11.4 Juvenile Idiopathic Arthritis (JAI), Cerebral Palsy (CP), Muscular Dystrophy Flashcards
Juvenile Idiopathic Arthritis (JAI)
- Chronic autoimmune disease characterized by inflammation of synovial joints with joint effusion and eventual destruction, erosion, and fibrosing (thickening) of cartilage within those joint capsules.
S/S
- Swelling and lost of motor function in affected joints.
- Possible fever
- Can be painful and isolating
Goal
- Control pain
- Promote proper functioning of joint
Treatment
- NSAIDs (first-line treatment)
- Dmards, steroids, biologic modifiers
- Requires PT and OT
Cerebral Palsy (CP)
- NON-PROGRESSIVE impairment of muscle control and coordination with poor muscle tone and altered posture.
S/S (DOES NOT JUST AFFECT MOTOR SYSTEM)
- Loss of muscle control and coordination
- Motor disturbances
- Changes with sensation, perception
- Altered communication, cognition, behaviors
Causes
- Anomalies in brain structure, shaken baby syndrome, anoxia at birth, maternal infection, placental failure, trauma, drugs/alcohol abuse.
Spastic CP
- Hypertonicity that causes persistent primitive reflexes and/or lack/delay of normal posture control
S/S
- Spastic muscles (high tone)
- Impaired fine/gross motor skills
- Altered gait (move in rigid faction)
Dyskinetic CP
Athetoid
- Involuntary jerking movements (slow, writhing, wormlike).
- Involves trunk, neck, face, and tongue
Dystonic
- Slow twisting movements
- Affects trunk, extremities pharynx, larynx which can lead to drooling and speech impairment
Ataxic CP
- Wide based gait and difficulty with coordination
S/S
- Difficulty with repetitive movements
- Lack of coordination with purposeful movements
CP Nursing Interventions
- SAFETY! (We do not want them to get injured or fall) - Promote normal development
- These patients may be increased risk for seizures
- More frequent rest periods due to increased energy expenditure to accomplish tasks
- Nutritional needs due to possible difficulty swallowing
Multi-Disciplinary Treatment
- PT
- OT
- Speech Therapy
- Nutrition/Dietician
- May need different communication tools
CP Medications
Baclofen
- Most common medication for spastic CP.
- Muscle relaxant to decrease muscle spasms
Diazepam
- Muscle relaxant and anti-epileptic to control seizures
- Used for older children and can cause drowsiness
Botulinum Toxin A
- Also reduces spasticity in muscles (usually used for lower extremities)
Muscular Dystrophy (DMD)
- Inherited progressive degeneration of symmetric skeletal muscle groups (degeneration of muscle fibers that leads to weakness and wasting away of muscle groups)
- Typically affects age 3-7
- Severely shortened lifespan due to respiratory and cardiac dystrophy
Most common type is Duchenne MD (DMD)
- If a baby was meeting developmental milestones but is no longer meeting them, muscular dystrophy is a possible concern
S/S
- Waddling Gait
- Severe lordosis
- Frequent falls
- Mental deficiency
- Respiratory/Cardiac involvement
- Fatigue
Gower’s Sign
- Uses hands to walk themselves up to a standing position due to weakness of muscle groups
Facioscapulohumeral MD
- Autosomal DOMINANT disorder
- Typically presented in adolescents
- Slower progression with more normal life expectancy
S/S
- Difficulty raising arms above head
- Lack of facial mobility
- Forward sloping of shoulders
Diagnosis and Medications MD
Diagnosis
- Observation and degradation of muscle groups
- Polymerase chain reaction to detect dystrophin gene mutation
- ELEVATED SERUM CREATINE KINASE
- Muscle Biopsy
Treatment
- NO CURE
- Prednisone to manage symptoms by increasing muscle strength
Nursing Interventions MD
- Supportive therapy (with respiratory and cardiac functioning)
- Genetic consultations
- Education and support of challenges parent’s may experience and altered life expectancy
