Managing patients with Oxygenation Disorders: Cystic Fibrosis and Larnygeal Cancer Flashcards
Exam 2
Cystic Fibrosis: Epidemiology
How many children and adults are effected?
30,000 children and adults in U.S. are affected
What percent of people are diagnosed with Cystic Fibrosis before age 2?
70% diagnosed before age 2
How many new cases of cystic fibrosis a year?
1,000 new cases annually
What causes cystic fibrosis?
Caused by defective gene carried by both parents
How many Americans are carriers of the the defective gene associated with cystic fibrosis?
More than 10 million Americans are carriers
Cystic Fibrosis: pathophysiology
Genetic disease of the exocrine glands
Cystic Fibrosis: pathophysiology
It is a dificiency of what?
Deficiency of protein cystic fibrosis transmembrane regulator
Deficiency of protein cystic fibrosis transmembrane regulator leads to
Disruption of ion transport
Mucus build up
Increased mucus in respiratory, gastrointestinal, and reproductive systems
Cystic Fibrosis- MEdical Management- Diagnosis
Sweat chloride test
Sweat chloride test normal results
Cystic Fibrosis results
Normal: <30 mEq/L
Cystic fibrosis: >60 mEq/L
What is a mandatory newborn screening?
Cystic Fibrosis
Cystic Fibrosis: Nursing Management
Assessment and analysis
Thick, sticky mucus
Persistent cough
Tachypnea
Frequent lung infections
Wheezing or shortness of breath
Poor growth or slight weight gain
Steatorrhea
Salty tasting skin
Cystic Fibrosis: Nursing Management
Nursing Diagnoses
Ineffective airway clearance related to increased sputum production
Impaired gas exchange related to increased sputum
Fatigue related to dyspnea
Risk for infection related to stasis of mucus
Imbalanced nutrition: less than body requirements related to impaired digestive process and absorption of nutrients, increased work of breathing
Cystic Fibrosis: Nursing Interventions- Actions
Monitor vital signs
Airway clearance: postural drainage, percussion
Administer medications as ordered
Maintain nutrition and supplements
Maintain record of weight
Cystic Fibrosis: Nursing Interventions- Teaching
Airway clearance techniques
Diet and supplements
Medication management
Knowing and avoiding risk factors of exacerbation
Genetic screening and counseling
