CLINICAL SCHOLARSHIP
Self-Care Status, Symptom Burden, and Reported Infections in Individuals With Lower-Extremity Primary Lymphedema Jie Deng, PhD, RN, OCN1 , Elise Radina, PhD, CFLE2 , Mei R. Fu, PhD, RN, APRN-BC, FAAN3 , Jane M. Armer, PhD, RN, FAAN4 , Janice N. Cormier, MD, MPH5 , Saskia R.J. Thiadens, RN6 , Jan Weiss, PT, DHS, CLT-LANA7 , Catherine M. Tuppo, PT, MS, CLT-LANA8 , Mary S. Dietrich, PhD9 , & Sheila H. Ridner, PhD, RN, FAAN10 1 Assistant Professor, School of Nursing, Vanderbilt University, Nashville, TN, USA 2 Professor, Department of Family Studies & Social Work, Miami University, Oxford, OH, USA 3 Associate Professor, College of Nursing, New York University, New York, NY, USA 4 Professor, Sinclair School of Nursing, University of Missouri, Columbia, MO, USA 5 Professor, UT MD Anderson Cancer Center, The University of Texas, Houston, TX, USA 6 Executive Director, National Lymphedema Network, San Francisco, CA, USA 7 Lymphedema Resource Therapist, CoxHealth Outpatient Rehabilitation, Springfield, MO, USA 8 Program Coordinator, Bariatric and Metabolic Weight Loss Center, Stony Brook Medicine, Stony Brook, NY, USA 9 Professor, School of Nursing, Vanderbilt University, Nashville, TN, USA 10 Professor, School of Nursing, Vanderbilt University, Nashville, TN, USA
Key words Primary lymphedema, lower extremity, symptom, infection, self-care Correspondence Dr. Jie Deng, School of Nursing, Vanderbilt University, 461 21st Ave. South, 516 Godchaux Hall, Nashville, TN 37240 USA. E-mail: [email protected] Accepted: October 4, 2014 doi: 10.1111/jnu.12117
126
Abstract Purpose: The purposes of this study were (a) to evaluate self-care, symptom burden, and reported infections among individuals with lower-extremity primary lymphedema; (b) to examine the differences in self-care, symptom burden, and reported infections between individuals with unilateral and those with bilateral lower-extremity primary lymphedema; and (c) to examine the associations among self-care status, symptom burden, and reported infections in individuals with lower-extremity primary lymphedema. Design: A secondary data analysis was used. Data were collected from a crosssectional survey study supported by the National Lymphedema Network from March 2006 through January 2010. The surveys were available both online and in hard copy in order to increase accessibility. Methods: Descriptive statistics were conducted and associations between variables were assessed using Mann-Whitney tests and chi-square tests of independence. Multiple logistic regression was used to test for associations while controlling for potentially confounding variables. Findings: A total of 803 participants reported having lower-extremity primary lymphedema. The majority of the participants were female (82.9%), White (74.2%), and from the United States (90.7%). Approximately two thirds of the respondents conducted some home daily lymphedema self-care. Over half of the respondents reported experiencing symptom burden and 44.8% reported at least one episode of infection. Compared to individuals with unilateral lower-extremity primary lymphedema, individuals with bilateral lower-extremity lymphedema were more likely to conduct skin care (p = .004), use alternative medications (p = .005), more frequently reported symptoms (p < .05), and more likely to report at least one episode of infection (p = .002). Respondents who reported use of compression garments also were less likely to have self-reported pain (p = .002), poor range of motion (p = .026), and numbness (p = .001). Participants who reported exercising also were less likely to have self-reported pain (p = .003). Participants who reported at least one episode of infection also reported experiencing more symptoms (p < .001). Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Individuals With Lower-Extremity Primary Lymphedema
Deng et al.
Conclusions: Individuals with lower-extremity primary lymphedema experienced substantial symptom burden and infection episodes. Significant associations were identified among self-care, symptom burden, and reported infections. Clinical Relevance: The findings support the need for clinicians to educate patients with lower-extremity primary lymphedema regarding the importance of self-care, symptom management, and infection control. It is critically important for clinicians to evaluate symptom burden and reduce infections in individuals with lower-extremity primary lymphedema.
Lymphedema is a chronic and serious condition, affecting more than 140 million people worldwide (Brorson, Ohlin, Olsson, Svensson, & Svensson, 2008). Lymphedema occurs when the lymphatic system fails to transport lymph fluid to the circulatory system due to either primary or secondary causes. Primary lymphedema is related to a developmental defect (malformation, ¨ dysplasia) of the lymph vessels or lymph nodes (Foldi, ¨ ¨ Foldi, Strossenreuther, & Kubik, 2007). Studies have reported an association between several genes, including VEGFR-3, VEGFC, and FOXC2, and primary lymphedema (Bernaudin, Kambouchner, & Lacave, 2013; Connell et al., 2009). Primary lymphedema can occur without obvious causes at different developmental stages, such as at birth, at puberty, or, as with lymphedema tarda, later in life (Zuther, 2009). Secondary lymphedema usually occurs as a consequence of cancer, cancer treatment, infection, or trauma (Lee, Bergan, & Rockson, 2011). Although no curative surgical or medical intervention is available for lymphedema, expert opinion supports that current standard for treatment of either primary or secondary lymphedema is two-phase complete de¨ congestive therapy (CDT; Foldi et al., 2007; Zuther, 2009), which includes the intensive phase (Phase I) provided by a certified lymphedema therapist and selfcare phase (Phase II) administrated by the patient or a caregiver. Phase II of CDT includes self-manual lymphatic drainage (self-MLD), compression (i.e., garments ¨ or bandaging), skin care, and exercise (Foldi et al., 2007; Zuther, 2009). Although the exact mechanisms of the two-phase CDT have not been clearly described, the underlying pathophysiological foundation of the CDT supports that both phases may be able to (a) improve the function of lymph vessels, (b) soften the fibrosclerotic indurations, (c) reduce increased connective tissue, (d) cleanse the skin to prevent opportunistic infections, and (e) reduce associated symptom burden (Lee et al., 2011). These potential mechanisms support the importance of self-care (Phase II of CDT) and suggest potential relationships among self-care, symptom burden, and infection.
Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Because lymphedema is a chronic and incurable condition, long-term daily self-care activities for lymphedema are critical to manage lymphedema-related symptom burden, functional impairments, and episodes of infection. Though empirical lymphedema self-care evidence is scant, a recent systematic review of the literature from 2004 to 2011 on self-care among patients with lymphedema identified only 16 articles focused on lymphedema self-care (Ridner, Fu, et al., 2012). Most of the articles evaluated self-care activities in individuals with secondary lymphedema and no article focused exclusively on self-care among patients with primary lymphedema. Four of the articles reported on a subgroup of study participants with primary lymphedema (Ridner, Fu, et al., 2012). Although little is known about the symptom burden experienced by patients with primary lymphedema, or infection, some studies have supported that individuals with primary lymphedema likely experienced distressful symptoms (e.g., pain or discomfort) and functional impairments (e.g., usual activities; Okajima et al., 2013; Schook et al., 2010; Symvoulakis, Anyfantakis, & Lionis, 2010), and reported frequent infection in the lymphedema-affected area. For instance, one article identified that 31.3% (26 out of 83) of the participants reported cellulitis (lymphedema-related infection; Okajima et al., 2013); another article found that 18.8% (26 out of 138) of the participants developed cellulitis (Schook et al., 2010). To date, there are no known studies that have investigated the associations between self-care, symptom burden, and infections in individuals with primary lymphedema. In order to address the gaps in the literature, the National Lymphedema Network (NLN) conducted a patient-reported survey study to collect data on lymphedema and its management. Among the respondents with primary lymphedema, the most common affected site was the lower extremity. The aims of this report are to identify (a) the current status of self-care, symptom burden, and infections among patients with lower-extremity primary lymphedema; (b) whether or not there are differences in self-care, self-reported symptom burden, and
127
Individuals With Lower-Extremity Primary Lymphedema
infection occurrence among respondents with unilateral or bilateral lower-extremity primary lymphedema; and (c) the associations among self-care status, symptom burden, and infections in this cohort group.
Design A secondary data analysis was used. All data were collected as part of the NLN cross-sectional survey study (Ridner, Deng, et al., 2012). Designed by the NLN Research Subcommittee, the survey collected the following self-reported information relevant to this study: demographics, type and anatomic sites of lymphedema, characteristics of primary lymphedema (e.g., onset age of lymphedema and family history), history of surgical treatment, associated symptoms (six symptoms were evaluated, including pain, poor range of motion, numbness, stiffness, heaviness, and swelling), infection status, self-care status (self-care defined as Phase II of the CDT, including self-MLD, compression garments or bandaging, skin-care, and exercise), and alternative self-care activities.
Data Collection The study was approved by the Institutional Review Board (IRB) at the University of Missouri Health Sciences. The study was conducted from March 2006 to January 2010. Individuals with or at risk for lymphedema were eligible to participate in this survey study. The survey was available and accessible to individuals within the United States and worldwide via the NLN website (www.lymphet.org). Study participants who had no access to the website were able to complete a hard-copy version of the survey and submit the survey via mail. Data were entered into the database by staff in the NLN office. Individuals’ completion or return of the survey served as consent to participate in the study. Detailed information about the survey development and administration has been reported in previous publications (Ridner, Deng, et al., 2012). Secondary data analysis was approved by the IRB at Vanderbilt University.
Sample From March 2006 through January 2010, 2,968 surveys were received. After the data cleaning process (i.e., deletion of duplicate surveys, n = 220; invalid responses, n = 14), a total of 2,734 respondent surveys were available for analysis. Out of these respondents, 830 individuals reported having primary lymphedema. Among individuals with primary lymphedema, the majority of respondents, 96.7% (n = 803), reported having 128
Deng et al.
lower-extremity primary lymphedema (including 269 with unilateral lower-extremity primary lymphedema and 534 with bilateral lower-extremity primary lymphedema). For the purpose of this report, we examined participant characteristics, self-care status, symptoms, and infections among respondents with either unilateral or bilateral lower-extremity primary lymphedema.
Data Analysis Data were analyzed using the statistical software package SPSS version 20.0 (International Business Machines Corp., Armonk, NY, USA). The data were verified and cleaned by screening for duplicate cases and missing data, which were omitted from the study sample. Descriptive statistics (frequency distributions, median and 25th-75th interquartile range [IQR]) were used to describe the sample characteristics and distributions of the variables. Tests of the differences between groups (e.g., between unilateral and bilateral) were conducted using Mann-Whitney tests. Chi-square tests of independence were used to test for associations between variables that were nominal in nature. Multiple logistic regression was used to test for associations while controlling for potentially confounding variables. Decisions regarding statistical significance used p values below .05.
Results Sample Characteristics Table S1 summarizes the characteristics of respondents with lower-extremity primary lymphedema. The majority of the respondents were female (82.9%), White (74.2%), and from the United States (90.7%). Approximately one third of respondents had a family history of lymphedema. Fifty percent of the respondents had insurance coverage. There were statistically significant differences noted for gender, ethnicity, country, family history of lymphedema (yes or no), insurance coverage, and type of insurance among respondents with unilateral or bilateral lower-extremity primary lymphedema. Compared to respondents with unilateral lower-extremity primary lymphedema, individuals with bilateral lower-extremity primary lymphedema were more likely to be from the United States, female, and White, have a family history of lymphedema, insurance coverage, and, if covered, insurance from government support. There was no statistically significant difference in the onset age of primary lymphedema between individuals with unilateral or bilateral lower-extremity primary lymphedema (see Table S1, available with the online version of this article). Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Deng et al.
AIM 1: Overall Self-Care Status, Symptoms, and Infections Self-care status. As displayed in Table S2 (available with the online version of this article), the majority (68.9%) of the respondents conducted some home daily lymphedema self-care. The self-care methods of Phase II CDT used (from high to low frequency) included compression garments (48.1%), skin care (40.1%), exercise (32.6%), bandaging (26.4%), and self-MLD (20.7%). Approximately 50% of the respondents reported that they spent more than 30 min per day on self-care. Also included in Table S2 are the reported alternative treatments of self-care. Less than 50% of the respondents used alternative treatments for self-care. The alternative treatments used among individuals with lower-extremity primary lymphedema (from high to low frequency) were bandage alternatives (19.1%), pumps (17.9%), medications (12.5%), herbal substitutes (5.9%), and yoga (3.6%). Of note, 13% of the respondents perceived that alternative treatment was more effective than Phase II CDT (see Table S2). Symptom profiles. Regardless of whether lymphedema involved unilateral or bilateral lower extremities, a high percentage of individuals reported associated symptoms (Table S3, available with online version of this article). The prevalence of reported symptoms in descending order were swelling (98.9%), heaviness (89.8%), stiffness (78.6%), pain (74.6%), poor range of motion (68.1%), and numbness (60.7%). Of the respondents experiencing each symptom, the respective reported severity was highest for heaviness (median = 4.0 on a scale of 1 [little] to 5 [severe]; see Table S3). Infection status. A high percentage of individuals with lower-extremity primary lymphedema reported infections since the first onset of lymphedema in their lower extremities. A total of 44.8% (360 of 803) reported at least one episode of infection, and 27.1% (218 of 803) with at least one admittance into the hospital due to lymphedema-related infection.
AIM 2: Differences of Self-Care Status, Symptoms, and Infections Among Respondents With Unilateral or Bilateral Lower-Extremity Primary Lymphedema Differences of self-care status. As displayed in Table S2, compared to individuals with unilateral lower-extremity primary lymphedema, individuals with bilateral lower-extremity primary lymphedema were more likely to conduct skin care (χ 2 [1] = 8.29, Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Individuals With Lower-Extremity Primary Lymphedema
p = .004) and use alternative medications (χ 2 [1] = 8.01, p = .005), but less likely to use compression garments (χ 2 [1] = 4.20, p = .040). After controlling for the demographic differences (i.e., gender, ethnicity, country, family history of lymphedema, insurance coverage, and type of insurance) between the individuals with unilateral and those with bilateral conditions, these differences in self-care activities between the two groups of lower extremity lymphedema remained statistically significant.
Differences of symptoms. As shown in Table S3, with the exception of swelling, which was reported in approximately 99% of the respondents, individuals with bilateral lower-extremity primary lymphedema more frequently reported symptoms (p < .05). After controlling for the demographic differences between the individuals with unilateral and those with bilateral conditions, differences in the prevalence of four symptoms (pain, poor range of motion, stiffness, and heaviness) between the two groups of lower extremity lymphedema remained statistically significant. After controlling for the demographic differences, the difference in rates of numbness for those with unilateral compared to those with bilateral were no longer statistically significant (Wald χ 2 [1] = 0.65, p = .422). In terms of severity of symptoms, individuals with bilateral lower-extremity primary lymphedema reported experiencing much more severe symptoms than individuals with unilateral lowerextremity primary lymphedema (Mann-Whitney test, p < .05; see Table S3). Differences of infections. Individuals with bil– ateral lower-extremity primary lymphedema reported more infections than those with unilateral lower extremity primary lymphedema (257 of 469, 54.8% vs. 103 of 241, 42.7%, χ 2 [1] = 9.26, p = .002). Within the group of individuals with insurance and responses to the question regarding infections (n = 317), different rates of infection were reported dependent on the type of insurance held by the individual (χ 2 [1] = 14.19, p = .001). Within the group with private insurance, approximately 46% (95 of 209) reported at least one episode of infection. However, within the other insurance groups, rates of reported infections were higher (government: 45 of 65, 69.2%; other insurance: 28 of 43, 65.1%). After controlling for these differences in the type of insurance, the difference in rates of infection for those with unilateral compared to those with bilateral were no longer statistically significant (Wald χ 2 [1] = 0.12, p = .724). If an infection was reported, no statistically significant difference in the rates of hospitalizations due to infection were identified between the two groups (149 of 253, 58.9% vs. 69 of 103, 67.0%, χ 2 [1] = 2.02, p = .155). 129
Individuals With Lower-Extremity Primary Lymphedema
AIM 3: Associations of Self-Care With Symptoms and Infections Associations between self-care and symptoms. Among the self-care methods evaluated in this study, statistically significant associations were found between several methods of self-care and types of symptoms reported (Table S4, available with online version of this article). Compared to the group of participants who did not report using compression garments (n = 376), the group who reported their use of compression garments (n = 357) had lower percentages of reports of pain (69.5% vs. 79.5%, χ 2 [1] = 9.77, p = .002), poor range of motion (64.1% vs. 71.8%, χ 2 [1] = 4.95, p = .026), and numbness (53.2% vs. 76.8%, χ 2 [1] = 16.36, p < .001). To the contrary, higher rates of heaviness were reported for those who used bandaging (n = 189) than those who did not (n = 544, 93.7% vs. 88.4%, χ 2 [1] = 4.18, p = .041). Finally, relative to those who did not report using exercise (n = 491), those who did (n = 242) reported lower rates of pain (67.4% vs. 78.2%, χ 2 [1] = 10.08, p = .001; see Table S4).
Association between self-care and infection status. Summaries of the associations between self-care activities and reported infections are also presented in Table S4. Unexpectedly, higher rates of infection were observed within the group reporting the use of skin care than the group that did not (60.3% vs. 44.6%, χ 2 [1] = 15.89, p < .001; see Table S4). Among the alternative self-care activities (not shown), there were statistically significant associations of the use of pump and bandage alternatives with infection. Compared to respondents who did not report the use of pumps (n = 578), those who reported the use of pumps (n = 132) had higher rates of infectious episodes (68.2% vs. 46.7%, χ 2 [1] = 19.82, p < .001). A similar pattern was observed for the use of bandaging alternatives. Of those who reported the use of bandage alternatives (n = 137), 67.9% reported having an infection compared to 46.6% of those who did not report the use of bandage alternatives (n = 573, χ 2 [1] = 20.04, p < .001).
Association between symptoms and infection status. There was an association between the number of reported symptoms (of the six symptoms examined in this study) and reports of infection. Those reported having had an infection had a median of six symptoms (IQR: 4–6), while those not reporting an infection had a median four symptoms (IQR: 3–5, Mann-Whitney test, z = 7.80, p < .001). Furthermore, with the exception of swelling (experienced by almost all participants), as shown in Table S5 (available with the online version 130
Deng et al.
of this article), the presence of each of the individual symptoms was associated with a self-reported infection (χ 2 [1] > 20.94, p < .001).
Discussion There are no reports in the published literature focused exclusively on self-care in individuals with primary lymphedema, although several studies have examined selfcare status in individuals with secondary lymphedema. In addition to self-care, this study examined associations with symptoms and infections in individuals with lower-extremity primary lymphedema. Some important findings were identified.
Overall Self-Care Status, Symptoms, and Infections First, approximately two thirds (68.9%) of the participants with lower-extremity primary lymphedema conducted some daily self-care, which is a lower proportion than reported by individuals with secondary lymphedema. For example, one study reported that 88% (45 of 51) of breast cancer survivors performed some daily self-care activities for the management of arm lymphedema (Ridner, Dietrich, & Kidd, 2011). Another study found that 73% (60 of 82) of gynecologic cancer survivors with lower-extremity lymphedema conducted some daily lymphedema self-care activities (Ryan et al., 2003). According to the Oncology Nursing Society’s Putting Evidences into Practices (ONS PEP) recommendations (Oncology Nursing Society, 2013), NLN Position Papers (National Lymphedema Network, ¨ 2011), and professional textbooks (Foldi et al., 2007; Zuther, 2009), individuals with lymphedema need to conduct a self-care activity (Phase II CDT) including self-MLD, compression garments or bandaging, skin care, and exercise. Lymphedema self-care should be directed by certified lymphedema therapists prior to embarking on home self-care activities. However, we found that only 13.7% of the participants performed all of the above self-care activities. This finding indicates that studies are needed to investigate the underlying reasons that may impact lymphedema self-care. Interventional studies are desired to promote lymphedema self-care. Among the self-care components or methods of Phase II CDT, compression garments were the most frequently used method by the participants. Less than half of the participants used skin care, exercise, bandaging, and self-MLD. This finding is consistent with other reports. Two studies found that compression garments were the most commonly used method in individuals with breast cancer-related arm lymphedema (Ridner et al., 2011; Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Deng et al.
Ryan et al., 2003). One study reported that compression garments were the most common form of prescribed treatment in women with gynecologic cancer-related lower-extremity lymphedema (Ryan, et al., 2003). Currently no data are available to explain the reason why compression garments were the most frequently used method in individuals with extremity lymphedema, but there are several possible explanations. First, the compression garments are often a treatment option prescribed by primary healthcare providers (Ryan et al., 2003). Second, patients are often informed that the compression garments are necessary to keep the swelling controlled and reduce discomfort (Ryan et al., 2003). Third, the compression garments may be more easily used daily compared to other self-care methods (e.g., exercise and self-MLD). Although we did not survey the reasons why self-MLD, skin care, bandaging, and exercise were less frequently used by the participants in this study, one study described multiple barriers and burdens (e.g., lack of knowledge about how to complete self-care, physical discomfort, and time management) that influenced selfcare activities in breast cancer-related arm lymphedema (Ridner et al., 2011). In this study, approximately one third of the participants performed exercise as part of their daily self-care methods. There have been debates and controversies regarding which type of exercises would benefit lymphedema management. A recent systematic review of lymphedema self-care reports that there is evidence to support that whole body exercise is a beneficial self-care activity (Ridner et al., 2012). Although weight-lifting has been investigated in breast cancer survivors in the past few years, studies are needed to determine if weight-lifting or a similar exercise is safe and applicable for individuals with lower-extremity lymphedema (U.S. Department of Health and Human Services, National Institutes of Health, & National Cancer Institute, 2013). Thus, this population may be uncertain as to the risks versus benefits of exercise. In addition, one third of the study participants reported no self-care activities to manage their lymphedema, which raises concerns, especially as the patients who sought out participation in the study are likely to represent relatively highly-motivated patients with lymphedema, and this finding may actually overestimate self-care in this patient population. Although no research studies are available to document time for daily lymphedema self-care, we identified that approximately 50% of the participants used more than 30 min per day on daily self-care of lymphedema. This time range indicates a significant patient burden due to lymphedema self-care. One of the most important findings from this survey study was that a high percentage of individuJournal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Individuals With Lower-Extremity Primary Lymphedema
als with lower-extremity primary lymphedema reported a high symptom burden, most commonly including swelling, heaviness, stiffness, pain, poor range of motion, and numbness. Similar symptom burden has been reported in individuals with secondary upper or lower-extremity lymphedema (Armer, Radina, Porock, & Culbertson, 2003; Ridner, Deng, et al., 2012; Ridner et al., 2011; Ryan et al., 2003). In addition, 44.8% of the individuals with lower-extremity primary lymphedema reported lymphedema-related infection. Although medical records were not available to confirm the accuracy of self-reported lymphedema-related infection, it is assumed that such infections represent distinct manifestations and unique symptoms of infection (e.g., red blotches, itching, erythema, increased swelling), which can easily be identified by educated individuals with lymphedema. In our previous qualitative study of breast cancer survivors with arm lymphedema, patients indicated their fear of lymphedema-related infection and their efforts to reduce infection risk (Ridner, Bonner, Deng, & Sinclair, 2012). These studies clearly demonstrated the need and importance of improvement of symptom assessment, management, and infection control in individuals with primary lymphedema.
Differences of Self-Care Status, Symptoms, and Infections Among Respondents With Unilateral or Bilateral Lower-Extremity Primary Lymphedema Another notable finding in this study is that significant differences of self-care, symptoms, and reported infections were identified between individuals with unilateral or bilateral lower-extremity primary lymphedema. First, compared to those with unilateral lower-extremity primary lymphedema, individuals with bilateral lower-extremity primary lymphedema were more likely to conduct skin care and use alternative medicines, although they were less likely to use compression garments. Second, it was found that individuals with bilateral lower-extremity primary lymphedema had a much higher symptom burden and increased likelihood of infections compared to those with unilateral lower-extremity primary lymphedema. Thus, healthcare professionals need to be aware that differences in self-care, symptoms, and infections may exist among individuals with different anatomical sites of lymphedema.
Associations Among Self-Care, Symptoms, and Infections Due to the limitations associated with a cross-sectional study, we were unable to identify causal relationships 131
Individuals With Lower-Extremity Primary Lymphedema
among self-care, symptoms, and reported infections. However, our preliminary findings did suggest possible associations among these variables. Although no statistically significant associations were found between symptoms and self-MLD, we did find that individuals who reported using compression garments were less likely to have self-reported pain, poor range of motion, and numbness. Although no randomized controlled trials (RCTs) are available to evaluate benefits of compression garments, it has been reported as the most commonly used self-care method in individuals with secondary lymphedema. Compression garments may generate appropriate pressure on muscles and soft tissues to facilitate lymph flow, which may decrease symptoms caused by accumulated lymph fluid in the lymphedema-affected area. We found that individuals conducting exercise were less likely to have self-reported pain. Two RCTs have reported the benefits of exercise in decreasing secondary lymphedema-related symptoms (Sandel et al., 2005; Schmitz et al., 2009). We also identified that there was a statistically significant association between increased use of bandaging and higher rate of heaviness. This finding may indicate that individuals with heaviness were more likely to use bandaging to manage their symptom. Although we cannot confirm an underlying relationship between the two variables due to a cross-sectional design, this finding implies that future studies are needed to identify if bandaging improves a patient’s symptom of heaviness. Moreover, we found that there was a statistically significant association between a reported history of infection and the use of skin care. Due to the nature of the cross-sectional study, we could not determine a causal relationship between these two variables. This finding may imply that infection may be a driving factor that stimulates patients’ self-care activities directed toward the skin because meticulous and appropriate skin care is pivotal for infection control. One study found that skin care, when combined with self-MLD, provided symptomatic relief and reduced limb volume (Barclay, Vestey, Lambert, & Balmer, 2006). Nevertheless, if patients do not receive or follow proper instructions about skin care (i.e., if they do not perform skin care correctly), patients may be at risk for infection episodes. We also identified that a low percentage of participants used alternative treatments for lymphedema self-care. Interestingly, the study identified that the participants with a history of infection were more likely to use pumps and bandage alternatives. These findings indicate that more studies are needed to evaluate the effect of alternative treatments on symptom management and infection control in individuals with primary lymphedema. 132
Deng et al.
Study Strengths There are several strengths associated with this study. The study focused on an under-reported condition (lower-extremity lymphedema) in an understudied population (individuals with primary lymphedema). Due to the relatively large sample size of the cohort, we were able to examine the self-care status, symptom burden, and reported infections in individuals with lower-extremity primary lymphedema. Some possible associations among these variables were identified, which could provide a basis for future descriptive and interventional studies. We also compared and identified differences in self-care, symptom burden, and infections among individuals with unilateral and bilateral lowerextremity primary lymphedema. These findings highlight the importance of self-care, symptom assessment and management, infection control, and risk reduction practices in individuals with primary lymphedema, and also identify areas for future research in this population.
Study Limitations There are also a number of limitations in the study. First, data were collected from a survey study, which is associated with selection bias. It is possible that individuals with more severe symptoms and episodes of infection were more likely to participate in this survey than those with mild symptom burden and fewer infections. Further, these participants may have been more (or less) motivated for self-care than nonparticipants. We did not know the respondent rates via this survey study. This also increases the risk for selection bias. Secondly, as in all observational studies, causal relationships cannot be identified. Thus, we must be particularly cautious to explain our findings. In other words, we only evaluated associations among self-care status, symptoms, and reported infections and did not intend to predict any underlying relationships among the study variables. Third, participants’ infection data were also based on self-report; thus, the accuracy of reported infections may be another confounding factor. Fourth, we did not collect data on severity of lymphedema in the sample, which may be a critical variable to influence self-care, symptoms, and infections.
Conclusions One third of the respondents with lower-extremity primary lymphedema reported no self-care activities. Future studies should aim to explore the underlying reasons why some patients with lower limb primary lymphedema conduct self-care and some do not, such as whether those without self-care are aware of the Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Deng et al.
importance of self-care and whether this can be attributed to difficulty in gaining access to necessary resources of self-care (e.g., financial support, educational information, hands-on self-care skills, etc.), whether time constraints exist for self-care, whether other chronic conditions or comorbidities limit the ability to conduct self-care activities, or whether patients have conducted self-care in the past and quit because they did not perceive a benefit. Opportunities exist for healthcare professionals to educate patients with lymphedema regarding the importance of self-care and to evaluate whether patients have the capabilities and resources to conduct self-care activities. Interventional studies may be required to identify effective strategies to improve their self-care status and tailoring of self-care tasks to meet individual patient needs. In this cohort group, symptom burden was high and infection episodes were frequent, which implies that improvement of symptom management and infection control is critically important. We also identified that the respondents with bilateral lower-extremity primary lymphedema experienced more severe symptom burden and infectious complications compared to the respondents with unilateral lower-extremity primary lymphedema. Tailored interventions need to be developed for this patient population. Clearly, more studies are required to address this under-recognized and underinvestigated health issue to improve the quality of life among patients with lower-extremity primary lymphedema.
Clinical Resources
r r
National Lymphedema Network, NLN Position Papers: http://www.lymphnet.org/le-faqs/nlnposition-papers Oncology Nursing Society, Lymphedema: https:// www.ons.org/practice-resources/pep/lymphedema
References Armer, J. M., Radina, M. E., Porock, D., & Culbertson, S. D. (2003). Predicting breast cancer-related lymphedema using self-reported symptoms. Nursing Research, 52(6), 370– 379. Barclay, J., Vestey, J., Lambert, A., & Balmer, C. (2006). Reducing the symptoms of lymphoedema: Is there a role for aromatherapy? European Journal of Oncology Nursing, 10(2), 140–149. doi:10.1016/j.ejon.2005.10.008 Bernaudin, J. F., Kambouchner, M., & Lacave, R. (2013). [Lymphatic vascular system, development and lymph formation. Review]. Revue de Pneumologie Clinique, 69(2), 93–101. doi:10.1016/j.pneumo.2013.01.005
Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Individuals With Lower-Extremity Primary Lymphedema
Brorson, H., Ohlin, K., Olsson, G., Svensson, B., & Svensson, H. (2008). Controlled compression and liposuction treatment for lower extremity lymphedema. Lymphology, 41(2), 52–63. Connell, F., Ostergaard, P., Carver, C., Brice, G., Williams, N., Mansour, S., et al. (2009). Analysis of the coding regions of VEGFR3 and VEGFC in Milroy disease and other primary lymphoedemas. Human Genetics, 124(6), 625–631. doi:10.1007/s00439-008-0586-5 ¨ ¨ ¨ Foldi, M. D., Foldi, E., Strossenreuther, R. H. K., & Kubik, S. (Eds.). (2007). F¨oldi’s textbook of lymphology: For physicians and lymphedema therapists (2nd ed.). Munich, Germany: Elsevier, Urban & Fischer Verlag. Lee, B.-B., Bergan, J. J., & Rockson, S. G. (2011). Lymphedema: A concise compendium of the theory and practice. London, England: Springer. National Lymphedema Network. (2011, February 1). NLN position papers. Retrieved from http://www.lymphnet.org/ resources/nln-position-paper-the-diagnosis-and-treatmentof-lymphedema Okajima, S., Hirota, A., Kimura, E., Inagaki, M., Tamai, N., Iizaka, S., et al. (2013). Health-related quality of life and associated factors in patients with primary lymphedema. Japan Journal of Nursing Science, 10(2), 202–211. doi:10.1111/j.1742-7924.2012.00220.x Oncology Nursing Society. (2014, April 4). Lymphedema. Retrieved from http://www.ons.org/Research/PEP/ Lymphedema Ridner, S. H., Bonner, C. M., Deng, J., & Sinclair, V. G. (2012). Voices from the shadows: Living with lymphedema. Cancer Nursing, 35(1), E18–E26. doi:10.1097/NCC.0b013e31821404c0 Ridner, S. H., Deng, J., Fu, M. R., Radina, E., Thiadens, S. R., Weiss, J., et al. (2012). Symptom burden and infection occurrence among individuals with extremity lymphedema. Lymphology, 45(3), 113–123. Ridner, S. H., Dietrich, M. S., & Kidd, N. (2011). Breast cancer treatment-related lymphedema self-care: Education, practices, symptoms, and quality of life. Supportive Care in Cancer, 19(5), 631–637. doi:10.1007/s00520-010-0870-5 Ridner, S. H., Fu, M. R., Wanchai, A., Stewart, B. R., Armer, J. M., & Cormier, J. N. (2012). Self-management of lymphedema: A systematic review of the literature from 2004 to 2011. Nursing Research, 61(4), 291–299. doi:10.1097/NNR.0b013e31824f82b2 Ryan, M., Stainton, M. C., Jaconelli, C., Watts, S., MacKenzie, P., & Mansberg, T. (2003). The experience of lower limb lymphedema for women after treatment for gynecologic cancer. Oncology Nursing Forum, 30(3), 417–423. doi:10.1188/03.ONF.417-423 Sandel, S. L., Judge, J. O., Landry, N., Faria, L., Ouellethe, R., & Majczak, M. (2005). Dance and movement program improves quality-of-life measures in breast cancer survivors. Cancer Nursing, 28(4), 301–309.
133
Individuals With Lower-Extremity Primary Lymphedema
Schmitz, K. H., Ahmed, R. L., Troxel, A., Cheville, A., Smith, R., Lewis-Grant, L., et al. (2009). Weight lifting in women with breast cancer-related lymphedema. New England Journal of Medicine, 361(7), 664–673. doi:10.1056/NEJMoa0810118 Schook, C. C., Mulliken, J. B., Fishman, S. J., Grant, F. D., Zurakowski, D., & Greene, A. K. (2010). Primary lymphedema: Clinical features and management in 138 pediatric patients. Plastic and Reconstructive Surgery, 127(6), 2419–2431. doi:10.1097/PRS.0b013e318213a218 Symvoulakis, E. K., Anyfantakis, D. I., & Lionis, C. (2010). Primary lower limb lymphedema: A focus on its functional, social, and emotional impact. International Journal of Medical Sciences, 7(6), 353–357. U.S. Department of Health and Human Services, National Institutes of Health, & National Cancer Institute. (2013, R April 10). Lymphedema (PDQ ): Management. Retrieved from http://www.cancer.gov/cancertopics/pdq/supportivecare/ lymphedema/healthprofessional/page2 Zuther, J. E. (2009). Lymphedema management: The comprehensive guide for practitioners (2nd ed.). New York, NY: Thieme.
134
Deng et al.
Supporting Information Additional Supporting Information may be found in the online version of this article at the publisher’s web site: Table S1: Characteristics of Respondent Cohort Stratified by Unilateral (n = 269) Versus Bilateral (n = 534) Lower Extremity Primary Lymphedema (Overall n = 803). Table S2: Self–Care of Respondent Cohort Stratified by Unilateral (n = 269) Versus Bilateral (n = 534) Lower Extremity Primary Lymphedema (Overall n = 803). Table S3: Reported Symptoms Among Respondents With Unilateral and Bilateral Lower–Extremity Primary Lymphedema. Table S4: Self–Care, Reported Symptoms, and Infections Among Respondents With Lower–Extremity Primary Lymphedema. Table S5: Reported Symptoms and Infections Among Respondents With Lower–Extremity Primary Lymphedema (N = 665).
Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Copyright of Journal of Nursing Scholarship is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Self-Care Status, Symptom Burden, and Reported Infections in Individuals With Lower-Extremity Primary Lymphedema Jie Deng, PhD, RN, OCN1 , Elise Radina, PhD, CFLE2 , Mei R. Fu, PhD, RN, APRN-BC, FAAN3 , Jane M. Armer, PhD, RN, FAAN4 , Janice N. Cormier, MD, MPH5 , Saskia R.J. Thiadens, RN6 , Jan Weiss, PT, DHS, CLT-LANA7 , Catherine M. Tuppo, PT, MS, CLT-LANA8 , Mary S. Dietrich, PhD9 , & Sheila H. Ridner, PhD, RN, FAAN10 1 Assistant Professor, School of Nursing, Vanderbilt University, Nashville, TN, USA 2 Professor, Department of Family Studies & Social Work, Miami University, Oxford, OH, USA 3 Associate Professor, College of Nursing, New York University, New York, NY, USA 4 Professor, Sinclair School of Nursing, University of Missouri, Columbia, MO, USA 5 Professor, UT MD Anderson Cancer Center, The University of Texas, Houston, TX, USA 6 Executive Director, National Lymphedema Network, San Francisco, CA, USA 7 Lymphedema Resource Therapist, CoxHealth Outpatient Rehabilitation, Springfield, MO, USA 8 Program Coordinator, Bariatric and Metabolic Weight Loss Center, Stony Brook Medicine, Stony Brook, NY, USA 9 Professor, School of Nursing, Vanderbilt University, Nashville, TN, USA 10 Professor, School of Nursing, Vanderbilt University, Nashville, TN, USA
Key words Primary lymphedema, lower extremity, symptom, infection, self-care Correspondence Dr. Jie Deng, School of Nursing, Vanderbilt University, 461 21st Ave. South, 516 Godchaux Hall, Nashville, TN 37240 USA. E-mail: [email protected] Accepted: October 4, 2014 doi: 10.1111/jnu.12117
126
Abstract Purpose: The purposes of this study were (a) to evaluate self-care, symptom burden, and reported infections among individuals with lower-extremity primary lymphedema; (b) to examine the differences in self-care, symptom burden, and reported infections between individuals with unilateral and those with bilateral lower-extremity primary lymphedema; and (c) to examine the associations among self-care status, symptom burden, and reported infections in individuals with lower-extremity primary lymphedema. Design: A secondary data analysis was used. Data were collected from a crosssectional survey study supported by the National Lymphedema Network from March 2006 through January 2010. The surveys were available both online and in hard copy in order to increase accessibility. Methods: Descriptive statistics were conducted and associations between variables were assessed using Mann-Whitney tests and chi-square tests of independence. Multiple logistic regression was used to test for associations while controlling for potentially confounding variables. Findings: A total of 803 participants reported having lower-extremity primary lymphedema. The majority of the participants were female (82.9%), White (74.2%), and from the United States (90.7%). Approximately two thirds of the respondents conducted some home daily lymphedema self-care. Over half of the respondents reported experiencing symptom burden and 44.8% reported at least one episode of infection. Compared to individuals with unilateral lower-extremity primary lymphedema, individuals with bilateral lower-extremity lymphedema were more likely to conduct skin care (p = .004), use alternative medications (p = .005), more frequently reported symptoms (p < .05), and more likely to report at least one episode of infection (p = .002). Respondents who reported use of compression garments also were less likely to have self-reported pain (p = .002), poor range of motion (p = .026), and numbness (p = .001). Participants who reported exercising also were less likely to have self-reported pain (p = .003). Participants who reported at least one episode of infection also reported experiencing more symptoms (p < .001). Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Individuals With Lower-Extremity Primary Lymphedema
Deng et al.
Conclusions: Individuals with lower-extremity primary lymphedema experienced substantial symptom burden and infection episodes. Significant associations were identified among self-care, symptom burden, and reported infections. Clinical Relevance: The findings support the need for clinicians to educate patients with lower-extremity primary lymphedema regarding the importance of self-care, symptom management, and infection control. It is critically important for clinicians to evaluate symptom burden and reduce infections in individuals with lower-extremity primary lymphedema.
Lymphedema is a chronic and serious condition, affecting more than 140 million people worldwide (Brorson, Ohlin, Olsson, Svensson, & Svensson, 2008). Lymphedema occurs when the lymphatic system fails to transport lymph fluid to the circulatory system due to either primary or secondary causes. Primary lymphedema is related to a developmental defect (malformation, ¨ dysplasia) of the lymph vessels or lymph nodes (Foldi, ¨ ¨ Foldi, Strossenreuther, & Kubik, 2007). Studies have reported an association between several genes, including VEGFR-3, VEGFC, and FOXC2, and primary lymphedema (Bernaudin, Kambouchner, & Lacave, 2013; Connell et al., 2009). Primary lymphedema can occur without obvious causes at different developmental stages, such as at birth, at puberty, or, as with lymphedema tarda, later in life (Zuther, 2009). Secondary lymphedema usually occurs as a consequence of cancer, cancer treatment, infection, or trauma (Lee, Bergan, & Rockson, 2011). Although no curative surgical or medical intervention is available for lymphedema, expert opinion supports that current standard for treatment of either primary or secondary lymphedema is two-phase complete de¨ congestive therapy (CDT; Foldi et al., 2007; Zuther, 2009), which includes the intensive phase (Phase I) provided by a certified lymphedema therapist and selfcare phase (Phase II) administrated by the patient or a caregiver. Phase II of CDT includes self-manual lymphatic drainage (self-MLD), compression (i.e., garments ¨ or bandaging), skin care, and exercise (Foldi et al., 2007; Zuther, 2009). Although the exact mechanisms of the two-phase CDT have not been clearly described, the underlying pathophysiological foundation of the CDT supports that both phases may be able to (a) improve the function of lymph vessels, (b) soften the fibrosclerotic indurations, (c) reduce increased connective tissue, (d) cleanse the skin to prevent opportunistic infections, and (e) reduce associated symptom burden (Lee et al., 2011). These potential mechanisms support the importance of self-care (Phase II of CDT) and suggest potential relationships among self-care, symptom burden, and infection.
Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Because lymphedema is a chronic and incurable condition, long-term daily self-care activities for lymphedema are critical to manage lymphedema-related symptom burden, functional impairments, and episodes of infection. Though empirical lymphedema self-care evidence is scant, a recent systematic review of the literature from 2004 to 2011 on self-care among patients with lymphedema identified only 16 articles focused on lymphedema self-care (Ridner, Fu, et al., 2012). Most of the articles evaluated self-care activities in individuals with secondary lymphedema and no article focused exclusively on self-care among patients with primary lymphedema. Four of the articles reported on a subgroup of study participants with primary lymphedema (Ridner, Fu, et al., 2012). Although little is known about the symptom burden experienced by patients with primary lymphedema, or infection, some studies have supported that individuals with primary lymphedema likely experienced distressful symptoms (e.g., pain or discomfort) and functional impairments (e.g., usual activities; Okajima et al., 2013; Schook et al., 2010; Symvoulakis, Anyfantakis, & Lionis, 2010), and reported frequent infection in the lymphedema-affected area. For instance, one article identified that 31.3% (26 out of 83) of the participants reported cellulitis (lymphedema-related infection; Okajima et al., 2013); another article found that 18.8% (26 out of 138) of the participants developed cellulitis (Schook et al., 2010). To date, there are no known studies that have investigated the associations between self-care, symptom burden, and infections in individuals with primary lymphedema. In order to address the gaps in the literature, the National Lymphedema Network (NLN) conducted a patient-reported survey study to collect data on lymphedema and its management. Among the respondents with primary lymphedema, the most common affected site was the lower extremity. The aims of this report are to identify (a) the current status of self-care, symptom burden, and infections among patients with lower-extremity primary lymphedema; (b) whether or not there are differences in self-care, self-reported symptom burden, and
127
Individuals With Lower-Extremity Primary Lymphedema
infection occurrence among respondents with unilateral or bilateral lower-extremity primary lymphedema; and (c) the associations among self-care status, symptom burden, and infections in this cohort group.
Design A secondary data analysis was used. All data were collected as part of the NLN cross-sectional survey study (Ridner, Deng, et al., 2012). Designed by the NLN Research Subcommittee, the survey collected the following self-reported information relevant to this study: demographics, type and anatomic sites of lymphedema, characteristics of primary lymphedema (e.g., onset age of lymphedema and family history), history of surgical treatment, associated symptoms (six symptoms were evaluated, including pain, poor range of motion, numbness, stiffness, heaviness, and swelling), infection status, self-care status (self-care defined as Phase II of the CDT, including self-MLD, compression garments or bandaging, skin-care, and exercise), and alternative self-care activities.
Data Collection The study was approved by the Institutional Review Board (IRB) at the University of Missouri Health Sciences. The study was conducted from March 2006 to January 2010. Individuals with or at risk for lymphedema were eligible to participate in this survey study. The survey was available and accessible to individuals within the United States and worldwide via the NLN website (www.lymphet.org). Study participants who had no access to the website were able to complete a hard-copy version of the survey and submit the survey via mail. Data were entered into the database by staff in the NLN office. Individuals’ completion or return of the survey served as consent to participate in the study. Detailed information about the survey development and administration has been reported in previous publications (Ridner, Deng, et al., 2012). Secondary data analysis was approved by the IRB at Vanderbilt University.
Sample From March 2006 through January 2010, 2,968 surveys were received. After the data cleaning process (i.e., deletion of duplicate surveys, n = 220; invalid responses, n = 14), a total of 2,734 respondent surveys were available for analysis. Out of these respondents, 830 individuals reported having primary lymphedema. Among individuals with primary lymphedema, the majority of respondents, 96.7% (n = 803), reported having 128
Deng et al.
lower-extremity primary lymphedema (including 269 with unilateral lower-extremity primary lymphedema and 534 with bilateral lower-extremity primary lymphedema). For the purpose of this report, we examined participant characteristics, self-care status, symptoms, and infections among respondents with either unilateral or bilateral lower-extremity primary lymphedema.
Data Analysis Data were analyzed using the statistical software package SPSS version 20.0 (International Business Machines Corp., Armonk, NY, USA). The data were verified and cleaned by screening for duplicate cases and missing data, which were omitted from the study sample. Descriptive statistics (frequency distributions, median and 25th-75th interquartile range [IQR]) were used to describe the sample characteristics and distributions of the variables. Tests of the differences between groups (e.g., between unilateral and bilateral) were conducted using Mann-Whitney tests. Chi-square tests of independence were used to test for associations between variables that were nominal in nature. Multiple logistic regression was used to test for associations while controlling for potentially confounding variables. Decisions regarding statistical significance used p values below .05.
Results Sample Characteristics Table S1 summarizes the characteristics of respondents with lower-extremity primary lymphedema. The majority of the respondents were female (82.9%), White (74.2%), and from the United States (90.7%). Approximately one third of respondents had a family history of lymphedema. Fifty percent of the respondents had insurance coverage. There were statistically significant differences noted for gender, ethnicity, country, family history of lymphedema (yes or no), insurance coverage, and type of insurance among respondents with unilateral or bilateral lower-extremity primary lymphedema. Compared to respondents with unilateral lower-extremity primary lymphedema, individuals with bilateral lower-extremity primary lymphedema were more likely to be from the United States, female, and White, have a family history of lymphedema, insurance coverage, and, if covered, insurance from government support. There was no statistically significant difference in the onset age of primary lymphedema between individuals with unilateral or bilateral lower-extremity primary lymphedema (see Table S1, available with the online version of this article). Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Deng et al.
AIM 1: Overall Self-Care Status, Symptoms, and Infections Self-care status. As displayed in Table S2 (available with the online version of this article), the majority (68.9%) of the respondents conducted some home daily lymphedema self-care. The self-care methods of Phase II CDT used (from high to low frequency) included compression garments (48.1%), skin care (40.1%), exercise (32.6%), bandaging (26.4%), and self-MLD (20.7%). Approximately 50% of the respondents reported that they spent more than 30 min per day on self-care. Also included in Table S2 are the reported alternative treatments of self-care. Less than 50% of the respondents used alternative treatments for self-care. The alternative treatments used among individuals with lower-extremity primary lymphedema (from high to low frequency) were bandage alternatives (19.1%), pumps (17.9%), medications (12.5%), herbal substitutes (5.9%), and yoga (3.6%). Of note, 13% of the respondents perceived that alternative treatment was more effective than Phase II CDT (see Table S2). Symptom profiles. Regardless of whether lymphedema involved unilateral or bilateral lower extremities, a high percentage of individuals reported associated symptoms (Table S3, available with online version of this article). The prevalence of reported symptoms in descending order were swelling (98.9%), heaviness (89.8%), stiffness (78.6%), pain (74.6%), poor range of motion (68.1%), and numbness (60.7%). Of the respondents experiencing each symptom, the respective reported severity was highest for heaviness (median = 4.0 on a scale of 1 [little] to 5 [severe]; see Table S3). Infection status. A high percentage of individuals with lower-extremity primary lymphedema reported infections since the first onset of lymphedema in their lower extremities. A total of 44.8% (360 of 803) reported at least one episode of infection, and 27.1% (218 of 803) with at least one admittance into the hospital due to lymphedema-related infection.
AIM 2: Differences of Self-Care Status, Symptoms, and Infections Among Respondents With Unilateral or Bilateral Lower-Extremity Primary Lymphedema Differences of self-care status. As displayed in Table S2, compared to individuals with unilateral lower-extremity primary lymphedema, individuals with bilateral lower-extremity primary lymphedema were more likely to conduct skin care (χ 2 [1] = 8.29, Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Individuals With Lower-Extremity Primary Lymphedema
p = .004) and use alternative medications (χ 2 [1] = 8.01, p = .005), but less likely to use compression garments (χ 2 [1] = 4.20, p = .040). After controlling for the demographic differences (i.e., gender, ethnicity, country, family history of lymphedema, insurance coverage, and type of insurance) between the individuals with unilateral and those with bilateral conditions, these differences in self-care activities between the two groups of lower extremity lymphedema remained statistically significant.
Differences of symptoms. As shown in Table S3, with the exception of swelling, which was reported in approximately 99% of the respondents, individuals with bilateral lower-extremity primary lymphedema more frequently reported symptoms (p < .05). After controlling for the demographic differences between the individuals with unilateral and those with bilateral conditions, differences in the prevalence of four symptoms (pain, poor range of motion, stiffness, and heaviness) between the two groups of lower extremity lymphedema remained statistically significant. After controlling for the demographic differences, the difference in rates of numbness for those with unilateral compared to those with bilateral were no longer statistically significant (Wald χ 2 [1] = 0.65, p = .422). In terms of severity of symptoms, individuals with bilateral lower-extremity primary lymphedema reported experiencing much more severe symptoms than individuals with unilateral lowerextremity primary lymphedema (Mann-Whitney test, p < .05; see Table S3). Differences of infections. Individuals with bil– ateral lower-extremity primary lymphedema reported more infections than those with unilateral lower extremity primary lymphedema (257 of 469, 54.8% vs. 103 of 241, 42.7%, χ 2 [1] = 9.26, p = .002). Within the group of individuals with insurance and responses to the question regarding infections (n = 317), different rates of infection were reported dependent on the type of insurance held by the individual (χ 2 [1] = 14.19, p = .001). Within the group with private insurance, approximately 46% (95 of 209) reported at least one episode of infection. However, within the other insurance groups, rates of reported infections were higher (government: 45 of 65, 69.2%; other insurance: 28 of 43, 65.1%). After controlling for these differences in the type of insurance, the difference in rates of infection for those with unilateral compared to those with bilateral were no longer statistically significant (Wald χ 2 [1] = 0.12, p = .724). If an infection was reported, no statistically significant difference in the rates of hospitalizations due to infection were identified between the two groups (149 of 253, 58.9% vs. 69 of 103, 67.0%, χ 2 [1] = 2.02, p = .155). 129
Individuals With Lower-Extremity Primary Lymphedema
AIM 3: Associations of Self-Care With Symptoms and Infections Associations between self-care and symptoms. Among the self-care methods evaluated in this study, statistically significant associations were found between several methods of self-care and types of symptoms reported (Table S4, available with online version of this article). Compared to the group of participants who did not report using compression garments (n = 376), the group who reported their use of compression garments (n = 357) had lower percentages of reports of pain (69.5% vs. 79.5%, χ 2 [1] = 9.77, p = .002), poor range of motion (64.1% vs. 71.8%, χ 2 [1] = 4.95, p = .026), and numbness (53.2% vs. 76.8%, χ 2 [1] = 16.36, p < .001). To the contrary, higher rates of heaviness were reported for those who used bandaging (n = 189) than those who did not (n = 544, 93.7% vs. 88.4%, χ 2 [1] = 4.18, p = .041). Finally, relative to those who did not report using exercise (n = 491), those who did (n = 242) reported lower rates of pain (67.4% vs. 78.2%, χ 2 [1] = 10.08, p = .001; see Table S4).
Association between self-care and infection status. Summaries of the associations between self-care activities and reported infections are also presented in Table S4. Unexpectedly, higher rates of infection were observed within the group reporting the use of skin care than the group that did not (60.3% vs. 44.6%, χ 2 [1] = 15.89, p < .001; see Table S4). Among the alternative self-care activities (not shown), there were statistically significant associations of the use of pump and bandage alternatives with infection. Compared to respondents who did not report the use of pumps (n = 578), those who reported the use of pumps (n = 132) had higher rates of infectious episodes (68.2% vs. 46.7%, χ 2 [1] = 19.82, p < .001). A similar pattern was observed for the use of bandaging alternatives. Of those who reported the use of bandage alternatives (n = 137), 67.9% reported having an infection compared to 46.6% of those who did not report the use of bandage alternatives (n = 573, χ 2 [1] = 20.04, p < .001).
Association between symptoms and infection status. There was an association between the number of reported symptoms (of the six symptoms examined in this study) and reports of infection. Those reported having had an infection had a median of six symptoms (IQR: 4–6), while those not reporting an infection had a median four symptoms (IQR: 3–5, Mann-Whitney test, z = 7.80, p < .001). Furthermore, with the exception of swelling (experienced by almost all participants), as shown in Table S5 (available with the online version 130
Deng et al.
of this article), the presence of each of the individual symptoms was associated with a self-reported infection (χ 2 [1] > 20.94, p < .001).
Discussion There are no reports in the published literature focused exclusively on self-care in individuals with primary lymphedema, although several studies have examined selfcare status in individuals with secondary lymphedema. In addition to self-care, this study examined associations with symptoms and infections in individuals with lower-extremity primary lymphedema. Some important findings were identified.
Overall Self-Care Status, Symptoms, and Infections First, approximately two thirds (68.9%) of the participants with lower-extremity primary lymphedema conducted some daily self-care, which is a lower proportion than reported by individuals with secondary lymphedema. For example, one study reported that 88% (45 of 51) of breast cancer survivors performed some daily self-care activities for the management of arm lymphedema (Ridner, Dietrich, & Kidd, 2011). Another study found that 73% (60 of 82) of gynecologic cancer survivors with lower-extremity lymphedema conducted some daily lymphedema self-care activities (Ryan et al., 2003). According to the Oncology Nursing Society’s Putting Evidences into Practices (ONS PEP) recommendations (Oncology Nursing Society, 2013), NLN Position Papers (National Lymphedema Network, ¨ 2011), and professional textbooks (Foldi et al., 2007; Zuther, 2009), individuals with lymphedema need to conduct a self-care activity (Phase II CDT) including self-MLD, compression garments or bandaging, skin care, and exercise. Lymphedema self-care should be directed by certified lymphedema therapists prior to embarking on home self-care activities. However, we found that only 13.7% of the participants performed all of the above self-care activities. This finding indicates that studies are needed to investigate the underlying reasons that may impact lymphedema self-care. Interventional studies are desired to promote lymphedema self-care. Among the self-care components or methods of Phase II CDT, compression garments were the most frequently used method by the participants. Less than half of the participants used skin care, exercise, bandaging, and self-MLD. This finding is consistent with other reports. Two studies found that compression garments were the most commonly used method in individuals with breast cancer-related arm lymphedema (Ridner et al., 2011; Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Deng et al.
Ryan et al., 2003). One study reported that compression garments were the most common form of prescribed treatment in women with gynecologic cancer-related lower-extremity lymphedema (Ryan, et al., 2003). Currently no data are available to explain the reason why compression garments were the most frequently used method in individuals with extremity lymphedema, but there are several possible explanations. First, the compression garments are often a treatment option prescribed by primary healthcare providers (Ryan et al., 2003). Second, patients are often informed that the compression garments are necessary to keep the swelling controlled and reduce discomfort (Ryan et al., 2003). Third, the compression garments may be more easily used daily compared to other self-care methods (e.g., exercise and self-MLD). Although we did not survey the reasons why self-MLD, skin care, bandaging, and exercise were less frequently used by the participants in this study, one study described multiple barriers and burdens (e.g., lack of knowledge about how to complete self-care, physical discomfort, and time management) that influenced selfcare activities in breast cancer-related arm lymphedema (Ridner et al., 2011). In this study, approximately one third of the participants performed exercise as part of their daily self-care methods. There have been debates and controversies regarding which type of exercises would benefit lymphedema management. A recent systematic review of lymphedema self-care reports that there is evidence to support that whole body exercise is a beneficial self-care activity (Ridner et al., 2012). Although weight-lifting has been investigated in breast cancer survivors in the past few years, studies are needed to determine if weight-lifting or a similar exercise is safe and applicable for individuals with lower-extremity lymphedema (U.S. Department of Health and Human Services, National Institutes of Health, & National Cancer Institute, 2013). Thus, this population may be uncertain as to the risks versus benefits of exercise. In addition, one third of the study participants reported no self-care activities to manage their lymphedema, which raises concerns, especially as the patients who sought out participation in the study are likely to represent relatively highly-motivated patients with lymphedema, and this finding may actually overestimate self-care in this patient population. Although no research studies are available to document time for daily lymphedema self-care, we identified that approximately 50% of the participants used more than 30 min per day on daily self-care of lymphedema. This time range indicates a significant patient burden due to lymphedema self-care. One of the most important findings from this survey study was that a high percentage of individuJournal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Individuals With Lower-Extremity Primary Lymphedema
als with lower-extremity primary lymphedema reported a high symptom burden, most commonly including swelling, heaviness, stiffness, pain, poor range of motion, and numbness. Similar symptom burden has been reported in individuals with secondary upper or lower-extremity lymphedema (Armer, Radina, Porock, & Culbertson, 2003; Ridner, Deng, et al., 2012; Ridner et al., 2011; Ryan et al., 2003). In addition, 44.8% of the individuals with lower-extremity primary lymphedema reported lymphedema-related infection. Although medical records were not available to confirm the accuracy of self-reported lymphedema-related infection, it is assumed that such infections represent distinct manifestations and unique symptoms of infection (e.g., red blotches, itching, erythema, increased swelling), which can easily be identified by educated individuals with lymphedema. In our previous qualitative study of breast cancer survivors with arm lymphedema, patients indicated their fear of lymphedema-related infection and their efforts to reduce infection risk (Ridner, Bonner, Deng, & Sinclair, 2012). These studies clearly demonstrated the need and importance of improvement of symptom assessment, management, and infection control in individuals with primary lymphedema.
Differences of Self-Care Status, Symptoms, and Infections Among Respondents With Unilateral or Bilateral Lower-Extremity Primary Lymphedema Another notable finding in this study is that significant differences of self-care, symptoms, and reported infections were identified between individuals with unilateral or bilateral lower-extremity primary lymphedema. First, compared to those with unilateral lower-extremity primary lymphedema, individuals with bilateral lower-extremity primary lymphedema were more likely to conduct skin care and use alternative medicines, although they were less likely to use compression garments. Second, it was found that individuals with bilateral lower-extremity primary lymphedema had a much higher symptom burden and increased likelihood of infections compared to those with unilateral lower-extremity primary lymphedema. Thus, healthcare professionals need to be aware that differences in self-care, symptoms, and infections may exist among individuals with different anatomical sites of lymphedema.
Associations Among Self-Care, Symptoms, and Infections Due to the limitations associated with a cross-sectional study, we were unable to identify causal relationships 131
Individuals With Lower-Extremity Primary Lymphedema
among self-care, symptoms, and reported infections. However, our preliminary findings did suggest possible associations among these variables. Although no statistically significant associations were found between symptoms and self-MLD, we did find that individuals who reported using compression garments were less likely to have self-reported pain, poor range of motion, and numbness. Although no randomized controlled trials (RCTs) are available to evaluate benefits of compression garments, it has been reported as the most commonly used self-care method in individuals with secondary lymphedema. Compression garments may generate appropriate pressure on muscles and soft tissues to facilitate lymph flow, which may decrease symptoms caused by accumulated lymph fluid in the lymphedema-affected area. We found that individuals conducting exercise were less likely to have self-reported pain. Two RCTs have reported the benefits of exercise in decreasing secondary lymphedema-related symptoms (Sandel et al., 2005; Schmitz et al., 2009). We also identified that there was a statistically significant association between increased use of bandaging and higher rate of heaviness. This finding may indicate that individuals with heaviness were more likely to use bandaging to manage their symptom. Although we cannot confirm an underlying relationship between the two variables due to a cross-sectional design, this finding implies that future studies are needed to identify if bandaging improves a patient’s symptom of heaviness. Moreover, we found that there was a statistically significant association between a reported history of infection and the use of skin care. Due to the nature of the cross-sectional study, we could not determine a causal relationship between these two variables. This finding may imply that infection may be a driving factor that stimulates patients’ self-care activities directed toward the skin because meticulous and appropriate skin care is pivotal for infection control. One study found that skin care, when combined with self-MLD, provided symptomatic relief and reduced limb volume (Barclay, Vestey, Lambert, & Balmer, 2006). Nevertheless, if patients do not receive or follow proper instructions about skin care (i.e., if they do not perform skin care correctly), patients may be at risk for infection episodes. We also identified that a low percentage of participants used alternative treatments for lymphedema self-care. Interestingly, the study identified that the participants with a history of infection were more likely to use pumps and bandage alternatives. These findings indicate that more studies are needed to evaluate the effect of alternative treatments on symptom management and infection control in individuals with primary lymphedema. 132
Deng et al.
Study Strengths There are several strengths associated with this study. The study focused on an under-reported condition (lower-extremity lymphedema) in an understudied population (individuals with primary lymphedema). Due to the relatively large sample size of the cohort, we were able to examine the self-care status, symptom burden, and reported infections in individuals with lower-extremity primary lymphedema. Some possible associations among these variables were identified, which could provide a basis for future descriptive and interventional studies. We also compared and identified differences in self-care, symptom burden, and infections among individuals with unilateral and bilateral lowerextremity primary lymphedema. These findings highlight the importance of self-care, symptom assessment and management, infection control, and risk reduction practices in individuals with primary lymphedema, and also identify areas for future research in this population.
Study Limitations There are also a number of limitations in the study. First, data were collected from a survey study, which is associated with selection bias. It is possible that individuals with more severe symptoms and episodes of infection were more likely to participate in this survey than those with mild symptom burden and fewer infections. Further, these participants may have been more (or less) motivated for self-care than nonparticipants. We did not know the respondent rates via this survey study. This also increases the risk for selection bias. Secondly, as in all observational studies, causal relationships cannot be identified. Thus, we must be particularly cautious to explain our findings. In other words, we only evaluated associations among self-care status, symptoms, and reported infections and did not intend to predict any underlying relationships among the study variables. Third, participants’ infection data were also based on self-report; thus, the accuracy of reported infections may be another confounding factor. Fourth, we did not collect data on severity of lymphedema in the sample, which may be a critical variable to influence self-care, symptoms, and infections.
Conclusions One third of the respondents with lower-extremity primary lymphedema reported no self-care activities. Future studies should aim to explore the underlying reasons why some patients with lower limb primary lymphedema conduct self-care and some do not, such as whether those without self-care are aware of the Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Deng et al.
importance of self-care and whether this can be attributed to difficulty in gaining access to necessary resources of self-care (e.g., financial support, educational information, hands-on self-care skills, etc.), whether time constraints exist for self-care, whether other chronic conditions or comorbidities limit the ability to conduct self-care activities, or whether patients have conducted self-care in the past and quit because they did not perceive a benefit. Opportunities exist for healthcare professionals to educate patients with lymphedema regarding the importance of self-care and to evaluate whether patients have the capabilities and resources to conduct self-care activities. Interventional studies may be required to identify effective strategies to improve their self-care status and tailoring of self-care tasks to meet individual patient needs. In this cohort group, symptom burden was high and infection episodes were frequent, which implies that improvement of symptom management and infection control is critically important. We also identified that the respondents with bilateral lower-extremity primary lymphedema experienced more severe symptom burden and infectious complications compared to the respondents with unilateral lower-extremity primary lymphedema. Tailored interventions need to be developed for this patient population. Clearly, more studies are required to address this under-recognized and underinvestigated health issue to improve the quality of life among patients with lower-extremity primary lymphedema.
Clinical Resources
r r
National Lymphedema Network, NLN Position Papers: http://www.lymphnet.org/le-faqs/nlnposition-papers Oncology Nursing Society, Lymphedema: https:// www.ons.org/practice-resources/pep/lymphedema
References Armer, J. M., Radina, M. E., Porock, D., & Culbertson, S. D. (2003). Predicting breast cancer-related lymphedema using self-reported symptoms. Nursing Research, 52(6), 370– 379. Barclay, J., Vestey, J., Lambert, A., & Balmer, C. (2006). Reducing the symptoms of lymphoedema: Is there a role for aromatherapy? European Journal of Oncology Nursing, 10(2), 140–149. doi:10.1016/j.ejon.2005.10.008 Bernaudin, J. F., Kambouchner, M., & Lacave, R. (2013). [Lymphatic vascular system, development and lymph formation. Review]. Revue de Pneumologie Clinique, 69(2), 93–101. doi:10.1016/j.pneumo.2013.01.005
Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Individuals With Lower-Extremity Primary Lymphedema
Brorson, H., Ohlin, K., Olsson, G., Svensson, B., & Svensson, H. (2008). Controlled compression and liposuction treatment for lower extremity lymphedema. Lymphology, 41(2), 52–63. Connell, F., Ostergaard, P., Carver, C., Brice, G., Williams, N., Mansour, S., et al. (2009). Analysis of the coding regions of VEGFR3 and VEGFC in Milroy disease and other primary lymphoedemas. Human Genetics, 124(6), 625–631. doi:10.1007/s00439-008-0586-5 ¨ ¨ ¨ Foldi, M. D., Foldi, E., Strossenreuther, R. H. K., & Kubik, S. (Eds.). (2007). F¨oldi’s textbook of lymphology: For physicians and lymphedema therapists (2nd ed.). Munich, Germany: Elsevier, Urban & Fischer Verlag. Lee, B.-B., Bergan, J. J., & Rockson, S. G. (2011). Lymphedema: A concise compendium of the theory and practice. London, England: Springer. National Lymphedema Network. (2011, February 1). NLN position papers. Retrieved from http://www.lymphnet.org/ resources/nln-position-paper-the-diagnosis-and-treatmentof-lymphedema Okajima, S., Hirota, A., Kimura, E., Inagaki, M., Tamai, N., Iizaka, S., et al. (2013). Health-related quality of life and associated factors in patients with primary lymphedema. Japan Journal of Nursing Science, 10(2), 202–211. doi:10.1111/j.1742-7924.2012.00220.x Oncology Nursing Society. (2014, April 4). Lymphedema. Retrieved from http://www.ons.org/Research/PEP/ Lymphedema Ridner, S. H., Bonner, C. M., Deng, J., & Sinclair, V. G. (2012). Voices from the shadows: Living with lymphedema. Cancer Nursing, 35(1), E18–E26. doi:10.1097/NCC.0b013e31821404c0 Ridner, S. H., Deng, J., Fu, M. R., Radina, E., Thiadens, S. R., Weiss, J., et al. (2012). Symptom burden and infection occurrence among individuals with extremity lymphedema. Lymphology, 45(3), 113–123. Ridner, S. H., Dietrich, M. S., & Kidd, N. (2011). Breast cancer treatment-related lymphedema self-care: Education, practices, symptoms, and quality of life. Supportive Care in Cancer, 19(5), 631–637. doi:10.1007/s00520-010-0870-5 Ridner, S. H., Fu, M. R., Wanchai, A., Stewart, B. R., Armer, J. M., & Cormier, J. N. (2012). Self-management of lymphedema: A systematic review of the literature from 2004 to 2011. Nursing Research, 61(4), 291–299. doi:10.1097/NNR.0b013e31824f82b2 Ryan, M., Stainton, M. C., Jaconelli, C., Watts, S., MacKenzie, P., & Mansberg, T. (2003). The experience of lower limb lymphedema for women after treatment for gynecologic cancer. Oncology Nursing Forum, 30(3), 417–423. doi:10.1188/03.ONF.417-423 Sandel, S. L., Judge, J. O., Landry, N., Faria, L., Ouellethe, R., & Majczak, M. (2005). Dance and movement program improves quality-of-life measures in breast cancer survivors. Cancer Nursing, 28(4), 301–309.
133
Individuals With Lower-Extremity Primary Lymphedema
Schmitz, K. H., Ahmed, R. L., Troxel, A., Cheville, A., Smith, R., Lewis-Grant, L., et al. (2009). Weight lifting in women with breast cancer-related lymphedema. New England Journal of Medicine, 361(7), 664–673. doi:10.1056/NEJMoa0810118 Schook, C. C., Mulliken, J. B., Fishman, S. J., Grant, F. D., Zurakowski, D., & Greene, A. K. (2010). Primary lymphedema: Clinical features and management in 138 pediatric patients. Plastic and Reconstructive Surgery, 127(6), 2419–2431. doi:10.1097/PRS.0b013e318213a218 Symvoulakis, E. K., Anyfantakis, D. I., & Lionis, C. (2010). Primary lower limb lymphedema: A focus on its functional, social, and emotional impact. International Journal of Medical Sciences, 7(6), 353–357. U.S. Department of Health and Human Services, National Institutes of Health, & National Cancer Institute. (2013, R April 10). Lymphedema (PDQ ): Management. Retrieved from http://www.cancer.gov/cancertopics/pdq/supportivecare/ lymphedema/healthprofessional/page2 Zuther, J. E. (2009). Lymphedema management: The comprehensive guide for practitioners (2nd ed.). New York, NY: Thieme.
134
Deng et al.
Supporting Information Additional Supporting Information may be found in the online version of this article at the publisher’s web site: Table S1: Characteristics of Respondent Cohort Stratified by Unilateral (n = 269) Versus Bilateral (n = 534) Lower Extremity Primary Lymphedema (Overall n = 803). Table S2: Self–Care of Respondent Cohort Stratified by Unilateral (n = 269) Versus Bilateral (n = 534) Lower Extremity Primary Lymphedema (Overall n = 803). Table S3: Reported Symptoms Among Respondents With Unilateral and Bilateral Lower–Extremity Primary Lymphedema. Table S4: Self–Care, Reported Symptoms, and Infections Among Respondents With Lower–Extremity Primary Lymphedema. Table S5: Reported Symptoms and Infections Among Respondents With Lower–Extremity Primary Lymphedema (N = 665).
Journal of Nursing Scholarship, 2015; 47:2, 126–134. C 2014 Sigma Theta Tau International
Copyright of Journal of Nursing Scholarship is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
