Cystic Fibrosis Case Cystic Fibrosis Case StudyStudy

• 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 DiagnosisNursing Diagnosis

• Ineffective Airway Clearance

Nursing DiagnosisNursing Diagnosis

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

HistoryHistory

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 Pharmacologic needs and pain managementmanagement

•Pancreatic enzymes

•Albuterol nebs

•Dornase (pulmozyme) 1 vial

•Megace

•Azithromycin

•Ceftazidime (fortaz)

•Ticarcilin + clavulanate

•Ibuprofen

Developmental levelDevelopmental 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-economicSocio-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.

CommunicationCommunication• 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 factorsSpiritual 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 careFamily 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 Patient and family teaching needsneeds

• 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 ConsiderationsEthical Considerations• There are seven children in this family, these children are not There are seven children in this family, these children are not the youngest.the youngest.

•Was it ethical for the parents to continue having children even Was it ethical for the parents to continue having children even though they knew they carried the gene for cystic fibrosis and though they knew they carried the gene for cystic fibrosis and one previous child had cystic fibrosis?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 This disease can be very limiting and costly as far as medical needs.medical needs.• Are we to judge the family for this?Are we to judge the family for this?

Age Appropriate Play & Activities for School Age Appropriate Play & Activities for School Aged ChildrenAged 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 PracticeBest 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 Best Nursing Practice ContinuedContinued

•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)

~Brought to you by~~Brought to you by~

Wendy Magana Danielle Hower Clint Studlow