CF 1
What ethnicity MC has this fatal hereditary disorder?
Caucasions
What is the pathophysiologic deficiency that causes CF?
*Deficiency causes depleted PCL and cause cilliary collapse & failure to clear overlying mucus
Cystic fibrosis transmembrane conducts regulator (CFTR)
What is the MC cause of severe chronic lung disease in young adults?
Cystic Fibrosis
What does a drug that is an alpha 1 agonist do?
Vasoconstriction
What does a beta 2 agonist do?
Causes relaxation of bronchiole smooth muscle resulting in bronchodilation
Cystic fibrosis not only affects the pulmonary system (obstructive lung disease), it also affects which system?
Endocrine (exocrine gland dysfunction)
*Pancreatitis/pancreatic dysfunction common
F508del mutation
*Omission of single phenylalanine residue (F) at CFTR position 508
Name 3 possible symptoms you may see in someone with CF.
Cough, sputum production, decreased exercise tolerance, recurrent hemoptysis, chronic rhino sinusitis, steatorrhea, diarrhea, abdominal pain, sterility in men & women
What is the drug Acetylcysteine used for?
Extra points - MOA & AEs
- Mucolytic/expectorant
MOA - Breaks open disulfide bonds in respiratory & GI tract
AE - Bronchospasm, malodorous, N/V
What is a recommendation for patients who are inhalant corticosteroids?
Extra points - Name one example of a CS inhalant
Rinse mouth out after each use to reduce the risk of thrush
Examples - Mometasone, Fluticasone, Budesonide (nebulizer)
The CFTR gene defect in CF affects the ability to open & close ion channel gate. What electrolyte is most affected & markedly elevated?
*Sweat Electrolyte (Iontophoretic Sweat) Test
Cl-
* Sweat Cl- concentration > 60 mEq/L (abnormal) on two occasions
Na+ can also be elevated
* Abnormal > 90 mEq/L
Name 3 possible signs that you would see in someone with CF?
Malnourished, low BMI, digital clubbing, increased anteroposterior chest diameter, hyper-resonance to percussion, apical crackle
What is the main lung function value that is the primary indicator of health in people with CF?
FEV1
*Forced exhaled Volume of air in the 1st second of exhalation
Give one example of a short acting beta 2 agonist (SABA)?
Extra points - MOA & AE
Ex. Albuterol, Levalbuterol
MOA - Increases cyclic AMP (cAMP), relaxes bronchial smooth muscle
AE - Tachycardia, tremor
What is the MOA of Azithromycin?
Extra points - AE
MOA - binds to the 50s subunit of bacterial ribosomes
AE - GI, QT prolongation
What 2 laboratory findings from an arterial blood gas test a/w CF?
- Hypoxemia
- Chronic compensated respiratory acidosis (advance disease)
Name one treatment management used for airway clearance in CF.
- Bronchodilator
- Mucolytics
- Abx
- Anti-inflammatory medications
- Decongestants
Name one kind of replacement therapy that you need to consider for someone with CF.
- Pancreatic enzyme replacement
- Vitamin A, D, E, K supplementation
What is serum level range for drug monitoring of Methylxanthines (Theophylline/Aminophylline)?
8-12 mcg/mL
Extra points - drug class, MOA
AE - Connective tissues dysfunction, CNS (agitation, confusion, delirium), photosensitivity
Class - Fluoroquinolone
MOA - Binds to DNA gyros, the enzyme responsible for uncoiling DNA
Name two findings from a pulmonary function test a/w cystic fibrosis?
- Decrease FVC, airflow rates, TLC
- Presence of air trapping noted by high RV:TLC ratio
- Decrease in pulmonary diffusion capacity
On CXR, what is the early sign of CF? What is the late stage sign?
- Bronchiectasis (long-term inflammation leads to remodeling in the lungs)
For CF patients with chronic Pseudomonas aeruginosa infections, what are the long-term antibiotics that you would put them on?
Azithromycin 500 mg 3x week
+ inhaled abx (Tobramycin, aztreonam, Coisitin, Levofloxacin) 2-3x wks
What is the MOA & AEs of Ivacaftor?
Extra point - What does it treat?
MOA - For selected genetic mutations, CF transmembrane conductance regulator (CFTR) modulator
AE - GI, elevated liver enzymes
Treats - Cystic fibrosis
Name 5 of the 7 drug classes for asthma & COPD.
- Corticosteroids
- Mast cell stabilizer
- Monoclonal antibodies
- B2 agonists
- Antimuscarinics (anticholinergic)
- Methylxanthines
- Antileukotrienes