A model for self-management of chronic filarial lymphoedema with acute dermato-lymphangio-adenitis

Abstract

Secondary lymphoedema due to filariasis is a leading cause of morbidity in India. We present a case of a 54-year-old female with lymphatic filariasis for 18 years, with three to four episodes of acute dermato-lymphangio-adenitis (ADLA) every year. The patient had voluntarily restricted social interaction and community participation. A combination of interventions provided in 17 physiotherapy sessions over a period of 20 days showed notable improvement, by reducing limb volume and ADLA episodes and by improving limb shape, functional capacity and quality of life. Interventions included aerobic exercise, decongestion therapy with faradism under pressure and exercises with elevation. This gain was maintained throughout the 8 months of the pandemic. Patient education and counselling along with home programme of self-bandaging and self-limb hygiene played a major role in the recovery of the patient. Self-reliance in management was important because of the pandemic, which restricted the patient from attending the outpatient department.

Background

Lymphatic filariasis (LF) is the second most vector-borne disease after malaria in India and the third globally after malaria and tuberculosis. LF is caused due to infection by Wuchereria bancrofti, which invades the lymphatics of humans, initiating pathological changes leading to lymphatic and extralymphatic disease manifestations.¹ India accounts for more than 40% of the global LF burden.² Deformities caused by LF, acute febrile episodes associated with it and acute dermato-lymphangio-adenitis (ADLA) adversely affect physical, social, psychological, sexual and economic domains of life.³ The pathogenesis of LF and ADLA is described in figure 1. Lymphatic system dysfunction leads to the accumulation of protein-rich fluid in lymphatics, which causes inflammation, adipose tissue hypertrophy and fibrosis, which makes lymphoedema not fully reversible but can be reduced and controlled significantly.^{4 5}

Figure 1.

Pathogenesis of lymphatic filariasis and vicious cycle of ADLA. ADLA, acute dermato-lymphangio-adenitis.

In 2000, WHO had launched a Global Programme to Eliminate LF by which India had expected eradication of filariasis from the country by 2020, but was not successful. LF, if left untreated, gradually progresses to elephantiasis.^{1 3} Multidimensional approach of physiotherapy plays an important role in reducing lymphoedema, delaying ADLA episodes and improving functional capacity, limb volume and hence the quality of life of the patient. Evidence is scarce regarding the role of physiotherapy management in LF. This case report brings out the improvement in outcomes following 20 days of daily physiotherapy sessions. It highlights the importance of cost-effective self-care education which helped our patient to retain the gains even after 8 months of lockdown.

Case presentation

A 54-year-old married female, diagnosed with secondary unilateral severe lymphoedema due to filariasis, presented with swelling of the right lower limb. Eighteen years ago, after an insect bite, she developed swelling starting from the right ankle, which gradually progressed to her mid-leg and then to the hip. She had three to four episodes of ADLA every year for the past 12 years. She undertook intermittent treatment for the same and was advised to use short-stretch multilayered bandages and stocking but was not referred for physiotherapy. She did not continue with the treatment as prescribed due to frustration with the disease not responding favourably. The last episode of ADLA was 10 months ago, which was medically managed and she was referred for physiotherapy for the first time then. The patient complained of pain, difficulty in walking, getting up from the floor and feeling of limb heaviness due to which she had left her job as a domestic cook. Muscle strength of plantar flexors and quadriceps was 3+/5, which indicated muscle weakness. The skin of the affected leg was dry, rough and darkly pigmented. Multiple skin folds, more near the ankle, and scaling of the skin of the leg and sole with clear fluid droplets oozing out of the skin were observed (figure 2). Trapping of sweat in the skin fold gave malodour because of which she had reduced social interaction. Dermatologist prescribed moisturising and skin softening cream.

Figure 2.

Skin changes.

Assessment

Limb volume measurement was undertaken with the tape measure method, using the cylinder equation formula every day in the morning.⁶ Quality of life and functional capacity were assessed by LF-Specific Quality of Life Questionnaire (LFSQQ) and 6 min walk test, respectively.^{7 8} On the fourth day, she developed tenosynovitis of the tibialis anterior tendon, causing pain.

Physiotherapy management offered to her is described in figure 3 and table 1. Home programme and medical treatment are described in table 2.

Figure 3.

Components of physiotherapy in lymphoedema management. ADLA, acute dermato-lymphangio-adenitis.

Table 1.

Physiotherapy management in outpatient department

Treatment	Description	Dosage
1. Patient education, counselling and therapeutic advice for lifestyle modification	Patient education consisted of: Encouragement to speak about her concerns and conflicts and counselling for the same Explaining the need for active participation in the therapy, the importance of limb hygiene and lifelong compliance to prevent recurrence and maintenance Explaining the effect of static postures for long duration like prolonged sitting, standing, sitting with legs hanging in a gravity-dependent position and cross leg sitting Advice about refraining from wearing tight-fitting clothes to avoid local compression and to wear comfortable modified footwear that would accommodate the excess volume of the foot due to lymphoedema and bandage Referral to dietician Helping her understand the necessity of wearing stockings or bandages	Once per 2 weeks and as required in between
			2. Self-care and limb hygiene procedure	It was performed as per Morbidity Management and Disability Prevention recommendations. Regular limb washing with mild, non-perfumed soap and water and cleaning web spaces and skin folds were recommended. A soft cotton cloth dipped in soap water was used for cleaning under the skin folds Inspection of the leg for any cuts or wounds Adequate moisturisation using oil, Vaseline or liquid paraffin over the foot and talcum powder in web spaces of toes Trimming nails carefully at regular intervals	Once per day
3. Relaxation	Jacobson’s technique was taught and instruction audio for the same was shared with her to be followed at home	Once per day
4. Faradism under pressure	Surged faradic stimulation was given with limb in elevation. One electrode was placed on the calf muscle and the other on the plantar aspect of the foot. The patient was asked to contract the plantar flexors in synchrony with the stimulus. Rubber bandage was tied in a figure-of-eight pattern with more compressive pressure applied distally, decreasing proximally to provide the necessary pressure gradient to drain the fluid towards the popliteal lymph nodes	Surge faradic current was given for 10 min every day with surge duration:interval ratio of 1:2
5. Pain relief	Ultrasound for tenosynovitis of Tibialis anterior	Pulsed-mode ultrasound on the anterior aspect of the right ankle over the area of the tibialis anterior tendon (5 cm in length) Intensity: 0.5 W/cm2 Duration: 8 min once per day for a week
6. Lymphatic drainage massage	Light self-strokes of effleurage in a distal to proximal direction, but first starting from the thigh progressing to the ankle	2 times per day for 20 min
7. Bandaging with short-stretch bandages	Multilayered short-stretch bandaging was to be worn day and night Self-bandaging from toes up to the groin was taught to the patient so that it can be untied for a bath and tied again after	24 hours
8. Lymphatic drainage exercises in elevation	Position: supine with the bandaged limb flexed at the hip to 60° placed against a wall The sequence of exercises from proximal to distal was as follows: Deep breathing exercises Gluteal setting and posterior pelvic tilts Kegels exercises in supine 20° head low position. Hip movements: flexion, abduction, adduction and rotations Hip-knee flexion by sliding the foot on the wall Supported cycling in supine with bed cycle Ankle movements	10 s hold for each repetition 1 set=10 repetitions 2 sets per session 3 times per day
9. Resistance exercises	For lower limb Resisted dorsiflexion and plantarflexion with weight cuffs of ½ and 1 kg Toe raises (gastrocnemius and soleus) Toe raises in a semi-squat position (isolated soleus) Toe walking Group class: strength training for the upper and lower body, core, using bodyweight, TheraBand and dumbbells Strengthening of hip extensors	1set=10 repetitions 3 times per day and 2 times per week
10. Aerobic exercises	Walking Stationary cycling Group aerobics	30–45 min 2 times per day 15 min per day 3 times per week

Table 2.

Home programme and medical treatment

Home programme	Contrast bath (once per day) Exercises in elevation and lower limb strengthening exercises (once per day) Self-care and limb hygiene and self-effleurage massage (once per day) Self-bandaging Walking for 10 min 3 times a day progressing to 45 min daily as tolerated
Medical treatment	Inj. benzathine penicillin Lanolin lotion Tab. Paracetamol Tab. Albendazole Tab. Vitamin C Tab. FSFA (ferrous ascorbate and folic acid) Cero soft cream and Revlon cream.

Outcome and follow-up

Outcomes after 17 physiotherapy sessions in outpatient department are described in tables 3–5.

Table 3.

Improvement in right full-length lower limb volume and BMI

	Right (affected) full-length lower Limb volume (L)	Left (normal) full-length lower limb volume (L)	Volume excess of normal lower limb (%)	BMI (kg/m2)
Pre	7.41	3.93	88.54	23.7 (overweight)
Post	4.22	3.93	7.37	21.5 (normal weight)
Difference	3.19	0	81.17	2.2

BMI, body mass incex.

Table 4.

Improvement in 6MWD, percentage of predicted distance

	6MWD (m)	% of predicted (Indian formula)
Pre	469	103.40
Post	610	131.54
Difference	141	28.14

6MWD, 6 min walking distance.

Table 5.

Improvement in quality of life

LFSQQ	Total	Mobility	Self- care	Usual activities	Disease burden	Pain, discomfort	Psychological health	Social Discomfort
Pre	31.25%	18.7%	62.5%	12.5%	12.5%	12.5%	67.85%	25%
Post	85.52%	96%	93%	91%	75%	83.3%	92.85%	30%

LFSQQ, LF-Specific Quality of Life Questionnaire.

Discussion

The patient attended the Physiotherapy Outpatient Department daily. Education and counselling were provided during the initial sessions to establish rapport in addition to providing guidance. It led to gradual acceptance of the disease by the patient. Daily attendance attests to improved adherence to therapy.

Self-care education included skincare and limb hygiene, which was performed as per Morbidity Management and Disability Prevention recommendation.^{3 9} Demonstration was given and the patient was asked to repeat it under supervision so that she could learn it correctly and repeat it independently at home. Skin is an effective barrier against any infection. Skin changes in lymphoedema make the skin sensitive and dry due to alteration of its micro-circulation and macro-circulation, thus causing impaired healing of skin and making it prone to ADLA.^{2 5} Direct contact of the compressive garment increases mechanical pressures on the corneal layer of skin. Self-care and moisturisation helped to maintain the integrity of the skin.^{3 10} Compression garments are not a contraindication in ADLA. As ADLA is associated with lymphoedema, compression garments play a major role in reducing ADLA episodes by reducing lymphoedema, improving immune response and providing physical protection for the skin.¹¹ Antibiotics controlled the infection. The patient did not report any episode of ADLA in the last 10 months. This is impressive considering three to four occurrences of ADLA per year for 12 long years before this therapy.

Compression compensates for the elastic tissue deficiency and inhibits outflow from capillaries by exerting pressure on subcutaneous tissue. This prevents tissue fluid transfer associated with gravity, thus improving venous return. Multilayered short-stretch bandages have a low resting pressure and high working pressure. Thus, short-stretch bandages likely helped to maintain the integrity of lymphatics, providing semirigid support and more compressive forces with muscle contraction. This bandage was kept for 24 hours and removed only for self-cleaning of the limb and for limb measurement by the therapist.^10–14 A certified lymphoedema therapist trained the patient to apply a bandage on herself until she mastered self-bandaging. She was self-reliant in applying the bandage, which she mastered within two sessions as seen in figure 4. The technique was observed and refined every day for perfection. In chronic lymphoedema, self-bandaging and self-limb hygiene are important, cost-effective and bring satisfaction to the patient.¹⁵ Rubber and gauze pieces were used as padding during bandaging over thickened adipose tissue. This helped to soften the area and to transmit the compression to the subcutaneous compartment effectively.¹⁴

Figure 4.

Self-bandaging by the patient.

Low-frequency currents and exercise have been proved to be beneficial in lymphoedema. Faradism under pressure with active ankle movement was given in limb elevation for added benefits of compression with muscle pump. This may have helped in draining lymphoedema faster and initiate good muscle contraction.^16–19

Gravity assisted lymphoedema exercises in limb elevation coupled with deep breathing exercises assisted lymphatic drainage by changes in intrathoracic pressure, thereby favourably influencing the venous return, which contributed to a reduction in swelling of the limb.¹⁴ Exercises began with contraction of the proximal followed by distal muscles, which helped in filling and emptying proximal lymph nodes causing proximal decongestion, thereby allowing drainage of more distal lymphatic fluid. It included a contraction phase of 1 s and a relaxation phase of 5 s to fill the lymphatics.^{14 20} During exercises, the muscle stretch may have caused a pull-on anchoring filament of the initial lymphatics, which helped the fluid to move into the vessel. The increase in muscle fibre cross-sectional area during contraction led to compression of connective tissue and therefore of the lymphatics, which led to lymph being pushed towards lymph collectors.²¹

Self-lymphatic drainage with Vaseline was taught to the patient. It consists of light effleurage strokes from the distal to proximal, decongesting the inguinal area followed by the popliteal and ankle area. The addition of self-lymphatic drainage to compression bandaging has proved to be beneficial in breast cancer-related lymphoedema.²² It assists in the drainage of lymphatic fluid which enhances the function of the immune system, helps in tissue cleansing, and improves circulation and tissue oxygenation. Massage leads to the release of endorphins, serotonin and dopamine, thus reducing stress and promoting relaxation. The effect of touch during massage purportedly stimulates the autonomic nervous system, which thereby influences the contractions and propulsion of larger lymphatic trunks through nervous system innervation.^{9 23} The patient learnt this technique in two sessions.

Plantar flexors are major pumps for lymphatic and venous drainage. In this case, weakness of plantar flexors due to inactivity caused by long-standing oedema and disuse of lower limb muscles was observed. Individual strengthening of soleus muscle along with combined strengthening of both gastrocnemius and soleus led to significant improvement in muscle strength and gait pattern. The decrease in weight of the limb resulted in improved locomotion and reduced disfigurement, which was the most disabling feature. The patient stated she was joyful over the improvement in the appearance of the limb. Compliance with the home programme as shown in table 2 was ensured by asking her every day about her previous day routine after going home.

All the above-mentioned components of lymphoedema management led to a significant reduction in limb volume as seen in table 3 and figure 5. On the first day, the percentage difference in excess limb volume was 88.54%, which was reduced to 7.37% after 20 days with combination of interventions provided in the Physiotherapy sessions.

Figure 5.

Improvement in limb volume pre-treatment and post-treatment.

Due to chronic filarial lymphoedema, there was fibrin deposition and adhesions in the tendon sheath. As the swelling reduced, the range of dorsiflexion increased and excursion of the tibialis anterior tendon in the sheath increased, thereby breaking adhesions and creating friction and inflammation. Although the swelling and mobility were improved, the tendon strength had not increased proportionally. These factors caused tenosynovitis of the tibialis anterior, leading to severe pain in the anterior aspect of the ankle on the fourth day which was aggravated during walking. The patient had complete resolution of symptoms after a week of ultrasound therapy on the anterior aspect of the right ankle over the area of the tibialis anterior tendon (5 cm in length) and relatively looser bandaging with extra padding over the pain site. Ultrasound therapy helps to improve tissue extensibility, relieves pain and reduces inflammation.²⁴ Contrast bath through its mechanism of alternate vasoconstriction and vasodilation creates a pumping action to drain the metabolic waste products and improves lymphatic drainage. There was a reduction in pain from 7 to 0 (no pain) on the Visual Analogue Scale after 20 days.

A combination of aerobic, strength and flexibility exercises with short-stretch multilayered bandages enhanced effective drainage of lymph. Footwear with modification accommodated the increased size of the foot caused by bandaging and swelling. Participation in aerobic group class promoted social interaction and boosted her confidence.^{25 26} Jacobson’s relaxation technique helped to reduce anxiety.²⁷ Body mass index (BMI) reduced from 23.7 kg/m² (overweight according to Asian standards) to 21.5 kg/m² (normal category) on day 20, thus reducing the BMI-related risk.²⁸ Exercise induces the release of endorphins which has a positive effect on well-being and happiness. Although the patient’s prefunctional capacity as predicted by percent predicted 6 min walking distance was 103.40% (Indian prediction formula) as seen in table 4, she was unable to translate it in function as seen from the mobility score being just 18% because of the psychological impairment.⁸ After 20 days, the mobility scores increased to 96%, indicating that she was able to do her daily activities more efficiently.

Table 5 shows a significant improvement in all the different domains of LFSQQ except the social distress domain. This may reflect the need for promoting social acceptance of filariasis. She had given up on her cooking job and never had thought she would ever be able to resume it. The main reason was the physical disfigurement and the offensive odour of the limb. After 20 days of treatment, she was eager to resume her work but could not, due to the pandemic. An increase in the scores of psychological and disease burden domains of LFSQQ suggests her improved social participation. Once the percentage difference in excess limb volume was less than 20%, the use of grade 2 stockings was encouraged as it was more convenient to wear.^{6 29} She found short-stretch bandaging inconvenient and time-consuming but more effective in reducing the swelling and used stockings to maintain the reduced limb volume.

Encouraging self-management was found to be highly effective and feasible. It boosted up confidence, self-acceptance, self-reliance and community participation.

Even in lockdown due to the COVID-19 pandemic, the patient was able to regularly partake in all aspects of the treatment regime. It was encouraging to see that the improvements documented were maintained through 8 months of lockdown and there was no deterioration in the patient’s health.

Patient’s perspective.

For 12 years, I had severe swelling in my right leg due to which I faced a lot of problems. I also had multiple frequent episodes of fever. I started physiotherapy treatment on first January 2020, ever for the first time, and since then due to the treatment and advice given by the physiotherapist, I am not facing any problems. My swelling has reduced and there are no more episodes of fever. I am also able to maintain the improvement in swelling with self-bandaging.

Learning points.

• Emphasising patient education, psychological counselling and improving social interaction through group exercises may help to avoid physical disability and extreme mental anguish.

• Prevention of acute dermato-lymphangio-adenitis episodes by teaching self-care of limb hygiene helps the patient to take responsibility for his or her health.

• This case report includes the use of exercises simultaneously with faradism under pressure in limb elevation for lymphoedema drainage.

• Self-bandaging is an effective and a very practicable tool in lymphoedema management.

• Social acceptance remains a challenge for filariasis.

Ethics statements

Patient consent for publication

Obtained.