domingo, 6 de septiembre de 2020

WHAT IS THE BEST TREATMENT OPTION FOR PAEDIATRIC AND PRIMARY LYMPHEDEMA - Milroy Disease – Meige Disease - Lymphoedema Tarda - Congenital Hereditary Lymphoedema – Quality of life & Disability - Children with Lymphedema - Lymphostatic Elephantiasis - Lymphatic Filariasis/Podoconiosis - Best Practices Management Guideline / Patient Care Pathway

Primary lymphoedema can be hereditary or sporadic. Hereditary lymphoedema, also known as Primary Congenital Lymphoedema, is a genetic disorder affecting the lymphatic system and is inherited as an autosomal dominant trait. There are three forms of hereditary lymphoedema:

  • Lymphoedema that appears at birth (MILROY DISEASE)
  • Lymphoedema that appears at puberty/adolescence (LYMPHOEDEMA PRAECOX or MEIGE DISEASE)
  • Lymphoedema that appears after the age of 35 (LYMPHOEDEMA TARDA)

Hereditary lymphoedema may be associated with several genetic multisystem disorders. Sporadic lymphoedema is referred to when there is no obvious cause or family history (the condition is not inherited).

Lymphoedema is characterized by chronic progressive inflammation and swelling (edema), which may be present in the trunk, face, genitalia, hands, arms, legs, feet, and also internal organs. Lymphatic dysfunction (Organic Lymphatic Vessel Disease) is due to obstruction, malformation, or underdevelopment of the lymphatic vessels, which causes the accumulation of protein-rich fluid (lymph) in the soft layers of tissue under the epidermis. 

WHAT IS THE GOLD STANDARD TREATMENT FOR PEDIATRIC AND PRIMARY LYMPHOEDEMA

After Clinical evaluation and imaging tests to confirm the diagnosis of Lymphoedema, COMPLETE DECONGESTIVE THERAPY (CDT) is recommended as the first-line Gold Standard treatment, consisting of intensive and long-term management phases. Conservative therapy in childhood needs to be suitably adapted to the respective age and involves the direct participation of the parents. The initial intensive phase aims to reduce swelling and normalize the tissue pressure and consists of Manual Lymph Drainage (MLD) in combination with multilayered compression bandaging, meticulous skin care, exercise, and the use of well-fitted compression garments. CDT and MLD should be conducted by lymphedema therapists with training at the specialist level. After the volume of the limb is stabilized, the patient enters the maintenance phase in which she/he continues with skin care, compression garments, and exercises. In Phase II, the components of CDT are applied according to the individual edema findings, and depending on the course of the disease, Phase I may have to be repeated, or in the case of intercurrent diseases.

The ideal setting and approach for treating lymphoedema are in a Multidisciplinary Unit, where the special needs of each patient are addressed. The team should be composed of a lymphedema therapist, dermatologist, dietician, internist, pediatrician, and vascular and/or plastic surgeon, who should work together in the assessment and management of all aspects of lymphoedema. Read more about what kind of doctor treats lymphoedema HERE.

There is no cure for lymphoedema, but as well as other chronic diseases that need life-long treatment or medication to control the condition, in the majority of cases lymphoedema also needs continued treatment for maintenance and control of progression. Patients require regular revisions and therapy, so as to improve their physiological response to the disease and ultimately, also enhance their quality of lifeWith proper diagnosis and management, the progression and potential complications of lymphoedema may be limited in many cases. Read more about which country has the best lymphoedema treatment coverage HERE.

Patients and their parents/caregivers should also be counseled on the importance of adhering to lymphoedema basic-care recommendations, to help prevent the progression of the disease and possible complications. 



IMPORTANT NOTICE 

Lymphedema is a serious chronic and progressive disease due to lymphatic dysfunction (Organic Lymphatic Vessel 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 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

CUTANEOUS COMPLICATIONS
INFECTIOUS COMPLICATIONS
CELLULITIS / DERMATOLYMPHANGIOADENITIS
TUMOURS

TREATMENT

COMPRESSION GARMENTS

DISABILITY

DISFIGUREMENT

PSYCHOSOCIAL IMPACT

PEDIATRIC LYMPHEDEMA

LYMPHATIC FILARIASIS / PODOCONIOSIS


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