Transcript Cystic Fibrosis

Cystic Fibrosis
Stacey Simon
Statistics
 Most common lethal, hereditary disorder
among Caucasians
1 in 1,000 live births
Prevalence: 30,000 children and adults in
US
About the Disease
 Chronic
genetic disorder of the endocrine
system that effects all organ systems
• Respiratory
• Digestive
• Pancreas
• Kidney
• Liver
• Reproductive
 Glands secrete thick,
sticky mucus
Pulmonary System
Mucus accumulates in the airways
Causes obstruction and infection
Over time: permanent damage to lungs
Primary cause of death: respiratory
insufficiency
Digestive System
Pancreatic insufficiency: Missing enzymes that
break down certain proteins
Occurs in 85-90% of CF patients
Causes malabsorption of proteins, fats, fatsoluble vitamins
Underweight, poor growth, delayed onset of
puberty
Diagnosis
 Typically during 1st year of life
 Presenting symptoms:
• resp. infections • malnutrition • intestinal obstruction
 Early detection: better growth,
cognitive functioning, lung functioning
 Sweat Test
 Newborn Screening
Genetics
Transmitted by recessive gene from both
parents
Homozygotes: have CF
Heterozygotes: carrier
2-4% of Caucasian
population are carriers
Life Expectancy
Chronic illness that ultimately results in
premature death
Severity: varies, affects life expectancy
Mean life expectancy:
1950: 4 years
1990: 28 years
2006: 37 years
Cause of death: lung disease
Treatment Regimen
Currently no cure – but CF gene identified
Treatment: targets symptoms –
Goal = increase longevity
Responsibility: Parents, with shift to children
Time/labor intensive: (Ziaian et al, 2006)
Daily time spent on treatment: 60-74min
Average treatments per day: 4-6
Children with CF spent more time on treatment than
children with other chronic illnesses.
Treatment Components
 Complex daily regimen
 Lung treatments
 Inhaled and oral
medications
 Nutritional components
Pulmonary Regimen
Goals: • airways open/clean • prevent lung infections
• avoid pulmonary disease • delay damage
Antibiotic therapy: oral, IV, aerosol delivery
Resistance: serious problem
Airway clearance:
Chest physiotherapy: 1-4x/day for approx. 30min
Methods:
Manual percussion and postural drainage
Vest
Others: PEP, Flutter, Acapella
Inhalation therapy: to wet/thin mucus or deliver meds
Exercise
Dietary Regimen
 High calorie diet
 Recommendations: 120-150% RDA
 35-40% calories from fat
 Enzymes with every meal and snack
 Vitamins, supplements
 Tube feedings at night
Adherence
Rates of adherence
Medications: 63-97%
CPT: 40-91%
Diet: 16-81%
Enzymes: 75-97%
Vitamins: 57-70%
Barriers to Adherence
Study: (Modi & Quittner, 2006) What makes it hard to do CF
treatment?
Most common: • forgetting • bad taste
• oppositional behavior • time management
Patient-provider communication: Disagree on 20% of rx
Treatment complexity: Fewer treatments associated
with better adherence
Knowledge: On tests of CF-related knowledge-• Parents: 68% correct • Children: 55% correct
Adolescence

Period of physical and emotional change
Typical adolescent peer and social concerns
 Increased responsibility for treatment
regimen


Critical period for nutrition
• Growth spurts
• Onset of puberty
• Frequent pulmonary infections

Less adherence
Psychosocial
Illness-related issues:
• Fear of death • med procedures • different
physically
At risk for:
Psych. problems: depression, anxiety
Behavioral problems: peer conflict, social
adjustment, aggression, social withdrawal
Quality of Life
 Important to understand the relation of CF and
treatment on patients life
 In general, CF patients have similar quality of life
to healthy controls
 Wahl et al, 2005: CF patients with poorer health
report lower quality of life than patients with better
lung functioning
Coping
Definition: Methods to prevent/avoid/control
emotional distress.
Study: (Abbott, Dodd, Gee & Webb, 2001) differences in coping
style for adherent and non-adherent patients
Good adherence: Optimistic/hopeful coping
Partial adherence: Distraction
Poor adherence: Avoidance
The Family - Parents
Balance between: personal needs, family
needs and treatment needs of child w/CF
Extra demands on time, energy, finances
Psych. Functioning: Depression, anxiety
Discipline issues: Overly permissive, overprotective
Marital Satisfaction: Less communication, decreased
time together.
Family stress: CF families = other chronic illness, CF
families > healthy children
The Family - Siblings
Limited research
Increased risk compared to healthy children
Psychological functioning: depression/anxiety
Behavioral problems:
More aggression, jealousy,
avoidance, fighting
Interventions
Psychoeducation: (Goldbeck & Babka, 2001) Family group
intervention
Components: Lessons about CF, group discussion, role-play, led
by CF medical team
Results: Parents rated children as less competent,
less optimistic, more withdrawn after treatment
Nutritional Intervention: (Stark et al, 1993, 2003; Powers et al, 2003, 2006)
Child behavioral management strategies
Results: successful at increasing caloric intake,
comparable to medical interventions