By
Sarah Kamper
PSY 1100
1606: Dr. Iuan
Alonfo makes
references to CF in
his literature.
1952: Jocelyn Reed
introduces
physiotherapy. Paul
di Saint’ Agnese
develops a Sweat
Test.
1905: Karl
Landsteiner first
describes Meconium
ileus.
19th Century: Karl
von Rokitansky
reports a fetal death
due to Meconium
peritonitis.
1957: Dr. William
Wallace develops a
prophylactic
treatment program.
Vital for survival.
1938: Dorothy
Hansine Anderson
first describes
characteristics of CF.
Uses pancreatic
enzymes
replacement
therapy.
1989: Lap-Chee
Tsui discovers CF
gene and CFTR
protein.
1976: Professor
Neils Hoiby
discovers crossinfection between
CF patients.
2012: FDA approval
of VX 770.
1990s: The discovery
that the CFTR defect
can be corrected.
Gene therapy begins
development.
Medications
•Nebulizers
•Enzymes
•Antibiotics
Physiotherapy
and exercise
•CPT with vest
•Expelling sputum
•Cardio
• Clean outs
• Cultures
Dr. Visits &
Hospitalizations
• Pulmonary tests
• Blood oxygen level tests
Nutrition
•High calorie/fat
intake
•2900-4500 p/day
•Supplements
Nature
Input
•Genetics
•Recessive
gene
•Inherited
depression
Input
• Nature
• Development
• Mother provides
nutrition
directly to
bloodstream via
umbilical cord.
Nature
Output
•Development
•Mother’s
nutrition
provides
healthy
growth.
Nature
Input
• Development
• Organs are
predetermined
to form with
defective CFTR
proteins.
Nature
Input
Input
•Physical
•Mucus inhibits
enzyme
secretions.
•pH imbalance in
stomach.
• Nurture
• Physical
• Parents provide
manual physiotherapy
and nebulizer
treatments.
• Parents encourage
consumption of
medicine and solid
foods.
• Parental distress,
depression, anxiety
Output
Output
• Physical
• Lower than norm in
weight and height.
• Increased vulnerability
to C. diff.
• Psychological
• Physical stress triggers
mental stress.
• Forced 2-3 hour
treatments lead to
confusion, stress, and
exhaustion.
• Children learn to fear the
nebulizer and percussion
cups, causing anxiety.
• Infants struggle to
swallow enzymes
necessary for digestion,
causing stress.
•Impacts attachment
Trust vs Mistrust
•Parental care
Input
•Special needs
require specific
care
Output
•Parental
dependency
Autonomy vs
Shame & Doubt
Input
• Parental guidance
• Promotes autonomy.
• Parental dependency
• Administering
enzymes & medication
Output
• Trust
• Trust is developed with
parents when proper
care is given.
• Mistrust
• Can be developed with
doctors and nurses
• Can be developed with
aunts, uncles,
grandparents, and
daycare centers.
• Autonomy
• Children become self
sufficient in many
activities.
• Shame and Doubt
• Dependence on parents
for enzymes before
eating/drinking places
some doubt in their
ability to be self
sufficient for meals.
Family Time
Family Time
Emotional Development
• Proximal parenting
for CF child
• Distal parenting for
other siblings
• Internalizing or
externalizing stress,
anxiety, depression.
Emotional
Development
• ½ children had
sleep and eating
disorders
• 40% lacked ACT
compliance
• Mothers spend
more quality
time with CF
child.
• Stress on
sibling
relationships
form.
Initiative vs Guilt
• Parental limitations
• Limit child’s
activities.
• Limit purchases of
items (pets, plants)
• Limit childcare
options
• Doctor limitations
• Limits use of public
facilities
Inputs
Outputs
Initiative
• Child develops a sense of
independence with
essential activities.
• Guilt
• Child learns limitations
that can lead to guilt if the
limitations are disobeyed.
• Can effect ability to cope
with stress management.
Brain Development
• Concrete Operational Thought
established
• By age 9, children understand
death
• Can take ownership of chronic
illness.
Emotional
Development
Industry vs
Inferiority
• Physical limitations
• Self-reliance
• Fear is of a
premature
death.
• Psychological
• Anxiety
manifests
Industry
Inputs
Physical Limitations
• Smaller stature
• Decreased lung function
Outputs
Physical
Limitations
• Effects play
• Loss of esteem
• Increases stress
• Prejudice
• Effects education
• Struggle to comply with
treatments adds to stress
on family relationships.
• Struggles to comply with
school work
• Inferiority
• Decreased lung functions
limit play with peers;
limits practice of
emotional regulation,
empathy, & social
understanding.
• Increased calorie intake
increases output,
different than peers
• Increased food intake
yields small results in
growth, below norms
Identity vs Role
Confusion
Peer and Adult
Relationships
• Social norms not met
• Chronic illness
limitations
Identity
• Identity is postponed & difficult to achieve
identity is predetermined by peers and
teachers “CF kid.”
• Adolescent will keep illness status a secret.
• Causes anxiety and stress
• Role Confusion
• Role diffusion typically occurs due to
known premature death.
• Causes depression.
•Teasing and cruel
behavior from peers.
•Special treatment or
overprotection from
teachers.
Physical
Development
• Poor nutrient
absorption
• Poor blood &
oxygen
circulation
Inputs
Family Closeness
• Parents
• Communication, support,
connectedness,& control are evident.
• Siblings
• Sibling rivalry decreases communication
• Sibling support diminishes with
increased medical needs.
• Connectedness is influenced by sibling
rivalry.
Physical
Development
• Thin, small,
discolored
teeth, and
clubbing of
fingertips.
• Postponed
puberty
Peer & Adult
Relationships
Outputs
Family Closeness
• Adolescent gains some
autonomy, but continues to
rely on parents.
• Parents stress medical
adherence
• Poor sibling relationships put
stress on family relations.
• Negative peer
reactions and adult
over protection
increases stress and
anxiety.
• Subjected to being
an outcast by
cliques
• Cannot indulge
in drug
experimentation
• 53% have eating
disorders
Emotional Health
• 46% showcase anxiety
and depression.
• Stress triggered by
increased
responsibilities.
Identity
• Illness and cost
of medical
needs trump
higher
education
• Vocational
identity can be
developed
Emotional Health
• 44% admit guilt for not
adhering to medical
treatments
• 32% admit to rebelling
once out of the house
• Young Adults
dependent on parents
lack independence.
Identity
• Education
• 36% HS>
• 8% HS/GED
• 8% Some
college
• 32% BA/BS
• 16% MA/MBA
• Work/School
• 12% Not
attending
• 36% Attending
Intimacy
Inputs
Intimacy vs Isolation
• Treatments and
medications increase
embarrassment and
loneliness.
• SES
• Health
Social Demands
• Peer pressure and
balancing the need
to do treatments
while keeping up
with social
demands.
• 4% has a
partner
• Isolation
• 36% admit to
hiding or
skipping
treatments &
not taking
enzymes
with friends.
Outputs
Social Demands
• Desiring a ‘normal’ life
• 64% admit treatment burden
reduces medical adherence.
• 60% admit treatments are
replaced with social events.
• 60% admits that work
demands trump treatments.
Physical Health
• Pulmonary function
varies; the mean FEV
69%, SD 18%
• Median symptoms
showing is 10
• Possible transplants
are necessary.
Identity vs. Role
Confusion
Physical Health
• Cough, shortness of breath,
lack of energy, & irritability.
• Lack of energy and
irritability cause the most
stress and depression.
• Transplant list increases
anxiety
• Sexual, religious,
political identity
achieved
• Some vocational
identity achieved
Integrity
Integrity vs
despair
• Health &
Premature
death
Inputs
Intimacy vs.
Isolation
Generativity vs
Stagnation
• Family vs no
family
• Health
limitations
• Lack of long
term
relationships
• Trust issues
• Anxiety
over
revealing CF
condition
• Infertility
• Difficult to
achieve
• Achieved with
support from
loved ones
• Despair
• Predominant
Identity
• 52% FT/PT
work status
• Lack of work
attributes to
depression &
stress
• Role
confusion
• Some
struggle with
religion
Outputs
Generativity
• Parenting provides distress or hope
• Achieved though public speaking
• Charity events
• Stagnation
• Depression occurs if isolation occurs
• Occurs from limitations from health
issues/dependency
Intimacy
• 8% married
• 16% divorced
• Isolation
• 72%
single/never
married
The psychosocial effects on those with CF
vary. Experiences of anxiety, depression, and
stress are evident throughout much their
lifespan, but the degree of their distress is
highly influenced by the severity of their
health condition and family/friend support.
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