The Lifespan of the psychosocial effects of Cystic Fibrosis

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.