jueves, 3 de agosto de 2023

WHAT IS THE BEST TREATMENT OPTION FOR PODOCONIOSIS? - MOSSY FOOT DISEASE - Organic Lymphatic Vascular Disease - Organic and physical Impairment - Secondary Lymphedema - Pediatric Lymphedema - Infectious Cellulitis - Dermatolymphangioadenitis (DLA) - Lymphostatic Elephantiasis - Best Clinical Practice Guideline - Treatment Protocol - Patient Care Pathway - Education and Health care - Disability and Morbidity Prevention


Podoconiosis is a non-filarial, non-infectious disease, caused by long-term exposure to red clay soil derived from volcanic rock which causes destruction of the lymphatic circulatory system in the feet and legs. Once lymphedema (Organic Lymphatic Vessel Disease) is established, the well-known cutaneous manifestations and natural progression of the disease are clear. 


The infection is stated to occur mostly in childhood and the disease first becomes symptomatic in the early teens and affects both sexes. It starts first from the feet and then spreads up the legs to the level of the knees, and is caused by blockage of regional lymph nodes and vessels. Although both legs are involved, leg swelling tends to be asymmetrical. 

Podoconiosis disease is most prevalent in resource-limited countries and especially among agrarian people who work barefoot, particularly in the neighborhood of volcanoes.

 

Podoconiosis symptoms include leg and foot pain, flare with exertion, itching, burning, swelling in the sole of the foot, marked soft tissue thickening, underlying bone changes, and bacterial infection. If left untreated or undertreated, the condition progresses toward Stage III Lymphedema, also called elephantiasis, characterized by large edema, and leathery pigmented feet. and lower leg disability and disfigurement. Read more about the signs, symptoms, and complications of lymphoedema 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.


The good news for Podoconiosis is that established lymphatic dysfunction is treatable. Therapy of peripheral lymphedema is divided into conservative (non-operative) and operative methods. The best first-line non-surgical treatment for lymphedema, considered the "Gold Standard" treatment, is Complete Decongestive Therapy (CDT).


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. 



The success of Complete Decongestive Therapy (CDT) depends on it being administered by an experienced certified therapist. CDT is considered safe, however, patients should consult with their physician before beginning treatment. Indications, contraindications, and the application of individual therapeutic measures depend on several factors: the stage of lymphedema at the start of treatment, comorbidities, the age of the patient, and the patient’s individual life circumstances. 

Failure of CDT should be confirmed only when intensive non-operative treatment in a clinic specializing in the management of peripheral lymphedema and directed by an experienced clinical lymphologist has been unsuccessful. 


In no way should this highly specialized treatment be replaced, due to the possible serious side effects, by edema reduction treatments carried out by non-specialists. Aggressive reduction of swelling can cause skin and vessel damage. Isolated compression therapy of the extremities without Manual Lymphatic Drainage (MLD), particularly in patients with lymphedema also affecting the quadrants of the trunk, may cause the shift of edema and increase swelling at the root of the extremity, and may even lead to genital lymphoedema.

 


Due to derangement of lymphatic function (Organic Lymphatic Vessel Disease) and resulting weak body defense, the limb with lymphedema is prone to repeated bacterial and fungal infections. Concerning Dermatolymphangioadenitis (DLA), also known as infectious cellulitis, studies have shown that swelling control is associated with a lower risk of cellulitis, and furthermore, that advanced degrees of chronic edema are a strong risk factor. Infectious Cellulitis can lead to sepsis (Septicaemia). Read more about lymphedema and Infectious Cellulitis HERE.


At the personal and work level, the majority of individuals affected by lymphedema cannot devote all hours of the day to exercise and limb elevation to retain the progression of edema, for they also need to attend to their daily chores and jobs, and this can only be achieved by wearing compression garments. 


The daily use of standard bandages is not practical, for they tend to loosen and slip off when undertaking work that requires continuous physical activity, and likewise, soiled bandages can also be an important source of infection. 


For the maintenance phase of lymphedema management, compression is the most important intervention. Compression garments are easy to use and achieve significant improvements in most patients without causing much discomfort. The beneficial value of applying compression stockings in the treatment of venous and lymphatic disease is supported by recommendations rated as Grade 1 evidence.  Read more about what is the best treatment for elephantiasis HERE.



The use of footwear is very important for the prevention of Podoconiosis, but once chronic lymphatic dysfunction (lymphedema) is established, shoes alone are not enough to stop the advance of the disease. Scientific studies highlight the importance of compression therapy in controlling the progression of lymphedema


Clinical results of interventions to control lymphoedema show that simple hygiene-based measures (skin care, exercises, elevation of affected limbs, and use of footwear) are necessary, but these alone are not enough to control the volume progression of the extremities. 


Self-management strategies according to the chronic care model (CCM), where patients with chronic diseases should assume a leading role in their treatment to improve health status, daily functioning, and quality of life, should not be a substitute for the proper and necessary clinical treatment that this disease requires. Read about why the world is not treating a treatable disease like lymphedema HERE.


It has been scientifically demonstrated that the majority of individuals that suffer from Stage III Lymphedema, can´t be free of disability, disfigurement, and stigma that symptoms bring, and can´t be productive and live more fulfilling lives, without access to proper treatment. Strategies for morbidity management and disability prevention with simple intervention packages, that only focus on the care of acute infections and not on the necessary control of edema progression, will not halt the advance of lymphedema toward the final elephantiasis stage.

 


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 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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