domingo, 6 de septiembre de 2020

WHAT IS THE BEST TREATMENT FOR PAEDIATRIC AND PRIMARY LYMPHOEDEMA - Milroy Disease – Meige Disease - Lymphoedema Tarda - Congenital Hereditary Lymphoedema – Children with Lymphoedema – Quality of life & Disability - Rare Disease

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 a chronic progressive inflammation and swelling (oedema), which may be present in the trunk, face, genitalia, hands, arms, legs, feet and internal organs too. The Lymphatic dysfunction 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. 

ORGANIC DYSFUNCTION AND FUNCTIONAL LIMITATIONS - DISABILITY AND QUALITY OF LIFE OF PATIENTS WITH PEDIATRIC AND PRIMARY LYMPHEDEMA

With regard to functional limitations and quality of life of patients with lymphatic dysfunction (this is the Heath Condition) and disability recognition, in the case of lymphoedema there is a dual Functioning impairment. On the one hand there is a PERMANENT ORGANIC DISABILITY ( this is the Body Function) due to failure in the Lymphatic System, and on the other hand it may lead to a PERMANENT PHYSICAL DISABILITY (this is the Body Structure), due to the functional deficit produced by the consequent inflammation and progressive chronic oedema. Depending on the disease location and severity, patients may experiance difficulties in executing activities (this is the Activity Limitation), or problems relatedted to involvement in life situations (this is the Participation Restriction), which make up the physical, social and atttitudinal circumstantial considerations (these are the Personal and Enviromental Contextual Factors). Organic dysfuncion in lymphoedema means that the cause of the disability is physically based, and not due to difficulties caused by social or enviromental factors. Read more about lymphoedema and disability HERE.


WHAT IS THE GOLD STANDARD TREATMENT FOR PEDIATRIC AND PRIMARY LYMPHOEDEMA

After Clinical evaluation and imaging tests to confirm diagnose of Lymphoedema, COMPLETE DECONGESTIVE THERAPY (CDT) is recommended as first option “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 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 physiotherapist with training at specialist level. After the volume of the limb is stabilized, the patient enters the maintenance phase in which she/he continues to skin care, compression garments, and exercises. In phase II, the components of CDT are applied according to the individual oedema findings, and depending on the course of disease, phase I may have to be repeated in the case of intercurrent diseases.

The ideal setting and approach for treating lymphoedema is in a Multidisciplinary Unit, where the special needs of each patient are addressed. The team should be composed of a physiotherapist, dermatologist, dietician, internist, pediatrician, and vascular and/or plastic surgeon, which should work together in the assessment and management of all aspects of lymphoedema. Read more about 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 counselled on the importance of adhering to lymphoedema basic-care recommendations, to help prevent progression of the disease and possible complications. 

References:

GERMAN SCIENTIFIC SOCIETY GUIDELINE: Diagnostics and Therapy of Lymphoedema.

THE INTERNATIONAL SOCIETY OF LYMPHOLOGY: Consensus Document.

NORD - National Organization for Rare Diseases. Primary Lymphedema.




LYHMPHEDEMA INCIDENCE 
AND PREVALENCE
(click on the texts) 








CAMPAIGN
 


For global awareness it is being asked that the 
WHO - WORLD HEALTH ORGANIZATION
name: 
"LYMPHEDEMA - AWARENESS & CURES"
as the World Health Day campaign 

  

  KATHY BATES
LYMPHEDEMA EMBASSADOR


SIGN THE PETITION HERE







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