Unit 40 Musculoskeletal, Neuromuscular and Cognitive Disorders in Children Flashcards
What are musculoskeletal disorders?
Scoliosis
Developmental Dysplasia of the Hip (DDH)
Juvenile Rheumatoid (Idiopathic) Arthritis
Describe Scoliosis.
- Most common spinal deformity
- Usually involving lateral curvature of spinal rotation causing rib asymmetry and thoracic hypokyphosis
- Can be congenital, or can develop during infancy or childhood
- Most common presentation is during the growth spurt of early adolescence
What is the therapeutic management and surgical intervention for Scoliosis?
Therapeutic management:
- Brace if lateral curve 20-40 degrees (Boston brace)
- Brace is not curative; slows the progression of the curve
Surgical intervention:
-Harrington Rod, Spinal fusion, Spinal stabilization for curves greater than 40 degrees
What is important for nurses regarding Scoliosis after brace or Sx? What are important post op points to remember?
- Screen children
- Observe child’s reaction to device used
- Need to assess compliance
-Provide post-op care:
Pain priority
Logrolling, wound care, elimination, mobility
What are the signs and symptoms of Developmental Dysplasia of the Hip (DDH)?
- Restricted abduction
- Shorter limb affected side
- Asymmetrical though and gluteal folds
- Positive Ortolani-Barlow maneuver (tests used to check for DDH)
What is the treatment for DDH?
- Pavlik harness (6months - 1 year old)
- Skin traction
- Surgery and Spica Cast (hard cast, can also be used for fractures)
Describe nursing spica cast care in the PERSON format.
P -Encourage independence E -Protect cast as child urinates or has BM -Use fracture pan as trained R -Turn child q2h -Provide bedside activity S -Pillows under legs with heels off mattress -Pillow under chest if lying prone -Do NOT turn child by grabbing abduction bar -Keep HOB flat -Lubricate skin -No object put in cast for itchy skin* -Keep cast dry* O -Check circulation/RR q2h -Encourage deep breathing N -May need to eat in prone position with head flexed upward -Use straws -Stay with child while eating
Describe Juvenile Rheumatoid (Idiopathic) Arthritis. What is the treatment?
- Chronic autoimmune inflammatory disorder affecting the joints (also skin, tissues, tendons, eyes)
- Clinical Dx, often presents in toddlers with joint pain and physical disabilities
Treatment:
- NSAIDS
- methotrexate (decreases immune system function to decrease damage, however increased risk of infection)
- Steroids
- Physical and Occupation therapy
- Heat and whirlpool therapy
- Non weight bearing exercise (keeping joints mobile is crucial)
- Biologic drugs such as: etanercept, infliximab (side effect flu like symptoms)
What are neuromuscular disorders in children?
Hydrocephalus
Spina Bifida
Cerebral Palsy
Muscular Dystrophy
What is Hydrocephalus?
Increased accumulation of CSF in ventricles which compresses the brain and increases pressure.
-Anything that increases CSF in brain
Symptoms of underlying brain disorder resulting in:
- Impaired absorption of CSF
- Obstruction to the flow of CSF within the ventricles
- Destruction of brain
Basically TOO MUCH FLUID = PRESSURE IN THE BRAIN
Why does Hydrocephalus happen?
- Abnormal development of CNS
- Bleeding in the ventricles (premies)
- Intrauterine infection (rubella, syphilis)
In older age from:
- Lesions, tumors in brain
- CNS infections
- Bleeding from stroke, head injury
- Trauma
What are the defining characteristics of Hydrocephalus in Infancy?
- Irritability
- Lethargy
- May have changes in LOC
- Increased BP decreased HR
- Abnormally rapid head growth
- Bulging anterior fontanel
- Dilated scalp veins
(Late signs include: depressed eyes, setting sun sign- eyes, pupils sluggish with unequal response to light)
-In advance cases: difficulty sucking and feeding
, shrill pitched cry
What are defining characteristics of Hydrocephalus in childhood?
-Headache on awakening
-Extrapyramidal tracts signs (ataxia) loss of control of
body movements
- Papilledema (optic nerve swelling because of increased pressure)
- Confusion
- Vomiting
What is the Dx for Hydrocephalus in Infants and Children?
Infants:
-Head circumference that crosses one or more grid lines on the chart within a period of 2-4 weeks**
Both infants and children:
- Neurological signs that are present and progressive
- Ultrasound on anterior fontanelle
- CT/MRI (both require sedation)
What is the management of Hydrocephalus/CSF?
- Treat underlying brain disorder if applicable
- Look for early signs
- Teach parents
- Diagnoses usually requires Sx to prevent brain damage
**VP Shunt which provides drainage of the ‘CSF’ from the ‘ventricle’ to an extra-cranial compartment usually the ‘peritoneum’
What is done postoperatively for Hydrocephalus VP shunt? What are the complications?
- Position flat* (for GRADUAL drainage)
- Stay off operated side
- Pain management
- Observe for signs of ICP (pupil changes, LOC, BP up HR down
Infants - should see a gradual decrease in head circumference if successful
Complications: Infection, Malfunction (ICP)
What is Spina Bifida?
Congenital defect of incomplete closure of the vertebrae and *neural tube during fetal development.
- Neural tube fails to close at 3-5 weeks gestation
- Hydrocephalus plays a role
(Folic acid is important to take)
What are the types of spina bifida?
Occulta - defect is not visible externally (very tiny, occurs in lumbar-sacral area, little hair spot)
Cystica - visible defect with an external saclike fluid
2 types:
>Meningocele- encases meninges and spinal fluid; CFS (may have normal function)
> Myelomeningocele - contains meninges, spinal fluid and spinal cord nerves!, spinal tissue as well (spinal dysfunction likely)
What are Spina Bifida risk factors during pregnancy?
Genetic mutation in FOLATE pathways
IDM (Insulin dependent diabetes)
Anticonvulsant meds
Low VitB12/ Low folic acid
Exposure to high temps and radiation
More in Caucasian women
What is the Dx for Spina Bifida and the clinical manifestations?
Fetal ultrasound (may due intrauterine Sx) MRI
Manifestations:
- Short, small lower extremities
- Club feet
- Dysplastic hips
- Uppe extremities normal
What are Myelomeningocele nursing considerations?
- Application of sterile, moist non-adhesive dressing over the defect
- Place child prone position in warmer
- Check head circumference
- Latex precautions
- Prevent Infection
- Anticipate Sx
What is spasticity treated with in Spina Bifida?
spasticity (treat with baclofen/Lioresal)
What are Spina bifida Bowel and GU considerations?
- Brain cannot tell when rectum as stool = no signal for keeping sphincter closed
- Constipation due to decreased activity and meds
- Will need bowel regimen of high fiber foods/fluids, laxitives/enemas, and bowel training
- Neuropathic bladder dysfunction, GU goal is to preserve renal function
- Prone to UTI’s
- May need oxybutynin or other musculotropics/antispasmodics
What are Spina Bifida orthopedic problems and cognitive challenges?
Orthopedic problems:
- Paralysis of lower extremities (no feeling below defect)
- Contractures (*prevent them!)
- Scoliosis/kyphosis
- Dislocated hips/club foot
- Obesity can result from mobility challenges (lower their BMI*)
Cognitive problems:
- Difficulty with time management
- Difficulty with planning skills/abstraction
- May have leaning disabilities/visual perception challenges/attention challenges
What is the prevention of Neural Tube Defects?
Folic acid 0.4 mg prevents 50-70% of neural tube defects
All women of childbearing age should take!
Neural tube closes often before women realizes she’s pregnant.
What is Cerebral Palsy (CP)? What can accompany it?
- Early onset, NON-progressive impaired muscle movements and posture
- Coordination deficits
- May be accompanied by language deficits, intellectual problems, and swallowing difficulties
- Most common physical disability in childhood
Why does CP happen?
- Birth trauma
- After birth could be various congenital intrauterine insults to brain
- Unexplained reasons
- Premie status
- Low birth weight
- Multiples
- Breech
- Maternal factors such as: thyroid, seizures, infections, drug use
What are the clinical classifications of CP?
Spastic: constantly contracted/tight (premies) painful
Dyskinetic: athetoid, slow involuntary writhing (shakey movements), difficulty controlling voluntary movements, can be hypotonic
Ataxic: cerebellar injury - coordination, depth perception, balance, tremors effected
Mixed:
What is the Diagnosis for CP?
May be missed at birth!
- Missing developmental milestones*
- Abnormal reflexes (may keep primitive ones)
What is the CP treatment?
Treat EARLY for best outcomes/reach child’s potential
-Meds for seizures
-Meds for spasms:
baclofen
botox injections if an isolated muscle spasms.
- Orthotics for balance/body support
- Mechanical aids: utensils/ communication devices
- Speech/behavior therapy
- Sx if needed to decrease spasticity and contractures
What are Cerebral Palsy medical problems?
Seizures:
- can cause more brain damage
- often high doses of AED’s (antiepileptic drugs)
Aspiration:
- inability to cough/clear secretions
- often have GT or JT for meds/feeding
Incontinence
Constipation: from inactivity and meds
Wounds: from pressure of same positions
Osteopenia: because lack of weight bearing
What is important socially regarding cerebral palsy?
Physically impaired NOT socially!
- Don’t assume that they are also cognitively impaired!
- They understand, but can’t communicate verbally
- Speak TO them, not AROUND them
- Use normal tone of voice
What are the nursing care/therapeutic interventions for CP?
- Care is preventative for complications and symptomatic
- maximize quality of life**
- Address families needs
- May need help with ADL’s
What is Duchene’s Muscular Dystrophy (DMD)? who is exclusively effected and who are the carriers?
An inherited disorder of progressive muscular weakness, males effected exclusively.
- sex linked recessive
- prenatal Dx
- lack Dystrophin, protein found in skeletal muscle
- Females are carriers
- Deadly
- Family hx present in more than half of the cases
What are the defining characteristics of Duchene’s Muscular Dystrophy (DMD)?
- Early onset usually 3-5 years
- Progressive muscular weakness, wasting and contractures
- *Calf muscle hypertrophy
- Loss of independent ambulation by 9-11 years old
- May have cardiorespiratory involvement in adolescence (palpations, irregular HR)
- Relentless progression until death from respiratory or cardiac failure
- Gower’s sign
What is Gower’s sign?
Patient uses their hands and arms to “walk” up their own body from a squatting position due to lack of hip and thigh muscle strength.
How is the dx for DMD made? What is the therapeutic management?
-Dx is made by muscle biopsy, EMG and a serum enzyme level
Therapeutic management:
- Maintain function in unaffected muscles for as long as possible
- Sx to release contractures deformities
- Bracing
- Help with performing ADL’s
- Genetic counseling for family
What are Cognitive Disorders? What is the etiology?
Cognitive impairment, intellectual disability, developmental disability or delayed
(Down syndrome is example)
Etiology: Hereditary/chromosomal Early fetal alterations Acquired childhood diseases Environmental factors
Describe Cognitive Impairment/Developmental Disability.
- Intellectual functioning IQ 70-75 or below
- Functional impairment in at least 2 of 10 adaptive skills
- Dx at 18 years of age or younger
- Social behaviors immature or outside norm for age
What is the difference between chronological age and developmental age? Which should be considered when developing care plan?
Chronological age - actual age
Developmental/Functional - Age group where skills and milestones are met, etc
*(Key for care plans)
What is Down Syndrome?
Most common disorder causing moderate to severe Developmental Disability.
- Trisomy 21
- Higher incident with advanced maternal age
- Etiology unknown
What is the Diagnoses for Down Syndrome? What are they are higher risk for?
- Chromosome analysis will confirm the genetic abnormality
- Congenital heart defects are at a higher incident for them
- Upper respiratory infections at a higher risk for them
- Hypotonicity of best and abdominal muscles
- Increased incidence leukemia
What are the characteristics of down syndrome?
Musculoskeletal :
- short stature
- muscle weakness
- hypotonia
Head:
- flat occiput
- seperated saggital suture
Eyes:
- Oblique fissures (slant)
- Short sparse eyelashes
Ears:
-Low set and small
Mouth:
-Protruding tongue
Hands:
-Simian crease (line going through entire hand)
What is first priority regarding parents and them finding out they have a child with a cognitive disorder?
- First priority is to have parents express their feelings about the loss of a normal child
- Feelings of grief, anger, guilt, and acceptance are part of the process all parents must go through
- Encourage parents to be involved in meetings parents with similar children
