Juvenile Rheumatoid Arthritis (JRA)

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MUSCULOSKELETAL DISORDER
DR. REEM ALI
Fall Semester 2015-2016
Musculoskeletal

The main organs and
tissues that are part of
the musculoskeletal
system in humans are
 the cartilages
 the
bones
 the muscles
Musculoskeletal

Main functions of MS are:
To support & protect vital organs (the brain, heart and
lungs)
 To keep structure and maintenance of the body spatial
conformation.
 Allows the body to move (walking, standing, bending).
Because soft tissues are resilient in children, dislocations
and sprains are less common than in adults


Nutrient storage (glycogen in muscles, calcium and
phosphorus in bones)
Musculoskeletal: Physical
Assessment







Inspect child undressed
Observe child walking
Spinal alignment
ROM
Muscle strength
Reflexes
Assessment of concerns
Musculoskeletal: Physical
Assessment

Assessment of concerns:




Pain or tenderness & Muscle spasm
Masses
Soft tissue swelling
assess injury site at the last
Musculoskeletal: Diagnostic
procedures

X-ray & Bone scan

Alkaline phosphatase (ALP): ALP is an enzyme found
mainly in bone, liver, placenta, and kidney; levels may be
elevated in bone disease, fractures, trauma, or liver disease
and during periods of rapid growth.
Electromyography (EMG): studies the electrical activity of
skeletal muscle and nerve conduction



Muscle or bone biopsy
Arthoscopy: direct visualization of a joint with a fiberoptic
instrument
Kyphosis

Is an abnormally increased
convex angulation in the
curvature of the thoracic spine

It can occur secondary to disease
process such as tuberculosis,
chronic arthritis

Treatment


Postural exercises
Bracing (Milwaukee) for more
marked deformity
Lordosis

Is an accentuation of the lumbar curvature
beyond physiologic limits

Lordosis is a normal observation in toddlers

It may be a complication of a disease process,
the result of trauma or idiopathic

In older children is often seen in association
with flexion contractures of the hip, obesity,
congenital dislocated hip
Scoliosis





Is a spinal deformity which may
involve lateral curvature, spinal
rotation causing rib asymmetry,
and thoracic hypokyphosis.
It is the most common spinal
deformity.
It can be congenital, or it can develop
during infancy or childhood, but it is
most common during adolescence
(peaks between 8-15 years)
It may be genetic and transmitted as
an autosomal dominant trait
It may be multifactorial
Scoliosis: Types

Functional scoliosis




caused by a secondary problem such as unequal leg length.
The curve tends to be a C-shaped curve
treated by treating the primary cause first
Structural scoliosis



the cause is idiopathic with a positive family history in some cases
It involves a permanent curvature of spine accompanied by
damage to the vertebra.
The curve tends to be S-shaped curve.
Scoliosis: Clinical Manifestation


From standing position ( feet together and
arms at sided)

Unequal shoulders level

Curved spinal column

Uneven level of the elbows
From bending position ( child bends and
touch his toes):

Rotation of the spine becomes more prominent.

Hump in the back

One shoulder blade is more prominent than
other is

In some cases there are back pain,
fatigue and dyspnea
12
Scoliosis: Management

Non-surgical management aimed to :







promoting self-esteem and positive body image
maintain spinal stability
prevent further progression of deformity until bone growth is complete and
surgical repair can be performed
mild cases (less than 20%), observation and exercises- swimming is
advised & Long-term monitoring.
Moderate (20-40%), exercises, traction, bracing. Bracing (Milwaukee
brace) is successful in halting or slowing the progression of
curvatures
Severe (more than 40%), bracing until the skeletal system mature
and then surgical intervention
Surgery includes realignment and straightening of the spine
with internal fixation
Internal fixation
14
External fixation: Milwaukee Brace
15
Congenital clubfoot (Talipes)

Deformity of the ankle and foot

Categories of Talipes

Positional clubfoot (transitional, mild
or postural),



Syndromic (tetralogic ) clubfoot



may occur from intrauterine crowding
responds to simple stretching and
casting.
associated with other congenital
abnormalities such as
myelomeningocele,
more severe form of clubfoot that is often
resistant to treatment.
Congenital clubfoot (idiopathic )

has a wide range of rigidity and prognosis
Congenital clubfoot: Management

goal of management: is Correction of the
deformity & Maintenance of the correction until
normal muscle is gained

Management
 Casts
 begin
immediately or shortly after birth and continue
until marked overcorrection is reached.
 Weekly manipulation and cast changes proceed for
the first 6 to 12 weeks of life.
 Surgery
 If casting and manipulation are not successful
 Followed by brace and cast
Congenital clubfoot: Management

Nursing Care
Observation
of skin and circulation
(particularly important in young infants
because of their normally rapid growth
rate): swelling in the toes, foot
temperature
Parents
need to understand the
diagnosis, the overall treatment
program, the importance of regular cast
changes
Developmental/congenital hip
dysplasia/dislocation (DDH/CHD)


Improper formation and function of
the hip socket.
Cause of DDH is unknown, but
there are predisposing factors such
as:




Genetic factors & birth order
Physiologic factors: maternal
hormone
Mechanical factors: intrauterine
position (breech), oligohydraminos,
twining and fetus size, delivery type,
postnatal positioning
DDH occurs more commonly in
females.
DDH/CHD: Degrees
Acetabular dysplasia
(or preluxation)


Subluxation

Dislocation
DDH/CHD: Degrees

Acetabular dysplasia (or preluxation)
 The
mildest form
 The femoral head remains in the acetabulum
DDH/CHD: Degrees

Subluxation




Accounts for the largest percentage
of DDH.
It implies incomplete dislocation
The femoral head remains in
contact with the acetabulum, but a
stretched capsule and ligamentum
tears cause the head of the femur to
be partially displaced.
Dislocation

Femoral head loses contact with
the acetabulum and is displaced
posteriorly and superiorly over the
fibrocartilaginous rim
DDH/CHD: diagnosis

Ortolani test




Abduction of the thighs with external
rotation.
If the femoral head can be felt to slip
forward into the acetabulum on
pressure from behind, it is dislocated
(positive Ortolani sign)
Sometimes an audible “clunk” can be
heard.
Barlow test


Pressure from the front
If the femoral head is felt to slip out
over the posterior lip of the acetabulum
and immediately slips back in place
when pressure is released, there is
dislocation or “unstable” (positive
Barlow sign)
DDH/CHD: diagnosis

Other signs of DDH are:
Shortening of the limb on the
affected side (limping and toe
walking
 Positive Trendelenburg sign or
gait)
 Asymmetric thigh and gluteal
folds
 Broadening of the perineum(in
bilateral dislocation)


Ultrasounds
DDH/CHD: Management

Newborn to six months

Pavlik harness




The harness is used to maintain the infant’s
hips in flexion and abduction and external
rotation
Pavlik harness device is to be worn
continuously.
The child in a Pavlik harness needs special
attention to skin care because the infant’s
skin is sensitive and the harness may cause
irritation.
6-8 months:


Gradual reduction by traction is used for
approximately 3 weeks.
If the hip is not reducible, an open
operative reduction is performed.
Following reduction the child is placed in
a hip spica cast for 2-4 months
DDH/CHD: Management

Older child:
 Operative reduction
 After cast removal
and before
weight bearing is permitted,
range-of-motion exercises &
rehabilitative measures

The former practice of doubleor triple-diaper for DDH is
not recommended because it
promotes hip extension, thus
worsening proper hip
development
DDH/CHD: Nursing Diagnosis &
Management






Knowledge deficit regarding care of harness or cast
Impaired physical mobility
Risk for impaired skin integrity related to pressure from casts or
braces
Risk for altered skin perfusion due to casts or braces
Risk for altered growth and development due to limited mobility
Nursing Management





Compliance with corrective devices by parents
Not removed for bathing
Prevent skin irritation
Cast care & Diaper area
Because DDH may reoccur it is important to follow-up until the
child reaches skeletal maturity
Juvenile Rheumatoid Arthritis (JRA)
(self-reading)




Is an inflammatory disease of the body joints and
sometimes affects blood vessels and connective
tissue
Unknown cause but a slight tendency to occur in
families
Peak ages : 2 – 5 years and between 9 - 12 years of
age
JRA is similar to the adult disease with some
distinguishing features
 onset
before puberty
 a negative rheumatoid factor (RF).
Juvenile Rheumatoid Arthritis (JRA):
Courses

Pauciarticular

Polyarticular

Systemic
Juvenile Rheumatoid Arthritis (JRA):
Pauciarticular

4 or fewer joints are affected

The most common form of JRA; about half of all children
with JRA have this type

Affects large joints, such as the knees

Girls under age 8 are most likely to develop this type of
JRA.
Some children have special kinds of antibodies in the
blood.



Antinuclear antibody (ANA)
Rheumatoid factor
Juvenile Rheumatoid Arthritis (JRA):
Pauciarticular


Eye disease
 affects about 20 to 30% of children with pauciarticular JRA

Up to 80% of those with eye disease also test positive for
ANA

The disease tends to develop at a particularly early age in
these children

Regular examinations by an ophthalmologist are necessary to
prevent serious eye problems such as iritis or uveitis
Some children with pauciarticular disease outgrow arthritis by
adulthood, although eye problems can continue and joint
symptoms may recur in some people
Juvenile Rheumatoid Arthritis (JRA):
Polyarticular

30% of all children with JRA have polyarticular disease

5 or more joints are affected. The small joints (hands and
feet) are most commonly involved, though large joints may
be affected

symmetrical; that is, it affects the same joint on both sides
of the body

Some children have an antibody in their blood called IgM
rheumatoid factor (RF)
These children often have a more severe form of the
disease

Juvenile Rheumatoid Arthritis (JRA):
Systemic (Still’s disease)

Joint swelling, & fever and a light skin rash

May also affect internal organs such as the heart, liver,
spleen, and lymph nodes

Almost all children with this type of JRA test negative for
both RF and ANA

Affects 20% of all children with JRA. A small percentage of
these children develop arthritis in many joints and can
have severe arthritis that continues into adulthood.
Juvenile Rheumatoid Arthritis (JRA):
Clinical Manifestations






Joint swelling
Stiffness that typically is worse in the morning or after a nap
Pain may limit/loss movement of the affected joint
Commonly affects the knees and joints in the hands and feet
One of the earliest signs of JRA may be limping in the
morning because of an affected knee.
Besides joint symptoms, children with systemic JRA have



A high fever and a light skin rash. The rash and fever may appear and
disappear very quickly.
Swelling in the lymph nodes located in the neck and other parts of
the body
In some cases (< 50%), internal organs (heart and, very rarely, the
lungs) may be involved.
Juvenile Rheumatoid Arthritis (JRA):
Clinical Manifestations

Eye inflammation


Sometimes occurs in children with pauciarticular JRA.
Not present until some time after a child first develops JRA

Typically, there are periods when the symptoms of JRA are
better or disappear (remissions) and times when symptoms are
worse (flare-ups).

Growth problems


Depending on the severity of the disease and the joints involved it
may cause joints to grow unevenly or to one side. causing one leg or
arm to be longer than the other.
Overall growth may also be slowed.
Juvenile Rheumatoid Arthritis (JRA):
Diagnosis

Diagnosis of JRA is based on:




Age of onset
Arthritis in one or more joints for 6 weeks or longer
Exclusion of other etiologies.
Laboratory tests-- Blood may be taken to test for RF and ANA,
and to determine the Erythrocyte Sedimentation Rate (ESR).




positive RF is detected in just 10% of the cases. The RF test helps the
doctor tell the difference among the three types of JRA.
ANA is found in the blood more often than RF, and both (ANA & RF)
are found in only a small portion of JRA patients.
ESR indicates inflammation in the body. Not all children with active
joint inflammation have an elevated ESR.
Lab. tests may include elevated WBCs
Juvenile Rheumatoid Arthritis (JRA):
Therapeutic Management
• Preserve joint
function,
• Prevent
physical
deformities,
and
• Relieve
symptoms
Goals
• Nonsteroidal
antiinflammatory
drugs
• Diseasemodifying antirheumatic drugs
• Corticosteroids
• Biologic agent
• Physical therapy
Treatment
• Reye
Syndrome
Free of
treatment
related harm
Juvenile Rheumatoid Arthritis (JRA):
Treatment

NSAIDs:
Aspirin, ibuprofen; may cause Reye’s Syndrome
Disease-modifying anti-rheumatic drugs (DMARDs): most
given in combination with NSAIDs to slow the progression of JRA
Corticosteroids: to control severe symptoms; can
interfere with a child's normal growth, a round face, weakened bones, and
increased susceptibility to infections.
Biologic agents: Etanercept (Enbrel) blocks the actions of tumor necrosis
factor, a naturally occurring protein in the body that helps cause inflammation
Physical therapy: Exercise to maintain
muscle tone and preserve and recover the range of motion of the joints; rest of
affect body part and heat application
Juvenile Rheumatoid Arthritis (JRA): Side
effect of Aspirin

Bleeding
Stomach upset
Liver problems
Reye’s Syndrome:
Is sudden (acute) brain damage (encephalopathy) & liver
function problems
Abnormal accumulations of fat begin to develop in the liver
and other organs of the body, along with a severe increase of
pressure in the brain
Without proper treatment death is common within a few days
Juvenile Rheumatoid Arthritis

During painful episodes of the disease


Proper positioning is important to support and protect
affected joints. Isometric exercises and passive range-ofmotion exercises will prevent contractures and deformities.
Swimming in warm water provides strengthening and
range-of-motion exercises while protecting the joints.
After discharge


routine ophthalmologic examinations
Swimming
Cerebral palsy (CP)*

It is a group of non-progressive disorders (meaning the
brain damage does not worsen, but secondary orthopedic
difficulties are common) of motor neuron impairment
that result in


motor dysfunction
may be accompanied by perceptual problems, language
deficits, and intellectual involvement.

The disabilities usually result from injury to the
cerebellum, basal ganglia, or motor cortex.

The exact cause is unknown; it may result from
injury to the brain before, during, or shortly after
birth
Cerebral palsy (CP)

Risk factors include:
Prematurity
 LBW
 Asphyxia
 Infections (meningitis, encephalitis)
 Head injuries
 Metabolic problems such as hyperbilirubinemia and
hypoglycemia
 Sever dehydration

Cerebral palsy (CP): Types

Spastic

Dyskinetic/ athetoid

Ataxic

Mixed type/dystonic
Cerebral palsy (CP): Types

Spastic : (most common type)




May involve one or both sides
Hypertonicity with poor control of posture, balance, and
coordinated motion (rigid & jerky movement).
Impairment of fine and gross motor skills
Dyskinetic/ athetoid:




Abnormal involuntary movement
Slow, wormlike, writhing (rolling & twisting) movements
that usually involve the extremities, trunk, neck, facial
muscles, and tongue.
Involvement of the pharyngeal, laryngeal, and oral muscles
causes drooling and dysarthria (imperfect speech
articulation)
Involuntary irregular jerking movements
Cerebral palsy (CP): Types

Ataxic
 Rapid, repetitive movements performed poorly.
 Disintegration of movements of the upper
extremities when the child reaches for objects

Mixed type/dystonic:
 Combination of spasticity and athetosis.
Cerebral palsy (CP): Clinical
Manifestations





Reflex abnormalities: persistence of primitive infantile
reflexes is one of the earliest clues to CP
Delayed gross motor development.
Alteration in muscle tone: increased or decreased
resistance to passive movements.
Abnormal posture :opisthotonic postures (exaggerated
arching of the back) and may feel stiff on handling or
dressing
Possible Associated disabilities and problems:




Convulsion/seizure
Visual and hearing impairments
Communication and speech difficulties
Some may have varying levels of mental retardation.
Cerebral palsy (CP): Treatment

Goals of treatment:





Establish locomotion, communication and self-help.
Gain optimum appearance and integration of motor functions.
Correct associated defects.
Adaptation.
Management:







Provide safe environment to prevent injury.
Prevent physical deformities by using braces and provide ROM
exercises.
Appropriate motor activities.
Medications such as sedatives, muscle relaxants, anticonvulsants.
Encourage self-care
Occupational therapy to improve small muscles development
Speech therapy
Cerebral palsy (CP): Treatment
Fracture: Clinical Manifestations
(self-reading)

Swelling, pain or tenderness

Diminished functional use of the affected part; inability to bear weight

Bruising, severe muscular rigidity

Sometimes crepitus

Less frequent neurological and vascular damage (ischemia), which can be
assessed using 5 Ps ( Pain, Pallor, Pulselessness ,Paresthesia ‫تنمل‬,
Paralysis)
 Fractures in children younger than 1 year are unusual because a large
amount of force is necessary to fracture their bones. A child younger
than 1 year with a fracture should be evaluated for possible physical
abuse or an underlying musculoskeletal disorder that would cause
spontaneous bone injury
Fracture: Therapeutic Management

Cast: fiberglass or plaster application to immobilize
affected body part

Tractions :Is the direct application of force to
produce equilibrium at the fracture site

Distraction: involves the use of an external device to
separate opposing bones to encourage regeneration
of new bone & used to immobilize fractures or
correct defects when the bone is rotated or angled
Fracture: Cast

Risk for altered tissue perfusion R/F pressure from
cast
Keep extremity elevated to decrease edema.
 assess circulation Q 15 minutes after applying the cast
then hourly
 assess skin warmth and 5 Ps


Risk for impaired skin integrity R/F pressure from
cast
Cast edges must smoothed/covered
 Cast remains in place for 4-8 weeks
 Discourage itching under the cast

Fracture: Cast

Possible concerns
Unusual odor under the cast
 Drainage from cast
 Tingling ‫وخز‬, numbness and swelling in the casted body
part
 Loose or cracked cast
 Unexplained fever
 Unusual fussiness or irritability and pain
 Discoloration of finger or toes

Musculoskeletal:
Neurovascular Assessment

Neurovascular checks should be done at least every
1 to 2 hours during the first 48 hours, and usually
for as long as the child is in traction
Pain: (location, alleviated and aggravating factors; Does the
pain become worse when fingers or toes are flexed)
 Sensation: Can the child feel/ touch on the affected
extremity
 Motion: Can the child move fingers or toes below area of
injury / nerve injury
 Temperature: Is the extremity warm or cool to touch
 Capillary refill; Color; Pulses (distal to injury or cast)

Fracture: Complications

Circulatory impairment:
 Careful assessment of the
pulses, skin color, and
temperature is crucial

Nerve compression
syndromes (e.g., carpal
tunnel syndrome, tarsal
tunnel syndrome)
 Sensory testing with touch
and pinprick
 evaluating motor strength
by asking the child to
move the unaffected joint
distal to the injury
Fracture: Complications

Compartment syndromes
is a tissue ischemia due to a compression of nerves,
blood vessels, and muscle inside a closed space
(compartment) within the body due to increased
pressure in that part
 The most frequent causes are:



tight dressings or casts, hemorrhage, trauma, burns, and
surgery
Signs & symptoms include:



motor weakness and pain that does not decrease with medication
the muscle may feel tight or full
Burning sensation
Fracture: Complications

Epiphyseal damage: leads to unequal growth

Infection: osteomyelitis (potential problem in open fractures, from
pressure ulcers, or when bone surgery)

Pulmonary emboli: blood, air, or fat emboli may produce a lifethreatening vascular obstruction and ischemia.
 Primary symptom is shortness of breath and chest pain.
 Interventions should include:


Place patient in high fowlers
Administer O2 and check chest X-ray
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