Growth & development nurs 3340 fall 2017 update

Growth &
Development
Joyce Buck, PhD(c), MSN, RN-C, CNE
Joy A. Shepard, PhD, RN-C, CNE
1

Objectives
• Describe principles, patterns, and rates of growth and development
• Describe major developmental theories
• Plan for the nursing management of children with learning and
intellectual disabilities
• Describe the nursing role in prevention and treatment of child abuse
and neglect
• Summarize major nutritional concepts pertaining to the growth and
development of children
• Describe nursing interventions for children who are overweight or
obese
• Plan care for the child with an inherited biochemical abnormality
2

Nursing Diagnoses
• Delayed Growth & Development
• Readiness for Enhanced Parenting
• Readiness for Enhanced Family Processes
• Compromised Family Coping
• Dysfunctional Family Processes
• Caregiver Role Strain
• Risk for Impaired Parenting
• Risk for Injury
• Imbalanced Nutrition: More than body requirements
• Imbalanced Nutrition: Less than body requirements
• Sedentary Lifestyle 3

4
Principles of Growth & Development

Growth
• WEIGHT- most sensitive
• Birth weight: 2X by 6 mos
• 3x by 1 yr
• 4X by 2 yrs
• LENGTH/ HEIGHT
•  1.5 cm/ mo 1st 6 mos
•  1 cm/ mo. 7-12 mos
• Ave.  in ht. during 1st yr is 50%
• Approx. ½ of adult ht. at 2 yrs 5

Development
• DEVELOPMENT- in the skills or capacity to
function
• How to Measure:
• By simply observing a child doing specific
task
• By noting parent’s description of the child’s
progress
• By comparing child’s performance to
developmental milestones
• By a Developmental Screening Tool
• Developmental Screening tools include the
domains of:
• Cognition
• Fine & Gross Motor Skills
• Speech & Language
• Social-Emotional Development 6

Maturation
• MATURATION - synonymous with development, also known as READINESS
• COGNITIVE DEVELOPMENT- is the ability to learn (to change behavior) and
understand from experience, to acquire and retain knowledge, to respond to a
new situation and to solve problems
• Basis of Intellectual Disability
• IQ= mental age X 100
chronological age
• 0-20 profound intellectual disability (infant)
• 20-35 severe (0-2 yo)
• 35-50 moderate (2-7 yo) trainable
• 50-70 mild (7-12 yo) educable
• 70-90 borderline
• 90-110 normal (average IQ)
•  130 gifted
7

Basic Divisions of Life
• 1st Stage- Prenatal (from
conception to birth)
• 2nd Stage- Infancy
• Neonatal: 1st 28 days of life
• Infancy: Birth to 12 months
3rd Stage- Early Childhood
• Toddler: 1-3 yrs
• Preschool: 3-6 yrs
• 4th Stage- Middle
Childhood
• School age: 6-12 yrs
• 5th Stage- Late Childhood
• Adolescent: 12-18 yrs (or 21)
8

Principles of Growth &
Development
1. Growth and development is a continuous process (from
womb to tomb)
2. Not all parts of the body grow at the same time or at the
same rate (Principle of Asynchronism)
9
Watch Video “Growth and Development”

Patterns of Growth &
Development
• CV, MS, GI, & GU systems
- fairly smoothly during
childhood
• CNS- rapidly 1st 1-2 yrs
• Immune System- rapidly
during infancy & childhood
• Tonsils: adult proportion
by 5 yrs
• Reproductive System-
rapidly during puberty
10

Rates of Growth & Development
• Fetal and Infancy-
most rapid
• Toddler- slow
• Preschool-
alternating rapid and
slow
• School age- slower
• Adolescent- rapid 11

Each Child is Unique
• 2 Primary Factors
• Heredity (Non-modifiable) or
“Nature”
• Race
• Intelligence
• Sex
• Nationality
• Environment (Modifiable) or
“Nurture”
• Quality of Nutrition
• Socioeconomic status
• Health
• Parent-child relationship
12

Directional Trends
• Cephalocaudal: “ head to tail”. It occurs along body’s long
axis in which control over head, mouth and eye movements
and precedes control over upper body torso and legs.
• Proximodistal: from center of the body to extremities (e.g.
baby uses whole arm in crawling then hand pincers)
• Symmetrical: each side of the body develops in the same
direction at same time and rate
• Mass-Specific (Differentiation): the child learns from simple
operations before complex functions or move from a broad
general pattern of behavior to a more refined pattern (e.g.,
crying precedes speech)
13

Gross Motor vs. Fine Motor Ability
• Motor skills – carried out when the brain, nervous system,
and muscles work together
• Gross motor – larger movement child makes with arms,
legs, feet, or entire body
• Examples: rolling over, sitting, crawling, running, and jumping
• Fine motor – smaller, more skilled movements
• Small muscles of fingers, toes, lips & tongue
• Examples: picking things up between finger and thumb,
wriggling toes in the sand, using lips and tongue to taste and
feel objects
• Gross motor skills precede fine motor skills
15

Infant Primitive Reflexes
• Infant primitive reflexes must be lost first before
development can proceed (e.g., spitting/extrusion reflex must be
overcome before infant can be fed with solid foods; tonic neck reflex
must be diminished before the infant can turn over)
• REFLEXES- involuntary reactions to specific forms of
stimulation
• Importance:
• Survival (e.g., feeding reflexes: rooting, sucking, swallowing;
protective reflexes: blink, gag, cough, Moro)
• Reflects how well CNS is functioning
• Forms the basis for later, more sophisticated behavior
16

Sequential & Secular Trends
• SEQUENTIAL TRENDS- involves a predictable sequence
of growth and development to which the child normally
passes
a) Locomotion
b) Language and social skills
• SECULAR TRENDS-refers to the worldwide trend of
maturing earlier and growing larger as compared to
previous generations
17

Behavior & Play
• BEHAVIOR- most
comprehensive
indicator of
developmental status
• PLAY- universal
language of the child
• A great deal of skill and
behavior is learned by
PRACTICE
18
• Play is a reflection of every
aspect of development and a
method for enhancing
learning and maturation

Play in Infancy
• Solitary play
• Play by themselves
• Limited interaction
• Egs: grasping a rattle; shaking
and banging things;
manipulation of blocks
• Every new object or
situation that is introduced
is a new learning
experience for the infant 19

Play in Toddlerhood
• Parallel play
• Play with similar objects side
by side, with little
communication or interaction
• Absorbed in their own
activity
• Both gross motor and fine
motor abilities enhanced
20

Preschooler Play
•Associative play
• Similar activity but no rigid
organization
• Motor activity!
• On the go and moving
• Dramatic play
• Dress up, imitation
• Games
• Rules are absolute and
rigid in games 21

School Age Play
• Cooperative Play
• Teams, organized clubs
• Rules
• Likes competition
• Construction
• Enjoys building and constructing things
• Computer games
• Limit amount of TV or “screen” time
22

Anticipatory Guidance: Promote Use
of Protective Gear
24

Theories of Development
• Developmental Task:
• Skill or growth responsibility
arising at a particular time in the
individual’s life
• Successful achievement provides
a foundation for the
accomplishments of the future
tasks
• Freud: Psychosexual Theory
• Erikson: Psychosocial Theory
• Piaget: Cognitive Theory
• Kohlberg: Theory of Moral
Development 26

Psychosexual Theory/
Psychoanalytic Theory
• Sigmund Freud (1856-1939): Austrian neurologist, founder of
psychoanalysis
• 3 parts of personality:
• 1) Id: basic sexual energy
present at birth
2) Ego: realistic part of person;
develops around 4-5 months
3) Superego: moral/ ethical system; develops
at 3-6 years
27

Psychosexual Theory Cont’d….
• 1. ORAL- (Birth-12 mos) Infant
• Mouth- site of gratification
• Activity: biting, sucking, crying (for enjoyment and release
of tension)
• Offer pacifier when NPO
28

• 2. ANAL-(1-3 yrs) Toddler
• Anus- site of gratification where elimination takes place
• Principles in Toilet Training:
• “Holding on”- child wins, becomes stubborn or antisocial
• “Letting go”- mother wins, child becomes obedient, kind,
perfectionist, obsessive-compulsive
29

• 3. PHALLIC-(3-6 yrs) Preschool
• Genitalia- site of gratification
•  knowledge on 2 sexes , exhibitionism is normal
• Accept child fondling his/her genitalia as normal area of
exploration
• Answer child’s questions directly
• SUPEREGO is a necessary part of socialization; includes
internalization of values, ideas and moral standards of
parents and society; development of CONSCIENCE 30

Preschooler cont’d: Gender
Role Identity
• Becomes aware of their similarity to parent of the
same sex
• Usually like to be like the same sex parent
• Reinforced by the parent
• Girls—cooking with mom; Boys—working with dad in the
shop
• Not absolute—many identify with both parents 31

• 4. LATENT-(6-12 yrs) School Age
• Period of suppression, no obvious development
• Help child achieve positive experiences so that he/she will
become ready to face the conflicts of adolescence
• 5. GENITAL- ( 12yrs) Adolescent
• Focused on sexuality
• Developing sexual maturity; learning how to establish a
satisfactory relationship with opposite sex 32

Psychosocial Theory
• Erik Erikson (1902-1994) -
trained in psychoanalysis theory
• Focuses on psychosocial tasks
that are accomplished throughout
the life cycle
• Stresses the importance of culture
and society to the development of
one’s personality
• Unsuccessful resolution of
psychosocial crisis leaves the
individual emotionally
handicapped
33

Psychosocial Theory
• Crisis: particular challenge that exists for healthy
personality development to occur
• 8 Stages
34

Psychosocial Theory Cont’d….
• 1. Trust vs Mistrust (Birth to 1 year) *Feeding
Developed by:
• Satisfying needs at all times: feed upon demand
(because stomach capacity is low and baby easily gets
hungry)
• Giving an experience that will add to security (e.g. touch,
hugs and kisses, eye-to-eye contact, soft music)
• Parental caring must be consistent and adequate
36

• 2. Autonomy vs Shame and Doubt (1-3 yrs)
Toddler *Toilet Training
• If everything is planned or done for the child, autonomy is
not developed
• Developed by:
• Giving opportunity for decision-making, offering choices,
rather than judgement
• Setting limits is the parents’ moral obligation
37

• 3. Initiative vs Guilt (3-6 yrs) Preschool *Doing basic things
• Views play as work and takes it seriously; if failed the child feels
• Guilt: anger turned inward
Developed by:
• Giving opportunity to explore new places and events
• Provide activities that can enhance imagination, creativity and
fine motor skills e.g. modeling clay, finger painting
• If child fails, don’t say “It’s just a game”; instead encourage
child to accept defeat and to do his/her best next time
38

• 4. Industry vs Inferiority (6-12 yrs) School age *School
• Learns how to do things well
• Developed by:
• Giving opportunity on short assignments and projects
39

• 5. Identity vs Role Confusion (12-18 yrs) Adolescent
*Peers
• Learns who he/she is, what kind of person will he/she
become by adjusting to new body image
• Emancipation from parents: liberation/freedom
• Peers become more important
• Experiment with new roles
40

Cognitive Theory
• Jean Piaget (1896-
1980)- Swiss Psychologist
• Defines cognitive acts as
ways in which the mind
organizes & adapts to its
environment
• SCHEMA- individual’s
framework of thought
• 4 Stages of Cognitive
Development
41

Stages of Cognitive
Development: Sensorimotor
• Sensorimotor (Birth-2 yrs): “Practical Intelligence,” words
and symbols not yet available
43
A.
SCHEMA AGE BEHAVIOR
Neonatal Reflex/ Stimulus
Response
1 mo All reflexes
Primary Circular Reaction 1-4 mos Activities r/t body, discover persons, no object
permanence, repetition of behavior
Secondary Circular Reaction 4-8 mos Activities not r/t body, object permanence, memory
traces present, anticipate familiar events
Coordination of Secondary
Circular Reaction
8-12 mos Exhibit goal-directed activities,  sense of permanence
Tertiary Circular Reaction 12-18 mos Use trial and error in discovering places and events,
space and time perception
Invention of new means thru
mental combination
18-24 mos Invent new means by active experimentation,
Transitional phase to Preoperational thought period

Stages of Cognitive Development:
Preoperational Thought
• Preoperational Thought (2-7 yrs)
44
SCHEMA AGE BEHAVIOR
Pre-conceptual 2-4 yrs Thinking is literal and static
Concept of time: Now
Concept of distance: what can be seen
Symbolic Play
Irreversibility of thinking
Transductive reasoning
Magical thinking
Intuitive 4-7 yrs Beginning of causation
Egocentric in play, thought and behavior
Unidimensional classification (texture,
color, length one at a time)
Animism-inanimate objects have life

Concrete Operational Thought
• Concrete Operational Thought (7-11 yrs)
• BEHAVIOR
• Find solution to everyday problems with
systematic reasoning
• Concept of REVERSIBILITY
• Concept of CONSERVATION
• Cooperative Interaction- relates own point of view
with others
• Activity: Collecting
• Multidimensional classification 45

School Age Cognitive Development
• Concrete Thinking
• Conservation of matter
• Able to have mental representations
• Concept of time and body parts
• Likes to learn
• Interested in learning new skills and information
• Interested in friends and school
• Clubs and sports
46

Formal Operational Thought
• Formal Operational Thought (11 yrs)
• BEHAVIOR
• Cognition achieved its final form
• Solve hypothetical problems with scientific reasoning
• ABSTRACT THINKING and mature thought
• Concept of time: past, present and future
• Sort out opinions and current events
47

Formal Operational
Thought cont’d…
• Capacity for fully mature intellectual thought
• No longer depends on concrete experiences as basis of thought
• Capable of abstract thinking
• Likes independence and autonomy
• Establishing own identity and values
• Experimentation and risky behaviors
• Peer relationships are VERY important
• Keen awareness of body image (often comparing themselves
with peers)
• Idealistic: ripe for health teaching 48

Theory of Moral Development
• Lawrence Kohlberg
(1984) – recognized the
theory of moral
development as
considered to closely
approximate cognitive
stages of development
• Not all persons may reach
all stages; may be fixated
in one stage 49

Stages of Moral Development
STAGE DESCRIPTION
Pre-conventional (4 to 7 yrs) Punishment/ obedience orientation Child does right because parents
tell him/her to and to avoid punishment. Carries out action to satisfy
own needs rather than society. Interest shifts to rewards – effort is
made to secure greatest benefit for oneself. Will do something for
another if that person do something for the child (“Do for me and I do
for you”)
Conventional (7 to 11 yrs) Orientation to interpersonal relations of mutuality. Child follows rules
because of a need to be a “good” person in own eyes and the eyes of
others (“Good boy, Nice girl social concept”). Conscience becomes
important for maintenance of social order and authority. Orientation
towards fixed rules. Child finds following rules satisfying. Follows
rules of authority figures as well as parents in an effort to keep the
“system” working (“Law and Order Orientation”)
Post Conventional (12 yrs and
Older)
Social contract, utilitarian law- making perspectives. Follows
standards of society for the GOOD OF ALL people
Universal ethical principle orientation. Follows
INTERNALIZED STANDARDS of conduct
50

52
Learning & Intellectual Disabilities

Learning & Intellectual
Disabilities (pp. 813-818 [new], 884-888 [old])
• Learning disability: normal intelligence, but has difficulty
learning in a typical manner
• Brain cannot receive or process information in the normal
manner
• 5-10% school-age children
• Affects many parts of a child's life: school, daily routines,
family life, and sometimes even friendships and play
• Common types: dyslexia, dyscalculia, dysgraphia, dyspraxia
53

Learning & Intellectual
Disabilities Cont’d…
• Intellectual disability (NOT “mental retardation”)
• Limitation in intellectual functioning, adaptive behavior
• Differences in conceptual, social, and adaptive skills, before
age 18 yrs
• IQ below 70 to 75
• Adaptive functioning deficits in at least two areas (e.g.,
communication, self-care, social/ interpersonal skills,
academic skills, or work)
• Etiology: prenatal development of CNS, postnatal changes or
external forces leading to CNS damage 54

Down Syndrome (p. 814 [new], 885 [old])
• Trisomy 21: Chromosomal alteration
• Extra chromosome (47 instead of 46)
• 3 chromosomes 21
• Most common chromosomal condition in US
• 5,500 infants each year; 1 in 800 infants
55

Down Syndrome, cont’d
• Syndrome: umbrella term with characteristic features
and anomalies of several organs
• Hypotonia, single palmar crease, almond-shaped eyes that slant
up, protruding tongue, short neck, low-set ears, wide, short neck,
epicanthal eye folds
• IQ in mildly-to-moderately low range; developmental delays (e.g.,
slower to speak than other children)
• At risk for: congenital heart defects, hearing loss, otitis media,
obstructive sleep apnea, strabismus, Hirschsprung disease,
thyroid disease, leukemia, Alzheimer’s disease, congenital
cataracts, diabetes 56

Transverse Crease on Palmar Surface
57

Nursing Management for
Children with Disability
• Developmental approach rather than a
chronological one
• Assess the child for developmental milestones
• Assess the child for adaptive functioning (ability to
effectively interact with society on all levels and care for
one's self)
• Assess functioning and coping of ALL family members
• Facilitate early intervention 59

Nursing Management for
Children with Disability, cont’d…
• Partner with the family and multidisciplinary team
• Open communication
• Parents are an ESSENTIAL part of “the team”
• Observe how the family is managing (parents are often
stretched to the limit)
• Collaboration is a MUST – need well-coordinated, seamless
plan of care
• Goal: accessible, continuous, comprehensive,
family-centered, coordinated, compassionate,
and culturally effective care 60

Nurse’s Role with Families of
Children with Disabilities
• Assessment
• Strengths and focus on abilities (not disabilities)
• Support
• Accept reactions and encourage expression of feelings
• Identification of resources
• Discuss realistic Goal Setting
• “Child First” language
• A child with Down syndrome, NOT a Down syndrome
child
61

Child Maltreatment
(pp. 388-394; 930 [new], 426-432; 1007 [old])
• Physical, emotional, or sexual
• Abuse or neglect
• “Non-accidental trauma”
• Nurses are legally required to report
• Suspicions, signs of abuse
• Children as victims
• Abandonment, hazing, bullying,
domestic violence, dating violence,
Munchausen's Syndrome by Proxy
63

Table 17-8 Methods of Physical
Abuse in Children (p. 389 [new], 427 [old])
64

Differentiate cultural practices from child abuse
65

Munchausen Syndrome by
Proxy (pp. 393 [new], 432 [old])
• Fabrication of signs and symptoms of a health condition in a child;
potentially deadly form of child abuse
• Occurs most often in parent-child relationships; mothers most often
perpetrator; victim usually under 6 yrs
• Unexplained, recurrent, or extremely rare conditions; illness unresponsive
to treatment; symptoms change frequently; history and clinical findings
inconsistent; symptoms occur in presence of same caretaker, disappear
when child is separated from caretaker
• Diagnosis: difficult because perpetrator is expert at faking illness; must
carefully review health records, talk with friends or family
• Mandatory reporting to Child Protective Services & law enforcement; child
placed in safe environment 68

Munchausen Syndrome by
Proxy
• Most commonly reported signs & symptoms:
• CNS dysfunction
• Apnea
• Vomiting
• Diarrhea
• Seizures
• Fever
• Signs of bleeding (urine or stool)
• Rashes 69

Psychological Maltreatment
• Not all abuse is physical!
• Psychological maltreatment
• Occurs when parents or other
caretakers harm children’s behavioral,
cognitive, emotional, or physical
functioning
• May take form of neglect in which
parents may ignore or act emotionally
unresponsive
• Not as easily identified without outward
physical signs
70

What are Consequences of
Psychological Maltreatment?
• Some children survive and grow into
psychologically healthy adults
• Others suffer long-term damage
• Low self-esteem, depression, suicide
• Lying
• Misbehavior
• Underachievement in school
• Criminal behavior
71

Range of Abuse and Maltreatment of
Children in the US
72

Risk Factors Common in Families with
Child Victims of Violence
• Poverty
• History of mental illness, domestic violence, incarceration, or
substance abuse in the home
• Family stresses (e.g., single-parent households, high levels of
marital discord)
• Inadequate child care or supervision
• Inadequate family social support
• Gang membership in family or neighborhood
• High exposure to media violence
• Children with chronic illnesses and special health care needs
• Child hyperactivity or other developmental behavior disorders
73

True or False?
•Child abuse can occur in any home
or child care setting
74

Nursing Interventions & Goals
• Assess the family for ineffective coping and the potential for
abuse
• Make appropriate referrals to support services such as
mental health counseling, social services, and respite
• Work with family to:
• Understand present and future risks
• Change social and physical environment
• To achieve goals:
• Educate families, connecting them with resources
• Plan measurable behavior changes 76

Triple P-Positive Parenting
• On-line parenting program
• 8 sessions with video clips, worksheets & activities
• 30-60 minutes each
• Podcasts, emails & text reminders
• Evidence-based
• Outcomes: happy children w/ better behavior, less arguing,
confident parents
77

Suspect...
Dramatic changes or shifts in behavior
without logical explanation warrant inquiry
78

Infant Feeding: Breast is Best!
82

Unsuitable Milk for Babies Under 1 Year….
84

Childhood Obesity Epidemic
(pp. 293-295 [new], 325-326; 328-329 [old])
• 33% of children in the
US are overweight or
obese
• Associated with a wide
array of health
problems, as well as
depression & low self-
esteem
89

Childhood Obesity Epidemic,
cont’d
•Helps bring on during childhood many of the
chronic diseases of middle age and old age:
• Type II Diabetes, CVA, cardiovascular disease,
hypertension, dyslipidemia, gallbladder disease,
osteoarthritis, respiratory problems
(e.g. obstructive sleep apnea)
90

Evaluation Overweight Child
92

Early Intervention: Healthy Food
Choices, Active Lifestyle
• Early diagnosis with
interdisciplinary monitoring
and appropriate interventions
along with healthy food
choices beginning in
childhood and an active
lifestyle are the keys to better
health and a longer life
• See Nursing Care Plan:
The Child Who is
Overweight
• pp.294-295(new),328-329(old)
94

95
Avoidant/ Restrictive Food Intake Disorder
(FeedingDisorderofInfancyandEarlyChildhoodor FailuretoThrive)

• Inadequate growth resulting from
inability to obtain or use calories
required for growth
• Sign of undernourishment
• Slow growth/ inadequate weight gain
per standards for children ≤ 6 yrs
• Weight < 5th percentile; <10% ideal BMI
• Syndrome: not a single disease or
medical condition
• Multidimensional problem that requires
multidisciplinary approach
• Early intervention essential
Avoidant/ Restrictive Food Intake Disorder
(Formerly Feeding Disorder of Infancy and Early Childhood or
Failure to Thrive, p. 299 [new], 332 [old])
Tracking the growth rate
96

• Organic
• Physical cause identified:
heart defect, GER, renal
insufficiency, malabsorption,
endocrine disease, cystic
fibrosis, AIDS
• Non-organic
• Inadequate intake of calories
• Disturbed mother-infant
bonding
• No associated medical
condition
• Mixed
• Combination of organic &
nonorganic causes
• Complications:
• Poor intellectual, language, &
reading skills
• Social immaturity
• Behavioral disturbances
• Assessment
• Low growth for age
• Developmental delays
• Apathy
Food Intake Disorder:
Many Possible Etiologies
97

Collaborative Care
• Multidisciplinary team approach: provide adequate caloric &
nutritional intake; promote normal G & D; assist parents
• Thorough history & physical
• Accurate daily weights, I & O
• Monitoring of height / weight / HC
• Observation of infant / caretaker interaction
• Calorie count to determine actual calories consumed
• Meals & snacks: pleasant, regularly scheduled (e.g., every 3
hrs), not rushed, established routine, distractions minimized
• Grazing in between meals/ snack times should be eliminated
• Referral: Community resources
99

•Complete a comprehensive health history
•Perform a physical examination
•Educate regarding nutrition, feeding
techniques, feeding cues
•Offer support for caregivers and families
•Report abuse or neglect
Nursing Care
100

102
Inborn Errors of Metabolism

Inborn Errors of Metabolism
(pp. 893-896 [new], 969-972 [old])
• Large class of genetic diseases involving disorders of
metabolism
• Body cannot properly turn food into energy
• Caused by defects in specific proteins (enzymes) that help
break down (metabolize) parts of food
• Manifestations usually occur within days or weeks of birth
• Untreated: irreversible organ damage, life-threatening
problems, coma, death
103

Guthrie Test: Metabolic
Screening
104

Phenyketonuria (p. 893 [new[, 970 [old])
• Autosomal recessive inheritance pattern
• Missing an enzyme (phenylalanine hydroxylase), which is
needed to properly break down phenylalanine
• Untreated: irreversible brain damage, severe intellectual
disability
• Clinical manifestations: lighter skin, hair, and eyes; “mousy” or
“musty” odor; irritability; vomiting; hyperactivity, hypertonia, and
hyperreflexia; seizures and jerking motions; skin rashes 105

Phenyketonuria, cont’d
• Treatment: special formula (Lofenalac); low
phenylalanine-diet (avoid high protein foods, such
as milk, dairy products, meat, fish, chicken, eggs,
beans, and nuts; aspartame)
• Nursing: support & educate parents; confer with
nutritionist
• All states require PKU newborn screening
106

This target is an easy way to visualize the
foods allowed on the diet for PKU
107

PKU Diet
• Phenylalanine-free medical formula (Phenyl-Free)*
• Contains protein, vitamins, minerals & energy with no
phenylalanine
• Fruits, vegetables, bread, pasta, & cereals (carefully
measured amounts)
• Special low-protein breads & pastas
• * Registered trademark of Mead Johnson Co.
108

Galactosemia (pp. 894 [new], 970-971 [old])
• Unable to use (metabolize) the simple sugar
galactose
• Untreated: irreversible damage to eyes, liver,
kidney, and brain
• Clinical manifestations: poor feeding, poor weight
gain, jaundice, vomiting, irritability, lethargy,
seizures, coma, death 109

Galactosemia cont’d
• Treatment: lactose-free formula (e.g., soy formula,
Nutramigen), galactose-free diet
• Avoid all milk, breastmilk, milk-containing products
(including dry milk), and other foods that contain
galactose for life
• Nursing management: support & educate parents;
confer with nutritionist
• All states require galactosemia newborn screening 110

Maple Syrup Urine Disease (MSUD)
(pp. 895-896 [new], 971-972 [old])
• Cannot break down the amino acids leucine, isoleucine, &
valine
• Untreated: cerebral edema, progressive neurologic
impairment, death
• Clinical manifestations: poor appetite, feeding difficulties,
lethargy, vomiting, urine that smells like maple syrup,
ketoacidosis, seizures, coma, death
112

MSUD
• Treatment: specially-designed formulas, low-protein foods,
daily urine testing for ketones; removal of amino acids &
their metabolites from the tissues & body fluids, may require
dialysis; liver transplant
• Nursing management: support & educate parents; confer
with nutritionist
• Most states require MUSD newborn screening
113

Growth &amp; development nurs 3340 fall 2017 update

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