disabilities and deformities in leprosy patients and management

DISABILITIES AND DEFORMITIES IN

LEPROSY PATIENTS

"Leprosy work is not merely medical relief; it is transforming frustration of life into joy of dedication, personal ambition into selfless service" Mahatma Gandhi

REFERENCES:-----

• IADVL• IAL• PARK’ Preventive and Social Medicine• Journals

TERMINOLOGY• `Impairments' are defined as `problems in body function

or structure such as a significant deviation or loss'. An example of an impairment in body function would be loss of sensation; examples of impairments in body structure would be contractures and absorption.

• A `deformity' is a structural, usually visible, impairment.• A `defect' could be either a functional or structural

impairment.• `Disability' is used as an umbrella term for impairments,

activity limitations and participation restrictions.

Risk factors and Types Of Deformities• Risk factors are:- 1) Type of Leprosy- more extensive and highly

bacilliferous types carry a high risk if not treated early.

2) No. of nerve trunk involved- more than three nerve trunk involvement increases the risk manifold.

3) Attack of reaction and neuritis increases the risk.4) Duration of active disases- longer the disease

remains untreated, greater the risk of disability.

• Types of Deformities:-Specific Deformities:- arise due to local infection with M.lepra like loss of eyebrows, nasal deformities. (face>hands=feet)Paralytic Deformities:- result from damage to motor nerves like claw finger, foot drop, facial palsy. (hands>feet>face)Anesthetic deformities:- results from insensitivity because of damage to sensory nerves like ulceration, mutilation. (feet>hands>face)

WHO Classification and GradingHANDS AND FEET

Grade 0: no anaesthesia, no visible deformity or damage.Grade 1: anaesthesia present, but no visible deformity or damage.Grade 2: visible deformity or damage present.

EYES

Grade 0: no eye problem due to leprosy; no evidence of visual loss.Grade 1: eye problems due to leprosy present, but vision not severely affected as a result of these (vision: 6/60 or better; can count fingers at 6 m).Grade 2: severe visual impairment (vision: worse than 6/60; inability to count fingers at 6 m) also includes lagophthalmos, iridocyclitis and corneal opacities.

Grade

Hands and feet

0

1

No sensory impairment, no visible impairment Sensory impairment present, no visible impairment

Scars of healed ulcers, when sensation is normal Scars of healed ulcers, when sensation is impaired Hands or feet following successful reconstructive surgery Muscle weakness without clawinga Ulcers, severe cracks, severe atrophya

Degree of impairment Included Excluded

Scars of healed ulcers when sensation is present Minor skin cracks

2

Eyes

0

1

2

Visible impairments present

No eye impairment; no visible or vision impairment Eye impairment present (vision: > 6/60) Severe visual impairment (vision: < 6/60)

Absence of (regular) blink Unable to count ®ngers at 6 m Lagophthalmos

b Corneal sensation testing19

Facial impairments due to lepromatous leprosyc Corneal opacities, uveitis19

Nerve Involvement• Nerve damage occurs in two settings- in skin lesion– small dermal sensory and autonomic nerve fibres supplying dermal and subcutaneous structures are damaged. involving Peripheral nerve trunks– usually those which are superficial or are in fibrocasseous tunnels leading to dermato sensory loss and dysfunction of muscles.• Nerve involvement in leprosy can be said to occur in

5 stages:-- First two are recognized histologically while next three by clinical examination

Posterior tibial nerve is the most frequently affected nerve followed by ulnar, median, lateral popliteal and facial.

Stages Charecteristics

1 Parasitization A few leprae found in nerve

2 Tissue response Host tissue response(TT to LL)+, bacilli+

3 Clinical involvement Clinically thickened w or w/o pain. No NFD

4 Nerve damage NFD+, recovery possible

5 Nerve destruction Irreversible NFD, severe wasting +

Nerve Care Practice• AIM- to prevent permanent damage to nerve trunks• It involves- Recognizing acute or subacute “clinical neuritis” and treating it using steroid or other measures. Recognizing nerve function defect and instituting appropriate treatment without delay.

‘Clinical neuritis’ is diagnosed when a nerve trunk shows moderate to severe nerve pain. It may or may not be associated with NFD and similarly NFD may or may not be associated with clinical neuritis(Quiet Nerve Paralysis)

NERVE TENDERNESS SCALE-

Categorization of pt. acc. To NFD and Clinical Neuritis-

GRADE Clinical features

0 No tenderness Palpation not poanful

1 Mild tenderness Palpation hurts only when asked about it

2 Moderate tenderness Palpation hurts even w/o asking

3 Severe tenderness Palpation is very painful

4 Very severe tenderness Pt. is apprehensive of palpation

Nerve Function Deficit Clinical Neuritis Absent Present

Absent A B

Present C D

• Category A patients- pt is taught how to look for signs and symptoms of neuritis.• Category B patients-(Neuritis +, no NFD) Start Prednisolone 40-80 mg daily 4 wks taper dose 5mg/wk upto 30mg 2-3 wks and then taper it.• In BT leprosy cases(neuritis due to RR), if there is no

significant improvement in the clinical condition within 48-72 hrs then immediate surgical decompression is required so that haemperfusion to nerve can occur.

• In BL and LL cases(neuritis due to ENL), one can wait for six weeks or even longer

• Category C patients- ( No neuritis,NFD+) Clinically, one may assume that the nerve trunk has the potential to recover if NFD is :-• of recent onset - < 6 mnths involvement• incomplete- some sensibility is there• and if no severe muscle wasting present If NFD considered reversible:- prednisolone 30mg 4 wks then tapered off over 30 days. If NFD not recent:- prevent secondary impairement.

• Category D patients:-(NFD +, neuritis+) Prednisolone 40-80 mg daily 2-3 wks reduce to maintenance dose in 3-4wks

Maintenance dose 30mg daily 8-10 wks

If there is no improvement in neuritis within 3-7days then surgical decompression is required.To accelerate resolution of inflammation:- 1- splint affected nerve in slightly stretched position 2-supportive therapy like analgesics 3- short wave or microwave diathermy

• Nerve abscess is cold abscess occuring in a damaged fascicle usually in Tuberculoid Leprosy

• Ocassionaly, ‘hot’ abscess occurs in ENL related neuritis

Management :--• if nerve shows no NFD: wait and watch, drain

abscess only if risk of sinus formation is there.• if nerve is considered irrecoverably damaged:

same as above.• if NFD is considered likely to recover: evacuate

and excise the abscess.

Hand Problems in Leprosy Patients• Hands are affected because of damage to nerves

supplying them or directly affected by reactional process(especially in BL, LL).

• Ulnar nerve is affected most often than others.• In BL,LL cases usually Glove type extensive acral

anesthesia occurs without significant motor involvement.

• Therefore loss of sensibility in palm doesn’t necessarily indicate damage to nerve trunk, as it may also result from destruction of dermal nerve twigs.

• Muscle weakness is sure sign of damage of nerve trunk.

Impairement Direct consequences Late consequences

Damage to somatic sensory fibres

Loss of sensibility Anesthetic deformities(ulcers,shortening of digits.)

Damage to motor fibres Muscle paralysis Contracture

Damage to sudomotor autonomic fibres

Dry skin Deep cracks, hand infections

Lepra reaction Inflammatory odema, osteoporosis, bone destruction, pathological fractures

Severe fixed deformities(specific deformities,bizzare deformities)

Sensory loss leads to:- Loss of perception of pain and heat deprives the hand of its protective mechanism. Motor activities become clumsy and difficult because muscle action is not fine tuned. Frequently injuries results in anesthetic deformities(shortening of digits). Dryness of Palmar skin :- Lack of sweating Cracks at digital creases

• Care of Insensitive Hand:- Skin care practices:- daily soaking hands in water for 15 min. rubbing palms vigorously apply liquid parrafin or vegetable oil Injury care practices:- precaution against burns while cooking using utensils with insulated handles daily inspection of hands using bulky bandages in case injury occurs

• Paralytic deformities of hand:- ulnar nerve supplies--- flexor carpi ulnaris medial half of flexor digitorum hypothenar muscles adductor pollicis and all interossei medial two lumbricals median nerve supplies:--all flexor muscles of forearm thenar muscles first two lumbricals

• Ulnar palsy leads to:- Ulnar claw hand(hyper extended MCP and flexed PIP jts)

Loss of adduction and abduction• Combined Ulnar and Median nerve palsy:- all intrinsic muscles are paralysed complete claw hand handling of objects become very difficult

Corrective Surgery are:-- Lasso insertion Zancolli’s operation augment flexion forces at MCP jt

Srinivasan’s operation Bunnell’s Brand augments extension forces at PIP jt

Antia

• Specific Deformities of hand:- Banana Fingers (due to heavy infiltration)

Shortening of fingers (due to resorption and fragmentation)

“Reaction Hand” (when hand is involved in reactional states)

Foci of ac. Inflammation develops which eventually resolves with dense fibrosis. Foci may be located in dorsal skin, s/c adipose tissue, in small muscles or in small bones. Lession in interossei leads to Swan Neck deformity. Rx. Start systemic corticosteroids therapy(30 mg), Initially hand is rested using splint in functional position Wax baths Active movements after subsidising acute phase

Massage and Exercises for Hands:-• Massage :- it should be done gently, after applying oil, place hand and gently stroke it with other.• Exercise :- press hand(flexed at MCP) against thigh and open flexed fingers with other hand take a soft rubber ball for squeezing in recent onset deformity, splints should be used.Four main types of splints are used:-( delivered by H Workers) Adductor Band splint(in splayed fingers) Finger Loop Splint(maintain lumbricals in position and strengthen small muscles of hand) Opponens Loop Splint Gutter Splint(in late cases with stiffness)

Adductor Finger loop Gutter splint

Opponens loop

Grip Aids:--used after advanced deformities like absorption and amputation. Epoxy resins Grip Aids- applied on articles of work Instant Grip aid kit- immediate benefit in daily work

Foot Problem In Leprosy Patients• Common problems are:- Plantar ulceration Foot drop Fixed deformities of feet and toes Tarsal disorganisation.PLANTAR ULCERATION:-- found in 10% of patients manifestation of sensorimotor deficit mostly in front part of sole in MTP joint augmented by infection through fissures and paralysis of feet muscles(which counter the stress while walking)

• Stages and Types of feet ulcers:

Stages--- First stage – threatened ulceration(dorsal puffiness, deep tenderness)

Second stage – concealed ulceration(destruction of soft tissue has occurred)

Third stage – open ulceration(necrosis blister open and exposed)

Types--- Acute ulcer– frankly infected, purulent, covered with slough

Chronis ulcer– indolent ulcer with hyperkeratotic edges, covered with granulation tissue

Complicating ulcer– infection spread to deeper structure may lead to muscle paralysis, gas gangrene, tetnus or septicemia.

Management and Prevention• Management:-- absolute bed rest and elevate foot eusol bath, irrigation, dressing remove slough or other draining procedures start antibiotics protective foot wearing• Prevention:-- Protective footwear:-(type depends on state of foot) Feet with only sensory loss(no muscle paralysis), footwer should have tough outer sole, should not rub against toes. Eg using automobile tyre side pieces.

• Any footwear can reduce the pressure upto 25%• Appropriate footwear should have outer sole of 15-18mm thick and soft inner sole 18-22mm.• Iron nails and buckles are to be avoided.• Raja Model is most suitable one.

• learn to take short steps

• Gjhh

Female models Male models

Insensitive feet(with intrinsic muscle paralysis):- these require a resilient, non collapsing, shock absorbing insole that will dampen the impact during walking Microcellular rubber is most suitable.

In certain case where greater reduction of pressure is required; add metatarsal bar obliquely or molding the insole so that pressure can be distributed evenly over entire plantar surface. Certain orthosis like fixed ankle brace can also be used that may transfer a part of load to leg. Foot Care Practices:-- similar to those done for hand soaking, scrubbing and smearing routinely corn and callosities are removed carefully identify ‘safe limits’ of walking

• Foot drop:-- About 1-2% of leprosy patients develop due to damage to lateral popliteal nerve. Paralysis of anterior muscles give rise to foot drop characteristic ‘stepping gait’ occurs in which ball of foot instead of heel hits the ground inversion foot leads to overloading on outer part.

If paralysis is recent; manage under ‘Nerve Care’ therapy.If paralysis is of >1 year duration; it is satisfactorily corrected by anterior transposition of tibialis posterior tendon(Srinivasan’ operation)If surgical intervention is contraindicated; foot drop appliances like strap, stops or springs are used that hold foot at right angle.

• Splinting of knee:- fig. 36.10 this allows rest to inflamed nerve and result in quicker healing.• Droped foot should be supported to hasten recovery. Splint

• Stretching calf muscles: as in foot drop these are not used while walking so contracture may develop.

CONTRACTURES WITH ‘FOOT DROP’ IS TO STRETCH THE HEEL CORDS BY LEANING FORWARD AGAINST A WALL OR BY SQUATTING WITH HEELS ON THE GROUND

Deformities of Face• Most of deformities on face occurs due to

infilteration of facial skin but paralytic deformities can also occur(in borderline leprosy).

• Deformities are:-- loss of eyebrows(madarosis)

mega lobules of ear(Budhha ear)

premature senility(strecthing of skin due to heavy infiltration lead to loss of elastic tissue, when infiltration regresses skin become redundant)

Sunken Nose (due to infilteration in nasal mucosa in LL , granuloma formed erodes the supporting bony structure of nose).

Eye Problem• More commonly in BL and LL type leprosy.• Occurs due to:- Direct invasion- leprous conjuctivitis, scleritis and choroidal nodule.

Acute iridocyclitis- due to immune complex deposition

Damage to – facial nerve paralysis of eyelid muscles and lagophthalmos

- trigeminal nerve loss of corneal sensation leads to exposure keratitis and corneal ulceration.

Management- using spectacles,gogles or eyeshades. artificial tears and cover eyes during sleep treating ac iridocyclitis using topical corticosteroids surgical intervention for lagophthalmos or cataract

• Splint in facial palsy- use adhesive tape strips so that lower lid is not sagging due to gravity and angle of mouth isnt deviated

• Gynecomastia: embarrassing enlargement of breast in males, usually bilateral due to hormonal imbalances because of testicular and liver damage.

GPAS(Green Pastures Activity Scale):-• It assess the daily routine of patients• Can help the nurse to pick up early deformity

Daily activities are assessed as

4. Not difficult

3.A bit difficult

2. Very difficult

1. Impossible

Interpersonal relationship

4. No problem

3. Some problem

2. More problem

1. No relation

For use of assistive devices

4. Not necessary

3. Not difficult

2. Difficult

1. Very difficult

Economic Rehabilitation• Social ostracism is now on decrease following

extensive education about leprosy.• Appropriate economic rehabilitation is provided eg

sewing machines,handcrafts, carpentry ,etc.• CBR(community based rehabilitation) aims to

overcome activity limitation and participation restriction and thus improving QOL for disabled.

• WHO has endorsed the goal of reducing grade2 disabilities by 35% from baseline of2010

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