Eight abstracts were chosen for oral presentations and the remaining abstracts were eligible for a poster competition judged by the review committee. There was great variety in the poster presentations including submissions by several first-time participants who had never presented a scientific poster before. Winners were K. Dobbin (pictured above) et al. for Identification of Physical Effects and the Availability and Accessibility of Breast Cancer Rehabilitation and Lymphedema Management. Second place winner was S. Shallwani and her team for their case study titled Self-Management for Malignant Lymphedema. The People’s Choice award for best poster went to Jean Ann Ryan for her poster titled Improving Patients’ Quality of Life: Primary and Secondary Lymphedema.

up and discuss important issues. The National Lymphedema Advocacy Workshop was led by Niya Chari, the government relations manager for the Canadian Breast Cancer Network. The workshop was made possible through financial support from the Canadian Breast Cancer Foundation. Friday morning featured pre-conference industry sponsored symposiums. BSN Medical, Valco and 3M Canada all took this opportunity to present two- or three-hour

John Mulligan, RMT, CLT-LANA, is a registered massage therapist and Certified Lymphedema Therapist with a private practice in St. Catharine’s, Ontario. John has also taught manual lymph drainage, advanced bandaging and provides basic education on lymphedema to patient groups. He is a member of the Education Working Group of the Canadian Lymphedema Framework and co-founder of Lymphedema Depot.

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workshops focusing on how their products can be best utilized to help treat or manage lymphedema. While these programs were running, the Patient Advocacy workshop was presented. Also meeting at this time were the working group committees of the Canadian Lymphedema Framework. Again, this was a rare chance for volunteer committee members, who are helping to shape the future of lymphedema care in Canada, to have a face to face meeting instead of their usual teleconferences. At these meetings the agenda for the coming year is set. As a result, the meetings are often spirited and dynamic and much is accomplished. While virtually unseen by the general population of conference attendees the committee meetings are an important part of the CLF’s functioning. Ly m p h e d e m a p a t h w a y s . c a 5

The conference officially opened after lunch—with Dr. David Keast, co-chair of the CLF and Anna Kennedy, Executive Director of the LAO, welcoming the attendees. Dr. Anna Towers introduced the guest speaker, Dr. Paula Stewart, who delivered the Plenary Session on the topic of Obesity and Lymphedema. She discussed some causes of the obesity epidemic and outlined treatment for different levels of obesity. Dr. Stewart also reported on the changes in the scientific

world’s understanding of obesity. Adipose tissue, we learned, is an active endocrine organ regulating metabolic processes; essentially it tries to make more of itself. This adds a complicating factor to the issue of losing weight and helps us to understand why it is more difficult to reduce adipose tissue (fat) in people with the highest BMI ratings. The impact of obesity on lymphedema is understood to the degree that it is an exacerbating factor. Dr. Stewart emphasized

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Dr. S. Rockson

Dr. P. Stewart

that lymphedema patients should attempt to keep their weight as close to ideal as possible, using the sensible dietary and exercise guidelines we all strive to observe. The rest of the afternoon offered concurrent education topics and sessions. Research Snapshots were presented by Canadian therapists and researchers who are closest to the clinical experience in Canada. Subjects addressed were: the association between lymphedema and cellulitis, developing a support program for patients with secondary lymphedema and one on secondary lymphedema and activities of daily living, in which the patients were given a chance to document their own challenges by taking photographs. The final presentation dispelled some myths around breast reconstruction and the risk of lymphedema. While these research findings were being presented, there were concurrent sessions on teaching a method of selfbandaging, modeling self-management support, and a session of Aqua Lymphatic pool Therapy (ALT) for therapists. These sessions provided good hands-on experiences for the attendees and were all taught or presented by Canadians. An early evening networking reception and poster viewing was sponsored by the Canadian Breast Cancer Foundation. In many ways, networking is the heart of a conference. The Canadian lymphedema community is far-flung across the country and an event like this gives us all the chance to speak to colleagues and friends that we might only exchange emails and phone calls with during the course of the year. The networking session was also a chance to view the scientific posters that were displayed in the area. The winners were announced by Roanne Thomas, Chair of Wi n t e r 2 0 1 4

the CLF’s Research Group. Cheers and applause greeted the winners, Karen Dobbin What the delegates had to say and Shirwin Shallwani and their teams for their excellent posters. The networking 98% of respondents gave an overall rating of good – excellent provided a pleasant and rewarding finish to 96% said the conference was well organized the first day’s activities. 96% felt it was a worthwhile investment of their time and money Saturday’s sessions were open to patients as well as professionals. The day began 99% learned something new with opening remarks by Dr. Keast and Anna Kennedy. The plenary session guest speaker was Dr. Stanley Rockson, who, among Dr. Rockson explained that, after a long and is engaged in a two-year-long pilot study. other achievements, is the Director of the period where little lymphedema research He expects to publish results in the next Stanford Center for Lymphatic and Venous was taking place, from 2000 to 2011 the six-to-nine months. Disorders as well as the Editor-in-Chief of growth in research has been exponential, After this well-received and exciting the journal Lymphatic Research and Biology. a progression that lasts into today. presentation, the day carried on with another Dr. Rockson’s presentation was the most Dr. Rockson’s presentation featured set of Research Snapshots. Scheduled talked about session of the conference and graphics of the lymph system taken through concurrently was a pool session for Aqua gave us access to the very latest information lymphscintigraphy and electron microscopy, Lymphatic Therapy for the Arm, a presentation in our understanding of lymphedema. giving many in the audience a glimpse into on living with the challenges of lymphedema He stressed the importance of clinical the real world of the lymphatic system that by Dr. Thomas Hack, the CBCF Chair in diagnosis and early intervention in they may not have encountered before. Psychosocial and Supportive Care Oncology lymphedema, supporting the idea His presentation was a tour of where Research at St. Boniface Hospital in Winnipeg. that very early intervention the science of lymphology has Dr. Hack has done great work in Psychosocial can return the lymph come from, where it is now Oncology and coping among breast cancer He ended on system to normal function and a vision of where survivors and is a moving and informative a note of promise, over time, but the more it is headed in the near speaker, bringing his unique perspective his own research time that passes between borne of long research into how coping future. He spoke of gene involves looking for onset and intervention markers and family cohorts, skills affect quality of life. During this set of biomarkers for the more difficult it is concurrent sessions, lymphedema therapist and studies with zebra fish lymphedema. to return the lymphatic and mouse tails that showed and educator Jan McFarland shared her functioning back to normal. the promise of using human work in developing integrative approaches He also brought us through the growth factor as a treatment modality to enhance CDT (Complex Decongestive history of lymphatic science, and reminded someday. He ended on a note of promise, Physiotherapy) outcomes in the lymphedema us that the current medical curricula saying that his own research involves looking patient population. for physicians contains about 30 minutes for biomarkers for lymphedema, in the way Dr. Catherine McCuaig from Montreal about the lymphatics over the four years is the Associate Professor of Dermatology we use the PSA test to look for biomarkers of of medical education. in Pediatrics at University of Montreal. prostate cancer. He has isolated six proteins,

The Board Directors of the Lymphedema Association of Ontario and the Canadian Lymphedema Framework thank the following conference sponsors for their generous support. Wi n t e r 2 0 1 4

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The most popular session after lunch was the Panel of Experts. With Dr. Anna Towers moderating, there were five fifteenminute presentations: Dr. Keast on wound care; Dr. Winkle Kwan on radiation oncology; Guenter Klose on using intermittent compression pumps as an adjunct technology in the treatment and management of lymphedema; Robert Harris on studies supporting the efficacy of manual lymph drainage; and Dr. Paula Stewart who shared her knowledge about nutrition and lymphedema. There was a lively question and answer period at the end of these presentations. One more timeslot of concurrent sessions followed, with a balance of theoretical and practical topics, including hints and tips regarding self-management for patients, an aqua-lymphatic pool session, Dr. McCuaig presenting a more in-depth look at pediatric lymphedema and a presentation about assessing research literature by Dr. H. Bhanabhai, a palliative care physician at McGill University Health Centre in Montreal.

Photos: Tenovus

Her session focused on Skin Changes and Lymphedema and in particular her clinical experiences with childhood lymphedema patients. Saturday afternoon offered a yoga session and a self-bandaging workshop. There was also another edition of Research Snapshots featuring work being done across Canada and in Wales. Richard Pugh, representing a charity called Tenovus, that raised the funds to deliver chemotherapy and lymphedema treatment to patients in rural communities across Wales, described their state of the art mobile unit that features satellite access and modern interactive technology.

Interior of the Tenovus mobile chemotherapy and lymphedema treatment unit. 14801 Linotrade annonce 1/4 horiz._horizontal 12-10-25 2:09 PM Page 1

The following feedback from a patient represents the overall feeling at the conference:

“I was really encouraged knowing that the study of LE is making such headway. I was inspired to meet the enthusiastic professionals that are taking such an interest in this field”.

There were numerous opportunities throughout the two days to view the poster gallery and to visit with the exhibitors to see what industry is currently bringing to the treatment and management of lymphedema. On the whole, the 17th Annual Lymphedema Association of Ontario’s Lymphedema Conference, coupled with the Canadian Lymphedema Framework`s national meeting and presentations, was a wonderful mix of inspiring presentations and practical applications, of advancements in both theory and practice and a broadening sense of the lymphedema community itself, where it finds itself today and where it is headed in the future. The conference provided enough fuel to keep us pushing forward as individuals and to give each of us, patients and professionals alike, a sense of where we stand in relation to this field of lymphedema work. We hope to see you at the next event! LP

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Wi n t e r 2 0 1 4

Clinical Perspectives

Lymphedema

Distichiasis Syndrome By Vaughan Keeley

ymphedema distichiasis syndrome describes a type of primary lymphedema that affects the legs and is associated with a “double row of eye lashes” (distichiasis).1 It is an inherited condition and is due to mutation in the FOXC2 gene. It may also be associated with other features. Although lymphedema distichiasis is a rare condition, it is not clear exactly how many people have it. It may not always be recognised by health care professionals, so it is possible that it may be more common than currently believed. Clinical description Lymphedema The lymphedema associated with this condition typically affects the lower part of both legs and can appear at a variety of ages (range 7 to 40 years) although it most commonly develops around puberty (Figure 1). However, the swelling may affect one leg only and, as with many other inherited types of lymphedema, the severity of the problem can vary significantly amongst members of the same family. It seems to affect male members of the family more significantly, in that it tends to occur at an earlier age in males and they experience a greater incidence of cellulitis. Distichiasis The term “distichiasis” describes an extra row of eye lashes which arises from small glands in the eyelids (Figure 2). These lashes are closer to the eye itself than normal eyelashes and may cause problems by “growing in,” leading to irritation of the eye with repeated infections. However, about 25% of individu-

als with distichiasis have no symptoms from it. The distichiasis itself can be very variable ranging from a full set of extra eyelashes to a single extra lash. Distichiasis is said to be present in 94% of individuals with lymphedema distichiasis syndrome, but the lashes can be very small and not easy to see without careful examination of the eye. This, therefore, may mean that the condition is not always recognized. Varicose veins There is a greater incidence of varicose veins developing at a young age in individuals with lymphedema distichiasis syndrome. It has been estimated that about 50% of individuals with lymphedema distichiasis have evidence of varicose veins on examination of the legs and it may be that abnormalities of the veins are present before they become obvious on examination (Figure 3). Other clinical features Ptosis describes drooping of one or both eyelids and occurs in about 30% of people with lymphedema distichiasis. In the past, the term “lymphedema-ptosis” was used to describe a specific type of primary lymphedema. It is now thought that this is probably not a separate condition but is part of the lymphedema distichiasis syndrome. Congenital heart disease occurs in about 7% of people with lymphedema distichiasis and can take a variety of forms including, for example, ventricular and atrial septal defects (“holes in the heart”).

Vaughan Keeley is a physician who works full time diagnosing and treating lymphedema patients in Derby, UK. He sees both adults and children with all types of lymphedema. He is also a board member of the International Lymphoedema Framework (ILF) and Chair of the ILF 2014 Conference Scientific Committee.

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Cleft Palate occurs in about 4% of people with lymphedema distichiasis. Other features may include curvature of the spine (scoliosis), cysts in the spine, “webbing” of the neck, abnormalities of the womb (uterus) and kidneys and squint affecting the eyes. Genetics Mutations of the FOXC2 gene are the only known cause for lymphedema distichiasis syndrome at present. It is possible that others will be described in the future. FOXC2 is a type of transcription factor gene. It provides instructions for making a protein that is involved in the formation of a number of organs and tissues in the body before birth. This explains why mutations of FOXC2 cause a variety of different features, not solely related to lymphatic problems (Table 1). The gene is located on chromosome number 16. Normally each cell in the body will have two copies of this chromosome. However in lymphedema distichiasis, one of the copies of the FOXC2 gene will be abnormal. This abnormality results in a malfunction in the FOXC2 protein so that it does not control the normal way other genes work. At this stage, it is not clear how the FOXC2 mutation causes all the TABLE 1

Clinical features of FOXC2 mutation and frequency Distichiasis

94%

Early onset varicose veins

49%

Ptosis

31%

Congenital heart disease

Cleft palate

Scoliosis

(derived from Brice et al. 2002)

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FIGURE 1

FIGURE 2

FIGURE 3

Lymphedema affecting lower legs

Extra row of eyelashes

Venous abnormality

aspects of lymphedema distichiasis but it is known that FOXC2 is involved in the development of blood and lymph vessels. In lymphedema distichiasis, it seems that the valves in both the lymph vessels and the veins do not work properly.2 As a result, there may be a backflow of blood in the veins and lymph in the lymph vessels, instead of a continuous forward flow. This process leads to a build up of pressure and subsequently varicose veins and lymphedema. Lymphedema distichiasis is inherited in “an autosomal dominant” manner and as such, each child of a person with lymphedema distichiasis has a 50% chance of inheriting the mutation. However, as mentioned above, the severity of the condition is very variable even within a single family. In about 75% of individuals with lymphedema distichiasis, one of their parents is also affected. In the other 25%, however, this is not the case and the condition is felt to be due to a new mutation (de novo mutation) in that individual. The gene mutation for lymphedema distichiasis was only described in 2000 and since then a variety of mutations and clinical patterns have been described as discussed above. In the next few years genetic studies will likely yield more information which may help to clarify some of the uncertainties in the current understanding.

veins at an early age help to confirm the clinical picture. The presence of a family history of similar problems supports the diagnosis. Isotope lymphoscintigraphy can be used to investigate lymphatic function in the legs. This shows a typical picture of the backflow of lymph due to the malfunctioning valves in the lymphatics.

of vein disease in the form of varicose veins. There is currently interest in the role of lymphovenous anastomosis surgery in treating lymphedema. This technique joins lymph vessels to veins to improve lymph drainage, especially if the lymph vessels are blocked or damaged, e.g., by cancer surgery or radiotherapy. Because individuals with lymphedema distichiasis also have abnormal veins, this technique is unlikely to be helpful in this condition.

Clinical diagnosis The diagnosis of lymphedema distichiasis is largely based on the clinical assessment. The pattern is of a primary lymphedema that affects the lower part of the legs, with onset around puberty or later together with the presence of distichiasis. The typical appearance of lower leg swelling and the presence of varicose 10 L y m p h e d e m a p a t h w a y s . c a

Genetic testing Testing for mutation in the FOXC2 gene can be carried out to confirm the diagnosis. Further investigations Echocardiography may need to be considered particularly if there is evidence of any heart problems such as a murmur on clinical examination. If there is doubt about the presence of distichiasis, referral to an Ophthalmologist for a more detailed examination of the eyelashes may be helpful. Treatment of lymphedema distichiasis syndrome Lymphedema There is no special treatment at present for the lymphedema associated with this condition. It is treated in the same way as other primary lymphedemas with a combination of physical treatments that may include compression, exercise, lymphatic drainage massage and skin care. The question has been raised whether the use of compression garments before the development of lymphedema in people with known distichiasis or FOXC2 mutation may help to prevent the development of lymphedema. There is no specific evidence about this at present but it would seem to be a reasonable way forward, particularly if there is evidence

Distichiasis The distichiasis, if symptomatic, can be managed by plucking the eyelashes out or, if warranted, treatment with electrolysis or cryotherapy may be used. Varicose veins Varicose veins may be managed with compression treatments. In light of the presence of backflow through the valves in both the deep and superficial vein systems, surgery is unlikely to be helpful. Other problems Specific treatments for ptosis (surgery), spinal problems and heart problems may be appropriate where needed. Cellulitis As with other types of primary lymphedema, cellulitis may be experienced as a complication. Management of acute episodes with appropriate antibiotics, the prompt treatment of foot infections such as athlete’s foot and the consideration of prophylactic antibiotics for recurrent episodes of cellulitis are appropriate in lymphedema distichiasis as in other types of primary lymphedema. Wi n t e r 2 0 1 4

Future treatments The identification of the causal gene mutation in lymphedema distichiasis does raise the possibility of different treatments for the condition including gene therapies, but these are unlikely to be available in the near future. The greater understanding of the molecular mechanisms of the production of lymphedema in lymphedema distichiasis may give rise to future drug treatments. Again, this is not likely to be available in the very near future but remains a possible development.

that an individual with a FOXC2 mutation may not have major problems from it. At present it is not possible to predict the impact of the individual types of mutation.

3. M ellor R, Brice G, Stanton et al. (2007) Mutations in FOXC2 are Strongly Associated With Primary Valve Failure in Veins of the Lower Limb. Circulation 115:1912–1920.

Conclusion In the last 15 years, there has been a significant increase in the understanding of lymphedema distichiasis as a result of genetic and other related research. The discussion in this article reflects our current knowledge. There is still much to learn. LP

Antenatal testing Evidence of lymphedema distichiasis may be detected in the form of congenital heart problems on an ultrasound scan of the fetus in utero in families where FOXC2 mutation is known. Antenatal gene testing may also be an option but may not necessarily be helpful in treatment decisions in view of the noted variability in the effect of FOXC2 mutations within individual families. It is possible

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References 1. Brice G, Mansour S, Bell R et al. (2002) Analysis of the Phenotypic Abnormalities in Lymphoedema-Distichiasis Syndrome in 74 Patients With FOXC2 Mutations or Linkage to 16q24. J Med Genet 39:478–483. 2. Mansour S, Brice W, Jeffrey S et al. (2012) Lymphoedema-Distichiasis Syndrome. http://ncbi.nlm.nih.gov/books/NBK1547.

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Personal Perspectives

Learning About Primary Lymphedema By Joanne Small-Hopkins

n 2007, my husband and I were excited to have our first child, a beautiful baby boy. During that same time, my husband found a small lump on his forehead over his eye. However upon examination, he was told it was not cancerous and that it was nothing to worry about. Two years later, I gave birth to a beautiful baby girl. She was born with a droopy eyelid, otherwise known as “ptosis.” It was congenital and hereditary as my husband also displayed this condition when he was born. My husband’s ptosis was bilateral; however, oto: Wik ipe dia. org our daughter’s ptosis was only in the left eyelid. We knew her ptosis was more noticeable than my husband’s and understood plastic surgery was in her future. In her first year of life, I struggled to understand why both of them had this congenital condition of the eye. The doctors said sometimes these are hereditary, but there is rarely a father-to-daughter hereditary transmission with ptosis, rather it may be further down the bloodline. We pushed ahead treating my daughter’s

ptosis by patching her eye and clearing the tear ducts to maintain her eye health. In the spring of 2010, my husband came home from hockey after being hit in the leg with a puck. There was a large bruise and the leg was tender. Over the coming weeks, he experienced swelling of the right ankle. As time passed, the swelling did not recede and started in his other leg and ankle. For the next few months, doctors ran a series of tests but nothing showed up. In the meantime, his legs were swollen and it was very uncomfortable for him to be standing for long periods. Our frustration grew as time passed, and we could not get an answer regarding his symptoms. Almost every night, I was reading international publications and circulations about various conditions and diseases. I kept thinking about my daughter’s ptosis and how I felt it was related. What I found stunned me: As I was searching the genetics of a condition called lymphedema, I found many case studies where ptosis was an anomaly. Lymphedema occurs when your lymph vessels are unable to adequately drain

Joanne Small-Hopkins resides with her husband Gy and their two children in Portugal Cove St. Philips, Newfoundland. She is proud to be part of the proclamation passed in St. John’s, Newfoundland, designating March 6th of each year as National Lymphedema day. Her goal is to spread awareness of primary lymphedema and vascular diseases, especially in children.

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lymph fluid. I found that there is a gene called FOXC2—considered the master switch for your body; this gene is also responsible for lymphedema distichiasis syndrome. The same physician, who was seeing my daughter for her ptosis, was also consulted for my husband’s leg edema. I presented him with research I found concerning primary vascular failure in the lower limbs and the FOXC2 gene. I noted that with lymphedema distichiasis syndrome 50% of patients have deep vascular insufficiencies. I spoke to him about my concerns that my daughter was at risk for the same swelling my husband was experiencing. The doctor’s facial expression changed as he stated “your husband does not have ptosis.”

I have a strong desire to educate others and myself on lymphedema and rare diseases. I confirmed he did but it was not documented formally in his medical history. In the fall of 2010, my husband’s vascular testing showed insufficiencies, while the lymphoscintography test showed normal. Later, my husband was referred to a local geneticist, and in May 2011 blood tests returned from a lab in the United States confirmed that my husband tested positive for a mutation in the FOXC2 gene causative for lymphedema distichiasis syndrome. Currently there are 50 documented mutations in this gene in Wi n t e r 2 0 1 4

Photo: Nate & Nicole Photography

Our personal journey:

the scientific literature and his particular variant had never been recorded. When I received that phone call my heart broke into a million pieces. I was devastated but also relieved that we finally had an answer. I knew that my entire family was affected and I was not sure how to process this news and how I would cope. My daughter had a consultation visit with her plastic surgeon at Sick Kids that same month, followed by a ptosis repair surgery in June 2011 in Toronto. The surgery failed and we were devastated to know that we would have to bring her back. In July 2011, my husband and I flew to Toronto to attend an International Lymphedema Conference to see what exactly we are facing and make contacts with international specialists. We found the trip very upsetting but at the same time useful and educational. When we returned in July, we discovered that both my children aged 4 and 2 had inherited the exact gene mutation found in their father and are at high risk for developing

lymphedema. It seemed that my world had turned upside down. In October 2011, we drove our daughter back to Sick Kids Hospital for a second attempt at repairing the ptosis in her left eye. The surgery went well. The eyelid healed nicely, however, the results dropped slightly over the coming weeks. On September 17, 2013, our daughter had a Levator Resection Surgery at SickKids to repair her ptosis, and it seems thus far that the third time is a success! In summary, 2011 was the most stressful year of our lives—my entire family was diagnosed with a rare lymphatic disorder and my husband with an arteriovenous malformation on his forehead outside the brain. This was investigated further in 2011 and though never documented with this disorder was thought to be related somehow to his vascular insufficiency. I could not comprehend how this could happen with no family history of disease and the probability of all these things happening to us. Today we take one day at a time. I have a

strong desire to educate others and myself on lymphedema and rare diseases. I believe the correct diagnosis is extremely important for your family and your generations to come. I urge more lymphedema specialists, physicians and researchers to read about genetic diseases that include lymphedema so other patients are not left to suffer physically and mentally. Currently there are at least 45 known syndromes that include lymphedema. Let’s educate our fellow Canadians! LP

Academy of Lymphatic Studies Courses in Manual Lymph Drainage (MLD) and Complete Decongestive Therapy (CDT) Lymphedema Management Seminar (31 hours)* $895 This 4-day course serves as an introduction to the management of uncomplicated lymphedema affecting the upper and lower extremities, using Manual Lymph Drainage (Vodder Technique) and Complete Decongestive Therapy.

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Certification in Manual Lymph Drainage (40 hours) – NEw! $1150 This 5-day course will certify therapists in the MLD Techniques developed by Emil Vodder, Ph.D. and will enable you to create treatment sequences for the management of edema including post-operative/post-traumatic edema and pathologies such as fibromyalgia, migraines, chronic fatigue and more. CEU’s are available for all courses.

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Wi n t e r 2 0 1 3 4

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L y m p h e d e m a p a t h w a y s . c a 13

Clinical Perspectives

Exercise

and Lymphedema By Dorit Tidhar and DeCourcy Squire

eople need to exercise. Exercise is beneficial for overall health and well-being, and for many illnesses. Exercise reduces sugar levels in people with diabetes, improves cardiac function in people who suffer from heart disease, and reduces the risk for cancer and its recurrence. Yet many people with lymphedema are afraid of exercise, perhaps

Decongestive exercises are an integral part of lymphedema treatment.

because for years they were told to avoid lifting heavy objects and over-exertion (which may be valid post-op precautions, but not life-time restrictions), or because they found that exercise exacerbated lymphedema. In this article, we discuss the role of exercise in the treatment for lymphedema—exercise for lymphedema— and guidelines for a safe return to more vigorous activity—exercise with lymphedema.

Exercise for lymphedema Decongestive exercises are an integral part of lymphedema treatment. Muscle contractions improve lymph flow in the muscles, and walking or moving the arms improves lymph flow in the lymphatic vessels1,2. However in lymphedema, lymph flow will not necessarily drain centrally as it should but may instead be pushed to low resistance areas such as the skin (dermal backflow) making swelling worse3. Therefore, individual tailoring of an exercise program is needed. Casley-Smith demonstrated an improvement in lower and upper extremity lymphedema when a sequence of decongestive exercises that started with the neck and trunk and progressed to the limbs, combined with deep breathing and some self-MLD, was done

Dorit Tidhar PT was certified by Prof. Judith Casley-Smith as a teacher in Self Management and Exercise for Lymphedema in 2003. Dorit designed an Aqua Lymphatic Therapy program to help people who suffer from lymphedema maintain and improve the results of conventional treatment and conducted a randomized controlled trial as part of her Master’s degree in Tel Aviv University in Israel to examine this method. Currently, Dorit is working in Maccabi health care services as a clinician and the national director of the lymphatic therapy services. DeCourcy Squire PT, CLT-LANA, ALT, CI-CS has been treating lymphedema for 20 years and has been an instructor in the Casley-Smith method of Complex Lymphatic Therapy since 1999. She currently works in the Lymphedema Specialty Treatment Service at Hennepin County Medical Centre, Minneapolis.

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for a year4. Boris et al. found this sequence also led to continued reductions after a course of intensive treatment5. Katz-Leurer et al. found that contracting muscles near the trunk was more beneficial in reducing volume than contracting muscles near the hands and feet6. In these studies, people performed the exercises wearing bandages or garments. Wearing compression during exercise helped prevent fluid accumulating under the skin by moving it up the limb and assisted in the reduction of the limb volume7. In studies of women with breast cancer related lymphedema (BCRL), simple exercises with deep breathing and relaxation performed for short periods (10 and 15 minutes) and Aqua Lymphatic Therapy (ALT) led to reduced volume and improved symptoms8,9,10. In ALT, the hydrostatic pressure provides a gradient compression that increases with the depth of the water. When the goal is to improve or maintain control of lymphedema: • Gentle slow rhythmic shoulder exercises with deep breathing (for arms) • Aqua Lymphatic Therapy (for arms or legs) • Remedial/decongestive exercises (such as the Casley/Smith sequence) (for arms or legs) These have all been found to improve lymphedema and should be performed as part of lymphedema therapy in the intensive, transitional or long-term phase. Wi n t e r 2 0 1 4

Exercise with lymphedema What about other exercises such as working out, doing aerobic exercises, doing weight lifting? Recent research has shown that resistance exercises, when performed in a gradual, supervised manner, do not increase the risk of developing lymphedema. (11) Women with BCRL and women at risk for BCRL have participated in a variety of general exercise studies that included swimming and general water exercises, a combination of aerobic, water, and resistance exercises, and resistance exercises12,13,14. In general, BCRL was not induced or exacerbated by these programs. All the programs were monitored, supervised and performed in a gradual manner. The number of women who developed lymphedema was no greater in the exercise group than in the group who did not exercise. Schmitz et al. developed guidelines to help therapists and patients decide whether and when to start a resistance exercise program14. Lymphedema should be “stable” and is defined as “stable” when, for the past 3 months, the following conditions are met: • no increase in volume more than 10%; • no infections of the limb; • no functional deterioration; and • no necessity for lymphedema treatment. Although not all types of exercise programs were studied, and not for all areas of the body or types of lymphedema, using the “stable lymphedema” definition can serve as a guide for women who have BCRL to decide when they can safely start a resistance exercise program.

Schmitz recommends wearing a compression sleeve14. For resistance/weightlifting, Schmitz also advised the following guidelines: • Start low! Use 1–3 pounds • Do two to four sessions before progressing to a higher level. • If a week or two is missed, start back at a lower level and build back up. • If a month or more is missed, start back at the beginning.

Aerobic exercise McNeely et al. found that after 20 minutes of aerobic exercise with an ergometer (arm “bicycle”), subjects experienced an immediate increase in volume of both arms that subsided after an hour16. A pilot study found that pole walking for an hour (while wearing compression garments) did not cause exacerbation of the affected arm18. continued on page 16

Resistance exercises such as weight-lifting A recent systematic review concluded that for the long term, resistance exercises are safe for BCRL15. However, there is an immediate normal response that McNeely et al. and Johansson noticed: an increase in the volume of both the healthy and the affected arm that subsided after 24 hours 16,17. Wearing a compression garment did not prevent the temporary increase. Some women perceived greater exertion when wearing a compression garment versus not wearing the garment. Wi n t e r 2 0 1 4

L y m p h e d e m a p a t h w a y s . c a 15

never develop lymphedema, more research with larger numbers of subjects would be helpful to determine if wearing a compression sleeve during vigorous exercise does perform a preventive function for the people at risk. Self-monitoring Anyone with lymphedema or at risk who notices symptoms of lymphatic overload when exercising should start their next exercise session at a lower level and be sure to wear a compression garment. The symptoms of overload are feelings of heaviness, achiness, fatigue and warmth in the affected limb; the skin may feel taut and clothing may feel tighter. A person who experiences these symptoms, whether while exercising or doing another activity, should take these immediate steps: •S top that activity. •R est with the affected limb supported in elevation above the level of the heart. • Take deep slow breaths. • Wait for the symptoms to subside.

Safe exercise for the benefit of the lymphatic system and exercise that avoids aggravation of lymphedema are the goals of therapists and people with lymphedema.

Wearing compression While it is generally agreed that someone with lymphedema should be wearing compression, the data are not conclusive on the question of whether someone who is only at risk for BCRL should use a compression garment while exercising. Many lymphedema therapists advise a compression sleeve, not only for people with BCRL already but for those who are at risk. It is somewhat like wearing a seatbelt: it is a precaution. However since the majority of breast cancer survivors 16 L y m p h e d e m a p a t h w a y s . c a

As well as being aware of subjective symptoms, objective signs are important. A useful tool is the tape measure. Self-measurement by people with BCRL was tested in a pilot study and found to be accurate and reliable 19,20. We recommend that people with lymphedema learn how to measure their limbs. When starting a new sport or activity, measurements should be taken before, after the exercise session and 24 hours later. This is also useful to do when changing the exercise program (increasing the load, adding more sets of exercises, etc.) To be accurate, measurements need to be taken in the same place and in the same way each time.

same level and may consider increasing the effort. • An increase in circumference immediately after a session that subsides after 24 hours: this is a normal response; it is safe to continue at the same level. • An increase in circumference immediately after a session that does not disappear after 24 hours: the effort was excessive; need to reduce exertion, and self-manage lymphedema or seek treatment. If a compression garment was not being worn, consider doing so in the future. Caution: Most of the research on exercise has been on BCRL. Lower extremities respond differently from upper extremities due to gravity’s influence on the circulatory system. Consequently, not all the knowledge of the influence of exercise on BCRL transfers to the lower extremity. People with leg lymphedema should consult their lymphedema therapist for individualized advice before they start an exercise program in order to get guidance and to learn self-measuring. Exercise has many positive health-related outcomes. Safe exercise for the benefit of the lymphatic system and exercise that avoids aggravation of lymphedema are the goals of therapists and people with lymphedema. In summary, when starting a program of vigorous activity if you have lymphedema 1. B e sure your lymphedema is stable. 2. S tart gradually in a supervised situation. 3. W ear compression. 4. P ay attention to symptoms of overload. 5. M easure and monitor your limb.

The measurement outcome can be used to guide decision-making about when it is safe to progress: •N o change in circumference immediately after a session: safe to continue at the

6. Don’t let fear limit you---proceed with caution—but PROCEED! LP

Full references are available for review at www.pathways.ca Wi n t e r 2 0 1 4

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Research News

Research Funding and Priorities

Moving lymphedema understanding forward A turning point in Canadian lymphatic research An exciting announcement was made September 24, 2013 of a $5 million gift from the Dianne & Irving Kipnes Foundation to fund a comprehensive, sustainable Lymphedema Research and Education Program at the University of Calgary Snyder Institute for Chronic Diseases. This philanthropic investment will launch a world-class lymphedema program enabling the construction of a Lymphatic Imaging Suite to research the physiology of the lymphatic system including disorders such as lymphedema. This gift will help us understand how the lymphatic system works and what goes wrong when it does not, as is the case in lymphedema.

“This is a gift to every person in our country who wakes up each morning with swollen limbs, who is unable to work, who suffers chronic pain, weight gain, depression or who is unable to walk or lift.”

Dr. Pierre-Yves von der Weid, Associate Professor in the Department of Physiology and Pharmacology at the University of Calgary, suggests, “The first step to improving treatments and outcomes for patients suffering from lymphedema is to better understand the complex biology of the lymphatic system. In that regard, research is pivotal. As we expand our knowledge we can develop new diagnostic and therapeutic approaches.” In summarizing this donation, Dianne Kipnes, herself a lymphedema patient, states: “This is a gift to every person in our country who wakes up each morning with swollen limbs, who is unable to work, who suffers 18 L y m p h e d e m a p a t h w a y s . c a

chronic pain, weight gain, depression or who is unable to walk or lift or is in any way unable to go about their daily life, and who cannot access reasonable care because of this condition.” Source: Ian Soles, Salutaris Centre for Lymphatic and Massage Therapy, Edmonton, Alberta

An international epidemiological study LIMPRINT is an international research study that will provide an opportunity to not only gather data that will benefit individual services and the countries in which they are located, but will also enable an international perspective by using a single central database.

What is the background to the study? An important part of the International Lymphoedema Framework strategy is to support countries to develop data that can establish the size of the problem, provide the basis for evidence-based practice and be used to support reimbursement.

What does LIMPRINT stand for? The acronym stands for Lymphoedema IMpact and PRevalence – INTernational Lymphoedema Framework and is representative of the aim of the study, which is to determine the impact and prevalence of lymphedema/chronic edema at a national and international level.

What are the aims of LIMPRINT? The primary aim is to develop and review a toolkit for use by national lymphedema frameworks and partner organizations to assess the number of patients with lymphedema/ chronic edema and its impact on individuals and health services.

Dianne & Irving Kipnes. Secondary aims include reviewing the data collection for case ascertainment and classification of chronic edema, develop an electronic case reporting system, piloting it in different health care settings and the development of educational and support tools required for its effective use. What epidemiology tools will be used? The principal focus of the first phase of the study is to develop electronic tools for the assessment and classification of lymphedema/chronic edema. The tools will be modular in design. n The

Core Tool must be completed and will be a case ascertainment questionnaire that does not require contact with the patient and can be completed by generalist practitioners.

n The

Module Tools will additionally assess the impact of lymphedema/chronic edema on patients and will require completion through contact with the patient. With the exception of the Demographic and Disability module, which must be completed, the continued on page 20 Wi n t e r 2 0 1 4

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module tools can be used at the discretion of the framework lead/organisation depending on the needs and priorities of the framework/organisation. The Module Tools cover a range of important areas including: Demographics and Disability, Quality of Life, Details of Swelling, Wounds and Cancer. Phase 1 Phase 1 of the project has five preparatory stages that started in July 2013 and will end in June 2014. They include the construction of the online database using Clindex, an assessment of the case studies by an expert panel, a review of the Core Tool and Module Tools that will be used to collect data and an assessment of the experience of using Clindex. Phase 2 Phase 2 represents the implementation and rollout of an international research study across national frameworks and participating organisations. It is anticipated that ethical

review and approval will be required by frameworks/organisations/sites for this phase of LIMPRINT. Work is under way to secure funding for the implementation of Phase 2. This funding will be used to support frameworks as well as ensure the necessary international project coordination. The research phase of LIMPRINT will take place from June 2014 and will be launched at the ILF conference in Glasgow, UK. Source: Phil Morgan, LIMPRINT Project Manager, International Lymphoedema Framework

International collaboration for lymphedema research In August 2013, a group of internationally recognized lymphedema researchers, clinicians and stakeholders met in Ottawa to plan an interdisciplinary program of qualitative research on lymphedema. Invited by Canada Research Chair and CLF Research Working Group Co-Chair, Roanne Thomas, the group met to identify and generate priorities and potential

projects, and to create and develop teams to work together globally in lymphedema research. Noted experts Christine Moffatt, UK and Mei Fu, USA attended, along with Kun Li, China, and Anna Kennedy, representing the CLF Executive Committee and Pamela Hodgson representing Anna Towers, one of Canada’s leading lymphedema experts. Other participants included health professionals and postdoctoral research fellows. Key discussions centred upon increasing international awareness of lymphedema, showcasing qualitative research through patient stories, understanding global incidence rates of chronic edema, and producing meaningful patient focussed outcomes. Several International collaborations were discussed and one new project has since been initiated. The group will meet again in June 2014 at the International Lymphedema Framework Conference in Glasgow, Scotland. LP

Source: Liz Piquot, University of Ottawa

Canadian Lymphedema Framework Initiatives The strength of the Canadian Lymphedema Framework (CLF) is its volunteers, extremely committed individuals who are passionate and devoted to the CLF mission, donating their time and own funding for annual face-to-face meetings and regular teleconferences. The current priority of the Education Working Group (EWG) is patient education, and work is well underway by a subcommittee for a standardized national patient educational pamphlet. This group is also undertaking educational initiatives for healthcare providers to ensure quality, evidence-based and consistent care. Examples of this are the establishment of the speakers/writers bureau, material for the CLF new website, and the nursing module. Their recent contribution is the CLF’s first Consensus Statement— Canadian guidelines with respect to training of therapists in lymphedema management using Complete Decongestive Therapy (CDT). The Research Working Group (RWG) is pleased with the positive response to the research element they incorporated into the recent lymphedema conference. 24 abstracts were presented in either oral or poster formats. The Research Snapshots (I and II) were two of the most popular sessions. The group recommends adding research workshops next year, to encourage more students and new researchers to participate. In addition, this group worked on applications to the CIHR Planning Grant competition and created a Research Advisory Network – to join the RWG in developing a research agenda for lymphedema in Canada.

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The Partnership Working Group (PWG) prioritized their efforts by launching and building industry support for the Pathways magazine. This educational tool supports the mission of the The CLF Executive Committee (left to right): Pamela Hodgson, Dr. David Keast, CLF, and has become Anna Kennedy, Dr. Anna Towers and a sustainable revenue Rachel Pritzker. source. This group also was responsible for offering a Patient Advocacy workshop at the fall conference and organizing bursaries and sponsorships to allow representation from all lymphedema community organizations and affiliates across Canada. The PWG’s next initiative is the creation of the new CLF website, to be launched in the spring of 2014. The CLF Executive thanks everyone across the country who has contributed to the organization’s many initiatives and successes since inception (2009). We are proud of this message from Christine Moffatt (Chair of the International Lympheodema Framework): “Canada has set an important example of how a new lymphedema framework can work with existing lymphedema patient and clinical organizations to unite in one voice for furthering lymphedema care in Canada”. LP

Wi n t e r 2 0 1 4

Ask the Expert

To Pump or Not to Pump By Guenter Klose Pneumatic Compression Devices (PCDs), also known as “pumps,” have been utilized in the treatment of lymphedema since the early 1950s. The initial devices used single-chamber pressure cuffs that applied a uniform level of compression to the entire limb. “New generation” PCDs use improved technology and are equipped with multiple chambers that provide calibrated, gradientsequential inflation. In the early 1990s, PCDs fell out of favor with practitioners after Complete Decongestive Therapy (CDT) was established as the standard of care for the treatment of lymphedema. Adverse effects were cited among some patients who used the device at unreasonably high pressure, and some people were using PCDs without medical supervision. However, the benefits of a properly used modern-day PCD device may make it a valuable component of a home-care regime.

I have Stage 2 lower extremity primary lymphedema. Should I use a pump to control my lymphedema at home?

Initially, it is best to have your lymphedema condition assessed and treated by a Certified Lymphedema Therapist (CLT). The number of treatment visits required will depend on the severity of your lymphedema. Once a maximum level of reduction is achieved, it may be beneficial to obtain a PCD to assist with your in-home maintenance. This device will not eliminate the need for daytime compression garments, nightly bandaging or any other items of self-care your therapist recommends.

Nevertheless, when used in conjunction with other daily self-care measures and weight loss (if indicated), a pump may lead to further limb volume reduction, improved range of motion, a decrease in subjective complaints such as heaviness and achiness, and ultimately, an improved quality of life.

What advice do you give to patients with lymphedema who are interested in purchasing a pump?

Always consult with your Primary Care Physician (PCP) and/or CLT for advice on whether a PCD is right for you. Most patients with lymphedema will benefit from a PCD that is equipped with multiple chambers and provides calibrated, gradient-sequential inflation. Since lymphedema often affects the adjacent trunk quadrant (chest and back in upper extremity lymphedema and buttock and groin region in lower extremity lymphedema) it is preferable to choose a PCD that includes a garment that also treats these core body areas. If the patient’s lymphedema only involves the distal areas of the limb—and there is no reason to believe that proximal areas will become congested—a PCD that only covers the extremity may be sufficient.

What is the optimal pressure setting and what are the potential side effects of using a pump?

Because every patient’s lymphedema and response to treatment is unique, there is no universal pressure setting that works for all. Also, there are no established guidelines for the optimal pressure value.

Guenter Klose, MLD/CDT Certified Instructor, CLT-LANA is the Founder of Klose Training and Consulting LLC, located in Lafayette, Colorado. He received initial Complete Decongestive Therapy training in 1984 at the Foeldi Clinic in Germany. Guenter has lectured extensively in the United States and abroad.

22 L y m p h e d e m a p a t h w a y s . c a

A recent systematic literature review published in the Journal of Lymphology (2012) stated: “A peak inflation pressure of 25–50 mmHg might be sufficient for most patients in the absence of significant fibrosis.” As a Certified Lymphedema Therapist and Instructor, I usually recommend approximately 40 mmHg pressure for patients with upper extremity lymphedema and 50 mmHg for patients with lower extremity lymphedema, with an upper limit of 50 mmHg and 60 mmHg, respectively. To ensure safe and effective treatment, patients using a pump should be advised to discontinue use of their PCD and contact their PCP or CLT if any of the following is observed: a new occurrence or increase in proximal swelling, swelling of the genital or breast regions, discomfort and pain in the affected limb or redness and increased swelling with or without fever. LP

References Lymphology. 2012 Mar; 45(1):13-25. Intermittent pneumatic compression therapy: a systematic review. Feldman JL, Stout NL. Wanchai A, Stewart BR, Cormier JN, Armer JM. Glossary Distal: area of the body that is remote from the trunk (hand and forearm of the upper extremity, and foot and lower leg of the lower extremity). Proximal: an area of the body close to the trunk including the adjacent trunk quadrant. Fibrosis: medical term to describe build up of connective tissue and hardening of the skin within the affected extremity. Wi n t e r 2 0 1 4

Letters...

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FITTER’S AID® APP

INTRODUCING A NEW AND REVOLUTIONARY WAY TO FIT COMPRESSION GARMENTS

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The Fitter’s Aid is a super-easy, super-fast fitting app for the iPod Touch, iPhone and iPad. With the Fitter’s Aid it is easy for professionals to get a LympheDIVAs sizing recommendation for a patient that wears off-the-shelf garments and is in-between sizes. Simply indicate your patient’s compression class, measurements and indicate whether they wear a hand compression garment and let the Fitter’s Aid do all the work. The Fitter’s Aid will provide you with the suggested sizing recommendation and all the other viable compression combinations to get your patient the compression they need to manage their lymphedema in style.

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Helpful Suggestions

Hints and Tips Pillow support Anyone who travels knows that there is always a little bit of trepidation when travelling, and traveling with lymphedema just adds to that stress. Here are a few things that I do to alleviate my stress. I always bring a small pillow to support my arm, as the airlines no longer automatically have pillows for you to use. I have left arm lymphedema so I always try to sit on the left side of the plane so there is no interference from other passengers. I wear a loose fitting top so I don’t constrict my arm and something warm to keep my arm warm during the take-off and landing. I usually wrap my arm a little

looser just before getting on the plane to accommodate the swelling that may happen during flights. Michelle Kinakin, British Columbia

A boost for feet and legs For over a year now I have been using the Circulation Booster on my feet and my legs. I have had some success with it, and in two or three sittings the swelling in my feet and legs has come down quite a bit. I don’t know if this would work for everyone but I thought I would pass it along anyway. Beckie Hutchinson, British Columbia

Garment aids Having trouble getting your compression garments on? There are many donning aids available. Check the fall 2012 issue of Pathways for a description of the various tools that can help you. Send us your hints and tips Why not make it easier for those living with lymphedema by sharing practical day-today living tips with us. We invite patient, caregivers and health professionals to send in your suggestions to canadalymph@live.ca and we will include your name and city. LP

Victoria Vancouver Winnipeg Toronto Montreal Chicoutimi Fredericton Halifax

Train in Canada

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Medical Updates

Did You Know? Preventing lymphedema “Separating fact from fiction” is a presentation from Dr. Mehrara, hosted by the Lymphatic Research Foundation. You can view the video presentation on YouTube.

lymphatic draining which could potentially be useful for lymphedema therapy.

This innovative methodology could lead to new technology to test and treat lymphatic disease.

Source: ETH Zurich

Source: Georgia Tech

An engineering approach to lymphedema

New function for a messenger molecule

Researchers from ETH Zurich have discovered that the molecule interleukin-7 (IL-7) has another important function; it enhances the drainage function of lymphatic vessels, which collect lymph fluid. In the future, this finding could become useful for lymphedema patients as researchers have discovered the molecule and mechanism for enhancing

canadacares.org

Thank you ...to everyone who told us about amazing caregivers from coast to coast, to our judges and community partners, to our sponsors and most of all, to our Canada Cares Caregiver Award winners for all that you do each and every day. Amy Furey

NATIONAL FAMILY WINNER Central Region

NATIONAL PROFESSIONAL WINNER Atlantic Region

Source: Journal of Clinical Oncology. Published September 16, 2013

canadacares.org

Thank you ...and congratulations to all of our Canada Cares Caregiver Award winners for all that you do each and every day. Bruce Ireland NATIONAL FAMILY WINNER

Amy Furey NATIONAL PROFESSIONAL WINNER

Central Region

Atlantic Region Get help . E

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A trial that randomized Canadian women with early lymphedema to receive either complex decongestive therapy (CDT) or compression garments was unable to show that CDT is better than the more conservative, less expensive approach. However, other researchers have noted that some of the limitations of the study were that CDT was tested as a first-line treatment and was not tested in women with advanced disease who have longer-standing, more extensive or progressive lymphedema.

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THE VITALITY OF THE SEA

The National Institutes of Health (USA) has awarded Georgia Tech a $2 million research grant to use an engineering approach to studying lymphedema. Solving the biological problem with engineering is an ideal strategy, states J. Brandon Dixon, a Georgia Tech assistant professor, because the lymphatic system is an engineered system— a very complicated network of pumps.

Source: Lymphatic Research Foundation

Compression sleeves first for early lymphedema

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To find out more about our remarkable winners

Hugh Finlay ONE WISH AWARD WINNER

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To find out more about our remarkable winners

visit canadacares.org

Canada Cares is a not-for-profit that supports, celebrates and recognizes family and professional caregivers.

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visit canadacares.org

Canada Cares is a not-for-profit that supports, celebrates and recognizes family and professional caregivers.

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L y m p h e d e m a p a t h w a y s . c a 25 canadacares.org

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You Are Not Alone

Education

You have access to information, resources, contacts and support from people who know what you’re going through from

their own personal experience. You have the following organizations on your side.

Canadian and International Events Alberta: Lymphedema Association of Alberta

www.albertalymphedema.

April 3-5, 2014 10th Australasian Lymphology Association Conference British Columbia: BC Lymphedema Association www.bclymph.org Auckland, New Zealand Koru: Life, Growth, Movement will be held in Auckland, providing workshops and interactive panel debates. Manitoba: Lymphedema Association of Manitoba www.lymphmanitoba.ca n www.alaconference.com.au

Ontario: Lymphedema Association of Ontario

www.lymphontario.ca

Saskatchewan: Lymphedema Association of Saskatchewan

www.sasklymph.ca

June 5-7, 2014 International Lymphedema Framework Conference Glasgow, Scotland Reduce Incidence, Manage Prevalence: A Realistic of Goal? The ILF is proud to hold its 5th international Quebec: Lymphedema Association Quebec www.infolympho.org conference at the University of Glasgow. n www.lympho.org September 3-7, 2014 11th National Lymphedema Network International Conference Washington, DC The Campaign for Lymphedema Care: Perspectives, Evidence & Practices. Registration begins October 15th, 2013. Conference will be held at Washington Marriott Wardman Park. n www.lymphnet.org/2014conf/2014conf September 19-20, 2014 Lymphedema Association of Quebec Conference. Quebec City, Quebec This event will be targeting patients, health professionals, government and other stakeholders. n www.infolympho.ca

You Are Not Alone

You have access to information, resources, contacts and support from people who know what you’re going through from their own personal experience. You have the following organizations on your side.

Alberta: Alberta Lymphedema Association

www.albertalymphedema.com

British Columbia: BC Lymphedema Association

www.bclymph.org

Manitoba: Lymphedema Association of Manitoba

www.lymphmanitoba.ca

Ontario: Lymphedema Association of Ontario

www.lymphontario.ca

Quebec: Lymphedema Association of Quebec

www.infolympho.ca

Saskatchewan: Lymphedema Association of Saskatchewan Inc.

www.sasklymph.ca

The Canadian Lymphedema Framework (CLF) wishes to thank key industry partners for their financial support in making this magazine a reality. Platinum Sponsorship

Silver Sponsorship

About the CLF The Canadian Lymphedema Framework is an academic and patient collaboration, working to improve lymphedema management for lymphedema and related disorders in Canada.

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The CLF has affiliate agreements with its provincial partners for distribution of Pathways including customized inserts. Alberta Lymphedema Association Atlantic Clinical Lymphedema Network BC Lymphedema Association Lymphedema Association of Manitoba Lymphedema Association of Ontario Lymphedema Association of Quebec Lymphedema Association of Saskatchewan Charitable Registration 85160 1260 RR0001

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Compression

Club

Conversations in Changing Practice & Improving Patient Outcomes go.3m.com/CompressionClub Join the 3M Compression Club

As health care providers, you play an important role in improving the quality of life of your lymphedema & chronic edema patients, especially when it comes to compression bandaging. We invite you to visit our online compression club to network, ask questions, and learn from one another. Share tips & tricks, connect with colleagues, or ask the experts all your compression-related questions. • discuss & share your own experience treating patients with lymphedema • learn from colleagues and industry experts • stay up-to-date on industry news and events

Learn More To learn more about 3M Coban 2 Layer Compression System Products, visit us at www.3m.ca/coban2layer, contact your 3M Critical & Chronic Care Solutions representative or call the 3M Health Care Customer helpline at 1 800 364-3577. These products can be ordered from your local distributor.

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