martes, 30 de agosto de 2022

WHAT ARE THE SIGNS, SYMPTOMS AND COMPLICATIONS OF LYMPHEDEMA - LIST OF THE STRUCTURAL AND FUNCTIONAL IMPAIRMENTS - Organic Lymphatic Vascular Disease - Primary Lymphedema - Secondary Lymphedema - Lymphostatic Elephantiasis - Filariasis/Podoconiosis - Disability and Lymphedema

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 Imparment HERE.


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. Read more about lymphedema and Infectious Cellulitis HERE.


lymphedema arises when there is a disruption of lymphatic flow (Organic Disability), leading to the buildup of lymphatic fluid. Individuals affected by lymphedema may experience difficulties in completing activities of daily living and maintaining employment. Nontreatment and undertreatment of lymphedema are associated with an unfavorable prognosis of disease progression and severity, which are barriers to work and causes of social stigmatization, discrimination, and loss of Quality of Life (QoL). 


Lymphedema is a disease that may affect arms, legs, fingers, toes, head, neck, abdomen (belly), genitalia, internal organs, or the whole body, which causes chronic inflammation and swelling in the affected area. Read more about disability and lymphedema HERE.


Treatment for lymphedema depends on the severity and extent of the condition. Prevention and controlling symptoms are important since there is no cure. To properly understand and evaluate how lymphedema affects people’s overall health, impairments, and Quality of Life (QoL), the following questions should be considered:

  1. What is it like to live day by day with lymphedema?
  2. How does lymphatic dysfunction limit an individual?
  3. What is the principal cause of disease progression?
  4. What complications and degrees of disability can lymphedema cause?
  5. What levels of anguish and suffering can be experienced by those affected?

These are some of the symptoms and complications resulting from lymphatic dysfunction (lymphedema) of the upper and lower limbs:


SIGNS AND SYMPTOMS

Lymphedema signs and symptoms may include:

  • Edema (chronic inflammation).
  • Lymphatic stasis (Lymphostasis / stagnant lymphatic flow).
  • Progressive swelling (increased edema volume).
  • Structural change of the skin.
  • Loss of hair on the affected limb.
  • The dorsum of the second toe cannot be pinched (positive Kaposi-Stemmer Sign)
  • Skin dents when pressed in the early initial stages (pitting edema).
  • Skin does not dent when pressed in late advanced stages (non-pitting edema).
  • Edema does not subside with limb elevation (only in the mild first stages).
  • Abnormal wound healing (Functions of the Skin and the Immune System).
  • Nail structure disorders (Functions of Nails).
  • Visible disfigurement (deformed/enlarged body parts).
  • Fullness sensation (puffiness).
  • Heaviness sensation (feeling of weight).
  • Weakness sensation (debilitation).
  • Fatigue sensation. (urging rest).
  • Burning sensation (warmth and redness).
  • Pins and needles/itching sensation (paresthesias).
  • Tightness or stiffness sensation.
  • Tenderness and soreness to the touch.
  • Numbness sensation.
  • Discomfort, aching, or pain sensation.


COMPLICATIONS THAT MAY DEVELOP 

Lymphatic dysfunction may include the development of:

  • Coagulation in the lymph (Lymphatic Thrombosis).
  • Inflammatory episodes involving the skin (erythema).
  • Soft-tissue changes (Skin Structure Disorder).
  • Tissue induration-scarring (fibrosis).
  • Dry, hard, thick, and scaly skin (hyperkeratosis).
  • Papules, often described as frogspawn (cutaneous lymphangiectasia).
  • Wart-like lesions/cobblestone appearance (papillomatosis).
  • Velvety growth with warty projections (verrucosis).
  • Deformities of the dorsal skin of toes (square or box toes).
  • Upslanting ''ski-jump'' toenails (dysplastic toenails).
  • Disturbed nail growth/dorsal deviation (nail hypoplasia).
  • Ingrown toenails (onychocryptosis).
  • Yellowing of nails.
  • Skin discoloration.
  • Skin fold retractions (deepened folds that cannot be widened).
  • Restricted range of flexibility (limited mobility).
  • Difficulties with extreme hot or cold temperatures.
  • Abdominal swelling (bloating),
  • Genital lymphedema (internal/external genitalia).
  • Disturbed gastrointestinal lymphatic flow (Intestinal Lymphangiectasia).
  • Aching swelling due to lymph pressure congestion (sensory nerves).
  • Pain due to the tension forces upon the subcutaneous tissue.
  • Aching joints in the affected limb.
  • Ear pain, nasal congestion, or affected vision (head, face, and neck lymphedema).
  • Cutaneous fungal infections.
  • Bacterial upper dermis skin infections (Erysipelas).
  • Bacterial inner layers of skin infections (Cellulitis / Dermatolymphangioadenitis-DLA).
  • Infection of the lymphatic vessels (Lymphangitis ).
  • Inflammation of lymph nodes (Lymphadenitis).
  • Inflammation involving lymph nodes and lymph vessels (Adenolymphangitis).
  • Poor healing wounds and recurring skin infections (Suppressed Immune Function).
  • Skin abscesses (areas of ulceration and/or tissue damage/necrosis).
  • Skin breakdown (fissures).
  • Lymph fluid leaking from the skin (Lymphorrhoea).
  • Malignant tumors (Lymphangiosarcoma).
  • Amputation.
  • Sepsis (septicaemia/septicemia). 


FUNCTIONAL PROBLEMS THAT MAY DEVELOP

Lymphedema may include experiencing:

  • Difficulties with restricted limb mobility (increased volume and weight).
  • Difficulties with the involvement of limb rotation.
  • Difficulties with standing or sitting for long periods.
  • Difficulties with walking long distances (walking or Gait Disorders).
  • Difficulties with bending or getting up (Functionality Disorders).
  • Difficulties in performing basic daily household activities.
  • Difficulties in self-care.
  • Difficulties in sexual performance (Genital Lymphedema/Dyspareunia).
  • Difficulties in engaging in some sports or leisure activities.
  • Poor quality of sleep.


PERSONAL AND SOCIAL IMPACT ON INDIVIDUALS

The impact of Lymphedema at the personal level may include experiencing:

  • Problems in finding well-fitted clothing or footwear.
  • The need to use orthopedic shoes.
  • The need to use Compression Garments all day long.
  • Problems with time-consuming daily self-management regimes.
  • Hours or days unable to perform usual activities due to edema symptoms.
  • Appearance-related social discrimination (visible physical disfigurement).
  • Negative social situations from the community or family members (Social Stigmatization).

The impact of Lymphedema at the family and partner level may include experiencing:

  • Being a financial burden to the family.
  • Difficulties regarding relationships with family and others.
  • Impaired sexual function (genital lymphedema or physical disfigurement-related issues).
  • Inability to get a partner or to get married.
  • Abandonment by their partner or spouse.
  • Needing help to carry out daily activities.
  • Requiring constant care and attention.


The impact of Lymphedema at the occupational level may include experiencing:
  • Difficulties with jobs involved in heavy lifting with the affected limb (heavy work).
  • Difficulties with jobs involved in pushing or pulling with the affected limb.
  • Difficulties with jobs involved in constant use of the affected limb (overstressing work).
  • Difficulties with jobs involved in standing or sitting for long periods.
  • Difficulties with jobs involved in the possible trauma/injuries of the affected limb (Immunity deficiency risk).
  • Difficulties with jobs involved in exposure to hot/cold temperatures of the affected limb.
  • Difficulties with jobs involved in precision work with hands and fingers of the affected limb.
  • Difficulties with jobs with restrictions in specific occupations due to wearing compression garments.
  • Difficulties with jobs involving long periods of work without rest (work performance issues).
  • Need to modify the workspace to accommodate the aching limbs.
  • Disadvantage to accessing jobs due to the visible disfigurement or physical impairment.
  • Discrimination in the workplace due to the impairment or visible difference.
  • Being worse off in terms of work performance and career.
  • Problems with full-time employment due to requiring more time for complex care.
  • Need to reduce work hours to accommodate the aching limbs.
  • Consequent low-paid employment and productivity losses (limited earning capacity).
  • Need to stop employment (days of work lost).


The impact of Lymphedema at the health care level may include experiencing:
  • Problems to access publicly financed treatment.
  • Problems with copayments in medical consultations (medical expenses).
  • Problems with direct/indirect out-of-pocket therapy-related expenses.
  • Problems with direct costs for compression garments/orthopedic footwear.
  • Requiring recurrent hospitalization (bacterial infections).
  • Developing the need for continuous antibiotic therapy.
  • Need to depend on other people.
  • Becoming housebound.
  • Loss of Quality of Life (QoL).


PSYCHOSOCIAL IMPACT

The consequent psychosocial impact of Lymphedema on individuals may include developing:

  • Distress caused by negative social experiences (Social Stigmatization).
  • Distress caused by disease progression and complications.
  • Distress caused by not having access to proper treatment (nontreatment or undertreatment).
  • Distress caused by a reluctance to participate in public events (social avoidance).
  • Distress caused by embarrassment to exposing oedematous limbs (disfigurement/Visible physical difference).
  • Distress caused by negative body image (self-stigmatization).
  • Distress caused by difficulties finding personal relationships or getting married.
  • Distress caused by a reluctance to engage in sexual activity (Physical and Disfigurement-Associated Impairments).
  • Distress caused by unsupportive spouse or family.
  • Distress caused by fear of losing the family or spouse.
  • Distress caused by an unsupportive employer.
  • Distress caused by fear of losing their job.
  • Distress caused by having to rely on others to accomplish housework or a job.



The ICF (International Classification of Functioning, Disability, and Health), is the international standard to describe and measure health and disability, and it includes disfigurement, sensory impairments, nails and congenital malformations, skin swelling and infections, pain, immunity response, functions of the lymphatic vessels and nodes, and general mobility and functional impairments which have the potential to interfere with the personal and social levels. 




Regarding disability and Quality of Life (QoL) in disorders of the lymphatic circulation, the physical impairments and consequences (symptom and function domains), are the most important factors and what most conditions individuals, and which consequently may also trigger the psychological and emotional outcome on their personal and social functioning. Read more about what is the psychological and quality of life impact of nontreatment or undertreatment in lymphedema HERE.


The direct and indirect out-of-pocket expenses of lymphedema management and overall healthcare may include payments for outpatient physician visits, physical therapy visits, complementary and integrative therapy visits, emergency department visits, hospitalizations, labs, X-rays and tests, lymphedema-specific healthcare needs (compression garments, bandages), medications or other health-related-specific product (antibiotics, antifungal creams, moisturizing ointments), etc.


If the disease is not identified and treated in the earlier stages, the patient prognosis is worse, and treatment is more costly. Higher costs may delay retirement and increase the inability to access proper health care. 



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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