jueves, 23 de febrero de 2023

WORLD RARE DISEASE DAY 2023 - PEDIATRIC AND PRIMARY LYMPHEDEMA – Children with Lymphedema - Lymphatic Circulatory System Insufficiency - Lymphostatic Elephantiasis - Lymphatic Filariasis/Podoconiosis - Best Practices Management Guideline /Treatment Protocol/Patient Care Pathway

Rare Disease Day is celebrated around the world on the 28 of February to bring awareness to conditions that often escape notice. The common thread between these diseases is their lack of knowledge among the general public and even in the healthcare field. While rare and neglected diseases take up different spaces in the health sphere, both experience a lack of awareness, research, and treatment funding.

Approximately 300 million people are affected globally by 6000 to 8000 different rare diseases and in Europe more than 30 million. Many of these diseases manifest during childhood, and some are devastating and even life-threatening.

Primary Lymphedema is not only a rare but also a neglected disease in many countries, especially in the world’s poorest communities. As the case for most rare diseases, inaccessibility to Lymphedema treatment, or the implementation of low-cost alternative ineffective solutions, due to the health policies of some national systems, can cause disease progression towards the severe ELEPHANTIASIS stage, which means suffering serious infections, disfigurement, disability, loss of income, and even social discrimination for those affected. Read more about the symptoms and important complications of lymphoedema HERE.

Lymphedema is a disease of the skin due to lymphatic valve incompetence (Organic Disability) which causes inflammation and lymph fluid buildup. Lymphatic dysfunction can lead to skin changes and infections, disfigurement, and physical impairment (Physical Disability). Scientific studies highlight the importance and implications of compression therapy in controlling the progression of Lymphoedema. Clinical outcomes on interventions to manage lymphedema show that simple hygiene-based measures are necessary, but these alone are not enough to reduce limb size and volume. 

Complete Decongestive Therapy (CDT) is not limited to lymphoedema stages I and II, and even late Stage III lymphoedema (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.

With reference to pediatric lymphedema, it is essential that children diagnosed with lymphatic dysfunction, receive early preventive compression treatment with the first signs, to minimize fluid build-up and prevent later life severe clinical complications, disfigurement, and disability. With reference to lymphatic filariasis, recent tests have revealed that LF is first acquired in childhood, often with as many as one-third of children infected before age 5. Prevention and controlling early warning signs and symptoms are important since there is no cure. Read more about what is the best treatment for children with lymphoedema HERE.

Rare Disease Day is an observance held on the last day of February to raise awareness for rare diseases and improve access to treatment and medical representation for individuals with rare diseases and their families. Treatment for numerous rare diseases is insufficient, and many patients are not treated or undertreated by their healthcare providers. Patients not only need access to a prompt diagnosis but also their human right to access the best treatment possible, which the healthcare providers should make available.


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.


(Click on the texts to read the research articles)











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