1. Cystic Fibrosis Case Study Gender: 1 Male & 1 Female Age: 12 yrs & 11 yrs old Setting: 4N at MAMC Ethnicity: Irish, Scottish, German (Western European) Cultural considerations: none

2. Nursing Diagnosis Ineffective Airway Clearance

3. Nursing Diagnosis Nutrition imbalance: less than required -Encourage 3 meals and 2 snacks daily (or as directed)

4. History Cystic Fibrosis 2 weeks of increasing cough Had viral URI 3 weeks before and cough evolved into dry hacking cough. Did not respond to short course of Ciprofloxacin. Cystic Fibrosis 3 weeks of worsening cough Prior admission pt. diagnosed with S. aureus and Pseudomonas in culture. (Nov 2007) Recently discontinued insulin for DM. Lightheaded with insulin and now thought to be normoglycemic.

5. Pharmacologic needs and pain management Pancreatic enzymes Albuterol nebs Dornase (pulmozyme) 1 vial Megace Azithromycin Ceftazidime (fortaz) Ticarcilin + clavulanate Ibuprofen

6. Developmental level Appropriate development status for age. Mental intelligence is extremely high for age. Girl: Appropriate social skills demonstrated through constant talk about friends and engaging in long conversations. Boy: Extremely knowledgeable for a 12 year old, excellent communication skills and curious about politics, government, and wants to be a doctor. Knew more correct answers than student nurses in the game, “So you think you are smarter than a 5 th grader”.

7. Socio-economic -Health care barriers –Very fortunate that father is in military where health care is readily available and mostly everything is paid for. –Father is a doctor in the hospital However being from a family of 8 and only one income puts a strain on finances, but he and siblings are very grounded.

8. Communication Language barriers- No noticeable barriers except when a flair-up occurs and is unable to speak due to coughing Level of communication for the best understanding by patient and family: -Open conversations, tell them the truth, use medical terms; pts very comfortable with usage of medical terminology.

9. Spiritual factors Roman Catholic- Family tries to follow Catholic guidelines, but is not too heavily involved in church. Passionate about politics and very patriotic: -Won’t buy anything that is made in China!!!

10. Family centered care -Parents are now divorced and 7 kids live with dad. Siblings ages are: 18, 17, 14, 12, 11, 10, & 8 -Family closeness is important and care by father is limited because he works long hours, thus other siblings are relied upon to care for pts.

11. Patient and family teaching needs Minimal due to father being doctor at MAMC Continue patient education as new information and articles become available Concentrate on coping mechanisms for potential future progression of disease

12. Ethical Considerations There are seven children in this family, these children are not the youngest. There are seven children in this family, these children are not the youngest. Was it ethical for the parents to continue having children even though they knew they carried the gene for cystic fibrosis and one previous child had cystic fibrosis?Was it ethical for the parents to continue having children even though they knew they carried the gene for cystic fibrosis and one previous child had cystic fibrosis? –“Tis better to have loved and lost than never to have loved at all” –“The way to love anything is to realize that it might be lost.” This disease can be very limiting and costly as far as medical needs. This disease can be very limiting and costly as far as medical needs. Are we to judge the family for this? Are we to judge the family for this?

13. Age Appropriate Play & Activities for School Aged Children Activities are limited due to CF, but should be encouraged as tolerated to increase independence and loosen mucus. Reading Board, card, video, and computer games Personal activities- journaling or scrapbooking Allow resting periods between activities to prevent fatigue Physical exercise is permitted as tolerated to promote mucus secretion and cardiopulmonary conditioning.

14. Best Nursing Practice Ineffective airway clearance: –Outcome: Child will maintain open airway, easy work of breathing, and respiratory rate within parameters of age –Priority Interventions Position with open airway Chest physiotherapy: –flutter valve device  provides high frequency oscillation to the airway as they exhale through mouthpiece. –positive expiratory pressure therapy  exhale through flow resistor which provides positive expiratory pressure. This is repeated until coughing yields expectoration of secretions. –high frequency chest compression vest  high frequency chest well oscillation to increase airway velocity creating cough like shear forces and decrease viscosity of secretions.

15. Best Nursing Practice Continued Imbalanced Nutrition: less than body requirementsImbalanced Nutrition: less than body requirements – Outcome: Child will maintain adequate nutritional intake and weight gain will occur. – Interventions: Calorie counts to ensure adequate intake (may need up to 20-50% more than RDI) Assist family in choosing higher calorie, protein rich food, and high fat snacks to optimize growth Offer favorite foods to encourage eating Encourage supplements (pancreatic enzymes) Continuous monitoring including weights, heights, BMI, ideal body weight percentile, skin fold thickness, and upper arm circumference Goal sheets (journal article)

16. ~Brought to you by~ Wendy Magana Danielle Hower Clint Studlow