lunes, 15 de enero de 2024

WORLD NEGLAECTED TROPICAL DISEASES DAY (NTD) 2024 - Organic Lymphatic Vessel Disease - Skin-Related Neglected Disease - Lymphostatic Elephantiasis - Lymphatic Filariasis - Podoconiosis - Mossy Foot Disease - Pufy Foot Syndrome - Secondary Lymphedema - Best Practices Guidelines - Management Interventions - Preventive Medicine - Rehabilitation Strategies - Organic and Physical Impairment (Disability) - Quality of Life – Human Rights - Awareness Campaign

 

LYMPHATIC ELEPHANTIASIS


World Neglaectd Tropical Diseases Day is an opportunity to celebrate the lives of those affected, raise awareness of the signs and symptoms of these conditions, and tackle the misconceptions and stigma that too often surround them, as is the case with Lymphatic Dysfunction (Lymphedema).


Lymphedema is a chronic inflammatory disease of the skin due to valvular impairment (Organic Lymphatic Vascular Disease) resulting in a progressive lymph fluid buildup. Lymphostatic elephantiasis (Stage III lymphedema ) results from non-treatment or under-treatment of lymphedema, which leads to consequent severe disfigurement, cutaneous complications, infections, physical disability, and low quality of life. Read more about what are the signs, symptoms, and complications of lymphedema HERE.


Episodes of acute dermatolymphangioadenitis (Infectious Cellulitis) and angiosarcoma (lymphangiosarcoma) are among the most severe clinical consequences of lymphedema, which can even be a direct cause of mortality. Read more about what are the recommendations and preventive measures to reduce dermatolymphangioadenitis HERE.


Lymphedema is classified as either:

  • Primary Lymphedema (genetic), is caused by a malformation of lymph vessels or nodes.

  • Secondary Lymphedema (acquired), resulting from trauma, chronic lymphatic or venous system overload, or the sequelae of cancer treatments.

    In developing countries, Secondary Lymphedema is also acquired by filariasis (parasites) or podoconiosis (barefoot exposure to irritant soil of volcanic origins). Progression to the elephantiasis stage is due to the nontreatment or undertreatment of the underlying lymphatic dysfunction.


Despite the fact that lymphedema is not a difficult disease to diagnose or manage, many patients are untreated or undertreated with low-cost ineffective methods. Lymphedema is easily managed in the majority of cases with adequate compression therapy (Complete Decongestive Therapy), and if spotted early, requires neither pharmaceuticals nor complex surgeries. For the maintenance phase, the majority of patients usually only need two compression garments per year, which sums much less than the medical expenditure of the majority of chronic diseases. The daily use of standard bandages for lymphedema is not practical, for they tend to loosen and slip off when undertaking work that requires continuous physical activity, and likewise, dirty bandages can also be an important source of infection.


Complete Decongestive Therapy (CDT) is not limited to lymphedema stages I and II, and even late Stage III lymphedema (elephantiasis) improves greatly with good results in the majority of cases, thus preventing the need for debulking techniques. Radical reductive ablative surgery (Charles Procedure), aimed to remove the diseased skin and subcutaneous tissue, should always be the last option, for it is frequently associated with significant blood loss, morbidity, infections, permanent disfigurement, and recurrence of symptoms. Read more about what is the best treatment for lymphedema HERE.


The progression of lymphedema toward the elephantiasis stage stems from a lack of access to adequate medical care, including the correct compression therapy and the necessary compression garments for the maintenance phase. Once the chronic swelling sets, it becomes difficult to manage the edema without permanent compression, affecting likewise the disease progression and thus the capacity to undertake daily tasks and work. Inadequate treatment or treatment carried out by therapists without certification in lymphatic disease, can aggravate the condition and even cause irreversible side effects.


Health literacy concerning lymphatic vascular disease in health workers is low. Misconceptions concerning measures to prevent the causes that lead to the elephantiasis stage are common among healthcare providers. There is the mistaken yet widespread belief that elephantiasis is of sudden onset, that it is only related to filariasis, or that it is not the result of undertreatment of lymphedema. Clearly, health care is held back as a result of inadequacies in both training and resources. Read more about what is the difference between elephantiasis and lymphatic Filariasis HERE.


Despite the high impact of the elephantiasis stage, in many countries treatment and control of lymphedema are hampered by a range of issues. There is a general lack of awareness of Lymphatic Vessel Disease by health professionals, Ministries of Health, and the general public, which leads to extreme neglect of individuals suffering from this debilitating disease. While permanent daily compression garment-wearing is one of the important keys to eliminating the elephantiasis stage, one cannot expect the practice to become widespread and enduring if it is not founded on an improved understanding of the disease by health workers and the general public.


A series of complex political and economic interrelated determinants collaborate to produce barriers to accessing effective treatment, and because lymphatic dysfunction (lymphedema) doesn't tend to result in immediate mortality, it has been treated as low priority by many national health systems, as such, lymphedema is one of the most neglected chronic diseases. It should be openly acknowledged, that particular human rights have been denied for many decades to those suffering from lymphostatic elephantiasis. Rights apply to individuals, and the well-being of those affected by lymphatic dysfunction relies on the responsibilities and duties of governments and international organizations, to provide the necessary resources for their health care. Read more about why elephantiasis is a forgotten and neglected disease HERE.


The answer to the question of who is responsible for the well-being of those affected by Lymphatic Vessel Disease must lie with governments, who are the principal duty-holders for preventing human rights abuses and for providing adequate health care. The key area for intervention is increasing the accessibility of compression garments, and public health efforts to subsidize compression garment distribution, which must be treated as a public health priority rather than a mere market opportunity. The violation of the right to access health treatment by duty-holders leads to consequent and additional human rights violations, principally restricted employment possibilities, social stigma, discrimination, psychological burden, and loss of quality of life.


Many health systems, even at the European level, are weak and under-resourced due to their low gross domestic product spent on health care. These countries only have the capacity to provide care to part of the population, and with a disproportionate share of funding focused on curative health care for very severe cases, and thus to the detriment of the patients suffering mild and severe lymphedema. Very few national Health systems advocate for the integration of lymphedema into the National Plans. Many promise to introduce lymphedema management services into government clinics and improve staff training, but the scheme is under-resourced, and government healthcare facilities do not serve all patients. Read about what country provides the best treatment coverage for lymphedema HERE.


CONCLUSION

Lymphostatic elephantiasis persists worldwide as the result of failures to provide the basic health care required, for the treatment of the underlying determinant which is lymphedema. This situation is caused by inadequacies and iniquities in access to appropriate compression therapy and the necessary compression garments. These factors constitute violations of the right to a standard of living appropriate for health, well-being, and quality of life. 



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 Expertise Center of Reference/Excellence 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 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

CUTANEOUS COMPLICATIONS
INFECTIOUS COMPLICATIONS
CELLULITIS / DERMATOLYMPHANGIOADENITIS
TUMOURS

TREATMENT

COMPRESSION GARMENTS

DISABILITY

DISFIGUREMENT

PSYCHOSOCIAL IMPACT

PEDIATRIC LYMPHEDEMA

LYMPHATIC FILARIASIS / PODOCONIOSIS



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