WHAT IS THE BEST TREATMENT OPTION FOR ELEPHANTIASIS - Organic Lymphatic Vascular Disease - Pediatric and Primary Lymphedema -Secondary Lymphedema - Lymphatic Filariasis - Podoconiosis - Dermatolymphangioadenitis (DLA) - Choosing an Lymphedema Expert Reference/Excellence Centre

First, it must be analyzed whether the patient's basic home setting management alone, consisting of skin hygiene, motion exercises, standard bandaging, limb elevation, and footwear, is enough to reduce and stop the progression of lymphedema toward the Elephantiasis Stage.

The effectiveness of basic hygiene, consisting of skin care, exercises, standard bandaging, and elevation of lymphoedematous extremities, are a part of the daily routine of patient self-care and are aimed at the prevention of possible dermal infections, but this is not sufficient in many cases to stop complications such as dermatolymphangioadenitis - DLA (infectious cellulitis), erysipelas or lymphangitis, without the necessary lymphatic therapy. Clinical results of interventions to control lymphedema show that simple hygiene measures are necessary, but these alone in many cases are not enough to stop the progression of the disease. Read more about the signs, symptoms, and complications of lymphedema HERE. 

Lymphedema is a progressive disfiguring and disabling disease of the skin, classified as a functional, immune, and lymphatic circulatory system disorder (Organic Lymphatic Vascular Disease). The lymphatics are an active and integrated component of the immune response, and in lymphedema, there is always an increased susceptibility to infection due to the compromised immune system. Read more about what is an Organic Impairment HERE. 

Lymphedema arises when there is a disruption of lymphatic flow (Organic Impairment), leading to the buildup of lymphatic fluid. It is clear that no patient or their lymphedema is the same and neither is its progress. Some patients with lymphedema have few symptoms, and can easily do different degrees of physical activity, while others feel the strain, experience increased swelling with minimum effort, or even suffer varying degrees of disability and severe complications. Read more about lymphedema and Infectious Cellulitis HERE.

WHAT IS LYMPHATIC FILARIASIS AND PODOCONIOSIS?

Lymphedema is a chronic progressive disease, and an uncomplicated lymphedema can easily turn complicated with inadequate management options. There is a safe and effective treatment for lymphedema, which helps to reduce edema and halt the progression of the disease towards its advanced stages. Simple health care packages (foot hygiene, compression with standard bandages, exercises, limb elevation, and use of foot wear) alone are usually not enough to control disease progression and subsequent complications (lymphatic dysfunction).  

The safe and effective first-line treatment for lymphedema is Complete Decongestive Therapy (CDT) carried out by a lymphatic therapist at specialist level, which is considered the “Gold Standard” conservative treatment. This specialized treatment consists of Manual Lymphatic Drainage (MLD) and Multi-layered Bandaging, as well as other components such as skin care, diet, exercise, and patient education. Read about the best treatment option for lymphedema HERE.

Compression therapy for the initial reduction phase applied without Manual Lymphatic Drainage (MLD), or treatment that is not carried out by a lymphatic therapist at specialist level, can cause very severe complications such as displacement of lymph to previously non-affected areas, which can provoke Genital Edema in the case of lower limb lymphedema. People with lymphedema need access to specialized care throughout their lives, both to manage the disease and to prevent progression to more advanced stages such as elephantiasis. Read more about the principal functions of Manual Lymphatic Drainage HERE.

Conservative treatment is indicated for all edema stages, and even advanced lymphedema can be successfully treated with CDT. Avoiding the initial progression toward the elephantiasis stage can help evade the need for ablative surgery. Radical reductive ablative surgery, aimed at removing subcutaneous tissue, should always be the last option, for it is associated with possible significant blood loss, morbidity, infections, permanent disfigurement, and recurrence of symptoms.

Apart from the patient's daily basic self-care, patients also need to wear compression garments (orthotic devices) throughout the day. Without compression, it is impossible to stop the progression of lymphatic edema. The daily use of standard bandages for lymphedema is not practical. Bandages lose pressure after application due to edema reduction, and they also tend to loosen and slip off when undertaking work that requires continuous physical activity, likewise, dirty bandages can also be an important source of infection. While bandages are mainly used for the initial phase of reduction therapy, compression garments are recommended for the maintenance and long-term management. Read more about the importance of compression garments in treating lymphedema HERE.

International protocols and best practice guides recommend simple hygiene-based measures for almost all diseases, but in the case of lymphedema, this is not enough for its volume control, and to prevent the progression of its severe clinical manifestations. Lymphatic edema without treatment or under-treated is more likely to develop lethal complications, such as infectious cellulitis, septicemia (sepsis) or lymphangiosarcoma. Read more about which country provides the best lymphedema treatment coverage HERE. 

CLICK TO SEE A VIDEO OF THE BEST TREATMENT OPTION FOR ELEPHANTIASIS-STAGE-4-LYMPHOEDEMA (NON-SURGICAL TREATMENT):

Regarding Secondary Lymphedema due to filarial infection (lymphatic filariasis), some countries have successfully interrupted transmission of filariasis employing mass treatment with antiparasitic drugs, but access to good compression treatment and compression garments to prevent morbidity management and disability in established lymphedema has lagged behind, and so people continue to suffer from the disabling and stigmatizing effects of Lymphedema Stage III-Elephantiasis.

IMPORTANT NOTICE 

Lymphedema is a serious chronic and progressive disease due to lymphatic dysfunction (Organic Lymphatic Vascular Disease). 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 Expert 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 the best treatment option for Lymphedema HERE.

• Read about the principal functions of Manual Lymphatic Drainage HERE.

• Read about the differences between the two principal lymphedema treatment protocols HERE. 

• Read about the strategies for the implementation of low-cost treatment options for Lymphedema HERE.

WHAT IS ELEPHANTIASIS / MYTHS AND FACTS

REFERENCES

(Click on the texts to read the research articles)

SCIENTIFIC SOCIETIES

• German Scientific Society Guideline: Diagnostics and Therapy of Lymphoedema.

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

CUTANEOUS COMPLICATIONS

• 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.

INFECTIOUS COMPLICATIONS

• Lymphatic Flow: A Potential Target in Sepsis.
• Infectious complications of lymphedema.
• Acute inflammatory exacerbations in lymphoedema.
• Inflammatory Manifestations of lymphedema.

CELLULITIS / DERMATOLYMPHANGIOADENITIS

• 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.

TUMOURS

• 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

PAIN

• 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.

COMPRESSION GARMENTS

• 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.

LYMPHATIC FILARIASIS / PODOCONIOSIS

• 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.

BLOOD VASCULAR SYSTEM 

• 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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LYMPHEDEMA INCIDENCE 
AND PREVALENCE
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