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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
3. Nursing Diagnosis
4. History
5. Pharmacologic needs and pain management
6. Developmental level
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.
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.
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 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) Journal article
A study found that on average children skipped one meal or snack per day and they met the RDI 1 out of 4 days per week
Education plays a vital role in maintaining adequate nutrition in CF patients
Goal sheet: allows for active participation in nutritional therapy
Given at each clinical visitJournal article
A study found that on average children skipped one meal or snack per day and they met the RDI 1 out of 4 days per week
Education plays a vital role in maintaining adequate nutrition in CF patients
Goal sheet: allows for active participation in nutritional therapy
Given at each clinical visit
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