the lifespan of the psychosocial effects of cystic fibrosis
DESCRIPTION
The Lifespan of the psychosocial effects of Cystic Fibrosis. By Sarah Kamper PSY 1100. " Woe to that child which kissed on the forehead tastes salty. He is bewitched and will soon die .“ - 1606, Alonfo y de los Ruyzes de Fontecha. CFTR protein transport defect: . - PowerPoint PPT PresentationTRANSCRIPT
THE LIFESPAN OF THE PSYCHOSOCIAL EFFECTS OF
CYSTIC FIBROSISBy
Sarah KamperPSY 1100
"Woe to that child which kissed on the forehead tastes salty. He is bewitched and will soon die.“
- 1606, Alonfo y de los Ruyzes de Fontecha
CFTR protein transport defect:
Lack of Cl- and water transfer leads to…
1606: Dr. Iuan Alonfo makes
references to CF in his literature.
19th Century: Karl von
Rokitansky reports a fetal death due to Meconium peritonitis.
1905: Karl Landsteiner first
describes Meconium ileus.
1938: Dorothy Hansine Anderson
first describes characteristics of
CF. Uses pancreatic enzymes
replacement therapy.
1952: Jocelyn Reed introduces physiotherapy. Paul di Saint’
Agnese develops a Sweat Test.
1957: Dr. William Wallace develops
a prophylactic treatment
program. Vital for survival.
1976: Professor Neils Hoiby discovers
cross-infection between CF
patients.
1989: Lap-Chee Tsui discovers CF gene and CFTR
protein.
1990s: The discovery that the CFTR defect can
be corrected. Gene therapy
begins development.
2012: FDA approval of VX
770.
CF TreatmentMedication
s
• Nebulizers• Enzymes
• Antibiotics
Physiotherapy and exercise
• CPT with vest• Expelling sputum
• Cardio
Dr. Visits & Hospitalizati
ons
• Clean outs• Cultures
• Pulmonary tests• Blood oxygen level tests
Nutrition
• High calorie/fat intake
• 2900-4500 p/day
• Supplements
Psychosocial impacts on those with CF include…
Embryo-Birth
Nature• Genetics• Recessive
gene• Inherited
depression
Input
• Nature• Development
• Mother provides nutrition directly to bloodstream via umbilical cord.
Input
Nature• Development
• Organs are predetermined to form with defective CFTR proteins.
Input
Nature• Development• Mother’s nutrition provides healthy growth.
Output
First 2 Years
Nature• Physical• Mucus inhibits enzyme
secretions.• pH imbalance in
stomach.
Input• Physical
• Lower than norm in weight and height.
• Increased vulnerability to C. diff. • Psychological
• Physical stress triggers mental stress.
Output
• Nurture• Physical
• Parents provide manual physiotherapy and nebulizer treatments.
• Parents encourage consumption of medicine and solid foods.
• Parental distress, depression, anxiety
Input
• 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
Output
First 2 Years
Trust vs Mistrust• Parental care• Special needs require specific care
• Parental dependencyInput
• 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.
Output
Autonomy vs Shame & Doubt
• Parental guidance• Promotes autonomy.
• Parental dependency• Administering enzymes &
medication
Input• 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.
Output
Early Childhood: 2-6 years old.
Inputs
Emotional Development• Internalizing or
externalizing stress, anxiety, depression.
Family Time• Proximal parenting
for CF child• Distal parenting for
other siblings
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
Outputs
Emotional Development
• ½ children had sleep and eating disorders
• 40% lacked ACT compliance
Family Time• Mothers spend
more quality time with CF child.• Stress on
sibling relationships form.
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.
Middle Childhood: 7-11 years old.
Inputs
Brain Development• Concrete Operational Thought
established• By age 9, children
understand death• Can take ownership of
chronic illness.
Industry vs Inferiority
• Physical limitations
• Self-reliance
Physical Limitations• Smaller stature• Decreased lung
function
Outputs
Emotional Development• Fear is of a
premature death.
• Psychological
• Anxiety manifests Industry
• 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
Physical Limitations
• Effects play• Loss of
esteem• Increases
stress• Prejudice
• Effects education
Adolescents: 12-17 years old.
Inputs
Identity vs Role Confusion
• Social norms not met
• Chronic illness limitations
Peer and Adult
Relationships•Teasing and cruel behavior from peers.
•Special treatment or overprotection from teachers.
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
• Poor nutrient absorption
• Poor blood & oxygen circulation
Outputs
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.
Peer & Adult Relationships
• 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
Family Closeness• Adolescent gains some
autonomy, but continues to rely on parents.• Parents stress medical
adherence• Poor sibling relationships
put stress on family relations.
Physical Developmen
t• Thin, small,
discolored teeth, and clubbing of fingertips.
• Postponed puberty
Young Adult: 18-25 years old.
Inputs
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
Social Demands• Peer pressure
and balancing the need to do treatments while keeping up with social demands.
Intimacy vs Isolation
• Treatments and medications increase embarrassment and loneliness.
• SES• Health
Outputs
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
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.
Intimacy• 4% has a
partner• Isolation• 36% admit
to hiding or skipping treatments & not taking enzymes with friends.
Adults: 26-65 years old.
Inputs
Physical Health• Pulmonary function
varies; the mean FEV 69%, SD 18%
• Median symptoms showing is 10
• Possible transplants are necessary.
Identity vs. Role Confusion
• Sexual, religious, political identity achieved
• Some vocational identity achieved
Intimacy vs.
Isolation• Lack of
long term relationships
• Trust issues
• Anxiety over revealing CF condition
• Infertility
Integrity vs despair• Health &
Premature death
Generativity vs
Stagnation• Family vs no
family• Health
limitations
Outputs
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
Identity• 52% FT/PT
work status• Lack of
work attributes to depression & stress• Role
confusion• Some
struggle with religion
Intimacy• 8% married• 16% divorced
• Isolation• 72%
single/never married
Integrity • Difficult to
achieve• Achieved
with support from loved ones• Despair
• Predominant
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
Conclusion
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.
References1. Anderson, D., Flume, P., & Hardy, K. (2001). Psychological Functioning of Adults With Cystic Fibrosis. Chest, 119 (4),
1079-1084.
2. Basic Defect in Cystic Fibrosis Part 1 [Video file]. Retrieved from http://www.youtube.com/watch?v=jDzmgXsgmwM
3. Basic Defect in Cystic Fibrosis Part 2 [Video file]. Retrieved from http://www.youtube.com/watch?v=Ev3yKU93Vl0&feature=relmfu
4. Ernst, M., Johnson, M., & Stark, L. (2011). Developmental and psychosocial issues in CF. Pediatric Clinics of North America, 58 (4), 865-885.
5. Eva, 65 Red Roses-#31 [video file]. Retrieved from http://www.youtube.com/watch?v=0uwZf2Sm0KI
6. Julia’s Warriors 2010 [Video file]. Retrieved from http://www.youtube.com/watch?v=HQGObNzignA
7. George, M., Rand-Giovannetti, D., Eakin, M., Borrelli, B., Zettler, M., & Reikert, K. (2011). Perceptions of Barriers and Facilitators: Self-management Decisions by Older Adolescents and Adults with CF. Journal of Cystic Fibrosis, 9(6), 425-432.
8. Littlewood, James. (2004). Looking back over 40 years and what the future holds. 27th European Cystic Fibrosis Conference 2004. Retrieved from http://www.cftrust.org.uk/aboutcf/whatiscf/cfhistory/Levy_Lecture_04_-_JL.pdf
9. Riekert, K., Bartlett, S., Boyle, M., Krishnan, J., & Rand, C. (2007). The Association Between Depression, Lung Functioning, and Health-Related Quality of Life Among Adults With Cystic Fibrosis. Chest, 132 (1), 231-236.
10. Segal, Terry Y. (2008). Adolescence: what the cystic fibrosis team need to know. Journal of the Royal Society of Medicine, 101 (S1), S15-S27.
11. Teicher, Joseph D. (1969). Psychological Aspects of Cystic Fibrosis in Children and Adolescents. California Medicine, 110
(5), 371-374. 12. Webb, A., Jones, A., & Dodd, M. (2001). Transition from paediatric to adult care: problems that arise in the adult
cystic fibrosis clinic. Journal of The Royal Society of Medicine,94 (S40), S8-S11.