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
Nursing Diagnosis
• Ineffective Airway
Clearance
Nursing Diagnosis
• Nutrition imbalance: less than required
-Encourage 3 meals and 2 snacks daily (or as directed)
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.
Pharmacologic needs and pain
management
•Pancreatic enzymes
•Albuterol nebs
•Dornase (pulmozyme) 1 vial
•Megace
•Azithromycin
•Ceftazidime (fortaz)
•Ticarcilin + clavulanate
•Ibuprofen
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 5th
grader”.
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.
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.
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!!!
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.
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
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?
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.
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.
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)
~Brought to you by~
Wendy Magana
Danielle Hower
Clint Studlow