(Printer friendly downloadable PDF version to take with you to
your doctor/health care provider)
Essential Lymphedema Information
for All Health Care Providers: from a Physician
with Secondary Lymphedema
I am a physician who acquired secondary lymphedema
while being treated for breast cancer. Despite being
a medical school educator, board certified in family
medicine and involved in the care of many women with
breast cancer, I had essentially no knowledge of
lymphedema--there was one question on my boards, and
no continuing education covered it.
After I developed lymphedema, I acquired both a
personal and professional education on the subject.
Following my diagnosis, I worked in an oncology
practice
and saw
many patients who
were
compliant and examined by many providers and yet
their lymphedema
was
over-looked.
Lymphedema is not addressed in medical school
education, residency or continuing medical
education. I'd like to share the information I've
learned in an effort to provide a short primer on
the subject and general advice in managing your
patients who are at risk for lymphedema.
This information is for
all members of the health care team--if
you touch, talk to, run tests or treat patients
at risk for lymphedema, knowledge of the
condition will enhance the care and health of
your patients.
Judith Nudelman, MD, CLT***
Associate Professor of Family Medicine, Clinical
Alpert Medical School
Brown University
“Therapeutic nihilism (i.e. no treatment at
all) is deplorable, although common. The fact
that the average clinician is ill prepared to
recognize the early signs of lymphedema must be
remedied, because the sooner the treatment is
started, the less treatment is required to
prevent further progression.”
Cancer, Principles and Practice of Oncology, 7th
Edition, DeVita et al, p.1485
1) LEARN ABOUT IT: Education about
Lymphedema and the lymphatic system is lacking:
Dr. Stanley Rockson (Stanford) has shown that
most physicians have received 15 minutes or less
of education on the lymphatic system. Lymphedema
is not a thing of the past: it is common and
providers need to understand the disease and
management. This is a chronic, progressive
disease and early diagnosis and proper treatment
is essential. Lymphedema is a disease of
inflammation and derangement of the lymphatic
and connective tissues of the affected area—it
is NOT just a swollen arm. Dr. Rockson’s
lectures can be accessed for free on YouTube:
http://www.youtube.com/watch?v=XR-9duCfgF0
Or via Stanford:
http://healthlibrary.stanford.edu/videolibrary/index.html
- rockson09
Provider training can be obtained through the
major lymphedema training schools and the
National Lymphedema Network. Here is a
free review article in the Journal Cancer:
Lymphedema Primer: CA Cancer J Clin 2009;
59:8-24
Current Links for DOI: 10.3322/caac.20001
2) LYMPHEDEMA IS NOT A DISEASE OF THE PAST:
The true incidence of lymphedema is unknown as
there is no single validated diagnostic
standard, but Jane Armer, PhD who has followed
over 250 women for greater than 8 years has
found the prevalence varies from 43-94%
depending on the measurement method employed:
this includes women with “just” sentinel node
biopsies
http://www.ncbi.nlm.nih.gov/pubmed/21226414
Lymphology. 2010 Sep;43(3):118-27.
3) LEARN TO RECOGNIZE AND DIAGNOSE
LYMPHEDEMA: Do a physical exam to look
for lymphedema at every follow up visit, or when
there are concerns: Examine limbs, chest, breast
and axilla for subtle changes and swelling.
Symptoms often precede swelling—take pain,
aching or tightness in the at risk area
seriously. Look for pitting edema, skin texture
changes—the swollen area will be either tight or
boggy or on the breast it can be reddened
thickened skin, skin color changes as lymph is
light colored and obscures blood vessels, in
hands check for swelling of the dorsum or palm
and have the patient make a fist and check the
MCP joints between the hands for obscuring, have
the patient bend their elbow and observe the
delineation of the olecranon and tendons between
limbs. If patients have bilateral lymphedema or
are at risk bilaterally, the exam becomes much
more difficult, as there is no “control” side to
use for comparison.
On this site there is an excellent series of
photographs of a patient with controlled, but
significant lymphedema—utilize the images and
realize the presentation may be more subtle:
http://www.stepup-speakout.org/What_does_lymphedema_look_like.htm
There is no diagnostic standard for diagnosing
lymphedema: common methods that are used are
inter-arm circumference discrepancy: measure the
arms at several points and compare the
circumferences, every 4 cm is the standard
method of calculating limb volume. While the 2
cm difference has been clinically adopted as
“the gold standard” for diagnosis, it is a
clinical standard that began with one clinical
trial and became widely adopted. The
sensitivity and specificity of the “2 cm rule”
are unknown. So, while a 2 cm size
discrepancy—at any one point on the two
arms—strongly suggests lymphedema, due to
fibrosis and “patchy” distribution of disease,
patients can have lymphedema without a 2
cm inter-arm circumference discrepancy. A.W.
Stanton, PhD has published that the clinician
must be alert to early changes in the limb:
obscuring of bony or tendon landmarks, and that
a 2 cm discrepancy is not required to diagnose
lymphedema. Link to Stanton, et al, Diagnosing
Breast Cancer-Related Lymphoedema, full article
Http://britishjournaloflymphoedema.com/journal/0101_arm.pdf
Early lymphedema, stage 1 or 0, is spontaneously
reversible and the swelling is highly variable.
More sophisticated measures of lymphedema are
perometry—a laser method of assessing volume,
cylinder water volume displacement, and a less
sensitive and specific method of serial
bioimpedance values can be utilized to measure
and with history and exam, confirm the diagnosis
of lymphedema.
--
History is crucial in evaluating patients.
Take a history of swelling, pain, achiness,
and/or tightness in the entire quadrant “at
risk.” Validated surveys have indicated high
incidences of intermittent swelling in women who
have been treated for breast cancer. Yet even if
swelling resolves spontaneously, it is still
Stage 0 lymphedema.
---Be aware of breast and truncal lymphedema:
The incidence has increased with sentinel node
biopsy: Lymphology. 2006 Mar;39(1):33-40
“Radiotherapy after breast conservation surgery
leads to increased long-term changes in basal
lymph circulation and smaller increases in lymph
flow in the contralateral breast receiving 2-4
Gy and after surgery. If maximal lymph transport
capacity is unchanged, edema may be more likely
in this circumstance of reduced lymphatic
transport reserve.”
http://www.ncbi.nlm.nih.gov/pubmed/16724508.
This full length article is an excellent review
of the subject:
Journal of Lymphoedema,
2006: http://www.lymphoedema-uk.com/journal/0101_breasttrunk.pdf
4) DISEASE MANAGEMENT:
Know the sequela and co-morbities of lymphedema:
High risk of severe
cellulitis—patients
should have prescriptions on hand of antibiotics
that treat cellulitis so that they can start
them at the first sign of redness, pain,
swelling or fever—and then contact their
physician. Please provide antibiotics for
patients with lymphedema—this is not a case of
inappropriate antibiotic use.
Cellulitis is both a risk factor for lymphedema
and a poor prognostic indicator as cellulitis
irreversibly destroys lymphatics.
From NE Journal Review of Cellulitis: “A
distinctive form of cellulitis, sometimes
recurrent, may occur weeks to months after
breast surgery for cancer. Cellulitis in the
ipsilateral arm has been well described after
radical mastectomy where it occurs because of
associated lymphedema; cellulitis in the
ipsilateral breast is more common now, occurring
after breast-conservation therapy. Local
lymphedema from the combination of partial
mastectomy, axillary lymph-node dissection, and
breast irradiation is a predisposing factor.”
http://www.nejm.org/doi/full/10.1056/NEJMcp031807
This link is to an image of breast/arm
celluliltis:
http://www.nejm.org/doi/full/10.1056/NEJMicm065836
Pain:
Lymphedema is often quite painful and that pain
should be validated and managed. The pain is
multi-factorial: neuropathic, mechanical, and/or
inflammatory. The National Lymphedema Network
issued a statement confirming that the condition
can be painful,
http://www.lymphnet.org/pdfDocs/NLN_Washington_Post_Reply.pdf
Emotional response:
Patients find lymphedema emotionally
distressing: patients feel abandoned,
stigmatized and generally distressed. The
disease is disfiguring, disabling and requires
daily care. They often find that the condition
is under-recognized—to the point of being
denied, and that their providers are not
educated enough about the disease to manage it
or supervise their treatment by lymphedema
therapists. Lymphedema reduces the quality
of life for breast cancer survivors. Multiple
studies have documented this distress. Please
address this with your patients and support
them.
http://www.ncbi.nlm.nih.gov/pubmed/20797846
: 2010 Study: ”CONCLUSIONS: In this cohort of
women with breast cancer, women with lymphoedema
after surgery for breast cancer had
significantly worse overall emotional well-being
and adjustment to life compared to women without
lymphoedema.”
---Learn about lymphedema therapy and form
therapeutic alliances with well- trained
therapists:NLN
Position Papers | National Lymphedema Network.
Read the
NLN position papers
on therapy,
therapist training, and utilize the therapist
locator web page to find therapists. There is no
standard for lymphedema therapy training,
although the NLN recommends that a properly
trained therapist receive 135 hours of training
and supervision. Even with adequate training,
therapists require supervision and consultation
from referring physicians—therapists should
not manage this chronic disease without
consultation from health care providers.
The standard physical therapy model of acute
intervention and transition to home management
is not sufficient for this chronic disease:
ideally patients should have regular maintenance
evaluations and follow up and treatment as
needed. This disease is chronic and progressive;
patients require hands on periodic evaluation.
---Understand Lymphedema “Gear”:
patients
will require compression garments, which should
be replaced every 6 months, and a sleeve should
not be dispensed without hand protection—either
a gauntlet or glove,
http://lymphedivas.com/lymphedema/gauntletandsleeve/
Treatment involves lymphedema compression
wrapping, specialized nighttime garments,
daytime compression garments and at times,
pneumatic pumps. None of these treatments are
fully covered by insurance and often they will
require prescriptions from a treating physician.
Again, review the National Lymphedema Network
Position paper on treatment, and please provide
prescriptions for your patients.
There are only a handful of rehabilitation
physicians in the country who specialize in
lymphedema. Your patients need you to know about
the condition, to be informed to diagnose it, to
treat the complications of it, to help manage
the therapy and to follow them for this chronic
condition.
5) WORK TO MINIMIZE LYMPHEDEMA IN YOUR
PATIENTS:
Education, Advice, Anticipatory Guidance:
---Educate your patients about lymphedema:
Most patients are not given accurate
information, and most physicians do not consider
lymphedema counseling or advice to be their
responsibility. An article from Breast by
Electra Paskett PhD revealed: “Overall, women
knew little to nothing about lymphedema before
they developed it . . . Most physicians reported that
they did not routinely counsel women or provide
written information on lymphedema prevention to
their patients, and the extent to which women's
daily living was affected by the condition was
not always recognized.”
http://www.ncbi.nlm.nih.gov/pubmed/11348395
---
Pre-operative lymphedema therapy evaluation
and post-operative limb measurements for early
detection of lymphedema should be standard
practice: The National Lymphedema Network
issued a position paper in 2011, "Screening and
Measurement for Early Detection of Breast Cancer
Related Lymphedema", calling for pre-treatment
baseline measurements, consistent follow up
measurements, inquiring about arm heaviness,
achiness and/or swelling of the affected arm and
trunk at all follow up visits:
http://www.lymphnet.org/pdfDocs/nlnBCLE.pdf
---Close and careful surveillance of patients
with early intervention minimizes lymphedema:
http://www.ncbi.nlm.nih.gov/pubmed/18428212
Cancer. 2008 Jun 15;112(12):2809-19. In this
study, women’s arms were measured pre-op and at
every post op visit and if a 3% increase
occurred, they were placed in compression
garments, with excellent results; “CONCLUSIONS:
A short trial of compression garments
effectively treated subclinical LE.”
Currently Massachusetts General Hospital is
replicating this study:
http://www.clinicaltrials.gov/ct2/show/NCT00959985
---Radiation increases the risk of lymphedema:
Even when women are receiving whole breast
radiation without nodes intentionally included
in the field, radiation oncologists should work
to minimize radiation to the level one nodes in
node negative women. Andrea Cheville, MD (Mayo)
is pioneering a radiation technique where lymph
nodes that drain the arm are blocked to minimize
lymphedema.
http://www.abstracts2view.com/sabcs10/view.php?nu=SABCS10L_1220
http://www.ncbi.nlm.nih.gov/pubmed/20951451
(publication)
interview:
http://newsblog.mayoclinic.org/2010/12/06/lymphedema-study/
- 2
Cheville AL, Petersen IA, Brinkman DH, Ward SB,
Mullan BP, Durski J, O'Connor MK, Schomberg PJ,
Yan E, Garces YI, Laack NN. Mayo Clinic,
Rochester, MN
”Lymphedema (LE) is a common complication of
cancer treatment, and has been identified as the
number one issue by breast cancer survivors with
incidence as high as 70%. Given the long-term
survivorship of these women, LE is a widely
prevalent condition. Irradiation of axillary and
supraclavicular lymph nodes increases a
patient's risk of LE onset and progression by as
much as 2-fold.”
---Inform
all of your patients of the National Lymphedema
Network’s risk reduction behaviors-and all
office staff should be aware of them as well:
NLN Position Papers | National Lymphedema
Network
The knowledge reduces the risk of developing
lymphedema:
http://www.ncbi.nlm.nih.gov/pubmed/21186149
“CONCLUSIONS: Exposing women to lymphedema risk
information at the time of breast cancer
diagnosis facilitates increased awareness and
enactment of risk-minimization behaviors.” Oncol
Nurs Forum. 2011 Jan 1;38(1):E27-36.
---Learn which patients should wear
compression garments while flying: all
patients with lymphedema and those at high risk
are advised to wear a sleeve and hand garment
while flying. They will need a prescription from
you for garments. Read the position paper by the
NLN on flying:
“It is the position of the National Lymphedema Network
that: Individuals with a confirmed diagnosis of
lymphedema should wear some form of compression
therapy while traveling by air. Individuals at
risk for developing lymphedema should understand
the risk factors associated with air travel and
should make a decision to wear compression based
on their individual risk factors.”
http://www.lymphnet.org/pdfDocs/nlnairtravel.pdf
---There is a movement to “debunk” risk
reduction behaviors as not evidence-based,
yet the clinical experience to support these
behaviors are based in broad experience and if a
permanent disease occurs due to an avoidable,
iatrogenic behavior, the patient’s incidence of
lymphedema becomes 100%.
---For patients with bilateral lymphedema or
bilateral risk, learn how to take leg blood
pressures, foot blood draws and foot or neck
IV’s if necessary.
http://www.medscape.com/viewarticle/471829
While popliteal thigh pressures can be obtained,
many patients find posterior tibial ankle blood
pressures are more comfortable:
http://www.ehow.com/how_5729015_check-blood-pressure-ankle.html
http://www.ncbi.nlm.nih.gov/pubmed/8912031:
“Since the mean blood pressure readings obtained
at the arm and at the ankle were statistically
equivalent, we concluded that the ankle cuff
placement provided a reliable alternative to the
placement of the cuff on the arm.”
Concerns have been
raised over the safety of intravenous lines in
the lower extremity: they have been associated
with a higher risk of infection, should be
avoided when artificial joints are in the limb
and this author could find no study that
documented increased risk of deep vein
thrombosis. The higher risk of infection needs
to be weighed against the risk of exacerbation
or triggering of lymphedema with the use of an
IV in a limb with compromised lymphatic
function.
---Learn about exercise and lymphedema:
Exercise is a key component of lymphedema
therapy and all patients are encouraged to
exercise. Patients with lymphedema or at risk
for it, should not exercise their upper
extremities to the point of fatigue and need to
monitor their arms for swelling or discomfort
after exercise. There are far too many cases of
anecdotal reports of women developing lymphedema
in exercise programs designed for cancer
survivors, using exercise trainers who are
unaware of lymphedema precautions or even with
standard physical therapy. Resistive exercise,
despite recent studies, can pose a risk to women
both with lymphedema and at risk for it.
From the NLN position paper on exercise-http://www.lymphnet.org/pdfDocs/nlnexercise.pdf:
"Studies: Resistive exercise has been formally studied in
“at risk” individuals, and when begun at a
low level and increased gradually, has not
been found to trigger or worsen lymphedema.
An isolated study in lymphedema patients
suggests that resistive exercise, in the
absence of compression, may lead to
increased lymph accumulation and eventually
worsen limb swelling. Potential Benefits:
Resistive exercise may enhance lymph flow
and prevent limb swelling from muscle
overuse. Potential Risks: Strength training
increases local blood flow and metabolic
waste production. These effects increase the demand on the lymphatic system,
potentially triggering or worsening limb
swelling.”
In carefully selected patients, who participated
in a specific exercise protocol, (PAL protocol),
utilizing specially trained trainers and
frequent assessment by trained lymphedema
therapists, in this highly controlled setting,
weight-lifting was shown to do little or no harm
to patients with lymphedema and at risk
patients, Schmitz et al—but weight-lifting does
not cure nor definitively prevent lymphedema,
and needs to be done slowly and carefully.
Read the articles, not the press releases.
The initial article on weight lifting with the
PAL protocol for women with lymphedema is a free
article on pubmed:
http://www.nejm.org/doi/full/10.1056/NEJMoa0810118
The second article reviewed use of the protocol
with women “at risk” for lymphedema:
http://www.ncbi.nlm.nih.gov/pubmed/21148134
http://www.abstracts2view.com/sabcs10/view.php?nu=SABCS10L_9027
“Slowly progressive weight-lifting had no
significant impact on arm swelling among
breast cancer survivors with lymphedema, and
resulted in a reduced incidence of
lymphedema flares and improvement in
symptoms. In breast cancer survivors at risk
for lymphedema, a program of slowly
progressive weight-lifting, compared with no
intervention, did not result in increased
incidence of lymphedema. In secondary
analyses, women with 5+ nodes removed who
did weight-lifting were less likely to
experience increases in arm swelling.”
Unfortunately, on the official University of
Pennsylvania PAL site:
http://www.penncancer.org/physical-activity-and-lymphedema
This statement will be found: “Several key findings from
the study include:
50% reduction of the likelihood of lymphedema
worsening among women with lymphedema
70% reduction of the likelihood of arm swelling
increases among women who had 5 or more lymph
nodes removed”
Please know the facts and limitations of the
study. Weight lifting does not cure or prevent
lymphedema, and can precipitate it in some
women. Read the NLN guidelines and counsel your
patients with the facts.
Peri-operative and Post-operative Management:
--Handle the tissues gently during surgery:
Janice Cormier, M.D., NLN lecture 2010,
Physicians’ Intensive.
Lecture slide: Critical Surgical Technique 1)
Gentle handling of tissues, 2) Orientation of
incisions (extremities) and 3) Hemostatic
control at time of surgery
--Treat seromas promptly: from San
Antonio Breast Conference 2010, “Seroma usually
leads to prolonged wound healing, tissue
inflammation and subsequent fibrosis and
necrosis. It has been hypothesized that seroma
formation is one of the main risk factors for
post-breast cancer lymphedema.” Seroma
Formation Is Associated with Lymphedema-Related
Symptoms.
http://www.abstracts2view.com/sabcs10/view.php?nu=SABCS10L_478
---Consider limiting stretch on the axillary
area for 10-14 days post-operatively, as
lymphatics have limited time to regenerate: NLN
Conference Lecture, 2010, Jodi Winicour PT
From Foldi Textbook of Lymphology: Lymphatic
regeneration occurs as the stumps of the
afferent or efferent collectors of a removed
node connect as the result of proliferation of
the endothelium at the terminal portion of the
damaged vessel. Regeneration of superficial
vessels in dogs takes 4 days, and deep vessels
in 8 days.
Have your patients limit their arm movement to
shoulder height for the first 10-14 days
post-op to allow the efferent and afferent
vessels to connect during the limited time of
lymphatic regeneration.
Systematic review of early vs. delayed exercise
has shown delayed exercise decreases seroma
formation:
http://www.ncbi.nlm.nih.gov/pubmed/15830140
A study in 2008, published in Physiotherapy,
showed higher risk of development of lymphedema
in women who had axillary node dissection and
performed exercise early vs. delayed exercise:
http://www.lymphoedemaleeds.co.uk/Pages/Research.aspx
---Axillary Web Syndrome is a risk factor for
lymphedema: In a study of early PT to
minimize lymphedema, a subset of patients who
developed axillary web syndrome at 3-4 weeks
post-op had an almost universal development of
lymphedema, despite intervention
http://www.ncbi.nlm.nih.gov/pubmed/20068255
BMJ. 2010; 340: b5396. Published online 2010
January 12. “We also found that 12 of the 18
women who developed secondary lymphoedema had
axillary web syndrome during the second and
third week after surgery. The axillary web
syndrome is a known but poorly studied
complication of surgery. No study has shown any
link between the axillary web syndrome and the
onset of secondary lymphoedema. We and others
suggest that the axillary web syndrome may be a
sign of injury to the lymphatic system and it
could produce a lymphatic overload as a result
of failure of the lymphatic system. This
overload, together with other factors, could be
responsible for the onset of secondary
lymphoedema”
Learn about axillary web syndrome:
http://www.stepupspeakout.org/Cording_and_Axillary_Web_Syndrome.htm
---Lymphedema Myths and Falsehoods: Learn why
they’re incorrect
1)
You can’t get lymphedema from “just” a sentinel node
biopsy:
the risk of lymphedema after sentinel biopsy is
unknown, but is at least 10%.
The incidence of lymphedema when radiation is
performed along with sentinel node biopsy,
approaches 25%.
Lymphedema
risk is not just due to mechanical disruption--it's
a systemic disease and
genetic predisposition, inherent lymphatic pump
capacities,
and inflammation all
play
important roles in its development.
2)
None of MY patients has ever gotten lymphedema:
statistically impossible, and it only indicates
that you need to do more careful examinations
and take more detailed histories. Lymphedema
doesn’t equate with poor medical care, and
ignoring it will lead to progressive disease.
There are behaviors, described above, that can
lessen the chances of your patients developing
it, but even with optimal medical care, some
patients will develop lymphedema.
3)
Risk reduction behaviors are myths:
they have not been proven with randomized,
double blind placebo controlled trials, due to
ethical and funding issues, but clinical
experience has validated that they are
reasonable and should be taught to patients and
staff and adhered to as medical practice.
Patients remain at risk for life—studies of
axillary node dissection patients show that the
majority develop lymphedema in the first few
years after surgery, and then at least 1%/year
develop it, so that the incidence at 20 years of
follow up is 50%. The triggering event that
overwhelms lymphatic filtration capacity can be
minimal and should be avoided.
4)
Lymphedema is just cosmetic:
it’s a derangement of the immune system, causes
chronic inflammation and connective tissue
derangement. There is the risk of life
threatening cellulitis with lymphedema, and the
psychosocial issues are considerable. It is a
“real” disease, and deserves prompt recognition
and treatment by informed medical staff.
5)
Why bother diagnosing it, there’s nothing that
can be done to treat it:
Lymphedema therapy is effective and most
effective when started early.
In summary:
lymphedema is common—even with sentinel node
biopsy, and its true prevalence is unknown as
there
is no diagnostic standard, it is
under-recognized, under-treated, and health care
providers are under-educated about the disease.
Your patients need you to understand the reality
of their disease and help them manage it.
Lymphedema is a disease of abandonment
and misunderstanding. It is NOT just swelling;
it is derangement of the lymphatic system,
derangement of connective tissue, progressive
disability and impaired lymphatic functioning. It is a dreaded complication for very good
reasons.
An excellent handout for quick reference:
http://www.livestrong.org/Get-Help/Learn-About-Cancer/Cancer-Support-Topics/Physical-Effects-of-Cancer/Lymphedema
***The author of this page works in radiation
oncology, although trained in primary care. In
my practice, the risk of lymphedema is on the
consent form for breast irradiation. The
practice protocol is to measure all breast
cancer patients’ arms at every visit, and every
week I diagnose lymphedema in compliant patients
who see their breast surgeons, medical
oncologists and primary care doctors—and I read
the notes, which state “no edema”. All the
patients are offered treatment and they
overwhelmingly accept it. As one patient said,
“I didn’t know there was anything I could do.”
If you don’t look, you won’t find it, and
patients will suffer needlessly. And I don’t
just diagnose lymphedema in breast cancer
patients: patients with sarcoma, endometrial
cancer, melanoma, head and neck cancer, rectal
cancer and prostate cancer have presented with
it as well. Any time medical therapy disrupts
the lymphatic system; patients are at risk for
lymphedema.
Page Last Modified
09/29/2015
