Whitehair, CL Chapter Nine v.6 Author-1: Curtis L. Whitehair, MD Associate Medical Director, MedStar National Rehabilitation Network Director, Cancer Rehabilitation MedStar National Rehabilitation Hospital Author-2: Yvonne Francis, PT MedStar NRH Network, Irving Street Chapter Nine Managing Lymphedema Key Points Lymphedema is a lifelong condition Although lymphedema is incurable, it is controllable The risk of developing lymphedema increases with the amount of different types of cancer treatment Maintaining proper exercise and a rehabilitation program by a lymphedema expert is the best way to control lymphedema “After my breast cancer treatment, I felt like I was home free. My oncologist said my cancer was gone and the likelihood of recurrence was low. I felt like I didn’t have much to be concerned about. However, 6 months after my surgery, I began to notice swelling in my arm. My first thought was: Is the cancer coming back so soon? I was so scared. Luckily, my oncologist referred me to a physiatrist – a specialist in physical medicine and rehabilitation. This physiatrist was an expert in problems caused by cancer and its treatment. The physiatrist told me I had lymphedema. While the bad news was that I will always be at risk for lymphedema, the good news was that it is treatable and manageable. The physiatrist referred me to a physical therapist with specialized training in lymphedema management. After just 6 weeks working with the therapist twice a week, my lymphedema was practically gone. I finished outpatient therapy and was given exercises and massage techniques to do at home on my own. I also have to wear a compression sleeve to keep the lymphedema away. This can be annoying, but it’s worth doing to keep my arm healthy. I am very thankful for my rehabilitation treatments.” Mary H. 1 1 INTRODUCTION 2 Lymphedema is a specific type of swelling that can be related to cancer treatment. As Mary 3 H.’s experience illustrates, lymphedema is both scary and challenging. This chapter will help 4 alleviate those fears by first providing an understanding of how the body works, what causes 5 lymphedema, and the risk involved in developing lymphedema. Furthermore, it will provide an 6 understanding of the complications that can occur with lymphedema if not treated. Lastly it will 7 discuss the different treatment options available to minimize or control lymphedema. 8 SECTION I: WHAT IS LYMPHEDEMA 9 How the body is constructed 10 The lymphatic system is composed of lymphatic channels or vessels which are located in every 11 part of the body (Figure 9.1). This system complements the circulatory system and runs along 12 the arteries and veins in what is known as the extracellular space. The circulatory system 13 carries the blood to and from the cells in the body, however, this system is somewhat leaky and 14 about 3 liters of a protein-rich fluid per day may leak out into the extracellular space. The 15 lymphatic system filters this fluid (known as lymphatic fluid) through lymph nodes to trap 16 bacteria, viruses and debris which help the immune system. It travels to places where the 17 circulatory system does not go and provides protection against illness. Part of the job of the 18 lymphatic system is to pick up any extra fluid that then gets deposited back into the circulatory 19 system. The right side of the head and neck, the right arm and that side of the upper body 20 drain into one particular lymphatic vessel known as the right lymphatic duct. The left side of the 21 head, neck, left arm, left side of trunk and both legs drain into the left side of the circulatory 22 system through the thoracic duct. Because this network is so extensive through the whole body 23 it, unfortunately, provides excellent routes for cancer cells to migrate to other locations resulting 24 in metastases of the primary tumor. Typically the cancer cells that loosen from the primary 2 25 tumor will be trapped by the lymph nodes thus becoming a secondary cancer site. This is why 26 lymph nodes are often biopsied or removed as part of cancer treatment. 27 28 29 [Figure 9.1] The Lymphatic System 30 What causes lymphedema 31 Most lymphedema associated with breast cancer is caused by an injured lymphatic system 32 leading to a buildup of protein-rich fluid in the extracellular space typically called lymphatic 33 fluid. This may be referred to as secondary lymphedema because it is cause by a known 34 problem. Primary lymphedema causes are unknown and are usually from birth. There are two 35 main ways in which this occurs. 36 The most common is when parts of the lymphatic system are removed either during 37 diagnosis, staging or during removal of the cancerous tumor and effected lymph nodes. Less 38 commonly, but just as problematic, is an obstruction due to scarring of the lymphatic system 39 which may occur with some treatments such as radiation or from the tumor itself. When the 40 system does not drain the extra fluid, two things occur. First, the fluid that leaked out of the 41 circulatory system is unable to get back in. Secondly, the proteins in the extracellular space 42 cause more fluid to come to that area than what would have normally leaked out. 43 The effects of lymphedema 44 In breast cancer, an abnormal lymphatic system may cause edema or swelling of the arm, 45 breast, chest wall or neck. The swelling may be extremely large or very minimal. Even if 46 lymphedema is kept under good control, there is a lifelong risk of developing a return of 47 increased lymphedema. 48 The complications due to lymphedema 49 Untreated lymphedema may result in an increased size of the affected limb or body part and 50 also create several risk factors for other medical problems. Cellulitis, which is a skin infection, is 51 a common complication that left untreated could result in a serious medical condition. The 52 protein rich fluid buildup is an excellent place for bacteria to grow. In addition, there may be 53 significant problems such as openings in the skin with leaking wounds and failure of these 54 wounds to heal. 55 How lymphedema is diagnosed 56 The typical diagnosis is made by a physician or lymphedema specialist using girth 57 measurements of the affected extremity with a comparison to the unaffected side. If there is 58 greater than a 2 cm difference at the measurement sites this is typically considered due to 59 lymphedema. There can be normal physiological swelling which makes one side of everyone’s 60 body larger than the other side, but there is rarely more than a 2 cm difference. The 61 lymphedema specialist should take the measurements from the same location each time during 62 an evaluation. If there is an increase of 2 cm from prior measurements this would then be 63 considered an exacerbation of the lymphedema. 64 New research is emerging about bio-impedance machines that may be used to diagnose 65 lymphedema. These machines measure the resistance of an electrical current in the 66 extracellular space. The electrical current is not felt during the test. If there is lymphedema this 67 electrical current will move faster to the recording site. The advantage of this new technology is 68 the ability to diagnose lymphedema prior to the girth size of the arm changing. One of the first 69 signs of lymphedema that a patient will notice is heaviness in the arm. A good example to think 70 of is the following: if two towels are lying on the floor and one is dry and one is wet, the 71 measurement around the towels would be the same, however, when lifting up the two towels the 72 one which is wet will feel heavier. This is the same concept that occurs within one's arm. More 73 lymph fluid results in a sense of heaviness. Bio-impedance may diagnose lymphedema even 74 before the heaviness is noted. Recent studies suggest that early treatment will help contain 75 lymphedema. 76 There are other potential causes of swelling in the arm that are related to the circulatory 77 system. If the veins are not pumping blood well, fluid can back up and cause swelling. One 78 potential cause of this would be a blood clot, which needs to be ruled out if suspected. In this 79 case the lymphatic system may be removing fluid at its normal rate but there is more fluid than 80 the lymphatic system can remove. An analogy would be a sink running over. Either the faucet is 81 turned on so much so the sink fills faster than it drains or the faucet is okay, but the drain is 82 clogged. Your doctor may request a special test called Lymphoscintigraphy. This involves 83 injecting a small amount of radioactive material between the webspace of the fingers. The arm 84 is then scanned as the radioactive fluid is absorbed and flows through the lymphatic 85 system. This is the only way to accurately determine whether there is a circulatory system or 86 lymphatic system problem. 87 The chances of getting lymphedema 88 The risk of developing lymphedema is directly related to how much of the lymphatic system is 89 damaged either by the cancer itself or the treatment to eradicate it. Almost 80% of all patients 90 that develop lymphedema do so within the first 3 years after cancer treatment, however, it has 91 been reported to occur as far as 30 years after treatment. All treatments to battle cancer can 92 have significant side effects. Therefore it is very important to have a frank conversation with the 93 oncologist or breast surgeon to examine the risk versus the benefit of each treatment. 94 Cancer has the lowest risk of causing lymphedema. In the past, the biggest risk factor 95 was related to the number of lymph nodes removed during surgery. The surgeons now use 96 sentinel lymph node biopsy to identify how far the cancer may have spread without need of 97 exploring or removing a large portion of the lymphatic system. This has significantly reduced the 98 risk of lymphedema in today's treatment. 99 During the removal of the tumors and or the lymphatic system the risk increases with 100 each lymph node that is removed. The surgeon will report how many lymph nodes were 101 removed during the surgery. There are approximately 35 to 45 in the region of the breast and 102 armpit. Additionally, radiation causes scarring of all of the vessels that are within the radiation 103 field. This may cause obstruction of the lymphatic drainage even if no lymph nodes have been 104 removed. 105 The National Lymphedema Network (www.lymphnet.org) publishes a position paper on 106 risk reduction practices. Most of these recommendations are based on health care provider 107 expertise as there is little research or data on this subject. Refer to the website for up-to-date 108 recommendations. In general, however, the following guidelines are recommended: 109 110 111 risk for lymphedema periodically. 112 113 Maintain regular medical checkups to include an evaluation of the arm at Report any increased size or heaviness in the arm as well as changes in sensation, color, temperature or skin conditions. Maintain a proper diet and body weight. This is critical as a close 114 correlation between body weight and lymphedema has been 115 demonstrated. 116 117 118 Proper exercise has been shown to improve lymphedema. CAUTION: incorrect or unsafe exercise may worsen lymphedema Use compression garments. Follow the manufacturer's recommendations 119 as well as your healthcare provider's instructions. Wear the garment 120 during air travel, exercise and extreme exertion. 121 122 123 124 Treat all skin infections or cellulitis urgently. More than three episodes of cellulitis in the year may require the use of preventive antibiotics. Appropriate skin care is extremely important. Use regular moisturizer to maintain good skin health and avoid 125 skin breakdown and cracking, especially during dry or cold 126 weather.. 127 When you go outside, wear protective clothing as well as bug 128 repellent and sunscreen on exposed skin. Avoid trauma to the 129 affected area. 130 131 132 Wear gloves when performing any activity that can injure the skin (such as gardening). Burns, broken bones or any kind of trauma may increase the risk 133 of lymphedema. 134 It is recommended regular venous puncture blood draws and 135 blood pressure readings should not be done on the affected side 136 if it all possible. However, in an emergency situation use that 137 arm if needed. 138 139 140 Avoid wearing constrictive clothing, this may cause additional swelling. Avoid extreme heat and cold temperature. 141 There is considerable controversy among healthcare 142 providers relating to heat and cold 143 recommendations. Specifically, these concern the use of 144 saunas and hot tubs. If one feels the need to get into a hot 145 tub, do not submerge the affected limb. 146 Running hot water over the affected limb in a shower is 147 allowed, however, it is not recommend to let do not let hot 148 water run over the arm long enough for it to turn red. 149 150 Your healthcare provider managing the lymphedema should be contacted before any surgery on the affected limb. 151 Air travel: There is an increased risk of developing or making 152 lymphedema worse while flying. The low pressure in the airplane 153 cabin promotes swelling. In addition, the flow of lymph fluid slows 154 down during the flight which can contribute to increased 155 swelling. Moving the arm frequently can reduce this 156 risk. Regardless of other risk factors, it is recommended to wear 157 a compression garment (Figure 9.2). It should be placed on your 158 arm at least 30 minutes before the flight. The compression 159 garment should remain on after the flight for 1 to 3 hours based on 160 the length the flight. The rule of thumb is 1 hour of wearing for up 161 to 2 hours of flying time, 2 hours of wearing for 3-5 hours of flying 162 time and 3 hours of wearing for any flight over 5 hours. 163 SECTION II: TREATMENT OPTIONS 164 Overview 165 There are many effective treatments for lymphedema such as manual lymphatic drainage, 166 multilayer bandages, vasopneumatic sequential gradient pumps, compression garments and 167 exercise. 168 lymphedema. However, several treatments in combination are even more helpful. When these 169 treatments are combined together, it is called Complete Decongestive Therapy (CDT.) The 170 individual components of CDT are described below. 171 Manual lymphatic drainage 172 Manual lymphatic drainage (MLD) is a light massage that helps to decongest an area and move 173 lymph fluid to healthy lymph vessels. MLD should be performed by a lymphedema specialist 174 who has received specialized training and certification. The arm is placed in a relaxed position Each of these treatments alone has been shown to be helpful to combat 175 while the specialist begins to drain proximal (closest to the shoulder) to distal (closest to the 176 hand). The massage strokes are performed to facilitate the movement of tissue fluid and 177 stimulate lymph nodes. Patients can be taught by the lymphedema specialist how to perform the 178 manual lymphatic drainage at home. Home maintenance of MLD will help to maintain the results 179 achieved in the clinic. 180 When performing MLD, It is important to avoid deep pressure to minimize the risk of a 181 backflow of fluid which can damage lymph vessels. Extra care should be taken when performing 182 MLD near radiated tissue to avoid further skin injury. Manual lymphatic drainage should be 183 performed prior to multilayer bandaging or to the vasopneumatic sequential gradient pump 184 treatment. 185 Multilayer bandages 186 Multilayer bandages (MLB) are a special type of short stretch bandages which are distinct from 187 typical ace-wrap bandaging. They are applied to the arm to assist with re-absorption of lymph 188 fluid and provide external support to the arm. A pressure gradient (tighter at the distal end and 189 looser at the proximal end) is created to facilitate a flow of fluid up the arm. The lymphatic 190 channels will begin to transport fluid away from the congested area. MLB will assist in breaking 191 up fibrosis or hardening areas of skin. MLB are wrapped along the arm to form a cone shape, 192 with greater pressure distal than proximal. The lymphedema specialist will inspect the skin prior 193 to applying the MLB for any breaks in the skin, infections, discolorations or increase in swelling 194 in the arm. Multilayer bandages will affect tissue texture changes which will reduce the size of 195 the arm. 196 Guidelines for multilayer bandages include: 197 Apply at the end of therapy treatment. 198 Don’t re-apply if they become undone between treatment sessions. 199 Continue to participate in activities of daily living with bandaging on. 200 If bandaging becomes too tight, remove outer most layers one at a time 201 until relief is experienced instead of removing entire wrap. 202 Keep bandaging dry. 203 Be aware of bandaging located around hand region when cooking with an 204 open flame. 205 The goal is to wear bandaging for 24 hours. A caregiver can be trained by a 206 lymphedema specialist how to apply MLB for home maintenance. Typically, a patient with 207 congestive heart failure should not have MLB applied unless cleared by a cardiologist. 208 Vasopneumatic sequential gradient pump 209 The vasopneumatic sequential gradient pump is a mechanical device sometimes used in 210 conjunction with MLB, and/or MLD, education and skin care. The pump is designed to move 211 fluid from the distal to the proximal arm. The, amount of pressure is usually pre-set on home 212 units at 30-40 mmHg. The treatment time should not be greater than 60 minutes per 213 session. The lymphedema specialist will place the arm in a sleeve and the flow of intermittent 214 air will move through multiple chambers causing a sequential squeezing effect. Conscientious 215 use of a home pump is critical for the reduction and long term maintenance of arm swelling.. 216 Patients with congestive heart failure or kidney problems should not receive pump therapy to 217 avoid pushing extra fluid to the heart and kidney which has impaired function. There are many 218 types of vasopneumatic sequential gradient pumps on the market. The lymphedema specialist 219 will help decide which machine is best to use which may also include a consideration of the cost 220 and how much is covered by insurance. 221 222 [Figure 9.2] Home compression pump. Copyright Lymphapress, reprinted with permission 223 Compression garments 224 After a lymphedema specialist provides the first phase of lymphedema treatment (discussed 225 above), a compression garment will need to be worn to maintain these improvements. A 226 compression sleeve provides external pressure and is worn during the day to maintain reduction 227 of lymphedema received in treatment. Compression garments are not worn to reduce swelling, 228 but to maintain the swelling reduced by other treatments. The garments are designed to have 229 greater pressure distally than proximally and to be worn in compliance with the lymphedema 230 specialist’s recommendations. The pressure in the sleeve is based on the amount of swelling in 231 the arm: the pressure for mild swelling should be 20-30 mmHg, mild-moderate 30-40 mmHg, 232 mod-severe 40-50 mmHg. For hand swelling, a gauntlet (a glove without fingers) can be worn. 233 For breast edema and fibrosis, a compression bra is useful fibrosis and breast edema. Custom 234 compression garments can be measured by a garment fitter when the size of the arm is too 235 large for a Pre-Fabricated sleeve. 236 It is important to wear compression garments during the day when doing regular 237 activities and exercising. The lymphedema specialist may also recommend a night time 238 compression garment while sleeping. There are many different brands of these garments 239 available. As discussed earlier, it is highly recommended to wear a compression sleeve and 240 gauntlet while flying. 241 Guidelines for the compression garments: 242 They typically last for 6 months before needing to be replaced. 243 Hand wash and air dry instead of putting in the washing machine and 244 dryer. 245 When experiencing difficulty adjusting to the garment, start by wearing it 246 two hours the first day, four hours the second day and six hours the third 247 day, etc. 248 249 Any time there is increased swelling and or pain the lymphedema specialist should be contacted immediately. 250 Exercise 251 It is essential for breast cancer patients with lymphedema to participate in some form of physical 252 activity to enhance lymph flow and maximize their quality of life. Muscles and joints can often 253 become stiff and hard to move after surgery or radiation. Exercise encourages a pumping 254 action of muscles to stimulate lymph fluid to flow. The lymphedema specialist will recommend a 255 specific exercise regimen based on individual impairments. There are three types of exercise: 256 flexibility, strength and aerobic (cardiovascular exercise). If pain is experienced during any of 257 these types of exercise, the exercise should be stopped immediately and the lymphedema 258 specialist should be notified. 259 Flexibility exercises consist of gentle stretches and range of motion which can be 260 performed postoperative Day 1. Deep breathing should be performed in conjunction with 261 flexibility exercises. MLB or a compression garment worn during exercise will support the arm, 262 serve as a protective measure and help to decrease swelling. It is important to follow the 263 exercise guidelines provided by the lymphedema specialist and progress gradually to avoid 264 overuse of the arm. Flexibility exercises can help with scar management, increase range of 265 motion and prevent joint tightness. These exercises are performed slowly to allow muscles to 266 stretch over a period of time. 267 Strengthening exercises are integral to rehabilitation management by providing physical 268 and emotional benefits. Muscle contractions help promote the flow of lymphatic fluid out of the 269 swollen region. It is important to gradually increase repetitions and resistance over a period of 270 time. Various equipment can be used safely to strengthen muscles in the arm including 271 dumbbells, weight machines, theraband and swimming (pool should be cool temperature) used 272 Some exercise principles consist of: 273 Exercise daily to achieve full symmetrical movement of the arm. 274 Deep breathing for relaxation and to increase venous blood and lymph fluid 275 return to the heart. 276 When using weights, begin with less than 10 pounds for the first six weeks. 277 Don’t wrap cuff weights on the arm. 278 Increase the amount of weight lifted at gradual intervals, as tolerated. Weight 279 lifting should never be more than “a little bit difficult”. 280 For aerobic exercise to be effective, it is recommended that the heart rate should reach 281 60—70% of the maximal heart rate. A person’s maximal heart rate is calculated by taking 220 282 minus their age. Some examples of aerobic exercise include; jogging, walking, cycling and 283 swimming. These activities will help maintain your weight, improve endurance and 284 cardiovascular fitness. It is important to drink plenty of water and take rest breaks while 285 exercising. While performing aerobic exercise, the intensity, speed and duration should not be 286 increased at the same time. Bowling, tennis and golf should be discussed with the lymphedema 287 specialist prior to returning to these activities due to the repetitive movements and stress on the 288 arm. 289 Deep Breathing exercises 290 Breathing deeply is a great contributor to good health. Among its many virtues, it helps get more 291 oxygen to all of the tissues and is a great stress reliever. Moreover, deep breathing exercises 292 are helpful to stimulate lymphatic pathways to healthy lymph nodes and the circulatory 293 system. This can be accomplished by diaphragmatic or abdominal breathing and lateral 294 expansion. 295 Diaphragmatic breathing is most easily done while lying on one’s back with your hand on 296 the abdomen. Breathe out (exhale) as much of the air as possible while pushing on the 297 abdomen with the hand. Next, breathe in (inhale), as much as possible. The hand should 298 be pushed up by the abdomen. Once comfortable with the lying position, try the exercise 299 sitting and standing. 300 Lateral expansion is most easily learned when sitting or standing in front of a mirror with 301 one’s hands on each side of the ribs. Exhale using the hands to squeeze in the ribs and 302 then inhale, flaring the rib cage so that the hands are pushed outward. Don’t let the 303 shoulders rise during this exercise. 304 Wellness 305 Wellness is your ability to maintain a healthy lifestyle, good nutrition and life-affirming habits. A 306 physically fit body decreases musculoskeletal problems, prevents strain on the affected arm and 307 reduces the chances of getting lymphedema. Gradual conditioning of the body is 308 best. Maintaining an ideal body weight is important to control obesity which increases the risk of 309 lymphedema. Lifestyle changes consist of minimizing alcohol, caffeine, unhealthy and salty 310 foods. Smoking can cause serious health issues for breast cancer patients; therefore quitting is 311 highly encouraged. 312 CONCLUSION 313 Everyone’s risk for developing lymphedema is different and therefore the treatments 314 vary. Maintaining a consistent relationship with a lymphedema specialist combined with 315 following the guidelines and recommendations in this chapter will help provide decreased risk of 316 developing or making lymphedema worse and provide the best possible treatment outcomes. 317 Glossary 318 319 Bio-impedance – a measurement utilizing a small and slow electrical current which records the time it takes to move through the body or specific tissues. 320 Biopsy – a small piece of tissue taken from the body for diagnostic purposes. 321 CDT – an abbreviation for Complete Decongestive Therapy. 322 Cellulitis – a bacterial infection of the skin and subcutaneous tissue. 323 Distal – near the end of a limb or body part such as an arm. 324 Edema – a term for swelling of any type. 325 Extracellular space – the space outside of the cells and the circulatory system. 326 Fibrosis –connective tissue that loses its normal elastic properties and becomes hardened.. 327 Gradient – going from a lower pressure area to one of higher pressure or vice versus. 328 Gauntlet – a glove like garment without the fingers that only covers the hand. 329 Interstitial space – same as extracellular space. 330 331 Lymph node – a place where several lymph vessels join together. These nodes contain lymph tissue which support the immune system. 332 333 Lymphedema – involves blockage of the lymph vessels, with a resulting accumulation of lymphatic fluid in the interstitial tissues of the body. 334 335 336 Lymphoscintigraphy – a test done in a Nuclear Medicine Department where a radio-active isotope is injected and then followed under a scanner as the isotope moves through the lymphatic system. 337 MLD – is an abbreviation for manual lymphatic drainage 338 mmHg– is an abbreviation for “millimeters of Mercury” , a measurement unit for pressure. 339 MLB – is an abbreviation for multilayer bandages 340 Physiological swelling – swelling that normally occurs and is not considered a problem. 341 342 Primary lymphedema – lymphedema that occurs when a person is born with a deficient lymphatic system. There is no known cause. 343 344 Primary tumor – the site of the original tumor. Proximal-- near the beginning of a body part such as an arm. 345 346 Secondary lymphedema – lymphedema that occurs after the lymphatic system was working normally. This may be due to trauma or illness . 347 348 349 350 351 Sentinel lymph node – the first lymphnode in a branch of lymphnodes. Biopsies are often done on this node as if it is clean than all that drain into it are considered cancer free. Vasopneumatic sequential gradient pump – a pump that causes pressure starting at one end and slowly moves it in a particular direction. 352 Symptom Index 353 Hardness 354 Heaviness 355 Leaking 356 Opening 357 Redness 358 Stiffness 359 Swelling 360 Weeping 361 Wound 362 [Figure 9-1] The Lymphatic System 363 [Figure 9-2] Home compression pump. Copyright Lymphapress, reprinted with permission