DOWN SYNDROME
Most common chromosomal abnormality in children
Results from a trisomy of chromosome 21, and in less than 5% of cases, a translocation of chromosome 21
Down syndrome is associated with maternal age over 35
DOWN SYNDROME Nursing Assessment:
Flat, broad nasal bridge
Inner epicanthal eye folds
Upward, outward slant of eyes
Protruding tongue
Short neck
Transverse palmar crease (simian crease)
Hyperextensible and lax joints (hypotonia)
DOWN SYNDROME Nursing Assessment:
Common associated problems:
Cardiac defects
Respiratory infections
Feeding difficulties
Delayed developmental skills
Mental retardation
Skeletal defects Altered
immune function
Endocrine dysfunctions
DOWN SYNDROME Nursing Diagnoses
Delayed growth and development
Risk for impaired parenting
DOWN SYNDROME Nursing Interventions:
Assist and support parents during the diagnostic process and management of child’s associated problems
Assess and monitor growth and development
Teach use of bulb syringe for suctioning nares
Teach signs of respiratory infection
Assist family with feeding problems
Feed to back and side of mouth
Monitor for signs of cardiac difficulty or respiratory infection
Refer family to early intervention program
Refer to other specialist as indicated: nutritionist, speech therapist, physical therapist, and occupational therapist
The nursing goal in caring for a child with Down syndrome is to help the child reach his or her optimal level of functioning.
CEREBRAL PALSY (CP)
Nonprogressive injury to the motor centers of the brain causing neuromuscular problems of spasticity or dyskinesia (involuntary movements).
Associated problems may include mental retardation and seizures.
Causes include:
Anoxic injury before, during, or after birth
Maternal infections
Kernicterus
Low birth weight (major risk factor)
CEREBRAL PALSY (CP)
Nursing Assessment:
Persistent neonatal reflexes (Moro, tonic neck) after 6 months
Delayed developmental milestones
Apparent early preference for one hand
Poor suck, tongue thrust
Spasticity (may be described as “difficulty with diapering” by mother or caregiver)
Scissoring of legs (legs are extended and crossed over each other, feet are plantar-flexed; a common characteristic of spastic CP
Involuntary movements
Seizures
CEREBRAL PALSY (CP)
Nursing Diagnoses:
Delayed growth and development related to …….
Risk for imbalanced nutrition: less than body requirements related to…….
CEREBRAL PALSY (CP)
Nursing Interventions:
Identify cerebral palsy through follow-up of high-risk infants such as premature infants.
Refer to community-based agencies.
Coordinate with physical therapist, occupational therapist, speech therapist, nutritionist, orthopedic surgeon, and neurologist.
Support family through grief process at diagnosis and throughout the child’s life. Caring for severely affected children is very challenging.
Administer anticonvulsant medications such as phenytoin (Dilantin) if prescribed.
Administer diazepam (Valium) for muscle spasms if prescribed.
CEREBRAL PALSY (CP)
NCLEX Hint: Feed infant or child with cerebral palsy using nursing interventions aimed at preventing aspiration.
Position child upright, and support the lower jaw.
INCREASED INTRACRANIAL PRESSURE
Nursing Assessment:
Signs of increased intracranial pressure:
Infant
Poor feeding or vomiting
Irritability or restlessness
Lethargy
Bulging fontanel
High-pitched cry
Increased head circumference
Separation of cranial sutures
Distended scalp veins
Eyes deviated downward
Increased or decreased pain response
INCREASED INTRACRANIAL PRESSURE
Nursing Assessment:
Signs of increased intracranial pressure:
Child
Headache
Diplopia
Mood swings
Slurred speech
Papilledema
Altered level of consciousness
Nausea and vomiting, especially in morning
SPINA BIFIDA
Definition:
Malformation of the vertebrae and spinal cord resulting in varying degrees of disability and deformity.
Spina bifida occulta
is a defect of vertebrae only. No sac is present, and it is usually a benign condition, although bowel and bladder problems may occur.
SPINA BIFIDA With meningocele and myelomeningocele
a sac is present at some point along the spine.
SPINA BIFIDA
Meningocele
contains only meninges and spinal fluid and has less neurologic involvement than a myelomeningocele.
SPINA BIFIDA
Myelomeningocele
is more severe than meningocele because the sac contains spinal fluid, meninges, and nerves.
SPINA BIFIDA
The severity of neurologic impairment is determined by the anatomic level of the defect.
SPINA BIFIDA
Every child with a history of spina bifida should be placed on latex precautions.
SPINA BIFIDA
Prevention:
folic acid 0.4 mg is taken daily for all women of child bearing age
SPINA BIFIDA Nursing Assessment:
Spina bifida occulta: dimple with or without hair tuft at base of spine
Presence of sac in myelomeningocele is usually lumbar or lumbosacral
Flaccid paralysis and limited or no feeling below the defect
Head circumference at variance with norms on growth grids
Associated problems:
Hydrocephalus (90% with myelomeningocele)
Neurogenic bladder, poor anal sphincter tone
Congenital dislocated hips
Club feet
Skin problems associated with anesthesia below the defect
Scoliosis
SPINA BIFIDA Nursing Diagnoses:
Risk for infection R/T
Impaired urinary elimination patterns R/T
Impaired physical mobility R/T
SPINA BIFIDA Nursing Diagnoses:
Risk for infection R/T
Impaired urinary elimination patterns R/T
Impaired physical mobility R/T
SPINA BIFIDA Nursing Interventions
Preoperative:
Place infant in prone position
Keep sac free of stool and urine
Cover sac with moist sterile dressing
Position child on his/her abdomen, with legs abducted
Measure head circumference every 8 hours; check fontanel
Assess neurologic function
Monitor for signs of infection
Empty bladder using Crede’ method, or catheterize if needed
Promote parent-infant bonding
SPINA BIFIDA Nursing Interventions
Postoperative:
place infant in prone position
Make same assessments as preoperatively
Assess incision for drainage and infection
Assess neurologic function
SPINA BIFIDA Nursing Interventions
Long-term care
Teach family catheterization program when child is young
Help older children to learn self-catherization
Administer urecholine ( a cholinergic medication)as prescribed to improve continence
Develop bowel program
- -High fiber diet
- -Increased fluids
- -Regular fluids
- -Suppositories as needed
Assess skin condition frequently
Assist with range-of-motion (ROM) exercises, ambulation, and bracing, if client is able
Coordinate with team members: neurologist, orthopedist, urologist, physical therapist, and nutritionist
Support independent functioning of child
Assist family to make realistic developmental expectations of child
HYDROCEPHALUS
Condition characterized by an abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles of the brain
It is usually caused by an obstruction in the flow of CSF between the ventricles
Hydrocephalus is most often associated with spina bifida. It can be a complication of meningitis.
HYDROCEPHALUS
Nursing Assessment:
Early manifestations of hydrocephalus in the INFANT:
Rapid head growth
Full, bulging fontanel
Irritability
Poor feeding
Distended, prominent scalp veins
Widely separated cranial sutures
HYDROCEPHALUS
Early manifestations of hydrocephalus in the CHILD:
Strabismus
Headache that occurs in the morning and is relieved by emesis or sitting upright
Nausea and vomiting (may be projectile)
Diplopia
Restlessness
Behavior or personality changes
Ataxia
Sluggish or unequal pupillary responses
Confusion
Changes in school work
Lethargy
HYDROCEPHALUS
Late manifestations in the infant
“Setting sun” sign
Frontal bone enlargement or bossing
Vomiting, difficulty swallowing or feeding
Increased blood pressure
Decreased heart rate
Altered respiratory pattern
Shrill, high-pitched cry
Sluggish or unequal pupillary responses
HYDROCEPHALUS
Late manifestations in the child
Seizures
Increased blood pressure
Decreased heart rate
Alteration in respiratory pattern
Blindness from herniation of the optic disc
Decerebrate rigidity
HYDROCEPHALUS
NCLEX HINT: Baseline data on the child’s usual behavior and level of development are essential so changes associated with increased ICP can be detected early.
HYDROCEPHALUS Nursing Diagnosis:
Delayed growth and development related to
Risk for injury related to
HYDROCEPHALUS Nursing Interventions:
Prepare infant and family for diagnostic procedures
Monitor for signs of increased ICP
Maintain seizure precautions
Prepare parents for surgical procedure
---Shunt is inserted into
ventricle
---Tubing is tunneled through
skin to peritoneum where it
drains excess CSFHYDROCEPHALUS Nursing Interventions:
Prepare infant and family for diagnostic procedures
Monitor for signs of increased ICP
Maintain seizure precautions
Prepare parents for surgical procedure
---Shunt is inserted into
ventricle
---Tubing is tunneled through
skin to peritoneum where it
drains excess CSFHYDROCEPHALUS Nursing Interventions:
Postoperative care
- Assess for signs of shunt malfunction
Infant:
—Changes in size, signs of bulging, tenseness, and separation in fontanels and suture lines
—Irritability, lethargy, or seizure activity
—Altered vital signs and feeding behavior
Older child: Increase in ICP
—Change in LOC
—Complaint of headache
—Changes in customary behavior (sleep patterns, developmental capabilities)
- Assess for signs of infection
- Monitor I&O closely
HYDROCEPHALUS Nursing Interventions:
Teach home care program
Teach to watch for signs of increased ICP or infection
Note that child will eventually outgrow shunt and show symptoms of difficulty
Note that child will need shunt revision
Provide anticipatory guidance for potential problems with growth and development
HYDROCEPHALUS
NCLEX HINT: The signs of increased intracranial pressure (ICP) are the opposite of those of shock:
Shock: Increased pulse, decreased blood pressure
Increased Intracranial pressure: Decreased pulse, increased blood pressure
HYDROCEPHALUS
NCLEX HINT: The signs of increased intracranial pressure (ICP) are the opposite of those of shock:
Shock: Increased pulse, decreased blood pressure
Increased Intracranial pressure: Decreased pulse, increased blood pressure
SEIZURES
Uncontrolled electrical discharges of neurons in the brain
Seizure are more common in children under the age of 2 years
Seizures can be associated with immaturity of the CNS, fever, infection, neoplasms, cerebral anoxia, and metabolic disorders
Seizures are categorized as generalized or partial
SEIZURES
Generalized seizures are:
Tonic-clonic (grand mal):
Absence (petit mal):
Myoclonic;
SEIZURES
Tonic-clonic (grand mal):
consciousness is lost.
Tonic phase: generalized stiffness of entire body
Clonic phase: spasm followed by relaxation
SEIZURES
Absence (petit mal):
momentary loss of consciousness, posture is maintained; has minor face, eye, hand movements
SEIZURES
Myoclonic;
sudden, brief contractures of a muscle or group of muscles,
no postictal state.
May or may not be symmetrical or include loss of consciousness
SEIZURES
Partial seizures
arise from a specific area in the brain and cause limited symptoms.
Examples are focal and psychomotor seizures.
SEIZURES
Nursing Assessment
Tonic clonic (grand mal)
Aura (a warning sign of impending seizure)
Loss of consciousness
Tonic phase: generalized stiffness of entire body
Apnea, cyanosis
Clonic phase: spasms followed by relaxation
Pupils dilated and nonreactive to light
Incontinence
Postseizure; disoriented, sleepy
SEIZURES
Nursing Assessment
Absence seizure (petit mal)
Usually occur between 4 and 12 years of age
Last 5 to 10 seconds
Child appears to be inattentive, daydreaming
Poor performance in school
SEIZURES
NCLEX HINT: Medication noncompliance is the most common cause of increased seizure activity
SEIZURES Nursing Diagnosis:
Risk for injury: trauma related to seizure activity
Noncompliance related to
SEIZURES
Nursing Interventions:
- Maintain airway during seizure; turn client on side to aid ventilation
- Do not restrain client
- Protect client from injury during seizure, and support head. Avoid neck flextion.
- Document seizure, noting all data in assessment.
- Maintain seizure precautions
–Reduce environmental stimuli
–Pad side rails or crib rails
–Have suction equipment and
oxygen quickly accessible
–Tape oral airway to the head
of the bed
SEIZURES
Nursing Interventions:
- Maintain airway during seizure; turn client on side to aid ventilation
- Do not restrain client
- Protect client from injury during seizure, and support head. Avoid neck flextion.
- Document seizure, noting all data in assessment.
- Maintain seizure precautions
–Reduce environmental stimuli
–Pad side rails or crib rails
–Have suction equipment and
oxygen quickly accessible
–Tape oral airway to the head
of the bed
- Support during diagnostic tests: EEG, CT scan
- Support during workup for infections such as meningitis
- Administer anticonvulsant medications as prescribed:
a. For tonic-clonic seizures:
phenytoin, carbamazepine,
phenobarbital, and
fosphenytoin
b. For absence seizures:
ethosuximide (Zarontin),
valproic acid (Depakene) - Monitor therapeutic drug levels
- Teach family about drug administration: dosage, action, and side effects.
SEIZURES
NCLEX HINT: Do not use tongue blade, padded or not, during a seizure.
It can cause traumatic damage to oral cavity.
BACTERIAL MENINGITIS
Description: Bacterial inflammatory disorder of the meninges that cover the brain and spinal cord.
A. Meningitis is usually caused by Haemophilus influenza type B (less prevalent), Streptococcus pneumonia, or Neisseria meningitides.
B. The usual source of bacterial invasion is the middle ear or the nasopharynx.
C. Other sources of bacteria from wounds include fractures of the skull, lumbar punctures, and shunts.
D. Exudate covers brain, and cerebral edema occurs.
BACTERIAL MENINGITIS
Lumbar puncture shows
- Increased WBC
- Decreased glucose
- Elevated protein
- Increased ICP
- Positive culture for meningitis
BACTERIAL MENINGITIS
Nursing Assessment:
Older children
- Classic signs of increased ICP
- Fever, chills
- Neck stiffness, opisthotonos
- Photophobia
- Positive Kernig sign (inability to extend leg when thigh is flexed anteriorly at hip)
- Positive Brudzinski sign (neck flexion causing adduction and flexion movements of lower extremities)
BACTERIAL MENINGITIS
Nursing Assessment:
Infants
- Absence of classic signs
- Ill, with generalized symptoms
- Poor feeding
- Vomiting, irritability
- Bulging fontanel (an important sign)
- Seizures
BACTERIAL MENINGITIS
Nursing Diagnoses
A. Disturbed sensory perception R/T
B. Risk for trauma R/T
BACTERIAL MENINGITIS
Nursing Interventions:
Isolate (Droplet precautions )for at least 24 hours
A. Administer antibiotics (usually ampicillin, penicillin, or chloramphenicol) and antipyretics as prescribed.
B. C. Monitor vital signs and neurologic signs
D. Keep environment quiet and darkened to prevent overstimulation
E. Implement seizure precautions
F. Position for comfort: head of the bed slightly elevated, with client on side if prescribed.
G. Measure head circumference daily in infants
H. Monitor I & O closely
I. Administer Hib vaccine to protect against H. influenza infection
BACTERIAL MENINGITIS
NCLEX Hint: Monitor hydration status and IV therapy carefully. With meningitis there may be inappropriate ADH secretions causing fluid retention (cerebral edema) and dilutional hyponatremia.
REYE SYNDROME
Description: Acute, rapidly progressing encephalopathy and hepatic dysfunction
A. Causes include antecedent viral infections, such as influenza or chickenpox
B. Occurrence is often associated with aspirin use
C. Disease is staged according to the clinical manifestations to reflect the severity of the condition
REYE SYNDROME
Nursing Assessment:
A. Usually occurs in school-age children
B. Lethargy, rapidly progressing to deep coma with marked cerebral edema
C. Vomiting
D. Elevated SGOT/AST, SCPT/ALT, lactate dehydrogenase, serum ammonia, decreased PT
E. Hypoglycemia
REYE SYNDROME
Nursing Diagnosis:
A. Excess fluid volume R/T
B. Ineffective Breathing Pattern R/T
REYE SYNDROME
Nursing Interventions:
A. Provide critical care early in syndrome
B. Monitor neurologic status: frequent noninvasive assessments and invasive ICP monitoring
C. Maintain ventilation
D. Monitor cardiac parameters (i.e., invasive cardiac monitoring system).
E. Administer mannitol, if prescribed, to increase blood osmolality
F. Monitor I & O accurately:
G. Care for Foley catheter
H. Provide family with emotional support
BRAIN TUMORS
Description: Second most common cancer in children
Most pediatric brain tumors are infratentorial, making them difficult to excise surgically
Tumors usually occur close to vital structures
Gliomas are the most common childhood brain tumors.
BRAIN TUMORS
NCLEX Hint: Headache on awakening is the most common presenting symptom of brain tumors.
BRAIN TUMORS
Nursing Assessment:
Headache
Vomiting (usually in the morning), often without nausea
Loss of concentration
Change in behavior or personality
Vision problems, tilting of head
In infants: --widening sutures, --increasing frontal occipaital circumference, --tense fontanel
BRAIN TUMORS
Nursing Interventions:
Identify baseline neurologic function
Support child and family during diagnostic workup and treatment
If surgery is treatment of choice, provide preoperative teaching: --Explain that head will be shaved --Describe ICU, dressings, IV lines, etc. --Identify child’s developmental level, and plan teaching accordingly
Assess family’s response to the diagnosis, and treat family appropriately
After surgery; position client as prescribed by the health care provider.
Monitor IV fluids and output carefully. Overhydration can cause cerebral edema and increased ICP.
Administer steroids and osmotic diuretics as prescribed.
Support child and family to promote optimum function postopertively.
BRAIN TUMORS
NCLEX Hint: Most postoperative clients with infratentorial tumors are prescribed to lie flat or turn to either side.
A large tumor may require that the child not be turned to the operative side.
BRAIN TUMORS
NCLEX Hint: Suctioning, coughing, straining, and turning cause increased ICP.
BRAIN TUMORS
NCLEX Hint: Suctioning, coughing, straining, and turning cause increased ICP.
MUSCULAR DYSTROPHY
Inherited disease of the muscles, causing muscle atrophy and weakness
A. The most serious and most common of the dystrophies is Duchenne muscular dystrophy, an X-linked recessive disease affecting primarily males.
B. Duchenne muscular dystrophy appears in early childhood (ages 3 – 5 years). It rapidly progresses, causing respiratory or cardiac complications and death, usually by 25 years of age.
MUSCULAR DYSTROPHY
Nursing Assessment:
A. Waddling gait, lordosis
B. Increasing clumsiness, muscle weakness
C. Gowers sign: difficulty rising to standing position; has to “walk” up legs using hands
D. Pseudohypertrophy of muscles (especially noted in calves) due to fat deposits
E. Muscle degeneration, especially the thighs, and fatty infiltrates (detected by muscle biopsy); cardiac muscle also involved
F. Delayed cognitive development
G. Elevated CPK and SGOT/AST
H. Later in disease; scoliosis, respiratory difficulty, and cardiac difficulties
I. Eventual wheelchair dependency, confinement to bed
MUSCULAR DYSTROPHY
Nursing Diagnoses:
A. Impaired physical mobility R/T
B. Chronic low self-esteem R/T
MUSCULAR DYSTROPHY
Nursing Interventions:
A. Provide supportive care
B. Provide exercises (active and passive)
C. Prevent exposure to respiratory infection
D. Encourage a balanced diet to avoid obesity
E. Support family’s grieving process
F. Support participation in the Muscular Dystrophy Association
G. Coordinate with health care team: physical therapist, occupational therapist, nutritionist, neurologist, orthopedist, and geneticist
