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