Hot off the Press March 2021
We have collated some great articles and material published over the last month. Click on the links below to read the abstract or download the full paper.
Anatomy / Physiology /Pathophysiology
Sex Hormones in Lymphedema - click for abstract
Sex Hormones in Lymphedema
Lymphedema is a disorder of the lymphatic vascular system characterized by impaired lymphatic return resulting in swelling of the extremities and accumulation of undrained interstitial fluid/lymph that results in fibrosis and adipose tissue deposition in the limb. Whereas it is clearly established that primary lymphedema is sex-linked with an average ratio of one male for three females, the role of female hormones, in particular estrogens, has been poorly explored. In addition, secondary lymphedema in Western countries affects mainly women who developed the pathology after breast cancer and undergo through hormone therapy up to five years after cancer surgery. Although lymphadenectomy is identified as a trigger factor, the effect of co-morbidities associated to lymphedema remains elusive, in particular, estrogen receptor antagonists or aromatase inhibitors. In addition, the role of sex hormones and gender has been poorly investigated in the etiology of the pathology. Therefore, this review aims to recapitulate the effect of sex hormones on the physiology of the lymphatic system and to investigate whether hormone therapy could promote a lymphatic dysfunction leading to lymphedema.
Tissue Engineering Strategies for Cancer-Related Lymphedema - click for abstract
Tissue Engineering Strategies for Cancer-Related Lymphedema
Tissue engineering has witnessed remarkable advancement in various fields of medicine and has the potential of revolutionizing the management of lymphedema. Combining approaches of biotechnology with the evolving understanding of lymphangiogenesis may offer promising treatment modalities for patients suffering from lymphedema. The strategies to lymphatic vessels tissue engineer can be grouped into four main categories: Delivery of chemokines, cytokines and other growth factors to induce lymphangiogenesis; cellular based-approach using LECs or stem-cells; scaffold-based tissue engineering; or a combination of these. This review will summarize the current approach to cancer-related lymphedema and advances in lymphatic tissue engineering strategies as well as the challenges facing the regeneration of lymphatic vasculature, particularly in an oncologic setting.
Comorbidity and Lymphatic Disease: The Lymphatic Continuum Re-Examined - click for abstract
Comorbidity and Lymphatic Disease: The Lymphatic Continuum Re-Examined
It has now been ∼20 years since the original Lymphatic Continuum conference was convened, and this continuum has transitioned from a compelling concept to a reality. The explosive growth in our comprehension of lymphatic genetics, development, and function has expanded and modified our traditional views regarding what is, and is not, lymphatic disease. Groundbreaking investigations over the past decade have now defined a large and growing list of pathological conditions in which morphological or function lymphatic alterations can be identified. This list includes atherosclerosis and dyslipidemia, hypertension and other cardiovascular diseases, inflammation and inflammatory bowel disease, obesity, narrow angle glaucoma, and, most recently and compellingly, neurodegenerative disease. The sometimes overlapping but largely disparate nature of these various aforementioned disease categories suggests that the presence, or absence, of structural or functional lymphatic derangements may represent a previously unrecognized unifying influence in the maintenance of health and the promotion of disease. Future investigation of lymphatic mechanisms in disease will likely continue to elucidate the influences of lymphatic dysfunction, perhaps subtle, that can invest other, seemingly unrelated, diseases. In future, such discoveries will provide mechanistic insights and may potentiate the development of a new lymphatic-based approach to human disease diagnosis and therapeutics
Lymphatic Development and Implications for Diagnosis and Therapy - click for abstract
Lymphatic Development and Implications for Diagnosis and Therapy
The lymphatic system was first described in the 17th century independently by Olaus Rudbeck and Thomas Bartholin. Since then, there has been deep-seated fascination with its development, function, and dysfunction.
Prevalence and Risk Factors
Predictors of Quality of Life in Patients with Breast Cancer-Related Lymphedema: Effect of Age, Lymphedema Severity, and Anxiety - click for abstract
Predictors of Quality of Life in Patients with Breast Cancer-Related Lymphedema: Effect of Age, Lymphedema Severity, and Anxiety
Patients with breast cancer-related lymphedema (BCRL) have lower quality of life (QOL). However, some important predictors, such as the effect of age, lymphedema severity, depression, and anxiety, have not yet been discovered. The overall objective of this study is to explore the QOL predictors associated with BCRL in China. Methods and Results: A cross-sectional design was conducted. Data were collected before treatment, including sociodemographic characteristics (height, heaviness, age, education level, work status, marital status, and economic status), clinical characteristics (surgical method, clinical cancer stage, lymphedema severity, and lymphedema duration), the hospital anxiety (HA) and depression scale, and the functional assessment of cancer therapy-breast quality of life instrument. Univariate analysis or bivariate correlation was first made to explore the correlation of QOL with sociodemographic/clinical characteristics, anxiety, and depression. The multiple linear regression model was used to identify the independent QOL predictors. Seventy-one patients with BCRL were recruited. Age, education level, work status, family income, lymphedema duration, lymphedema severity, and HA and hospital depression scale scores are significantly correlated with QOL (p < 0.05). Age, lymphedema severity, and HA accounted for 85.9% in QOL (F = 62.76, p < 0.001). Conclusions: Age, lymphedema, and anxiety are the most important QOL predictors. Therefore, it is very important to establish a BCRL prevention system and pay attention to psychological distress in the patients with BCRL. [/av_toggle] [/av_toggle_container] [av_hr class='invisible' height='50' shadow='no-shadow' position='center' custom_border='av-border-thin' custom_width='50px' custom_border_color='' custom_margin_top='30px' custom_margin_bottom='30px' icon_select='yes' custom_icon_color='' icon='ue808' font='entypo-fontello' av_uid='av-j17b77m'] [/av_one_full] [av_hr class='short' height='40' shadow='no-shadow' position='left' custom_border='av-border-thin' custom_width='50px' custom_border_color='' custom_margin_top='30px' custom_margin_bottom='30px' icon_select='yes' custom_icon_color='' icon='ue808' font='entypo-fontello' admin_preview_bg='' av_uid='av-iryww6a'] [av_one_full first min_height='' vertical_alignment='' space='' custom_margin='' margin='0px' padding='0px' border='' border_color='' radius='0px' background_color='' src='' background_position='top left' background_repeat='no-repeat' animation='' mobile_breaking='' mobile_display='' av_uid='av-iceb4yq'] [av_textblock size='' font_color='' color='' av-medium-font-size='' av-small-font-size='' av-mini-font-size='' admin_preview_bg='' av_uid='av-hpr2abm']
Assessment
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Agreement Between Optoelectronic Volumetry and Circumferential Girth Measurements to Diagnose Lymphedema in Patients Submitted to Axillary Radical Lymphadenectomy for Treatment of Cutaneous Melanoma
Aim: To assess the agreement between indirect and optoelectronic volumetries to diagnose lymphedema based on arm volume difference in patients with axillary lymph node dissection (ALND) for cutaneous melanoma.
Methods and Results: Patients were assessed by circumferential girth measurements (truncated cone formula) to determine the upper limb volumes (indirect volumetry) and by optoelectronic volumetry (Perometer®) of affected and control limbs. A diagnosis of lymphedema on each measuring method was defined as an absolute volume difference >200 mL or a relative volume >10%. Forty-six patients with ALND were included. There were no significant differences between the volume means or the mean absolute or relative differences measured by each method. Good correlation was observed between the volume of upper limbs for both the left (r = 0.998) and right (r = 0.985) arms. As for the diagnosis of lymphedema, an absolute volume difference >200 mL determined a prevalence of 28% (13/46) of lymphedema by indirect volumetry and 35% (16/46) by optoelectronics volumetry. The crude diagnostic agreement was 93% with a kappa = 85% (agreement adjusted by chance) between methods. If a 10% increase in the relative volume difference between the arms was used as the diagnostic criterion, prevalence was 20% (9/46) and 22% (10/46), respectively.
Conclusion: There is good agreement between perometry and circumferential girth measurements when classifying patients as having a difference between arm volumes >200 mL or 10%, the most frequently used cutoffs to diagnose lymphedema.
Feasibility of segmental bioelectrical impedance analysis for mild- to moderate-degree breast cancer-related lymphedema: Correlation with circumferential volume measurement and phase angle - click for abstract
Feasibility of segmental bioelectrical impedance analysis for mild- to moderate-degree breast cancer-related lymphedema: Correlation with circumferential volume measurement and phase angle
Segmental multi-frequency bioelectrical impedance analysis (s-MFBIA) has been adopted recently to evaluate the volume of breast cancer-related lymphedema (BCRL). This procedure uses the segmental phase angle (s-PhA) as an indicator of cellular integrity. In the smaller-built Asian population, the BCRL often has a small volume difference and can be overlooked by tape circumference volume measurement (TVM). This study aimed to investigate the clinical feasibility of s-MFBIA for the assessment of lymphedema severity compared with TVM and evaluate the association between lymphedema severity and cellular integrity of the affected arm based on s-PhA values for a patient with mild- to moderate-degree BCRL. Segmental PhA and extracellular water (ECW)/total body water (TBW) ratio of bilateral arms were measured using In Body S10, an s-MFBIA device, in 128 BCRL patients. Inter-limb volume ratio was measured using TVM. The inter-limb ECW/TBW ratio was correlated with inter-limb volume ratio. Inter-limb ECW/TBW ratio and inter-limb volume ratio were then correlated with inter-limb PhA ratio to demonstrate the association between lymphedema severity and arm cellular integrity. The inter-limb ECW/TBW ratio and inter-limb volume ratio were positively correlated (r = 0.654, P < .001). The same result was obtained after adjusting for age, body mass index, postoperative survival, and duration of lymphedema (r = 0.636, 0.653, 0.652, and 0.648, P < .001). The inter-limb PhA ratio demonstrated significant negative correlation with inter-limb ECW/TBW ratio and inter-limb volume ratio (r = -0.896, -0.562, P < .001).s-MFBIA has high consistency with the conventional TVM method, and its relation to cellular integrity by segmental PhA enables better understanding of the cellular state of the affected limb in mild- to moderate-degree BCRL. Therefore, it is clinically feasible for severity assessment and monitoring of mild- to moderate-degree BCRL in smaller-built Asian patients. [/av_toggle] [/av_toggle_container] [av_toggle_container initial='0' mode='accordion' sort='' styling='' colors='' font_color='' background_color='' border_color='' av_uid='av-fffy1pe'] [av_toggle title='A Comparison of Supine Versus Stand-on Bioimpedance Devices to Assess Breast Cancer-Related Lymphedema - click for abstract' tags='' av_uid='av-f4q7av6'] A Comparison of Supine Versus Stand-on Bioimpedance Devices to Assess Breast Cancer-Related Lymphedema
While supine bioimpedance devices are used to evaluate for lymphedema (LE), stand-on devices are gaining popularity. Because research on differences in bioimpedance values between the two devices is limited, this study’s purposes were to: (1) determine the average upper limb impedance values and inter-limb ratios for women who self-reported having (n = 34) or not having (n = 61) a history of LE, using a single-frequency supine device and a multifrequency stand-on device; (2) compare the level of agreement in inter-limb impedance ratios between the two devices; evaluate the percent agreement between the two devices in classifying cases of LE using established supine thresholds; and evaluate the percent agreement in classifying cases of LE between the supine device using previously established supine thresholds and the stand-on device using two published standing thresholds. Methods and Results: Bioimpedance measures were done using the two devices. For the entire sample, absolute impedance values for both the affected and unaffected limbs were significantly higher for the stand-on device in women with and without LE. Impedance values for the two methods were highly correlated. Bland-Altman analysis determined that for the entire range of impedance ratios the values for the two devices could not be used interchangeably. Conclusions: Findings suggest that the stand-on device can be a useful and valid tool to assess for LE. However, because agreement is not perfect, values obtained from the two devices should not be used interchangeably to evaluate for changes in impedance ratios, particularly for ratios of >1.20.
Assessment of Edematous Changes Using Three-Dimensional Body Scanning and Segmental-Bioelectrical Impedance Spectroscopy - click for abstract
Assessment of Edematous Changes Using Three-Dimensional Body Scanning and Segmental-Bioelectrical Impedance Spectroscopy
Purpose: The primary purpose of this study was to clarify the occurrence of sites of edematous changes using the measured circumferences of the thigh and lower leg via three-dimensional (3D) body scanning. The secondary purpose was to determine the relationship between the volume changes using 3D body scanning and the resistance changes as indicator of extracellular water (ECW) via segmental-bioelectrical impedance spectroscopy (S-BIS).
Methods and Results: Fifteen healthy women participated. Limb circumferences were measured using 3D body scanning at 10% intervals between 50% and 80% in the thigh circumference and between 0% and 80% in the lower leg circumference. The resistance of the ECW component (RECW) and total body water (RTBW) was measured using S-BIS in the thigh and lower leg segments. These measurements were conducted at baseline and 6 hours post observation. A paired t-test was conducted for the differences in these parameters, and the effect sizes (ESs) were calculated using Cohen’s d. Correlations between changes in segmental volume and RECW were analyzed. Measurement-site circumferences and segmental volume significantly increased in the lower leg at post observation but not in the thigh. The ES of circumference changes in the lower leg’s central region were larger (ESs were 0.40-0.71 at 30%-50%) than in other regions. A significant correlation was observed between changes in segmental volume and RECW of the lower leg (ρ = -0.79, p < 0.001). Conclusions: Assessing the circumference using 3D body scanning, we found the edematous changes to be significant in the lower leg’s central regions. Moreover, volumetry using 3D body scanning can detect edematous change in the lower leg.
Reliability of Jeweler Rings and a Revised Figure-of-Eight Circumference Protocol for the Assessment of Finger and Hand Circumferences - click for abstract
Reliability of Jeweler Rings and a Revised Figure-of-Eight Circumference Protocol for the Assessment of Finger and Hand Circumferences
Assessing the circumference of the hand and fingers can be challenging. Therefore, we studied the reliability of jeweler rings and a revised figure-of-eight (figure-8) protocol for the clinical assessment of finger and hand circumference. Methods and Results: Thirteen patients residing in a rehabilitation center were recruited. Inter- and intrarater reliability of the jeweler rings as well as the revised figure-8 protocol were assessed. Jewelers rings were used to assess the circumferences of the middle and ring finger of both hands. The figure-8 method was used to assess the circumference of both hands. Reliability analyses were performed in Statistical Package for the Social Sciences 26 and demonstrated an excellent reliability for the jeweler rings as well as the revised figure-8 protocol, represented by single random measure intraclass correlation coefficient(2,1) values of at least 0.973 and Cronbach alpha of at least 0.99. Conclusions: Circumference measurements are valid to use; however, reliability issues do exist in clinical practice. Assessing the circumference of fingers with our new jeweler rings protocol and the circumference of the hand by means of a revised figure-8 protocol is highly reliable in a clinical setting
Diagnostic Contribution of Ultrasonography in Breast Cancer-Related Lymphedema - click for abstract
Diagnostic Contribution of Ultrasonography in Breast Cancer-Related Lymphedema
Many assessments have been reported and used in evaluating lymphedema. The aim of this study was to investigate the diagnostic contribution of ultrasonography in unilateral breast cancer-related lymphedema. Methods and Results: Upper extremity circumferences were measured with a measuring tape from ulnar styloid at the wrist to the axilla at 4 cm intervals. The point with the highest difference between the upper extremities and the control point with no difference between the lower extremities were marked. Skin and subcutaneous thicknesses were measured from four quadrants (volar medial-lateral and dorsal medial-lateral) at the marked points and also subcutaneous tissue changes were graded according to the subcutaneous echogenicity grade (SEG) scale ultrasonographically. The correlations between circumferential and ultrasonographic measurements were investigated. Receiver operating characteristic curve analysis was made to estimate the diagnostic accuracy of the difference in ultrasonographic subcutaneous thickness measurements between the two arms. The study was completed with 34 female patients. Circumferential and subcutaneous tissue thickness measurements were moderately positively correlated in the volar quadrants and strongly positively correlated in the dorsal quadrants of the affected extremity. In the unaffected extremity, a strong positive correlation was identified in all quadrants. The clinical stages of lymphedema and SEG were weakly positively correlated in the volar medial quadrant. The difference between the two upper extremities was found to have a high (0.83%) sensitivity, and an acceptable (0.75%) specificity in the differentiation of Grade II and Grade III lymphedema. Conclusion: A correlation was established between circumferential measurements and ultrasonographic measurements. Ultrasonography can be used complementary to circumferential measurements in diagnosing lymphedema.
Sexual Functions and Quality of Life in Patients Developing Lymphedema After Total Mastectomy: A Pilot Study - click for abstract
Sexual Functions and Quality of Life in Patients Developing Lymphedema After Total Mastectomy: A Pilot Study
Sexual functions in women with lymphedema secondary to breast cancer surgery have not been investigated sufficiently. This study aimed to compare patients with and without lymphedema after total mastectomy in terms of emotional state, sexual functions, and quality of life. We also investigated the factors affecting sexual functions in these patients. Methods: Married women 20-55 years of age, who presented to lymphedema polyclinic of Health Sciences University Ankara Training and Research Hospital after having undergone total mastectomy at least 1 year earlier owing to breast cancer were included. Twenty-five patients with lymphedema were assigned to the lymphedema group, and 20 without lymphedema to the control group. Hospital Anxiety and Depression Scale (HADS) was used to assess emotional state. We evaluated sexual functions of the participants by Female Sexual Function Index (FSFI) and quality of life with European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC QOL-C30). Results: There was no statistically significant difference in age and body mass index between the groups (p > 0.05). The mean HADS score was 13.4 ± 6.5 in lymphedema group and 13.4 ± 6.0 in control group. The groups were also similar in aspect of the HADS score (p > 0.05). FSFI and global health and physical function scores of EORTC QOL-C30 were statistically significantly lower in the lymphedema group (p < 0.05). A statistically significant correlation was found between FSFI and age and time elapsed postmenopause (p < 0.05, for both). Conclusion: We evaluated sexual functions of the patients who underwent mastectomy in this study. Although the HADS score is similar in patients with and without lymphedema, both sexual functions and quality of life are adversely affected in patients who undergo mastectomy and develop lymphedema. This study is important for investigating whether lymphedema developing after total mastectomy affects sexual functions of the patients. [/av_toggle] [/av_toggle_container] [av_toggle_container initial='0' mode='accordion' sort='' styling='' colors='' font_color='' background_color='' border_color='' av_uid='av-ap616f6'] [av_toggle title='Use of bioimpedance spectroscopy for prospective surveillance and early diagnosis of breast cancer-related lymphedema - click for abstract' tags='' av_uid='av-ahxfqxu'] Use of bioimpedance spectroscopy for prospective surveillance and early diagnosis of breast cancer-related lymphedema
Breast cancer treatment-related arm lymphoedema and morbidity: A 6-year experience in an Australian tertiary breast centre - click for abstract
Breast cancer treatment-related arm lymphoedema and morbidity: A 6-year experience in an Australian tertiary breast centre
Prediction of the Presence of Fluid Accumulation in the Subcutaneous Tissue in BCRL Using Texture Analysis of Ultrasound Images - click for abstract
Prediction of the Presence of Fluid Accumulation in the Subcutaneous Tissue in BCRL Using Texture Analysis of Ultrasound Images
The usefulness of the Electronic Patient Visit Assessment (ePVA)() as a clinical support tool for real-time interventions in head and neck cancer
Management Strategies
From Bench to Bedside: The Role of a Multidisciplinary Approach to Treating Patients with Lymphedema- click for abstract
From Bench to Bedside: The Role of a Multidisciplinary Approach to Treating Patients with Lymphedema
Lymphedema is a condition characterized by dysfunction of the lymphatic system resulting in chronic, progressive soft tissue edema that can negatively impact individuals’ function, self-image, and quality of life. Understanding of the disease process has evolved significantly in the past two decades with advances in diagnostic modalities and surgical techniques revolutionizing prior treatment algorithms. Methods and Results: We reviewed our current approach at the University of Southern California to improving outcomes in lymphedema treatment. Given the complexity of this medical condition, patients are best served by a multidisciplinary approach. At our institution, this involves a collaborative effort between bench researchers, lymphatic therapists, medical physicians, and lymphedema surgeons. Basic science and translational research provide further understanding into the underlying mechanisms of lymphangiogenesis and the possibility for potential therapeutic interventions. Our surgical algorithms require patients to undergo a thorough diagnostic evaluation and consultation with certified lymphatic therapists prior to undergoing either physiologic or debulking operations. Patients are followed clinically following any interventions. Further community outreach and education is carried out in order to improve upon early diagnosis and symptom recognition. Conclusions: Optimizing lymphedema care requires a collaborative interplay between researchers, physicians, and therapists. Additionally, patient and provider education on early disease recognition and treatment options is an equally critical aspect of improving patient outcomes
Long-Term Benzathine Penicillin Prophylaxis Lasting for Years Effectively Prevents Recurrence of Dermato-Lymphangio-Adenitis (Cellulitis) in Limb Lymphedema - click for abstract
Long-Term Benzathine Penicillin Prophylaxis Lasting for Years Effectively Prevents Recurrence of Dermato-Lymphangio-Adenitis (Cellulitis) in Limb Lymphedema
The lymphedema-affected limbs are predisposed to acute and, subsequently, chronic dermato-lymphangio-adenitis (DLA) episodes in around 40%-50% of cases, irrespective of what the primary etiological factor is for the development of this condition. DLA is of bacterial etiology, and it needs antibiotic control and prevention of recurrence. Aim: To follow the effects of years-long continuous no-break administration of benzathine penicillin on the recurrence of acute DLA episodes. Methods and Results: Two hundred thirty-one patients were affected with lymphedema of lower and upper limbs. The mean duration of lymphedema was 10.2 ± 7.3 (range 2-30) years, and the number of DLA attacks/patient was 3.3 ± 3.2 (range 1-10). The total number of DLA episodes was 805. Benzathine penicillin injections 1,200,000 units were given i.m. at 14-21 days intervals (mean 18 ± 9 days) with short accidental breaks only. The period of therapy was 39.2 ± 38.7 (median 32) months. Recurrence occurred in 23 out of 231 (10%) (p < 0.01). There were 42 DLA incidents compared with 805 before introduction of therapy (5.2%) patients (hazard ratio 0.05, 95% confidence interval 0.034-0.079) (p < 0.01). Among patients with recurrence, there was a decrease of DLA episodes from 6.2% ± 3.6% to 1.7% ± 1.0%/patient. There were no differences in effectiveness of penicillin prophylaxis between etiological groups, depending on stages of lymphedema. Conclusions: Long-term years-long benzathine penicillin prophylaxis is extremely effective in prevention of DLA recurrence. It can be applied for years with no breaks, without clinical side-effects, and raising resistance to antibiotics. Microbial colonization and evoked inflammatory reaction of hosts should be controlled from the first symptoms of lymph stasis, irrespective of the etiology of lymphedema. [/av_toggle] [/av_toggle_container] [av_toggle_container initial='0' mode='accordion' sort='' styling='' colors='' font_color='' background_color='' border_color='' av_uid='av-3k3w682'] [av_toggle title='Challenges and opportunities identified for lymphoedema services in Wales during the COVID-19 pandemic - click for abstract' tags='' av_uid='av-b0pcf6'] Challenges and opportunities identified for lymphoedema services in Wales during the COVID-19 pandemic
BACKGROUND: During the COVID-19 pandemic, lymphoedema staff adapted services, providing care remotely, and worked in other NHS sectors. The impact on services and staff must be understood in order to safeguard patient care and foster workforce resilience. AIMS: To evaluate the experiences of clinical and non-clinical lymphoedema staff in Wales during the COVID-19 pandemic.
METHODS: An anonymous online survey, based on scoping work, was sent out via the Welsh lymphoedema services mailing list.
FINDINGS: 71% (68/96) of eligible lymphoedema staff completed the survey. More than half supported lymphoedema services (40/68) with the remaining staff deployed elsewhere. Overall, staff and services felt prepared for new ways of working. Concerns about others and the future burden on services when life returned to normal were reported. Opportunities identified included education initiatives and virtual services.
CONCLUSION: Lymphoedema services were well prepared to deliver virtually, enable effective care and share knowledge. Co-ordinated efforts to uphold patient advocacy will support virtual services to meet their needs
International Camps for Children with Lymphedema and Lymphatic Anomalies: When Education Links with Psychosocial Research - click for abstract
International Camps for Children with Lymphedema and Lymphatic Anomalies: When Education Links with Psychosocial Research
Lymphedema in children and adolescents is a rare and chronic condition. The management of their lymphedema is mainly driven by the adaptation of treatments used in adults. The aim of our study was to explore the needs and challenges the children and adolescents face during their management with the aim of finding ways to satisfy these needs and organize an hospital-based centre accordingly with an educational program. Methods and Results: Patients and their families were given the opportunity to meet other patients, their families and professionals during social activities organised annually and during two international camps. They were invited to take part in different semi structured focus groups and interviews. All patients and families described a long journey and relief when the diagnosis was obtained followed by the shock of being told that it was a chronic condition. Meeting other children with the condition was a relief. The impact of lymphedema on body shape and genitals was a source of distress. Rejection of the compression was part of journey. Lymphedema management had an impact on all the family members including siblings. Parents were responsible for their child self-management in young children which was described as demanding. It was followed by a complex transition phase to self-management. The impact was not the same according to the age the lymphedema had started. Conclusion: Acceptance and management of lymphedema is complex and invades many aspects of families’ life. Self-management is demanding. Based on these results, the management of lymphedema in the centre included meeting other children and families and an educational program based on individual needs and follow-up.
Guidelines for managing people with lymphoedema remotely: a post-COVID-19 response document
