Ch. 3, 19, 20, 5, 8, 11, 17 (Abnormal G&D) Flashcards
Growth & Development Pt. 2
congenital anomalies are
birth defects
- occur in 2-4% of all live-born children and are often classified as either deformations, disruptions, dysplasias, or malformations
- some are preventable, some are not
deformations
caused by extrinsic mechanical forces on normally developing tissue
- ie. uterine restraint causes club foot
disruptions
result from the breakdown of previously normal tissue
- ie. congenital amputations caused by amniotic bands
dysplasias
result from abnormal organization of cells into a particular tissue type
- ie. congenital anomalies of the teeth, hair, nails, or sweat glands may be manifestations of one of the more than 100 different ectodermal dysplasia syndromes
malformations
abnormal formations of organs or body parts resulting from an abnormal developmental process
- most occur before 12 weeks gestation
- ie. cleft lip (5 weeks gestation)
cleft lip is an example of
malformation
club foot is an example of
deformation
congenital amputation is an example of
disruption (think something is missing)
absent sweat glands is an example of
dysplasia
when can genetic screening occur?
- prenatal
- pre-conception (before pregnant): screening of selected populations for heterozogyous carriers
- post-conception (after pregnant)
- family medical history: screening of relatives of known carrier or affected individuals within a family, for purpose of reproductive decision making
- newborn
- any time a family asks
pediatric indications for genetic consultation
- family hx (of something metabolic, drug use)
- abnormal newborn screen (PKU)
- abnormal genetic test result
- progressive neurologic condition
- major congenital anomaly
- pattern of major or minor anomalies
- congenital or early-onset hearing/vision loss
- CI or autism
- abnormal sexual maturation or delayed puberty
- abnormally tall/short
- excessive bleeding or clotting
- parental requests
- if baby dies (SIDS), get testing done to determine reason for death
nurse’s role in caring for children and families with hereditary disorders
- apply/integrate genetic knowledge into nursing assessment
- ID and refer clients who may benefit from genetic information or services
- ID genetics resources and services to meet clients’ needs
- providing care and support before, during, and after providing genetic information and services
assessment clues of genetic disorders
- major or minor birth defects and dysmorphic features (think Downs)
- growth abnormalities (not growing/delayed)
- skeletal abnormalities (club foot)
- visual or hearing problems
- metabolic disorders (PKU test)
- sexual development abnormalities (ambiguous genitalia)
- skin disorders
- recurrent infection or immunodeficiency
- development and speech delays or loss of milestones
- cognitive delays
- behavioral disorders
major or minor birth defects and dysmorphic features may indicate
genetic disorder
visual or hearing problems may indicate
genetic disorder
metabolic disorders may indicate
genetic disorder
sexual developmental abnormalities may indicate
genetic disorder
skin disorders may indicate
genetic disorder
recurrent infection or immunodeficiency may indicate
genetic disorder
developmental or speech delays or loss of milestones may indicate
genetic disorder
cognitive delays may indicate
genetic disorder
behavioral disorders may indicate
genetic disorder
skeletal abnormalities may indicate
genetic disorder
growth abnormalities may indicate
genetic disorder
adaptive tasks for parents with a chronically ill child begins with
acceptance of child’s diagnoses
adaptive tasks for parents with a chronically ill child ends with
establishment of a support program
adaptive tasks for parents with a chronically ill child
- accept diagnosis
- establish support system
- family-centered care- make the family involved every step of the way through the child’s care
times of anticipated stress begins with
- time of child’s diagnosis (extent of it, what it means for the child’s future)
time periods of anticipated stress in parents
- dx and development of milestones (ability to meet or being delayed in meeting milestones)
- start of school (social acceptance by peers, how will the child in a wheelchair get to school)
- adolescence (child may not be allowed to be independent because of their disease impeding their level of functioning, puberty, social acceptance)
- parental realization (that child will not be able to live up the potential the parent originally thought due to disease; and future placement decisions)
times of anticipated stress ends with
death of child
ways to promote sibling relationships/help siblings cope
- get siblings involved (but don’t give them too many responsibilities- that will make them resentful)
- don’t expect the siblings to be involved
- making time for the children without illness
- recognizing when the child needs a break
- allow siblings to have battles and let them work it out on their own
- allow healthy sibling to learn how similar and different they are
- get resources involved: support groups, books
involving siblings with the care of their sibling with chronic illness
- get siblings involved (but don’t give them too many responsibilities- that will make them resentful)
- don’t expect the siblings to be involved
normalization
efforts of family members make to create a normal family life, their perceptions of the consequences of these efforts, and the meanings they attribute to their management efforts
- attempt to make life as normal as possible
- culture norm: ie. of town
- social norm: what society, other kids norm
age appropriateness
having the disabled child spend time with the age group that they are in
- when a disabled child is surrounded by their age group, they do better in life
what is the difference between normalization and age appropriateness?
normalization: family makes life as normal as possible for disabled child
age-appropriateness: theory of keeping the disabled child engaged with other children their age
approach behaviors
result in movement toward adjustment and resolution of the crisis
- accepting dx
- giving care
- utilizing resources: communicating with HC team about what they can do
avoidance behaviors
result in movement away from adjustment and represent maladaptation to the crisis
- denying its happening
- unrealistic expectations
- upset or abrupt or doesn’t want to talk to the HC team
coping behaviors
behaviors that are aimed at reducing the tension caused by a crisis (ie ill dx of child)
- include avoidance and approach behaviors
family centered care
philosophy that considers the family as the constant in the child’s life
- nurses must have effective communication and trust with parents to meet the needs of the parents and child
is it realistic to expect a chronically ill child to participate in their own care?
yes- we allow them to do as much as they can
- promotes sense of self-worth
- promotes independence
examples of dysmorphic syndromes
- down syndrome
- fragile x syndrome
early signs of cognitive impairment
- gross motor delay
- fine motor delay
- behavior difficulties
- language difficulties or delay
- major organ system dysfunction (ie feeding or breathing difficulties)
- irritability or non-responsiveness to their environment
- dysmorphic syndromes
global developmental delay
term used to describe a child who has delays in two or more areas of development and functioning
- motor
- language
- social-emotional
- self-care
- behavior
developmental delay
- descriptive term
- not a dx
- rarely an accurate designation beyond 3 years old
demonstration vs explanation for a child with CI
demonstration
- showing will usually be more effective than explanation
- normally visual learners
nursing care for a child with CI
- demonstration
- one-step directions (no medical jargon)
- positive reinforcement
- repetition (demonstration)
- non-verbal communication (sign language)
- set limits (discipline)
- social behaviors (hello, good-bye, please, thank you)
- mainstreaming peers (think age-appropriateness)
- sexuality (adolescence)
- promote interactive play vs self-stimulatory
- independence w/ ADLs (as much as they can do)
non-verbal communication skills to use with a child with CI
- sign language
- picture board
- computer technology
social behaviors to use with a child with CI
- say hi, bye
- thank you
explaining sexuality to an adolescent with CI
- simple explanations (ie menstruation)
- self-care/hygiene
- physical development changes
- conception
- supervision
clinical manifestations of down’s syndrome
- simian palm crease (1 big crease across their hand)
- almond-shaped eyes that turn up
- space between big toe and second digit
- hypotonic: low tone, very floppy, wet noodle
- hyperreflexive joints
- dots on eyes
- flat nasal bone (resp. issues)
- flat face
- protrude their tongue (drooling)
can down’s syndrome be prevented?
**no it cannot be prevented- it is genetic
older mother is at a higher risk
- under 35 years: 1/350
- over 40 years: 1/100
family history more at risk (genetic condition)
how would a nurse care for a child and family with down’s syndrome?
- ongoing emotional support
- let the parent grieve after the initial dx
- give resources
- assisting with physical problems
- supporting prenatal and genetic counseling
what physical problems of a pedi with down’s syndrome do nurses help with?
- feedings: suction mouth after feeding
- respiratory health: aspiration, suctioned nose for secretions, saline in nose
- decreased tone: positioning while eating
autism
a neurological and developmental disorder that affects how people interact with others, communicate, learn, and behave
how can you decrease stimulation in a child’s hospital room?
- close the door/ wear earphones
- close the blinds
- dim the lights
- shut the tv off/turn volume down
- private room
- limit people going in and out of the room
atraumatic care to an autistic child and their family
- ask the parent: likes/dislikes, what i can/can’t do
- getting family involved in care (medication, sitting on parent lap)
children with communication or CI are at a higher risk for inadequate treatment of ____
pain
- hard to measure pain because they can’t really “tell” you
non-pharmacologic pain interventions: infants
- hold
- rock
- feed sugar water
- feed them breastmilk/formula
- sing/play music
- kangaroo care (skin-to-skin)
non-pharmacologic pain interventions: young children
- distraction (call child life: blow bubbles, video games)
non-pharmacologic pain interventions: teenagers
diversion
- play music
- watch a tv show
- guided imagery (ie. think of a peaceful place/vacation you went on; can begin at school-age)
pain management without drugs with pedi patients
- describe sensation they will experience
- use pinching, pulling, pushing, heat
- avoid over-explanation/lies
- stay with child
- praise, positive reinforcement
- bubbles, imagery, movies, music, thought stopping, etc.
- consult child life specialist (if avail)
medication administration: which preparations are preferred?
- liquid/suspension
(*only use oral syringes)
what is the key to pediatric medication administration?
positioning- therapeutic hold PRN
pedi medication administration tips
*only use oral syringes
- no nostril pinching unless by child
- put in the cheek
- not in their bottle- may not finish
- flavoring if possible
- suppository (rectal route) as an alternative
- IVP (IV push) can be used in children
if a child is in pain and requires pharmacologic treatment, use the correct _____ for oral ingestion.
- syringe
what should you not mix oral medication with for pediatric patients?
favorite drink or any beverage
if the child has a CI and needs oral medication, the nurse should
empower the parent to help
- increases medication compliance in child
- gives nurse an opportunity to evaluate parent technique
what does a child’s pain rating need to be in order to receive drug treatment?
moderate rating
can PCA (patient controlled analgesic) pumps be used in pediatrics?
(patient controlled analgesic)
yes as long as child is …
- child has to be old enough to understand number sense and cause and effect
- 7-8 year old
how do you manage opioid induced respiratory depression in children?
- evaluate sedation level
- full set of VS
- taper off
- O2
- support respirations
- monitor
- naloxone
how can you prevent opioid induced respiratory depression in children?
- monitor VS
- taper off
- O2 should be in the room
- hooked up to a monitor
acute failure to thrive
- weight <5th percentile *if child started at 50%, then 25%, then 5%; height WNL
pearl
a consistent nurse is important in developing a trust with infants who have failed to thrive
acute failure to thrive is also called
growth failure
acute failure to thrive is classified (diagnosed) according to ____
pathophysiology
- inadequate caloric intake
- inadequate absorption (ie CF, celiac disease, lactose intolerant)
- defective utilization- genetic/metabolic (ie trisomy 21)
- metabolic: hyperthyroidism
why does inadequate caloric intake occur in patients with FTT?
- dilution
- breastfeeding- but baby is not latching properly
- family can’t afford formula
clinical manifestations of acute FTT
- gaze, don’t blink
- sucking on fingers
- small
- thin
- head looks big in relation to body
- frontal bossing: protrusion of forehead
- cognition and developmental delays
- no stranger anxiety (should kick in at 9 months, but doesn’t)
- irritable, not smiley
- stiff or hypotonic
management of FTT is focused on ____
reversing the cause(s)
who is involved in the interdisciplinary approach to FTT of a pediatric?
- nutritionist
- lactation consultant
- nurse
- physician
- parent
what are the goals of treatment for FTT?
- determine cause
- increase the calories
- educate parents about FTT
cleft lip and palate can be detected when?
in utero (at 16 weeks) or at birth
what is cleft lip/palate a result of?
- environmental and genetic factors
- not taking Folic Acid
- certain medication (anti-seizure, __)
- drug and alcohol use
environmental factors of cleft lip/palate
- maternal alcohol ingestion, smoking, teratogens
- not taking FA
- certain medication (anti-seizure,
genetic factors of cleft lip/palate
- familial occurence
how can cleft lip/palate be treated?
surgical correction during infancy
how can cleft lip/palate be prevented?
yes it can be prevented
- mom takes FA
- avoids teratogens during pregnancy
nursing goals for a patient with cleft lip/palate
- parental acceptance
- feeding and growing
- pre/post-op care and education
- long-term family guidance
incidence/inheritance: cleft lip
- 1:600
- multifactorial causes
- M>F
incidence/inheritance: cleft palate
- 1:2500
- associated with syndromes
- genetic and environmental causes
- F>M
anatomy: cleft lip
- unilateral or bilateral
- may involve external nose, nasal cartilage, septum, maxillary alveolar ridges, and dentition
anatomy: cleft palate
- soft and/or hard palate
- midline of posterior plate
- may involve nostril and absence of septum
management: cleft lip
- surgical repair early infancy (2-3 months)
management: cleft palate
- surgical repair late infancy (6-12 months)
- if it involves lip, repairs may extend into early toddlerhood
short-term problems (pre-op): cleft lip
- feeding
- weight gain
short-term problems (pre-op): cleft palate
- feeding efficiency
- growth failure (FTT)
special post-op care: cleft lip
- suture line care and protection
- positioning: elbow restraints, on back
- special feeding techniques (slow/flow; syringe)
special post-op care: cleft palate
- feeding cup
- syringe feeds
- special needs feeder
- pigeon nipple
- avoid utensils/straws (no sucking)
- squeeze bottle for feeding
- can go on stomach when supervised
long-term concerns: cleft lip
- social acceptance
- dental and/or orthodontic
long-term concerns: cleft palate
- speech
- OM
- middle ear effusion (ear infections)
- hearing loss
- URI’s
- dental and/or orthodontic
- feeding
- social acceptance
infants at risk for SIDS:
- low birth weight
- low APGAR scores
- recent viral illness
- male
- native american or african american ethnicity
- maternal ETOH and smoking
- co-sleeping
- prone and side lying positioning
- bedding (soft, bumpers, toys, blankets)
- sibling of two or more victims of SIDS
- hx of central hypoventilation
what ethnicities are at a higher risk for SIDS?
- african american
- native american
are males or females at a higher risk for SIDS?
males
are males or females are a higher risk for cleft lip? cleft palate?
CL: M>F
CP: F>M
if an infants has one or more severe BRUE requiring CPR or vigorous stimulation, the infant is at a high risk for ____
SIDS
preterm infants experiencing apnea at time of discharge from the hospital are at risk for ___
SIDS
anticipatory guidance to parents to prevent SIDS
- appropriate bedding surfaces
- association of SIDS and maternal smoking
- dangers of co-sleeping
modeling behaviors
- placing infant supine to sleep
BRUE stands for
brief resolving unexplained events
BRUE definition
- unexplained respiratory pause lasting 20 seconds or more
OR - unexplained respiratory pause lasting less than 20 seconds accompanied by pallor, cyanosis, bradycardia, or hypotension (term infant)
causes of BRUE
- 50% are unidentified
- premature
- LBW
- upper respiratory illness
- reflux- spit up/vomit
- seizure
- cardiac defect
BRUE was formerly called
ALTE
- apparent life-threatening event
ALTE
- may be present with or without accompanying apnea
if the etiology of ALTE is unknown…
at home monitoring
ALTE management is
dependent on cause
testicular torsion
partial or complete venous occlusion with rotation of testicle
- very sudden
- not associated with a fever
testicular torsion prevalence/occurence
1:4000 males
peak onset of testicular torsion?
age 13
testicular torsion treatment
surgical emergency to prevent necrosis
s/sx of testicular torsion
- severe/acute pain
- red, swollen, hot scrotum
- no fever
- absent cremasteric reflex
- nausea
- vomiting
- abdominal pain
what can the nurse do if testicular torsion is suspected?
refer for immediate medical evaluation
- have 6 hours to remedy this: untwist testes/epididymis manually
nursing considerations for testicular torsion
this is an emergency- 6 hours to remedy this or will need surgery to prevent loss of testicle
- if testicle is lost, fertility is reduced by 50%
teach parents of children with communication or CI to look for: (to assess pain)
- pain with ADLs
- inactivity or a reduction in activity
- irritability/ personality/ emotional change
- uncooperativeness
- change in sleep pattern
- change in muscle tone (ex. rigidity)
- vocal sounds (ex. moaning, crying)
syndrome
a recognized pattern of anomalies resulting from a single specific cause
association
a nonrandom pattern of malformations for which a cause has not been determined
sequence
when a single anomaly leads to a cascade of additional anomalies, the pattern of defects is referred to as a sequence
abnormalities in autosomes usually account for syndromes of ___ deficiency
cognitive deficiencies
gene transfer
an experimental technique used to introduce a normally functioning gene into the cells of a patient with a genetic disorder
causes of congenital anomalies
- family history
- teratogens during pregnancy
- alcohol
ambiguous genitalia: definition
when the genitalia is swollen to the point that you don’t know what sex the baby is
- have to send them for genetic testing to determine the sex
labs to be done with CP/CL
- PT/PTT
- Urinalysis (UA)
- CBC
- CMP/Electrolytes
cheiloplasty
lip repair of CL
palatoplasty
palate repair of CP
if CL is repaired with sutures: keep site
- antibiotic ointment for 3 days
- vasaline
if CL is repaired with glue: keep site
stay dry
- when feeding, if milk gets near site, dab dry
pain control post-op CL repair
24-48hr ATC
- Tylenol 10-15mg/kg q4-6hr
why do we wait to repair CP after CL?
wait because they want palate to expand and lip to heal
