Cystic Fibrosis Stacey Simon. Statistics Most common lethal, hereditary disorder among Caucasians ...
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Transcript of Cystic Fibrosis Stacey Simon. Statistics Most common lethal, hereditary disorder among Caucasians ...
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