Some international health organizations portray lymphatic filariasis, elephantiasis, or podoconiosis, as if they were unrelated diseases. Lymphatic filariasis, which is commonly but erroneously known as elephantiasis, is a mosquito-transmitted disease that occurs when filarial parasites (roundworms) damage the lymphatic system causing lymphatic dysfunction (lymphedema). Podoconiosis is a non-filarial, noninfective disease, usually caused by crystalline minerals clogging regional lymph nodes and vessels, affecting especially agrarian people who work barefoot, particularly in the neighborhood of volcanic soil. These two conditions develop chronic lymphatic dysfunction (lymphedema), and in many cases is acquired in childhood. Read more about childhood elephantiasis prevention HERE.
WHAT IS ELEPHANTIASIS
The medical term Elephantiasis represents lymphedema (whatever cause or setting) in its most advanced degree of severity. There are some organizations and clinical guides that make reference to lymphedema and elephantiasis as if they were two different and isolated conditions, referring to them as lymphedema "or" elephantiasis, which can give rise to confusion. Lymphedema and elephantiasis should not be portrayed as if two different conditions, for they are the same disease and consequence of one another, first comes initial mild lymphedema, and then the late severe stage called elephantiasis, and which is principally due to nontreatment or undertreatment. Read more about the best treatment option for elephantiasis HERE.
Elephantiasis (Stage III lymphedema) is a public health problem and represents the worst disfiguring visible manifestations of the disease, which causes not only physical disability and financial hardship, but also social stigma and isolation due to the disfigurement, and thus loss of Quality of Life (QoL). Regarding Filariasis, it is important to prevent the spreading of the disease, but just as much to prevent unnecessary suffering to individuals that already have established disorders of the lymphatic circulation. Read about why the world is not treating a treatable disease like elephantiasis HERE.
WHAT IS LYMPHATIC EDEMA
Lymphedema is a symptom of lymphatic failure and manifests as chronic connective tissue disease. Fluids, cells, and proteins usually removed by lymphatic pumping accumulate in the tissue spaces between the skin and underlying muscle, and over time chronic inflammatory processes change the tissue composition and architecture. Early fluid-rich stages are followed by fibrosis and fatty deposits in the middle stages, and advanced stages are marked by hyperkeratosis, papillomatosis, and other skin diseases. Lymphedema, whatever the cause, is incurable and requires life-long care, and effective lymphedema management to gain and improve "Quality of Life" (QoL).
Lymphedema in its advanced form, known as elephantiasis, is a significant cause of disability, disfigurement, and morbidity, and is principally due to nontreatment or undertreatment.
PRIMARY, SECONDARY, AND TERTIARY PREVENTION OF CIRCULATORY SYSTEM DISEASES
Two distinct fluids move through and form part of the circulatory system: blood and lymph. The blood system carries oxygen and nutrients to the body's cells and carries hormones, antibodies, and waste materials away. The lymphatic system serves as a defense system for the body, filtering out organisms that cause disease, producing white blood cells, generating antibodies, and supporting the circulatory system by draining excess fluids and proteins from tissues back into the bloodstream. Although they are totally different hemodynamic mechanisms, they are mutually dependent and complementary circulation systems.
Like most other clinical conditions, diseases of the circulatory system like Chronic Venous Insufficiency (CVI) in "Peripheral Vascular Disease", and Lymphedema (LE) in "Lymphatic Vascular Disease", is most treatable in their earliest stages. The goals of preventive treatment in diseases of the blood vascular system, are to reduce the pooling of blood in venous disease, and the goals of preventive treatment in diseases of the lymphatic vascular system, are to reduce the pooling of lymph fluid (dermal backflow) in lymphatic disease, thus preventing limb swelling, skin complications, and disease progression.
Evidence-based research provides the basis for sound clinical practice guidelines and recommendations for diseases of the circulatory system. Vascular medicine or vascular surgery specialists recommend a combination of preventive treatments for people with venous and lymphatic dysfunction. Some of the simple basic daily self-care preventive treatment strategies include:
- Avoid long periods of standing or sitting.
- Exercise regularly.
- Control weight.
- Elevate limbs while sitting and lying down.
- Wear compression garments all day.
- Take antibiotics as needed to treat skin infections.
- Practice good hygiene and skincare.
- Use adequate clothing and footwear.
THE BEST FIRST-LINE TREATMENT FOR LYMPHATIC DYSFUNCTION
Treatment for lymphedema is an evidence-based clinical need, that centers around reducing the edema which then influences all of the other problems. Education and improving access to health services are keys to the successful prevention of stage elephantiasis. The First-line non-surgical treatment for patients with established lymphedema is Complete Decongestive Therapy (CDT). Treatment is to control the clinical severity and progression of the disease.
Scientific studies highlight the importance and implications of compression therapy in controlling the progression of Lymphoedema, and evidence also supports that compression garments are essential. On the other hand, clinical outcomes on interventions to manage lymphedema, show that simple hygiene-based measures (washing and elevation of limbs, antifungal creams, exercise, and use of footwear) are necessary, but these alone without compression therapy, are not enough to reduce the limb volume in the majority of cases. People with lymphedema need access to continuing care throughout their lives, both to manage the disease and to prevent progression to more advanced stages.
With regard to dermatolymphangioadenitis (DLA), also known as "Infectious Cellulitis", studies have found that the control of swelling is associated with a lower risk of cellulitis and that advanced stages of chronic edema are a strong risk factor. Infectious cellulitis can lead to sepsis (septicemia). Read more about what are the preventive measures for Infectious Cellulitis in lymphedema HERE.
The psychological stress factors in lymphedema must also be taken into consideration regarding the "Quality of Life" (QoL) indicators of individuals. Lymphedema is not in itself a psychopathology, nor does it predispose individuals to poor mental health. In many cases, the negative situation of non-treatment or under-treatment of lymphedema leads to increased disease progression and, contributes to the cycle of disability, disfigurement, social stigmatization, poverty, suffering, and thus, the associated burden on the physical, economic, social, and psychological well-being of individuals. Read more information about psychological aspects surrounding the non-treatment of lymphedema HERE.
Lymphedema is a serious chronic and progressive disease due to an organic disability. The clinical treatment of lymphedema is neither a cosmetic nor an aesthetic treatment. The treatment of lymphedema is to control its progression and alleviate the symptoms related to dysfunction of the lymphatic circulatory system. To prevent and avoid serious complications associated with treatment, such as the possible displacement of edema to previously unaffected areas when compression therapy is applied for volume reduction, patients should use highly specialized and experienced therapists.
Professional qualification and instruction delivered remotely online are not the same as live hands-on practical instruction in the clinical training and certification of lymphedema therapists, similar to all other rehabilitation, medical, and surgical training programs, and especially for developing the necessary manual skills to treat a disease as complex as lymphedema.
Patients should also take special care when choosing a "Multidisciplinary Center of Reference for Lymphedema", as not all countries and centers provide the same treatment options. The best choice is a center of reference that provides “Complete Decongestive Therapy (CDT)”, which is recognized as the “Gold Standard” treatment for lymphedema.
- Read more about what is the best treatment option for Lymphedema HERE.
- Read about what are the principal functions of Manual Lymphatic Drainage HERE.
- Read about what are the main differences between the two principal lymphedema treatment protocols HERE.
- Read about what are the strategies for the implementation of low-cost treatment options for Lymphedema HERE.
REFERENCES
(Click on the texts to read the research articles)
SCIENTIFIC SOCIETIES
PATHOPHYSIOLOGY
- lymphedema.
- Biology of Lymphedema
- Lymph vessels: the forgotten second circulation in health and disease
- Lymphatic Vessel Network Structure and Physiology
- Lymphatic System Flows
- Organ-specific lymphatic vasculature: From development to pathophysiology.
- The lymphatic vascular system: much more than just a sewer.
- The unresolved pathophysiology of lymphedema.
- Lymphedema: A Practical Approach and Clinical Update.
- Coagulation in Lymphatic System.
- Regulation of immune function by the lymphatic system in lymphedema.
- Oxidative stress in chronic lymphoedema.
- Regulatory T Cells Mediate Local Immunosuppression in Lymphedema.
- Hemostatic properties of the lymph: relationships with occlusion and thrombosis.
- Secondary lymphedema: Pathogenesis
- Gastrointestinal Lymphatics in Health and Disease
- Intestinal lymphangiectasia in adults
- Role of the lymphatic vasculature in cardiovascular medicine
- The lymphatic vasculature in disease
- Lymphoscintigraphic abnormalities in the contralateral lower limbs of patients with unilateral lymphedema.
- The Prevalence of Lower Limb and Genital Lymphedema after Prostate Cancer Treatment: A Systematic Review
- Peripheral Edema
- The lymphatic system and the skin. Classification, clinical aspects, and histology.
- Lymphedema and cutaneous diseases.
- Lymphedema and subclinical lymphostasis (microlymphedema) facilitate cutaneous infection, inflammatory dermatoses, and neoplasia: A locus minoris resistentiae.
- Serum Immune Proteins in Limb Lymphedema Reflecting Tissue Processes Caused by Lymph Stasis and Chronic Dermato-lymphangio-adenitis (Cellulitis).
- Lymphedema and subclinical lymphostasis (microlymphedema) facilitate cutaneous infection, inflammatory dermatoses, and neoplasia: A locus minoris resistentiae.
- Lymphatic Flow: A Potential Target in Sepsis.
- Infectious complications of lymphedema.
- Acute inflammatory exacerbations in lymphoedema.
- Inflammatory Manifestations of lymphedema.
- Cellulitis.
- Clinical features, microbiological epidemiology, and recommendations for the management of cellulitis in extremity lymphedema.
- Challenges of cellulitis in a lymphedematous extremity.
- Diagnosis and management of cellulitis.
- Cellulitis risk factors for patients with primary or secondary lymphedema.
- Oedema as a risk factor for multiple episodes of cellulitis/erysipelas of the lower leg.
- Prevention of dermatolymphangioadenitis by combined physiotherapy.
- Erysipelas: a common potentially dangerous infection.
- Malignant tumors as complications of lymphedema.
- Lymphedematous areas: Privileged sites for tumors, infections, and immune disorders.
- Lymphedema-related angiogenic tumors and other malignancies.
- Lymphedema: an immunologically vulnerable site for the development of neoplasms.
- Lymph stasis promotes tumor growth
- A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema.
- Stewart-Treves Syndrome
- Congenital lymphedema complicated by pain and psychological distress: case report
- Lymphatic Pain in Breast Cancer Survivors
- Does Manual Lymphatic Drainage Have Any Effect on Pain Threshold and Tolerance of Different Body Parts?
- Lymphedema therapy reduces the volume of edema and pain in patients with breast cancer
- Quality of life in patients with primary and secondary lymphedema in the community
TREATMENT
- Treatment of limbs lymphedema.
- Nonoperative treatment of lymphedema.
- Lymphedema: From diagnosis to treatment.
- Lymphedema-clinical picture and therapy.
- Physiotherapeutic rehabilitation of lymphedema: state-of-the-art.
- Diagnosis and management of lymphatic vascular disease
- Effective treatment of lymphedema of the extremities.
- Lymphoscintigraphic aspects of the effects of manual lymphatic drainage.
- Intensive Treatment of Lower-Limb Lymphedema and Variations in Volume.
- Effectiveness and safety of Complete Decongestive Therapy of Phase I.
- Therapeutic Efficacy of Complex Decongestive Therapy in the Treatment of Elephantiasis of the Lower Extremities.
- Effects of Phase I complex decongestive physiotherapy on physical functions and depression levels in breast cancer-related lymph edema.
- Does lymphoedema bandaging reduce the risk of toe ulceration?
- Study of 700 referrals to a Lymphedema Program.
- Worldwide assessment of healthcare personnel dealing with lymphoedema.
- Földi M, Földi E. Földi’s textbook of lymphology for physicians and Lymphoedema therapists.
- Fluid Shifts Induced by Physical Therapy in Lower Limb Lymphedema Patients
- Visualization of Accessory Lymphatic Pathways, before and after Manual Drainage, in Secondary Upper Limb Lymphedema Using Indocyanine Green Lymphography
- The risk of genital edema after external pump compression for lower limb lymphedema.
- The management of genital lymphoedema
- The management of deep vein thrombosis in lymphoedema: a review.
- Lymphedema-associated comorbidities and treatment gap.
- A study of the advantages of elastic stockings for leg lymphedema.
- Indications for medical compression stockings in venous and lymphatic disorders: An evidence-based consensus statement.
- Medical compression stockings for chronic venous diseases and lymphedema: Scientific evidence and results of a patient survey on quality of care.
- Impact of Compression Therapy on Cellulitis (ICTOC) in adults with chronic edema: a randomized controlled trial protocol.
- Compression Therapy Is Cost-Saving in the Prevention of Lower Limb Recurrent Cellulitis in Patients with Chronic Edema.
- Occupational leg edema-use of compression stockings.
DISABILITY
- Disability and lymphedema.
- Lymphedema and employability.
- Worse and worse off: the impact of lymphedema on work and career after breast cancer
- Functioning in lymphedema from the patient's perspective using the International Classification of Functioning, Disability and Health (ICF).
- Unilateral upper extremity lymphedema deteriorates the postural stability in breast cancer survivors
- Postural Stability in Patients with Lower Limb Lymphedema
- Disability, psychological distress and quality of life in breast cancer survivors with arm lymphedema
DISFIGUREMENT
- Adjusting to disfigurement: processes involved in dealing with being visibly different
- Quality of Life in Cancer Patients with Disfigurement due to Cancer and its Treatments
- Quality-of-life and body image impairments in patients with lymphedema
- Association of lower extremity lymphedema with pelvic floor functions, sleep quality, kinesiophobia, body image in patients with gynecological cancers
QUALITY OF LIFE
- The impact of lower limb chronic oedema on patients' quality of life
- Quality of life in patients with primary and secondary lymphedema in the community
- Evaluating the effect of upper-body morbidity on quality of life following breast cancer treatment.
- Functionality and quality of life of patients with unilateral lymphedema of a lower limb: a cross-sectional study
- The effect of complete decongestive therapy on the quality of life of patients with peripheral lymphedema
PSYCHOSOCIAL IMPACT
- Psychosocial Impact of Lymphedema.
- Associations between chronic disease, age and physical and mental health status
- A network analysis of psychological flexibility, coping, and stigma in dermatology patients
- 'Abandoned by medicine'? A qualitative study of women's experiences with lymphoedema secondary to cancer, and the implications for care
- People are neglected, not diseases.
- Chronic edema/lymphoedema: under-recognized and under‐treated.
- Lymphatic Medicine: Paradoxically and unnecessarily ignored.
- Medical education: a deficiency or a disgrace.
PEDIATRIC LYMPHEDEMA
- NORD: Rare Disease Database - Lymphedema
- Medical management of lymphedema.
- Primary lymphedema in childhood.
- An approach to familiar lymphedema
- Pediatric Children Lymphedema
- Primary Lymphedema French National Diagnosis and Care Protocol (PNDS).
- Lymphatic filariasis: an infection of childhood.
- Lymphatic filariasis in children: clinical features, infection burdens and future prospects for elimination.
- Podoconiosis: Clinical spectrum and microscopic presentations.
- Podoconiosis: A Possible Cause of Lymphedema in Micronesia.
- A cross-sectional study to evaluate depression and quality of life among patients with lymphoedema due to podoconiosis, lymphatic filariasis, and leprosy.
- The impact of acute adenolymphangitis in podoconiosis on caregivers.
- Neglected tropical diseases and disability-what is the link?
- Locomotor disability in bancroftian filarial lymphoedema patients.
- Neglected patients with a neglected disease?
- Neglected tropical diseases and mental health: a perspective on comorbidity.
- People are neglected, not diseases.
- Addressing Inequity: Neglected Tropical Diseases and Human Rights.
- Neglected Tropical Diseases, Conflict, and the Right To Health.
- Peripheral Edema
- Phlebolymphedema
- Understanding Chronic Venous Disease: A Critical Overview of Its Pathophysiology and Medical Management
- Role of the lymphatic vasculature in cardiovascular medicine
- Communication between lymphatic and venous systems
- Phlebopathies and occupation
- Phlebopathies and workers
- Evidence of health risks associated with prolonged standing at work and intervention effectiveness
- Leg edema formation and venous blood flow velocity during a simulated long-haul flight
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- WHAT KIND OF DOCTOR TREATS LYMPHEDEMA/LYMPHOEDEMA - WHICH MEDICAL SPECIALITIES ARE RELATED TO LYMPHEDEMA
LYMPHEDEMA INCIDENCE
AND PREVALENCE
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LYMPHEDEMA INCIDENCE
AND PREVALENCE
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