1. Cystic Fibrosis Stacey Simon

2. Statistics  Most common lethal, hereditary disorder among Caucasians  1 in 1,000 live births  Prevalence: 30,000 children and adults in US

3. 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

4. Pulmonary System  Mucus accumulates in the airways  Causes obstruction and infection  Over time: permanent damage to lungs  Primary cause of death: respiratory insufficiency

5. Digestive 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

6. 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

7. Genetics  Transmitted by recessive gene from both parents  Homozygotes: have CF  Heterozygotes: carrier  2-4% of Caucasian population are carriers

8. 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

9. 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.

10. Complex daily regimen Lung treatments Inhaled and oral medications Nutritional components Treatment Components

11. 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

12. 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 Regimen

13. Adherence  Rates of adherence  Medications: 63-97%  CPT: 40-91%  Diet: 16-81%  Enzymes: 75-97%  Vitamins: 57-70%

14. 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

15.  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 Adolescence

16. 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

17. 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 Life

18. 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

19. 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

20. The Family - Siblings  Limited research  Increased risk compared to healthy children  Psychological functioning: depression/anxiety  Behavioral problems: More aggression, jealousy, avoidance, fighting

21. 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