Cystic FibrosisCystic Fibrosis

Stacey SimonStacey Simon

StatisticsStatistics Most common lethal, hereditary disorder among Caucasians

1 in 1,000 live births

Prevalence: 30,000 children and adults in US

About the DiseaseAbout 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 SystemPulmonary System

Mucus accumulates in the airways

Causes obstruction and infection

Over time: permanent damage to lungs

Primary cause of death: respiratory insufficiency

Digestive SystemDigestive System

Pancreatic insufficiency: Missing enzymes that break down certain proteins

Occurs in 85-90% of CF patients

Causes malabsorption of proteins, fats, fat-soluble vitamins

Underweight, poor growth, delayed onset of puberty

DiagnosisDiagnosis

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

GeneticsGeneticsTransmitted by recessive gene from both parents

Homozygotes: have CF

Heterozygotes: carrier

2-4% of Caucasian population are carriers

Life ExpectancyLife ExpectancyChronic 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 RegimenTreatment RegimenCurrently 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: 60-74minAverage treatments per day: 4-6Children with CF spent more time on treatment than children with other chronic illnesses.

Complex daily regimen

Lung treatments Inhaled and oral medications Nutritional components

Treatment ComponentsTreatment Components

Pulmonary RegimenPulmonary RegimenGoals: • airways open/clean • prevent lung infections • avoid pulmonary disease • delay damage

Antibiotic therapy: oral, IV, aerosol deliveryResistance: serious problem

Airway clearance: Chest physiotherapy: 1-4x/day for approx. 30min

Methods: Manual percussion and postural drainageVestOthers: PEP, Flutter, Acapella

Inhalation therapy: to wet/thin mucus or deliver medsExercise

High calorie diet

Recommendations: 120-150% RDA

35-40% calories from fat

Enzymes with every meal and snack

Vitamins, supplements

Tube feedings at night

Dietary RegimenDietary Regimen

AdherenceAdherence

Rates of adherenceMedications: 63-97%

CPT: 40-91%

Diet: 16-81%

Enzymes: 75-97%

Vitamins: 57-70%

Barriers to AdherenceBarriers to AdherenceStudy: (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

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

AdolescenceAdolescence

PsychosocialPsychosocialIllness-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

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

Quality of LifeQuality of Life

CopingCopingDefinition: 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 - ParentsThe Family - ParentsBalance 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 - SiblingsThe Family - SiblingsLimited research

Increased risk compared to healthy children

Psychological functioning: depression/anxiety

Behavioral problems: More aggression, jealousy, avoidance, fighting

InterventionsInterventionsPsychoeducation: (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