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Rehabilitation Care for the

Child with Joint Disease


Evaluation of Children with Rheumatologic Disease
- history - laboratory exams
- signs and symptoms

Juvenile Idiopathic Arthritis (JIA)


- definition - subtypes
- diagnostic criteria - management
- signs and symptoms

Rheumatic Fever and RHD


- symptoms
- Jone’s criteria
Outline
Growing pain
- definition signs and symptom
- incidence growing pain vs restless leg syndrome

Rehabilitation Management
- pharmacologic
- Rehab management
▪ Constellation of results of physical
examinations, autoimmune markers and
Rheumatic other serologic test, tissue pathology
and imaging
Disease
▪ 1. Arthralgia
Symptoms
▪ 2. Fatigue
suggestive of
▪ 3. fever
Rheumatic
▪ 4. rash
Disease
▪ Arthritis is defined by presence of
intraarticular swelling or 2 or more of
the following finding on joint
examination:
▪ Pain on motion
▪ Loss of motion
Signs Suggestive ▪ Erythema

of Rheumatologic ▪ heat
▪ Photosensitive malar rash
Disease
▪ Mouth ulcers
▪ Raynaud’s phenomenon
▪ Pericardial rub/ orthopnea
▪ Essential blood tests for rheumatic
assessment
▪ CBC
▪ Inflammatory markers: ESR and C-
reactive protein (CRP)
▪ Muscle enzymes (aspartate transaminase
AST), alanine transaminase (ALT),
creatinine phosphokinase (CPK), lactate

Laboratory dehydrogenase (LDH)


▪ ANA (antinuclear antibody) is a
Testing screening test

▪ Imaging studies
▪ Plain x-ray
▪ MRI
▪ CT scan
▪ Bone scan
▪ Is a disease that occurs in children
under 16 years old
▪ Causes pain, stiffness, and swelling in
Arthritis in one or more joints. This pain, stiffness
and swelling is called inflammation
Children
Juvenile Idiopathic Arthritis
▪ Most common rheumatic disease in
children

Juvenile ▪ One of the more common chronic


disease of childhood
Idiopathic
▪ Represents a heterogenous group of
Arthritis disorders sharing the clinical
manifestation of arthritis
▪ Defined as definite arthritis of
unknown etiology beginning
before the age of 16 years
lasting for at least 6 weeks
Juvenile
Rheumatoid ▪ Arthritis – swelling or effusion
or the presence of >/= 2 of the
Arthritis following
(CRITERIA) ▪ Limitation of range of motion
▪ Tenderness or pain on
motion
▪ Increased heat in >/= 1 joint
▪ Onset type (type of articular
involvement in the first 6
months after onset)

Juvenile ▪ Polyarticular >/= 5 inflamed


joints
Rheumatoid
Arthritis ▪ Oligo articular </= 4 inflamed
joints
(CRITERIA)
▪ Systemic onset arthritis with
rash and a characteristic
quotidian fever
▪ Incidence
▪ Onset in childhood accounts for
Juvenile about 5% of rheumatoid arthritis
cases
Rheumatoid ▪ 13.9 per 100,000 per year with a
Arthritis prevalence of 113.4 per 100,000
children
▪ Complex genetic trait in which multiple
genes may affect disease susceptibility
▪ Autoimmune disease associated with
Etiology alteration in both humoral and cell
mediated immunity
Systemic Polyarticular

Fever >/= 2 weeks plus: Arthritis in four or more


joints during 6 months of
Typical rash disease

Generalized
Subtype:
Lymphadenopathy PoJIA RF –

Enlarged liver or spleen PoJIA RF+

serositis 5 or more joints during


first 6 mos of disease

classification RF detected in two or


more laboratory tests 3
mos apart
Oligoarticular JPsA

Arthritis in four or fewer Arthritis and psoriasis or


joints during first 6 arthritis plus 2 or more of:
months of disease Dactylitis
Nail abnormalities
Family history of psoriasis
confirmed by
dermatologist in one or
more first degree relative
ERA

Arthritis and enthesitis or arthritis or


enthesitis plus two of:

Sacroliac joint tenderness,


inflammatory spinal pain or both

HLA-B27
classification Family history in a first or second
degree relative of confirmed HLA
B27 associated disease

Acute anterior uveitis


▪ Arthritis in > 1 joint with or preceded
by fever of at least 2 weeks in duration
that is documented to be daily
(quotidian) for at least 2 days and
accompanied by > 1 of the following:
▪ 1. evanescent (non fixed) erythematous
Systemic JIA rash
▪ Generalized lymph node enlargement
▪ Hepatomegaly or splenomegaly or both
▪ serositis
▪ Arthritis affecting 1-4 joints during 1st 6
months of disease
▪ Has 2 subcategories:
Oligoarthritis ▪ Persistent Oligoarthritis – affecting < 4
joints throughout the disease course
▪ Extended oligoarthritis – affecting > 4
joints after 1st 6 months of disease
▪ Arthritis affecting >5 joints during 1st 6
Polyarthritis (RF months

negative) ▪ Test for Rheumatoid factor is negative


▪ Arthritis affecting > 5 joints during 1st 6
Polyarthritis (RF months of disease. > 2 tests for RF at
least 3 months apart during 1st 6 months
positive) of disease
▪ Arthritis and psoriasis or arthritis and at
least 2 of eh following
▪ Dactylitis
Psoriatric arthritis ▪ Nail pitting and oncycholysis
▪ Psoriasis in 1st degree relative
▪ Arthritis and enthesitis or arthritis or
enthesitis with at least 2 of the following
▪ Presence of or history of sacroiliac joint
tenderness or inflammatory lumbosacral
pain or both

Enthesitis-related ▪ Presence of HLA B27 antigen


▪ Onset of arthritis in a male > 6 years old
arthrits ▪ Acute (symptomatic) anterior uveitis
▪ History of AS, enthesitis related arthritis,
sacroiliitis with IBD, Reiter syndrome or
acute anterior uveitis in 1st degree
relative
▪ Causes
▪ Unknown
▪ Possible factors:
Juvenile ▪

Genetics
Immunologic abnormalities
Rheumatoid ▪ Infection
Arthritis ▪

Trauma
Invection (Mycobacterium
tuberculosis, Epstein Barr virus,
parovirus, Rubella virus
Overview of main
features of
Subtypes
Subtype Peak age of onset Female : Arthritis pattern Extraarticular
(years) male ration feature

Systemic arthritis 1-5 1:1 Polyarticular, often Daily fever,


knees, wrist, ankle, evanescent rash,
fingers, neck and hips pericarditis,
pleuritis

Oligoarthritis 2-4 3:1 Knees, ankles, fingers Uveitis in 30% of


cases

Polyarthritis RF 2-4 and 10-14 3:1 and 10:1 Symmetric or Uveitis 10%
negative asymmetric smalla nd
large joints, cerevical
spine TMJ

Polyarthrits RF 9-12 9:1 Aggressive symmetric Rheumatoid nodule


positive polyarthritis in 10%, low grade
fever

Psoriatric arthritis 2-4 and 9-11 2:1 Asymmetric arthritis of Uveitis in 19%,
small and medium sized psoriasis in 50%
joints

Enthesitis related 9-12 1:7 Predomnantly lower Acute anterior


arthritis limb joints affected; uveitis, association
sometimes axial with reactive
skeleton (but less than arthritis and
in adult, AS) inflammatory bowel
disease
Rheumatic Fever
▪ is a disease that can affect the heart,
joints, brain, and skin
▪ Rheumatic fever can develop if
streptococcus throat and scarlet fever
infections are not treated properly.
▪ Happens about 1 to 5 weeks after strep
Rheumatic fever throat or scarlet fever
▪ immune system. The immune system
responds to the earlier strep throat or
scarlet fever infection and causes a
generalized inflammatory response
▪ Most common form of acquired heart
disease in all age groups accounting to
50% of all cardiovascular disease
Rheumatic Heart
▪ a condition in which permanent damage
Disease to heart valves is caused
by rheumatic fever.
Major manifestation Minor manifestation

Carditis Clinical Manifestation


- Arthralgia
Polyarthrtis
- Fever
Erythema marginatum
Laboratory Features:
Subcutaneous nodule - Elevated acute phase
reactants (ESR, CRP)
Chorea
Diagnosis of initial - Prolonged P-R interval
ECG
or recurrent attack
of Rheumatic fever
Jone’s criteria
2 major or 1 major and 2 minor
manifestation with an evidence of recent
Streptococcus infection
Major criteria
▪ Antibiotic therapy
Rheumatic Fever ▪ Anti-inflammatory therapy
management ▪ Phenobarbital (for chorea)
Growing Pains
▪ Appropriately termed benign nocturnal
pains of childhood
▪ Affect 10-20% of children
Growing Pains ▪ Peak incidence 4 and 12 years
▪ Most common cause of recurrent
musculoskeletal pain
▪ Affected areas:
▪ Anterior thigh
▪ Shin
▪ Calf
▪ Note: not joints
▪ Triggered by
Growing pains ▪ Exercise, physical activity
▪ Patient wakes up at night because of pain
▪ Relieved by
▪ Massage
▪ Heat
▪ NSAID
▪ Restless Leg syndrome (RLS or Willis
Ekbom disease)
▪ Seen more frequently among
Growing Pain vs adolescents and adults
▪ Often familial
Restless leg
▪ Definition: difficult to control urge to
Syndrome move the leg that is exacerbated during
rest and at night and relieved by
movement
▪ 5 basic criteria
▪ Overwhelming urge to move legs
especially along with uncomfortable or
unusual feelings
▪ Urge that starts or gets worse while at
Restless leg rest
▪ Urge that goes away either partly or
syndrome totally when you move
▪ Urge that starts or gets worse at night
▪ Another conditions like leg cramps,
arthritis or muscle pain has been ruled
out
characteris Growing Growing Restless leg
tics pains pains syndrome
Inclusion Exclusion features
Nature of Intermittent, Persistent Urge to
pain some pain increasing move legs
free days intensity often,
and nights, pain during accompanie
deep day d by
aching, unpleasant
cramping sensation in
legs but may
not be
Growing Pains vs painful
Unilateral or Bilateral Unilateral
Restless Leg bilateral

Syndrome Location Anterior


thigh, calf,
Articular,
back or
Urge to
move and
posterior of groin discomfort
knee ; throughout
usually leg
muscle not
joint
Onset Late Pain still Worse late
afternoon or present net in day or
evening morning nigh but
present at
periods of
rest or
inacttvity
characterist Growing Growing Restless leg
ics pains pains syndrome
Inclusion Exclusion features
Physical normal Swelling,
findings erythema,
tenderness,
local trauma
or infection,
reduced joint
range of
motion, limp,
fever, weight
Growing Pains vs loss, mass
Laboratory normal Objective
Restless Leg findings evidence of
abnormalitie
Syndrome s, increase
erythrocyte
sedimentatio
n rate or CRP,
abnormal
CBC,
radiograph,
bone scan or
MRI
Assessment/ Evaluation
▪ Pain
▪ Oucher scale
▪ 3-12 year
▪ Measures pain intensity
▪ Includes six faces and a scale from 0 to 100
from happy to very sad scored 0-5

Evaluation ▪ Faces Rating Scale


▪ Suitable for children 3 year and older
▪ Scale includes 6 faes, from happy to very
sad scored as 0-5, record number of face
child chooses
▪ Child chooses the face that best represents
how he or she feels
▪ Measures pain intensity and effect
Evaluation
oucher scale
Evaluation
F.A.C.E.S.
▪ Examination of body structures and
functions
▪ Joint motion and integrity
▪ Muscle strength and function
Evaluation ▪ Aerobic capacity and performance
▪ Growth and postural alignment
▪ gait
▪ Class I Completely able to
perform usual activities of daily
living (self care, vocational,
avocational)
▪ Class II Able to perform usual self
American College of care and vocational activities but
limited in avocational activities
Rheumatology Revised
▪ Class III Able to perform usual self
Criteria for Classification care activities but limited in
of Functional Status in vocational and avocational activities
Rheumatoid Arthritis ▪ Class IV Limited in ability to perform
usual self care, vocational and
avocational activities
Management
▪ Over goal of management
▪ Quickly control the joint inflammation
using the most effective drug therapy
with the least adverse effects

Management ▪ Preserve joint mobility and integrity and


function
Goals ▪ Promote independence and competence
in necessary and desired activities
▪ Provide education and support o the
child and family
▪ Purpose:
▪ Suppress inflammation and decrease or
Pharmacologic eliminate pain with least possible toxicity

therapies ▪ Prevent or minimize disability


▪ First line
▪ NSAID (Non steroidal anti-inflammatory
drugs)
▪ Approved use for children
▪ Naproxen
▪ Ibuprofen
▪ Tolmetin
▪ Aspirin
Pharmacologic ▪ Choline magnesium trisalicylate

therapies ▪ Diclofenac
▪ Indomethacin
▪ Reduce fever and inflammation
▪ Have analgesic effect
▪ Adverse effect:
▪ GI upset
▪ liver and kidney toxicity
▪ pseudophorphyria
▪ DMARD (Disease modifying antirheumatic
drugs) – drugs that retard progression of
disease
▪ Most common is Methotrexate
▪ Side effects:
▪ liver toxicity
▪ Immunosuppression
▪ GI upset

Pharmacologic ▪ Steroids – has potent anti-inflammatory


effect but do not alter disease course
therapies ▪ Long term
▪ Cushing syndrome
▪ Generalized growth deficits
▪ Osteoporosis
▪ Fracture
▪ Diabetes mellitus
▪ Obesity
▪ Steroid myopathy
▪ Increase susceptibility to infection
▪ Goals:
▪ Pain relief that reduces mobility and limit
self care and ADLs
Rehabilitation ▪ Minimize deformity
Management ▪ Maintain as close to a normal lifestyle as
possible
▪ Pain
▪ Caused by stretching of the capsule
▪ Causes limitation in movement and
Rehabilitation hasten development of contracture
▪ Modalities (superficial heat and cold or
Management warm bath)
▪ Progressive muscle relaxation
▪ Breathing exercises
▪ Rest
▪ 3 forms: bed rest, local joint rest
with or without splints, daily rest
periods
▪ Children with systemic JRA require
period of bed rest for management
Rehabilitation of myocarditis or pericarditis

Management ▪ Resting in prone reduces hip and


knee flexion contracture
▪ Running, jumping or skiing are
discouraged
▪ Massage
▪ Used to relieve pain, reduce muscle spasm
and mobilize soft tissue
▪ Used in conjunction with heat and cold
modalities or prior to stretching and ROM
Rehabilitation exercises
Management
▪ Heat
▪ Reduce stiffness, increase the
extensibility of tissue, reduce
pain and muscle spasm
▪ Reduces sensitivity of muscle
spindle to stretch
▪ Warm bath, play activities for
Rehabilitation ROM can be incorporated
Management ▪ Moist heat can be provided to
localized joint and surrounding
muscle
▪ Ultrasound can be used but
can have an effect on growth
plate of children
▪ Cold
▪ Provide pain relief, increase the
pain threshold and reduce muscle
spasm and swelling by
vasoconstriction
Rehabilitation ▪ Used in acute injury
Management ▪ Cold is applied by cold packs or ice
▪ General preference of children is
for used of heat over cold
▪ Contraindicated in the presence of
Raynaud’s phenomenon
▪ Range of Motion
▪ Flexion is the usual position
of comfort
▪ Passive ROM is used to
preserve or regain joint
mobility
▪ Performed daily with
supervision
▪ AAROM should be
performed with therapist or
Rehabilitation parent assisting to achieve
Management maximal joint motion
▪ AROM may be done in
bathtub or in play activity as
reaching, throwing etc.
▪ Strengthening
▪ Isometric strengthening exercises are
done during acute inflammation or while
Rehabilitation on bed rest

Management ▪ Can include bicycle riding and


swimming
▪ Activities of Daily Living
▪ Includes age appropriate self care skills, play
and school related activities
▪ Aims to help child perform efficiently,
compensating for reduced mobility &
function, provide assistive devices &
environmental modification
▪ Adaptive Devices: help improve function
and reduce pain
▪ Clothing – use velcro closure
Rehabilitation ▪ Use of straw to reduce need to hold and lift cup
▪ Used adapted utensils
Management ▪ Wrist support pen to reduce grip
▪ Long handled brushes
▪ Walker wheelchair etc.
▪ Physical Fitness
▪ Modified physical education program
Rehabilitation may be required especially with cervical
spine involvement to avoid contact sports
Management ▪ Aerobic conditioning such as swimming,
walking, dancing and tai chi are suitable
▪ Splinting
▪ To reduce inflammation
▪ Provide rest and reduce contracture
▪ Support a weak structure

Rehabilitation ▪ May use dynamic splinting or casting to


improve ROM and for continuous stretching
Management ▪ Upper extremity:
Wrist 15 – 20 deg extension
Fingers slightly flexed
MCP flexed at 25 deg
Thumb in opposition
▪ Cervical Spine
▪ Manifestation includes pain, limited ROM,
Specific Joint spasm, limited mobility
▪ Treatment: collar, HMP or cold to reduce
Management pain and spasm, proper position
▪ Complication: subluxation
▪ Temporomandibular Joint
▪ May be involved in 50% of cases
▪ Due to increase synovial fluid causing
Specific Joint pain and swelling
▪ Complains of pain on chewing and mouth
Management opening
▪ Treatment: ice packs, exercises, surgical
reconstruction
▪ Shoulder
▪ Not common at onset of disease
▪ Lose abduction and IR causing difficulty
Specific Joint in ADL
▪ Treatment: exercises as throwing ball,
Management paining on an easel, throwing darts
▪ Gentle stretching exercises
Elbow
- must have 90 deg of elbow flexion for
ADL
Specific Joint - loss of 45 deg of extension limits use
Management of arm as leverage to rise from seated
position of makes toileting difficult
- dynamic splinting can be applied
▪ Wrist
▪ Usually causes loss of extension and
flexion contracture of wrist
▪ Wrist splint at 15 – 20 deg extension and
Specific Joint fingers in a few degrees of flexion

Management ▪ Stretching and splinting prevent


contracture
▪ Most head can be applied to reduce
spasm
▪ Hand Joint
▪ Loss of both flexion and extension range
in fingers
Specific Joint ▪ Ring splints control proximal
Management interphalangeal flexion and extension
▪ Clay can be used to strengthen fingers
▪ ROM exercises of fingers can be done
▪ Hip Joint
▪ Can develop flexion contracture with IR
and adduction
Specific Joint ▪ Compensatory lordosis
Management ▪ May be treated by supported standing
▪ Child can do homework in standing
position
▪ Knee
▪ Can be maintained in extension

Specific Joint ▪ Usually assume a flexed and in valgus


▪ Position knee at 30 deg flexion
Management ▪ Treatment: ROM ex, strengthen
quadriceps
▪ Affectation of MTP reduces push off
resulting in flat foot gait
▪ Molded foot orthosis used to reduce
Specific Joint pain

Management ▪ Metatarsal pad inserted to reduce


weight bearing over metatarsal head
▪ Bone Mineralization
▪ Can develop osteopenia due to failure to
achieve adequate skeletal bone mass
▪ Associated with prolong treatment with
Other Problems steroids and lack of physical activity,
inadequate vit. D and calcium intake, low
body weight
▪ Treatment: calcium supplement
▪ Leg Length Discrepancy
▪ Inflammation can cause bone overgrowth
at distal femur causing true LLD causing
pelvic asymmetry and later scoliosis

Other Related ▪ Steroids can cause reduce overall growth


of child
Problems ▪ Hip flexion contracture, hip subluxation
can cause apparent leg length
discrepancy
▪ Treatment: lift ¼ inch
▪ Nutrition and Growth
Other Related ▪ Early onset, polyarticular and sytemic
JRA can cause growth retardation
Problems ▪ Can be aggravated by steroids
Thank You
ANY QUESTIONS?
▪ Kleigman et al Nelson Textbook in
Pediatrics p. 1248
References ▪ Tecklin Pediatric Physical therapy
p.486- 529

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