Ch. 33 & 34 Flashcards
neuromuscular dysfunction pediatric assessment: inspection & palpation of
- motor function
- reflexes
- sensory function
spina bifida cystica is also called
aka myelomengocele
what is a neural tube defect
when the neural tubes of the brain and spine dont close properly
what is the difference between meningocele and myelomeningocele
meningocele: defect of posterior elements of spine w/ extrusion of meninges and CSF (w/out neural elements)
myelomeningocele: defect of posterior elements of spine w/ extrusion of meninges and CSF and neural elements such as the spinal cord elements and nerves
- most severe form of spina bifida
- develops during first 28 days of pregnancy
- 90% of spinal cord lesions
- located at any point on spinal column
how are meningocele and myelomeningocele abnormalities detected?
- in utero (prenatally) or at birth
- ultrasound of the uterus and elevated maternal AFP or MS-AFP, fetal specific gamma 1-globulin (tested between 16-18 weeks gestation)
myelomeningocele therapeutic management
Associated/acquired problems
Parental support
Care of the “sac”
Positioning
General care
Orthopedic concerns
GU function
Bowel control
Prevention
myelomeningocele post-op management
Monitoring
Positioning
Parent involvement
Latex allergy??
Home care prep
Prevention of complications and readmissions
teaching for parents of children with myelomeningocele
Positioning
Preventing infection
Feeding
Promoting urinary elimination through clean intermittent catheterization
Preventing latex allergy
Preventing signs and symptoms of complications such as increased ICP
duchenne muscular dystrophy
X-link trait (M>F)
Genetic counseling
Begin meeting most developmental milestones with delays in gross motor d/t muscle weakness around age 3-7 y.o.
Waddling gait
Muscular enlargement (early) atrophy (later)
Loss of independent ambulation by age 12
Immobility complications
Cognitive impairment
duchenne’s therapeutic management: goals
- maintain optimal function in all muscles (mobility)
- prevent contractures/complications and maximizing quality of life
- maintaining cardiopulmonary function
Duchenne’s: What collaborative services are required to maintain function?
- PT: passive ROM, stretching, splinting
- polysomnography: before onset of daytime sx,
- no CPAP, maybe sip-puff ventilator
- tracheostomy
- MIE (cough assist)
Duchenne’s: What problems/complications can result with disease progression?
- progressive muscle weakness
- no decline in cognitive function; BUT mild-moderate intellectual disability (lower IQ)
- neurodevelopment: autism, ADHD, anxiety and mood d/o, OCD (not as common)
Duchenne’s: What nursing interventions should be implemented into a DMD patient’s POC?
- assist patient and family in coping with dx (progressive nature of d/o)
- design program to promote independence of the child and reduce the predictable and preventable complications associated with the d/o
- adapt to limitations caused by d/o
- decisions regarding quality of life, achieving independence, transition to adulthood
- modify family activities to meet the needs of the child
Duchenne’s: promoting mobility
Administering corticosteroids and calcium supplements
Performing passive stretching and strengthening exercises
Duchenne’s: patient teaching
Teaching deep breathing exercises
Performing chest physical therapy
Duchenne’s: preventing complications and maximizing quality of life
Developing a diversional schedule
Providing emotional support
for neuromuscular disorders like DMD and spina bifida, consider:
Growth and development
Care of disabled patient
Family (sibling) support
Coping with ongoing stress & periodic crises
Palliative care
End of life care
cerebral palsy: causes
- prenatal
- perinatal: infection, trauma, hypoxia
- postnatal factors
80% of CP cases are linked to
perinatal or neonatal brain lesion or brain maldevelopment
4 primary movement disorders of CP
- spastic is most common (80% of cases): upper motor neuron muscular weakness
CP clinical manifestations
Delayed gross motor skills
Abnormal motor movements
Altered muscle tone
Abnormal posture
Abnormal reflexes
Associated disabilities
CP nursing management
Developmental assessment
MS assessment/neuro assessment
Parent participation and education for normalization
Parent and sibling support
Multidisciplinary approach
CP assessment and maintenance
Nutritional needs
Limited mobility concerns (skin, B/B)
Adaptive equipment for age and deficits
Safety precautions (meds, helmet, fall-risk car seat)
Health promotion (immunizations, dental, sensory etc.)
pain with CP
Intense pain may occur with muscle spasms in patients with CP as a result of:
- painful procedures such as injections used to control spasms
- surgical procedures intended to reduce contracture deformities
- anatomical position and gastroesophageal reflux
- physical therapy
medications for pain and spasticity with CP
Botulinum toxin A
baclofen (Lioresal)
dantrolene sodium (Dantrium)
diazepam (Valium)
BOTULINUM TOXIN A
used to reduce spasticity in muscles of the upper and lower extremities
when BOTULINUM TOXIN A is administered early in the course of the illness it can
Prevent muscle contractures
Decrease the need for surgical procedures with adverse effects
BOTULINUM TOXIN A: goal
allow relaxation and stretching of the muscle
permit ambulation with an AFO
BOTULINUM TOXIN A: prime candidates
children with spasticity confined to the lower extremities
BOTULINUM TOXIN A: side effects
Pain at the injection site and temporary weakness
Diazepam
- used frequently but should be restricted to older children and adolescents.
- Oral administration: little improvement with muscle coordination in children with CP. More effective in decreasing overall spasticity.
Diazepam side effects
drowsiness, fatigue, and muscle weakness
hallucinations, mood changes, seizures, nausea, and urinary incontinence.
Baclofen (Lioresal)
- Higher PO doses are associated with significant side effects (ex. drowsiness and confusion); often provide little to no relief of spasticity.
- Oral administration: little improvement with muscle coordination in children with CP. More effective in decreasing overall spasticity.
Baclofen (Lioresal): side effects
drowsiness, fatigue, and muscle weakness.
diaphoresis and constipation (with oral baclofen) hallucinations, mood changes, seizures, nausea, and urinary incontinence.
dantrolene sodium (dantrium)
Oral administration: little improvement with muscle coordination in children with CP. More effective in decreasing overall spasticity.
dantrolene sodium (dantrium): side effects
hepatotoxicity (dantrolene), drowsiness, fatigue, and muscle weakness.
hallucinations, mood changes, seizures, nausea, and urinary incontinence.
INTRATHECAL BACLOFEN
- Direct infusion of baclofen into the intrathecal space provides relief without as many side effects
- implantation of a pump to infuse baclofen directly into the intrathecal space surrounding the spinal cord to provide relief of spasticity
- Provides excellent improvement in comfort
- Outpatient pump refill q 4-6 weeks
Abrupt withdrawal, especially at high doses, may result in adverse effects (ex. rebound spasticity, pruritus, hyperthermia, rhabdomyolysis, disseminated intravascular coagulation, multiorgan failure, and death. - Goal of treating med withdrawal = reestablishing the medication dosage
INTRATHECAL BACLOFEN: med withdrawal
- in some cases intrathecal baclofen withdrawal may mimic sepsis. Needle inserted into pump to refill.
- Treatment of med withdrawal: Should see improvements within 1 to 2 hours.
- Hospitalization and surgery may be required for withdrawal that was the result of pump or catheter failure
gross motor milestones: 6-8 months
sits with support
gross motor milestones: 9 months
- concern if not sitting at this point
- stands while holding
gross motor milestones: 10 months
pulls to standing
gross motor milestones: 12 months
walks with assistance
gross motor milestones: 15 months
- walks wall, unaided
- gait is wide-based
gross motor milestones: 18 months
- runs well, unaided
- gait is wide-based
gross motor milestones: 2 years
- goes up and down stairs, two feet per step, without assistance
gross motor milestones: 2.5 years
- jumps on both feet
- may walk on tiptoes
- concern if cannot jump by school age
- hand dominance is appropriate around 2.5-3 years (earlier is a concern of CP)
gross motor milestones: 3 years
- stands on one foot (few seconds)
- goes up stairs 1 foot per step, comes down 2 feet per step
gross motor milestones: 4 years
- hops on one or both feet
- goes up and down stairs like an adult (1 foot per step)
- heel and tiptoe walk
gross motor milestones: 5 years
skips
gross motor milestones: 7 years
- balance on one foot for 20 seconds
- should be coordinated
growth and development is dependent on
the child
- not every child will be sitting at 6 months
- we give a little time before concerned (like around 3 months)
MS assessment should include
- gait
- posture
- curvature of the spine
- muscle strength: arms, hands, legs
- ROM: extremities, joints
signs and symptoms of fractures
- Swelling
- Pain or tenderness
- Deformity
- Diminished functional use of affected part
- Bruising
- Muscle rigidity
- Crepitus** (direct pressure on spot that hurts, hear crunching)
- Hx of injury may be lacking due to age
common variations in growth development
- bowleg or genu varum
- knock knee or genu valgum
- pigeon toe or toeing in
toddlers carry their weight in
their abdomen
- the abdomen will protrude
- lordosis
(developmentally this is okay, around 4/5 years this will go away)
bowleg (genu varum)
- Lateral bowing out of the tibia
- Bow seen when standing
- Outward curvature of femur & tibia
- Normal in TODDLERS
- Abnormal past the age of 2-3 years
- Common in African-American Children
- not > 2” between knees
- concern if causing issues to get or if one-sided
knock-knee
- Opposite of genu varum
- Measure distance between malleoli
- Normally present in 2-7 years of age
- past age 7 is concerning
- not > 3” between ankles
pigeon toe or toeing in
- Most common gait problem in young children
- Can result from a rotational deformity of the hips, legs, or feet
- internal rotation of bones in the hips, legs, feet
- Most common is r/t hips (W sitting)
- Can test leg/hip forms by assessing Babinski (plantar)
- Usually self-corrects with growth
- usually goes away by 8 years
- refer if unilateral or is getting worse
tibial torsion
tibia turn inward
- pigeon toe/toeing in
femoral antiversion
thigh bone (femur) turns inward
- pigeon toe/toeing in
metatarus adductus
feet turns inward
- pigeon toe/toeing in
vit D is given through
- nutrition and sun
(rice cereal, orange juice, whole milk)
Breastfed babies usually begin taking Vit D supplementation because there is a limited amount of Vit D in human milk.
affects cognition and development if not enough
rickets
- the softening and weakening of bones in children, usually because of an extreme and prolonged vit D deficiency
- consider family hx too
- very thin long bones, swollen joints
- protrusion of sternum (necklace appearance)
s/sx of rickets
Delayed motor development
Injury
Pain, loss of sensation, tingling
Muscle weakness, loss of function of an extremity
Interferes with usual ADL’s or play
common causes of immobilization
- Disability
- Congenital Defects
- Neuromuscular conditions
- Prolonged mechanical ventilation and sedation
- traction
- surgery
- infections and injuries impairing: MS, integumentary, neurological
immobility effects: muscular system
- decreased muscle mass
- atrophy
- wasting
interventions for muscular system
- ROM exercises
immobility effects: skeletal system
- bone damage (d/t calcium)
- hypercalcemia
- higher risk of fractures
skeletal system interventions
- calcitonin
- IVF/ fluids
- biphosophate therapy
immobility effects: CV system
- blood clots
- heart has to work harder
CV system interventions
- anticoagulants
- pulses above and below site of injury
immobility effects: respiratory system
- PNA
respiratory system interventions
- incentive spirometer
- blow bubbles
- blow cotton balls
- anything to encourage breathing
immobility effects: GI system
- constipation
GI system interventions
- increase fiber and fluids
immobility effects: integumentary system
- breakdown
- shearing with movement
integumentary interventions
lotions, massage, position changes
immobility effects: renal
renal calculi d/t hypercalcemia
immobility effects: neurosensory
- unusual pressure
- pain
- ultimately can lead to decreased sensation
renal system interventions
calcitonin (to prevent calcium kidney stones)
neurosensory interventions
position changes
immobility effects: metabolism
- decreased BMR
- increased diuresis, natriuresis
- depletion of nitrogen and calcium
metabolism interventions
psychosocial effects of immobilization
depression, isolation
possible regression in milestones
example: bedwetting, picky eater, personality changes
- should return to normal/ resolve within a few days of discharge
- it is normal for a child to regress in the hosptial setting
5 P’s of ischemia
pulselessness
pain
pallor
paresthesia
paralysis
- reassess post-intervention too
ischemia
lack of blood flow to a certain area
- concern with casts
fracture complications
Circulatory impairment
Nerve compression syndromes
Compartment syndromes
Physeal damage: growth plate damage
- deformity of doesn’t heal right
Nonunion: fracture was casted but still did not heal correctly
Malunion: casted but was misaligned
Infection: osteomyelitis
Renal calculi: d/t hypercalcemia, urinary stasis
Pulmonary embolism (chest pain & dyspnea): #1 thing we are concerned about
compartment syndrome
Severe pain
Decreased/lack/deficit sensation
Edema
Paleness
Pulse changes
Motor weakness
prevention of compartment syndrome
prevention = CSM assess/reassessment
compartment syndrome management
- assess the 5 P’s
- notify ortho
- remove any mechanically obstructive materials
- do not elevate if compartment syndrome is suspected
- if not improved, do a fasciotomy
why may compartment syndrome happen in a casted child?
cast became too tight
- if not treated and ischemia develops, will lose limb
fasciotomy
surgical intervention for compartment syndrome
goal:
- decrease arterial spasms
- increase blood flow
cast care: application
- Anticipatory guidance (burning with drying)
- Age approp. explanations
- Distraction with application (age approp.)
- Petal edges PRN
- Teach CSM checks
cast care: anticipatory guidance
- Cutter sounds
- Warmth
- Caked skin
- Atrophy
traction is used for
Provide rest for the extremity
Prevent deformity
Reduce muscle spasms
Immobilize fracture site
Proper realignment
Allow healing time before surgery or casting (callus formation)
pain relief
manual traction
- applied by the examiner’s hands
skin traction
- 5-7 lbs.
- attached to skin indirectly.
- Used if temporary or light force is needed.
skeletal traction
- 25-40 lbs.
- attached directly to the bone using tongs, pins, or screws
what to look out for with traction
infection:
- fever
- swelling
- pain
- heat
halo traction
- screws going into the scalp
traction managment
- maintain traction
- assess the skin and CMS
- distraction and education; quiet play
tortocollis
- kink in the neck
- can be congenital or acquired
- plagiocephaly: flattened back of head (one sided)
- brachiocephaly
treatment of torticollis
- get them off their head
- tummy time
- get their focus towards the opposite side as the kink
- gentle stretches/passive ROM (left and right motion of head)
- surgery if severe or unable to correct
developmental dysplasia of hip (DDH)
- Unknown/Multi-factorial cause (genetic and environmental)
- seen commonly in children
- 80% more likely in F than M
DDH (develpomental dysplasia of hip) physiologic factors
Maternal hormone secretion
Intrauterine position
Joint laxity
Co-existing orthopedic dx’s
DDH mechanical factors (think perinatal)
Breech presentation
Multiple fetuses
Oligohydramnios
Large infant size
Postnatal positioning
DDH genetic factors
- Parent
- Siblings of affected infants
DDH is diagnosed through
- hip ultrasound: for < 6 months
- x-ray of hip: for > 6 months
can do a physical exam: will hear and feel popping of hip
acetabular dysplasia DDH
- acetabular abnormality
subluxation DDH
- shifted out of hip socket
dislocation DDH
- shifted out of hip socket and goes superior and posterior
signs to look for DDH: infant 0-4 weeks
- Hip joint laxity
- Ortolani
- Barlow
- Galeazzi
- Shortening of thigh on affected side
- Asymmetric folds (thigh and buttocks)
- more folds on the affected side
- Unequal knee height
- Limited abduction (away from center point)
signs to look for DDH: older infant and child
- The leg of affected hip appears shorter
- Waddling gait
- Lumbar lordosis (bilat)
- Trendelenburg sign (pelvis tilts downward when the leg is raised)
- Spacing between the legs may look wider than normal
pavlik harness
- for DDH
- surgeon puts on
- stays on
- need to go back every couple of weeks to have it adjusted by surgeon
- concerned for skin integrity under the straps
pavlik harness: parent teaching
Maintain reduction
Removal for bathing (may not be allowed)
Do not adjust
Skin care: no lotion, no powder, massage is okay
Prevention of complications
Normalcy
spica cast: care of child, safety & diapering
used for DDH!
care of the child
- have to support the child
- will fall over
- emolskin for protection of skin under the arms
safety
- positioning
- clothing
diapering
- double diapering
- sanitary pad and then diaper
- water proof tape around the edges to prevent urine and stool from getting on the cast
swaddling DDH
- don’t put babies legs straight down- could pop hip out of socket
- let legs be in normal position
- stop swaddling the babies when they start rolling over (aspiration risk)
congenital clubfoot
- diagnosed as early as 20 weeks (in utero or at birth)
- assess for co-existing hip abnorms
affected foot: clubfoot
Foot and calf size variation
- Affected limb smaller and shorter
- Empty heel pad (no fat pad)
- Midfoot plantar crease
- Calf atrophy
Stiffness in the ankle or foot tendons
Affected foot (feet) lack full range of motion
clubfoot goals
Correct deformity
Maintenance of correction
Prevention of recurrence
treatment of clubfoot
- Ponseti method-serial casting with stretching
- Heelcord tenotomy
- Log leg cast
- Denis Browne bar and shoe brace
- If not effective surgical pinning
*this will delay walking
* usually in this several hours a day
osteogenesis imperfecta
- Most common pediatric osteoporosis syndrome
- caused by defect of the genes: colia-1 and colia-2
- > 11 classifications: type 1 is most common and mildest; type 2 is most fatal (stillborn or die within a few days of birth)
- Distinguish OI from child abuse
- NO CURE
assessment/ s/sx of osteogenesis imperfecta
- Blue sclera
- Hearing loss
- Dentinogenesis imperfect (discolored teeth)
- Growth restrictions (bc body isn’t growing; so organs cant grow- don’t work properly for age)
- they do have a normal IQ
- body doesn’t grow but head grows
therapeutic management of osteogenesis imperfecta
Supportive treatment
Bisphosphonate therapy
Bone marrow transplantation (experimental)
osteogenesis imperfecta: care goals
Decreasing incidence of fx’s
Decreasing pain
Increasing growth
Improving bone metabolism
Optimizing function
Collaborative care
osteogenesis imperfecta: nursing care
- Handling with care
- Diaper changes
- Support with positioning
- Manual b/p’s: decreases risk of fractures
- Bisphosphate therapy
- Caution with live vaccines
- Frequent head repositioning
- Routine dental care
- Child and Family education
- Support network
juvenile idiopathic arthritis (JIA)
Genetic susceptibility is not understood
Unknown cause (autoimmune)
s/sx before age 16
Peak onset:
2-4 yrs. and
10-14 yrs.
F>M
90% children have neg. RF
Chronic inflammation of synovium with joint effusion, destruction of cartilage, and ankyloses of joints as disease progresses
forms of JIA
Pauciarticular: involves two or more joints
Polyarticular: more then five joints
Systemic: affects organs, lympadeonpthy, fever
JIA s/sx
Stiffness (am)
Edema
Warmth (no erythema)
Tenderness
Loss of motion
Pain (variable)
Growth restrictions (severe cases)
Different from adult onset
*discomfort after rest is key
Symptoms may “burn out” and become inactive
medical management of JIA (drug therapy)
NSAIDS (start with)
DMARD’s (methotrexate)
- GI upset, infection, WBC, fetal risks
Biological DMARD’s (humara)
- causes malignancy
Glucocorticoids
physical management of JIA (non-pharm)
PT/ROM
Water therapy
Splinting overnight (to prevent footdrop)
Surgery if physical mgmt. unsuccessful
JIA goals
- relieve pain
- promote general strength: diet, exercise, sleep/rest
- encourage involvement in age appropriate activities
JIA nursing management
- facilitate compliance
- comfort measures
- participation in ADLs
- exercise with nighttime splinting
scoliosis
- most common spinal deformity
- may be congenital or develop during childhood
- most commonly idiopathic
- complex spinal deformity in 3 planes:
-lateral curvature
-spinal rotation causing rib asymmetry
-thoracic hypokyphosis
scoliosis screening
- want to screen young bc once puberty/growth plates close, not much can be done
- scoliosis x-rays
scoliosis exam
- scapulary hump
- lordosis
- kyphosis
scoliosis brace
- wear for 23 hours a day
- slows progression
- allows time for skeletal maturity
scoliosis checks
girls: 10 and 12 years
boys: 13 and 14 years
actually do them younger*
degrees/grades of scoliosis
mild: 10-25° - wait and see
moderate: 25-45° - bracing
severe: > 45° - surgery
scoliosis labs
- CBC
- CMP
- PT/PTT
- imaging
patient education post-surgery for scoliosis
- log roll
- pillow splinting for cough
scoliosis therapeutic management
- Treatment is based on magnitude, location, type of curve, age, skeletal maturity, and other disease dx’s
- bracing
- boston or wilmington braces
- exercise with bracing
scoliosis operative management
- For progressive or severe curves that does not respond to more conservative measures
- Spinal fusion-technique is surgeon’s preference
- Psychological and physiological care
scoliosis: pre-op care
- blood work/blood loss plan
- teaching (PCA, log-rolling, tubes)
scoliosis: post-op care
- Early recognition of complications
- PCA, early mobilization, skin, I/O, Resp
