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CRTEP 4th Edition Cystic Fibrosis
Terms in this set (20)
General Info: What is Cystic Fibrosis?
Genetic disease that is more common in white persons than people of ethnic backgrounds.
General Info: What does Cystic Fibrosis do to the body?
1. Affects the exocrine glands, causes respiratory infections, gastrointestinal dysfunction and pancreatic enzyme insufficiency
General Info: Is end-stage lung disease a cause of death for someone with CF? True/False
General Info: CF is diagnosed by looking at a person based on their cough. True/False
False; it is diagnosed based on genetic testing; sweat chloride test (>60 mmol/L is considered positive)
1. Assessment: What do you look for when assessing for CF?
A) History of sinusitis
B) Reoccurring respiratory infections
D) Failure to grow
E) Cough with sputum production
F) Fatty stool history
A, B, D, E, F
General Info. What causes the thick secretions in cystic fibrosis?
The disruption of the chloride transport causes the thick secretions in cystic fibrosis. Sodium is absorbed in the airways, pancreas, and bile ducts.
Assessment: What physical characteristics do you look for when examining for CF?
Abnormal breath sounds, failure to thrive/grow, presence of nasal polyps, digital clubbing, pancreatic insufficiency
Assessment: What are CF findings on an x-ray?
Hyperinflation, peribronchial thickening, infiltrates with or without lobar atelectasis, right ventricular hypertrophy, bronchiectasis
Assessment: How do you assess a baseline for assessing disease progression?
Decreased FEV1 and FEV1/FVC (Obstruction)
Increased RV/TLC Ration (Air Trapping)
Decreased TLC and Vc ("late stage only"; Lung scarring/fibrosis)
Assessment: What pathogen supports CF diagnosis?
1. ED Assessment: Patient being assessed for acute respiratory distress in the ED would include all the following EXCEPT which:
· Weight loss, fatigue, weakness, appetite suppression
· Abnormal breath sounds
· Increased productive cough/sputum production
· Increased use of assessor muscles
· Labored breathing with intercostal retractions
· Flailed chest
· CT Scan
· X-Ray of the chest
· Family history of diseases
· Lab work for sodium, chloride and potassium
· A picture of their favorite dog type
everything is correct EXCEPT: Flailed Chest and A picture of their favorite dog type
Treatment: What treatment would you give a CF patient in distress?
isolate the patient, provide supplemental O2 to maintain a SpO2 >90%, give medication
Treatment: What specific medication would you give a CF patient in respiratory distress?
AlbuterolàPulmozymeàHyperSal or Mucomyst
Treatment: The only treatment for CF is airway clearance therapy/directed cough. True/False
False; airway clearance/ directed cough are important but you need the medication to help mobilize the secretions
Treatment: If P. aeruginosa is colonized or infection is confirmed; what would you give the patient?
Aerosolized antibioticà Tobramycin (TOBI) or Polymyxin E (Colistin) or Aztreonam (Cayston)
Treatment: What would be the best recommendations for CF management?
Flu vaccine, High fat diet and increased salt intake, regular exercise
Treatment: What is patient education for CF?
Proper education on medication needed and how to use it, airway clearance techniques, what to do in case of worsening symptoms, and sanitation/hygiene/disinfection practices
Treatment: How often should a CF patient visit their physician?
every 2-3 months for proper assessment of developments and progression of the disease
General Info: Where can you find the defective gene for CF?
General Info: What is a typical ABG for cystic fibrosis patients?
A mixed of respiratory acidosis and metabolic alkalosis
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