LYMPHEDEMA: A BRIEF OVERVIEW
Edited by Dr. Brad Battaglia
WHAT IS LYMPHEDEMA?
WHAT CAUSES LYMPHEDEMA?
SYMPTOMS OF LYMPHEDEMA
LYMPHANGITIS
(INFECTION)
TREATMENTS FOR LYMPHEDEMA
CONTRAINDICATIONS
PREVENTION
PREVENTIVE STEPS FOR LOWER
EXTREMITIES
LYMPHEDEMA:
BIBLIOGRAPHY
Lymphedema occurs when “lymphatic fluid” builds up of in
certain tissues of the body. This
accumulation of fluid causes swelling, most often in the arm(s) and/or leg(s),
and occasionally in other parts of the body. As a general rule, Lymphedema that
develops when the lymphatic channels
(similar to veins) that carry the lymphatic fluid around the body are
damaged is called “Primary Lymphedema”.
Lymphedema that occurs when lymph vessels or lymph nodes are removed is
called “Secondary Lymphedema”.
In Lymphedema, an
abnormal amount of protein-rich fluid collects in the tissues of the affected
area. Left untreated, this stagnant, protein-rich fluid causes the tissue
channels to increase in size and number, reduces oxygen availability in the
transport system, interferes with wound healing, and provides a culture medium
for bacteria that can result in Lymphangitis (infection).
Lymphedema should not be confused with edema resulting from
venous insufficiency, which is not lymph-edema. However, untreated venous
insufficiency can progress into a combined venous/lymphatic disorder that is
treated in the same way as Lymphedema.
Primary Lymphedema, which can affect from one to as many as
four limbs and/or other parts of the body, can be present at birth, develop at
the onset of puberty (praecox) or in adulthood (tarda), all from unknown
causes, or associated with vascular anomalies such as Hemangioma, Lymphangioma,
Port Wine Stain, Klippel Trenaury.
Secondary Lymphedema, or acquired Lymphedema, can develop as
a result of surgery, radiation, infection or trauma. Surgical procedures that
usually require removal of lymph nodes put patients at risk of developing
secondary Lymphedema. These include:
·
Surgery for melanoma
·
Breast surgery
·
Gynecological surgery
·
Head and neck surgery
·
Prostate, Testicular and Bladder surgery
·
Colon cancer surgery
Secondary Lymphedema can develop immediately
post-operatively, or weeks, months, even years later. It can also develop when
chemotherapy is unwisely administered to the already affected area (the side on
which the surgery was performed) or after repeated aspirations of a seroma (a
pocket of fluid which occurs commonly post-operatively) in the axilla, around
the breast incision, or groin area. This often causes infection and,
subsequently, Lymphedema.
Aircraft flight has also been linked to the onset of
Lymphedema in patients who have had prior cancer surgery (likely due to the
decreased cabin pressure). Always be sure to wear a compression garment
(sleeve, stocking) when you fly, even if you do not have Lymphedema. (For more
information, see the "Aircraft Flight" reprint available through the
Educational Corner.
The medication Tamoxifen appears to be another cause of
lower extremity Lymphedema. This
medication can cause blood clots and subsequent DVT (deep venous thrombosis).
Radiation therapy, used in the treatment of various cancers
and some AIDS-related diseases (such as Kaposi-Sarcoma), can damage otherwise
healthy lymph nodes and vessels, causing scar tissue to form which interrupts
the normal flow of the lymphatic fluid. Radiation can also cause skin
dermatitis or a burn similar to sunburn. It is important to closely monitor the
radiated area for any skin changes, such as increased temperature,
discoloration (erythema) or blistering which can lead into the development of
Lymphedema. Be sure to keep the area soft with lotion recommended by your
radiation oncologist.
Lymphedema can develop secondary to Lymphangitis, an infection
that interrupts normal lymphatic pathway function. A severe traumatic injury in
which the lymphatic system is interrupted and/or damaged in any way may also
trigger the onset of Lymphedema. Although extremely rare in developed
countries, there is a form of Lymphedema called Filariasis which affects as
many as 200 million people worldwide (primarily in the endemic areas of
southeast Asia, India and Africa). When the filarial larvae from a mosquito
bite enters the lymphatic system, these larvae mature into adult worms in the
peripheral lymphatic channels, causing severe Lymphedema in the arms, legs and
genitalia (also known as Elephantiasis).
Lymphedema can develop in any part of the body or limb(s).
Signs or symptoms of Lymphedema to watch out for include: a full sensation in
the limb(s), skin feeling tight, decreased flexibility in the hand, wrist or
ankle, difficulty fitting into clothing in one specific area, or
ring/wristwatch/bracelet tightness. If you notice persistent swelling, it is
very important that you seek immediate medical advice (and get at least one
second opinion) as early diagnosis and treatment improves both the prognosis
and the condition.
Lymphedema develops in a number of stages, from mild to
severe (referred to as Stage 1, 2 and 3:
Stage 1 (spontaneously reversible):
Tissue is still at the "pitting" stage, which
means that when pressed by fingertips, the area indents and holds the indentation.
Usually, upon waking in the morning, the limb(s) or affected area is normal or
almost normal size.
Stage 2 (spontaneously
irreversible):
The tissue now has a spongy consistency and is
"non-pitting," meaning that when pressed by fingertips, the tissue
bounces back without any indentation forming). Fibrosis found in Stage 2
Lymphedema marks the beginning of the hardening of the limbs and increasing
size.
Stage 3 (lymphostatic
elephantiasis):
At this stage the swelling is irreversible and usually the
limb(s) is/are very large. The tissue is hard (fibrotic) and unresponsive; some
patients consider undergoing reconstructive surgery called
"debulking" at this stage.
When Lymphedema remains untreated, protein-rich fluid
continues to accumulate, leading to an increase of swelling and a hardening or
fibrosis of the tissue. In this state, the swollen limb(s) becomes a perfect
culture medium for bacteria and subsequent recurrent Lymphangitis (infections).
Moreover, untreated Lymphedema can lead into a decrease or loss of functioning
of the limb(s), skin breakdown, chronic infections and, sometimes, irreversible
complications. In the most severe cases, untreated Lymphedema can develop into
a rare form of lymphatic cancer called Lymphangio-sarcoma (most often in
secondary Lymphedema).
Signs and symptoms of Lymphangitis (infection) may include
some or all of the following: rash, red blotchy skin, itching of the affected
area, discoloration, increase of swelling and/or temperature of the skin, heavy
sensation in the limb (more so than usual), pain, and in many cases a sudden
onset of high fever and chills.
Treatment for infections: immediately discontinue ALL
current Lymphedema treatment modalities (including manual lymphatic drainage,
bandaging, pumps, wearing of compression garments) and contact your physician
as soon as possible. The antibiotics of choice for these types of lymphatic
infections are those in the penicillin family (note: people who develop side
effects, such as yeast infections or gastric upset can take Bicillin injections
for two weeks), if no allergies are present (for more information about
Bicillin, request article reprint "Efficacy of Benzathine Penicillin
Administration," $1.75, available through NLN). NOTE: Always carry
antibiotics or a prescription with you when you travel.
Planning the treatment program depends on the cause of the
Lymphedema. For example: If the initial signs and symptoms of swelling are
caused by infection (redness, rash, heat, blister or pain may indicate an
infection), antibiotics will first need to be prescribed. Treating an infection
often reduces some of the swelling and discoloration.
If the Lymphedema is not caused by infection: Depending on
the severity of the Lymphedema, the recommended treatment plan should be
determined using an approach based on the Complex Decongestive Therapy (CDT)
methods which consist of: a) manual lymphatic drainage; b) bandaging; c) proper
skin care & diet; d) compression garments (sleeves, stockings, devices such
as Reid Sleeve, CircAid leggings, Legacy Sleeve, as well as other alternative
approaches); e) remedial exercises; f) self-manual lymphatic drainage &
bandaging, if instruction is available; g) continue to follow prophylactic
methods at all times.
1. Post-cancer surgery Lymphedema patients who experience a
sudden marked increase of swelling should immediately cease treatment and be
checked by their physician for possible recurrent tumor or disease. Tumor
growth can block the lymphatic flow causing a worsening of the condition.
Although not yet proven in a controlled clinical study, many Lymphedema
specialists believe that patients with recurrent or metastatic disease should
not undergo Complete Decongestive Therapy (CDT) in order not to promote the
spreading of the cancer. Be sure to discuss this treatment with your doctor.
2. Patients with a sudden onset of Lymphangitis (infection)
should immediately discontinue treatment (see page 4) until the infection is
cleared. Patients with histories of vascular disease and who are taking
anticoagulants, should have a Doppler and ultra-sound to rule out deep-venous
thrombosis before being treated. During treatment, these patients should be
followed closely and regular laboratory tests should be performed (prothrombin
time).
3. Patients who have congestive heart failure must be
monitored closely to avoid moving too much fluid too quickly, for which the
heart may not be able to compensate.
4. If pain is present, discontinue all treatment until the
pain subsides or the underlying cause has been determined.
The information above is adapted from Lymphedema: An
Information Booklet, a classic NLN publication now in its sixth edition. This
16 page booklet addresses the lymphatic system, causes of Lymphedema, symptoms,
Lymphangitis (infection), Lymphedema prevention, treatments, contraindications,
diagnostic studies, special warnings and reimbursement issues. Booklets are
available through the NLN, $5.95 for NLN members, $6.95 for non-members, (if
ordering more than 12 copies, please call for bulk order information.)
Send orders to: NLN
Latham Square, 1611 Telegraph Avenue, Suite 1111
Oakland, CA 94612-2138
Tel: 510-208-3200
Fax: 510-208-3110
Infoline: 1-800-541-3259
(c) 1999 Saskia R.J. Thiadens, R.N. All rights reserved.
* 18 Steps to
Prevention for Upper Extremities
* 18 Steps to
Prevention for Lower Extremities
* Lymphedema
Awareness: Before, During And After Breast Cancer Surgery
* In Defense
of the 18 Steps to Prevention
* Tips for
Travel
------------------------------------------------------------------------
Eighteen Steps To Prevention - UPPER Extremeties
For the patient who is at risk of developing Lymphedema, and
for the patient who has developed Lymphedema.
WHO IS AT RISK?
At risk is anyone who has had either a simple mastectomy,
lumpectomy or modified radical mastectomy in combination with axillary node
dissection and/or radiation therapy. Lymphedema can occur immediately
postoperatively, within a few months, a couple of years, or 20 years or more
after cancer therapy. With proper education and care, Lymphedema can be
avoided, or, if it develops, kept well under control. (For information
regarding other causes of upper extremity Lymphedema, please refer to the
section: “What is Lymphedema?”)
The following instructions should be reviewed carefully
pre-operatively and discussed with your physician or therapist.
1. Absolutely
do not ignore any slight increase of swelling in the arm, hand, fingers, or
chest wall (consult with your doctor immediately).
2. Never allow
an injection or a blood drawing in the affected arm(s). Wear a LYMPHEDEMA ALERT
bracelet.
3. Have blood
pressure checked on the unaffected arm, or on the leg (thigh), if bilateral
Lymphedema/at-risk arms.
4. Keep the
edemic or at-risk arm(s) spotlessly clean. Use lotion (Eucerin, Lymphoderm,
Curel, whatever works best for you) after bathing. When drying it, be gentle,
but thorough. Make sure it is dry in any creases and between the fingers.
5. Avoid
vigorous, repetitive movements against resistance with the affected arm
(scrubbing, pushing, pulling).
6. Avoid heavy
lifting with the affected arm. Never carry heavy handbags or bags with over-the-shoulder
straps on your affected side.
7. Do not wear
tight jewelry or elastic bands around affected fingers or arm(s).
8. Avoid
extreme temperature changes when bathing or washing dishes, and it is
recommended that saunas and hot tubs be avoided (at least keep arm out of the
hot tub). Protect the arm from the sun at all times.
9. Try to avoid
any type of trauma (bruising, cuts, sunburn or other burns, sports injuries,
insect bites, cat scratches) to the arm(s). (Watch for subsequent signs of
infection.)
10. Wear gloves
while doing housework, gardening or any type of work that could result in even
a minor injury.
11. When
manicuring your nails, avoid cutting your cuticles (inform your manicurist).
12. Exercise
is important, but consult with your therapist. Do not overtire an arm at risk:
if it starts to ache, lie down and elevate it. Recommended exercises: walking,
swimming, light aerobics, bike riding, and specially designed ballet or yoga.
(Do not lift more than 15 lbs.)
13. When
travelling by air, patients with Lymphedema (or who are at risk) must wear a
well-fitted compression sleeve. Additional bandages may be required on a long
flight. Increase fluid intake while in the air.
14. Patients with
large breasts should wear light breast prostheses (heavy prostheses may put too
much pressure on the lymph nodes above the collar bone). Soft padded shoulder
straps may have to be worn. Wear a well-fitted bra: not too tight, ideally with
no underwire.
15. Use an
electric razor to remove hair from axilla. Maintain electric razor properly,
replacing heads as needed.
16. Patients with
Lymphedema should wear a well-fitted compression sleeve during all waking
hours. At least every 4-6 months, see your therapist for follow-up. If the
sleeve is too loose, most likely the arm circumference has reduced or the
sleeve is worn.
17. Warning:
If you notice a rash, itching, redness, pain, increase of temperature or fever,
see your physician immediately. An inflammation (or infection) in the affected
arm could be the beginning or worsening of Lymphedema.
18. Maintain
your ideal weight through a well-balanced, low sodium, high-fiber diet. Avoid
smoking and alcohol. Lymphedema is a high protein edema, but eating too little
protein will not reduce the protein element in the lymph fluid; rather, this
may weaken the connective tissue and worsen the condition. The diet should
contain easily digested protein (chicken, fish, tofu).
Unfortunately, prevention is not a cure. But, as a cancer
and/or Lymphedema patient, you are in control of your ongoing cancer checkups
and the continued maintenance of your Lymphedema.
Revised (c) January 2001 National Lymphedema Network.
Permission to print out and duplicate this page in its entirety for educational
purposes only, not for sale. All other rights reserved. For more information,
contact the NLN: 1-800-541-3259.
PREVENTIVE STEPS FOR LOWER
EXTREMITIES Return to top
For the patient who is at risk of developing Lymphedema, and
for the patient who has developed Lymphedema.
WHO IS AT RISK?
At risk is anyone who has had gynecological, melanoma,
prostate or kidney cancer in combination with inguinal node dissection and/or
radiation therapy. Lymphedema can occur immediately postoperatively, within a
few months, a couple of years, or 20 years or more after cancer therapy. With
proper education and care, Lymphedema can be avoided or, if it develops, kept
under control. (For information regarding other causes of lower extremity
Lymphedema, see What is Lymphedema?) The following instructions should be
reviewed carefully pre-operatively and discussed with your physician or
therapist.
1. Absolutely
do not ignore any slight increase of swelling in the toes, foot, ankle, leg,
abdomen, genitals (consult with your doctor immediately).
2. Never allow
an injection or a blood drawing in the affected leg(s). Wear a LYMPHEDEMA ALERT
Necklace.
3. Keep the
edemic or at-risk leg spotlessly clean. Use lotion (Eucerin, Lymphoderm, Curel,
whatever works best for you) after bathing. When drying it, be gentle, but
thorough. Make sure it is dry in any creases and between the toes.
4. Avoid
vigorous, repetitive movements against resistance with the affected legs.
5. Do not wear
socks, stockings or undergarments with tight elastic bands.
6. Avoid
extreme temperature changes when bathing or sunbathing (no saunas or hottubs).
Keep the leg(s) protected from the sun.
7. Try to avoid
any type of trauma, such as bruising, cuts, sunburn or other burns, sports
injuries, insect bites, cat scratches. (Watch for subsequent signs of
infection.)
8. When
manicuring your toenails, avoid cutting your cuticles (inform your pedicurist).
9. Exercise
is important, but consult with your therapist. Do not overtire a leg at risk;
if it starts to ache, lie down and elevate it. Recommended exercises: walking,
swimming, light aerobics, bike riding, and yoga.
10. When
travelling by air, patients with Lymphedema and those at-risk should wear a
well-fitted compression stocking. For those with Lymphedema, additional
bandages may be required to maintain compression on a long flight. Increase
fluid intake while in the air.
11. Use an
electric razor to remove hair from legs. Maintain electric razor, properly
replacing heads as needed.
12. Patients who have
Lymphedema should wear a well-fitted compression stocking during all waking
hours. At least every 4-6 months, see your therapist for follow-up. If the
stocking is too loose, most likely the leg circumference has reduced or the
stocking is worn.
13. Warning:
If you notice a rash, itching, redness, pain, increase of temperature or fever,
see your physician immediately. An inflammation or infection in the affected
leg could be the beginning or a worsening of Lymphedema.
14. Maintain
your ideal weight through a well-balanced, low sodium, high-fiber diet. Avoid
smoking and alcohol. Lymphedema is a high protein edema, but eating too little
protein will not reduce the protein element in the lymph fluid; rather, this
may weaken the connective tissue and worsen the condition. The diet should
contain easily-digested protein such as chicken, fish or tofu.
15. Always wear
closed shoes (high tops or well-fitted boots are highly recommended). No
sandals, slippers or going barefoot. Dry feet carefully after swimming.
16. See a
podiatrist once a year as prophylaxis (to check for and treat fungi, ingrown
toenails, calluses, pressure areas, athelete's foot).
17. Wear clean
socks & hosiery at all times.
18. Use talcum
powder on feet, especially if you perspire a great deal; talcum will make it
easier to pull on compression stockings. Be sure to wear rubber gloves, as
well, when pulling on stockings. Powder behind the knee often helps, preventing
rubbing and irritation.
Unfortunately, prevention is not a cure. But, as a cancer
and/or Lymphedema patient, you are in control of your ongoing cancer checkups
and the continued maintenance of your Lymphedema.
Revised (c) January 2001 National Lymphedema Network.
Permission to print out and duplicate this page in its entirety for educational
purposes only, not for sale. All other rights reserved. For more information,
contact the NLN: 1-800-541-3259.
------------------------------------------------------------------------
In Defense of the 18 Steps to Prevention(c)
William. J. Schuch, Chairman, NLN Board of Directors
During the 1998 NLN Conference, Prof. med. Michael Földi
addressed the issue of the insistence by some U.S. physicians for
"evidence-based" validation of the do's and don'ts contained in the
"18 Steps." As he pointed out, "there are cases in which
'anecdotal observations' are in harmony with scientific facts, with established
knowledge and looking for evidence by prospective, randomized clinical studies
is prohibited by ethical considerations."
Cancer survivors who have undergone the excision of lymph
nodes and/or radiation therapy are at risk for Lymphedema. Yet, the majority of
these individuals are unaware of this risk and what can be done to avoid or, at
least, delay the onset of Lymphedema.
The National Lymphedema Network's 18 Steps to Prevention for
both upper and lower limbs has been widely disseminated in an attempt to inform
those at risk and their health care providers as to the activities or events
which have been identified by experienced MD lymphologists as potential
triggers for the onset or exacerbation of Lymphedema.
In simplest terms, a regional lymphatic network that has
been subjected to nodal basin excision and/or radiation has had its capacity to
transport and filter the necessary lymphatic load - protein, water, metabolic
wastes, viruses and bacteria - curtailed to a greater or lesser degree. This
reduced transport capacity is frequently not enough to result immediately in
the swelling which is characteristic of Lymphedema.
From that point forward, however, any activity or event
which directly or indirectly further impairs the transport capacity of the
affected lymphatic network or increases the lymphatic load has the potential to
trigger the onset of chronic Lymphedema.
Clearly, the following can have the effect of further
reducing transport capacity of superficial impaired regional lymphatics: the
high-end pressures involved in the taking of blood pressure on the afflicted
limb, carrying heavy handbags with over-the-shoulder straps, wearing tight jewelry
or elastic bands around afflicted fingers or limbs, wearing heavy breast
prostheses, narrow bra straps, tight bras, underwire bras, tight socks,
stockings, shoes and underpants.Therefore, heavy lifting with the affected
limb, extreme climatic heat and cold, extreme water temperatures when bathing
or washing dishes, hot tubs, saunas, sunburn and vigorous repetitive movements
against resistance, all of which increase blood flow, should be avoided.
Airplane travel involves pressure changes which allow interstitial
fluid to pool in the dependent extremities while the vasomotor activity of the
lymphangia (the valved vessels which pump the lymph towards the regional lymph
nodes) is at a low level because the individual is essentially at rest during
flight. Hence, the need to wear compression bandages, sleeves or stockings and
to move around as much as possible to prevent the pooling which increases the
lymphatic load.
Infections have the potential for not only curtailing
transport capacity, but also increasing the lymphatic load. Cellulitis and
lymphangitis, which can become episodic, inflame the superficial lymphatic
vessels, rendering them progressively dysfunctional and, thereby, adversely
affecting the transport capacity.
It is important to avoid needle sticks of every kind into
the affected limb (cuts, insect bites, animal scratches, cuticle trimming,
shaving underarms and legs with a razor blade) and necessary to keep the
affected limb, hand or foot, scrupulously clean and supple, and as free of topical
bacteria and fungi as possible.
All of the above precautions make preeminent sense. However,
whether or not one or more of these events or activities will be the immediate
or ultimate precipitating trig-ger is dependent upon a number of factors. They
include, but may not be limited to, the initial degree of surgical
impairment(number of lymph nodes excised or the extent of other surgical
disruption, or collateral dam-age of nearby lymphatic networks and the amount
of lymphatic scarring from radiation therapy) , or the degree of impairment
from other causes, i.e. burns, infections, or severe physical trauma; the
degree of obesity, if any; the individual's specific lymphatic anatomy and the
level of anastomoses (connections) between neighboring lymphatic networks and
regions; the lymphangion fatigue factor resulting from long-term dynamic
compensation for the impaired networks; and the cumulative or progressive
effect of the previously cited events and activities subsequent to the initial
impairment.
Because cancer survivors and others with secondary lymphatic
impairment frequently are not afflicted with Lymphedema immediately and appear
able to carry on their normal activities without modification does not mean
that these activities and events will not trigger the onset of Lymphedema
eventually. Upwards of five percent of breast cancer survivors are afflicted
with Lymphedema in their first year of survival, but the lifelong affliction
rate is reported to be between 30 and 40% - secondary to en bloc excision of
lymph nodes and radiation therapy.
There is an abundance of reinforcing anecdotal reports that
experienced MD lymphologists and Lymphedema therapists, both here and abroad,
have heard from their patients concerning one or more events cautioned against
in the 18 Steps which immediately preceded the onset of their Lymphedema. I
would conclude, therefore, that those who choose to ignore these cautions place
themselves at risk for Lymphedema.
Observance of the 18 Steps is a matter for the at-risk
patient to weigh seriously and to decide. Tragically, Lymphedema is a serious
quality-of-life issue for many thousands of people who were uninformed as to
its prevention and treatment.
The 18 Steps are positive steps for improvement in the
life-styles of those afflicted with this disease.
William. J. Schuch is a patient advocate and advisor to the
Bosom Buddies Breast Cancer and Lymphedema support Group in Naples, Florida:
Tel: (941) 514-3150; Fax: 514-3846; email: wjschuch@email.msn.com; Website
www.go-icons.com/bosombuddies.htm.
Revised (c) January 2001 National Lymphedema Network.
Permission to print and duplicate this this article in its entirety for
educational purposes only, not for sale. All other rights reserved. For more
information, contact the NLN: 1-800-541-3259.
www.lymphnet.org/whatis.html
www.lymphnet.org/prevention.html
Lymphedema Prevention: A One-Year
Survey
Volume 13, No. 1 January-March 2001
By Allen G. Meek, MD
Chairman, Department of Radiation
Oncology, SUNY at Stony Brook, University Hospital
Cohen SR, Payne DK, Tunkel RS.
Lymphedema: strategies for
management.
Cancer. 2001 Aug 15;92(4
Suppl):980-7. Review.
PMID: 11519024 [PubMed - indexed
for MEDLINE]
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