©SOPHIABENNETT@2024-2025 Sunday, August 25, 2024 2:42 PM
CHA 1: Unit 6 Respiratory Exam Study
Guide with Complete Solutions
cystic fibrosis (CF) - Answer✔️✔️-Autosomal-recessive trait
Hereditary disorder; altered functions of exocrine glands: Lung, Pancreas,
Sweat Gland, Reproductive Organs
Characterized by: lung congestion, infection, and malabsorbtion of
nutrients by the pancreas
symptoms of cystic fibrosis - Answer✔️✔️-Thick mucus
Chronic cough
Dyspnea
Freq respiratory infections
Clubbing of fingers
Increased AP diameter with hyper-resonant chest
Rales, rhonci
Failure to gain weight/malnutrition
Meconium ileus
Always hungry
1
, ©SOPHIABENNETT@2024-2025 Sunday, August 25, 2024 2:42 PM
Abdominal distension
Steatorrhea
Fat soluble vitamin deficiencies (increased bruising)
Sweat, tears abnormally salty
Decreased or absent sperm count
Viscous cervical mucous
Cystic fibrosis pulmonary manifestations - Answer✔️✔️-Respiratory
infections
Chest congestion
Limited exercise tolerance
Cough and sputum production
Use of accessory muscles
Decreased pulmonary function
Changes in chest x-ray
Increased anteroposterior diameter
Cystic Fibrosis Diagnostic Test - Answer✔️✔️-sweat test-chloride test (excess
chloride release)
PFT-monitor current status
2
, ©SOPHIABENNETT@2024-2025 Sunday, August 25, 2024 2:42 PM
CXR-monitor current status
DNA Testing
Cystic Fibrosis Treatment Goals - Answer✔️✔️-Client will absorb adequate
nutrients daily
Client will maintain patent airway
Family demonstrated adequate coping
Cystic Fibrosis Treatment - Answer✔️✔️-Postural drainage and percussion
Good hygiene
Moistened oxygen
Nebulized meds
Antibiotics (Tobramycin solution for inhalation)
Dornase alfa
Pancreatic enzyme
Diet: High calorie & protein, moderate fat
Water-miscible forms of Vit A, D, E supp
3
, ©SOPHIABENNETT@2024-2025 Sunday, August 25, 2024 2:42 PM
Generally not breast fed, not enough protein
Pancreas-lung transplant
Tobramycin (TOBI) - Answer✔️✔️-used to treat chronic P. aeruginosa
infection in CF patients
Dornase alfa (Pulmozyme) - Answer✔️✔️-decreases viscosity of mucus and
improves lung function. improvement monitored by pfts. use once daily.
Pancreatic Enzyme Replacement - Answer✔️✔️-Pancreatic enzymes
(pancreatin and pancrelipase); action: aid in the digestion of starches, fats,
protein; side effects: anorexia, N/V, diarrhea, hypersensitivity, sneezing,
lacrimation, skin rashes; nursing responsibilities: do not use with antacids,
avoid inhaling powder, client education: take with meals
Cystic Fibrosis Family Teaching - Answer✔️✔️-Functions of oxygen and
how to regulate flow
Postural drainage
Encourage them to think about fitting the treatment/illness into family life
(DO both parents work)
Respiratory Distress Syndrome (RDS) - Answer✔️✔️-A respiratory disorder
that affects premature infants born without enough surfactant in the lungs.
It is treated with respiratory support and surfactant administration
4
CHA 1: Unit 6 Respiratory Exam Study
Guide with Complete Solutions
cystic fibrosis (CF) - Answer✔️✔️-Autosomal-recessive trait
Hereditary disorder; altered functions of exocrine glands: Lung, Pancreas,
Sweat Gland, Reproductive Organs
Characterized by: lung congestion, infection, and malabsorbtion of
nutrients by the pancreas
symptoms of cystic fibrosis - Answer✔️✔️-Thick mucus
Chronic cough
Dyspnea
Freq respiratory infections
Clubbing of fingers
Increased AP diameter with hyper-resonant chest
Rales, rhonci
Failure to gain weight/malnutrition
Meconium ileus
Always hungry
1
, ©SOPHIABENNETT@2024-2025 Sunday, August 25, 2024 2:42 PM
Abdominal distension
Steatorrhea
Fat soluble vitamin deficiencies (increased bruising)
Sweat, tears abnormally salty
Decreased or absent sperm count
Viscous cervical mucous
Cystic fibrosis pulmonary manifestations - Answer✔️✔️-Respiratory
infections
Chest congestion
Limited exercise tolerance
Cough and sputum production
Use of accessory muscles
Decreased pulmonary function
Changes in chest x-ray
Increased anteroposterior diameter
Cystic Fibrosis Diagnostic Test - Answer✔️✔️-sweat test-chloride test (excess
chloride release)
PFT-monitor current status
2
, ©SOPHIABENNETT@2024-2025 Sunday, August 25, 2024 2:42 PM
CXR-monitor current status
DNA Testing
Cystic Fibrosis Treatment Goals - Answer✔️✔️-Client will absorb adequate
nutrients daily
Client will maintain patent airway
Family demonstrated adequate coping
Cystic Fibrosis Treatment - Answer✔️✔️-Postural drainage and percussion
Good hygiene
Moistened oxygen
Nebulized meds
Antibiotics (Tobramycin solution for inhalation)
Dornase alfa
Pancreatic enzyme
Diet: High calorie & protein, moderate fat
Water-miscible forms of Vit A, D, E supp
3
, ©SOPHIABENNETT@2024-2025 Sunday, August 25, 2024 2:42 PM
Generally not breast fed, not enough protein
Pancreas-lung transplant
Tobramycin (TOBI) - Answer✔️✔️-used to treat chronic P. aeruginosa
infection in CF patients
Dornase alfa (Pulmozyme) - Answer✔️✔️-decreases viscosity of mucus and
improves lung function. improvement monitored by pfts. use once daily.
Pancreatic Enzyme Replacement - Answer✔️✔️-Pancreatic enzymes
(pancreatin and pancrelipase); action: aid in the digestion of starches, fats,
protein; side effects: anorexia, N/V, diarrhea, hypersensitivity, sneezing,
lacrimation, skin rashes; nursing responsibilities: do not use with antacids,
avoid inhaling powder, client education: take with meals
Cystic Fibrosis Family Teaching - Answer✔️✔️-Functions of oxygen and
how to regulate flow
Postural drainage
Encourage them to think about fitting the treatment/illness into family life
(DO both parents work)
Respiratory Distress Syndrome (RDS) - Answer✔️✔️-A respiratory disorder
that affects premature infants born without enough surfactant in the lungs.
It is treated with respiratory support and surfactant administration
4
