CF Flashcards
What is cystic fibrosis?
Autosomal recessive exocrinopathy affecting exocrine glands
What population is the most risk for CF?
White people
How are most cases of CF identified?
Newborn screening- results with high elevated immunoreactive trypsinogen levels
What is the pathophysiological problem with CF?
Dysfunction/absence of CFTR cystic fibrosis transmembrane conductance regulator.
Abnormally function _ _ results in altered _ + _ transport across the _ surface of epithelial cells.
chloride channels, chloride, water luminal
What is elevated in pt’s CF sweat?
High Na Cl
What is decreased in most mucous secretions in a CF patient’s body?
Decreased Na Cl
_ glands produce abnormally thick mucus that results in obstruction in CF
Exocrine
Explain the movement of chloride in sweat glands in the normal population vs a patient with CF
Normally, CL and sodium goes into the cell from the lumen in a normal person’s body. When a patient has CF, the cloride and sodium are blocked from getting access into the cell. They are stuck in the lumen.
Explain the movement of chloride + water in exocrine glands in the normal population vs a patient with CF.
Chlorine is usually expelled out of the cell to allow a small amount of Na and H2O to enter the cell. When a patient has CF, the chlorine is blocked from leaving the cell, causing LARGE amounts of Na and H2O to enter the cell. This dehydrates the mucus, making it thick and crusty.
Other than respiratory compromise, why are copious viscous pulmonary secretions harmful to patients?
Bacterial growth
If you have an unscreen patient, what three things could signal towards CF?
Chronic lung disease
Pancreatitis
Infertility
What are symptoms of CF?
Cough with sputum production
Decreased exercise tolerance
Recurrent hemoptysis
Symptoms not related to lungs
-chronic rhinosinitis
-steatorrhea
-diarrhea
What are some clinical findings of CF?
-malnourish, low BMI
- digital clubbing
- increased A/P diameter
- hyper-resonance to percussion
- Apical crackles
- Sinus tenderness w/ purulent discharge
- Nasal polyps
-Absence of vas deferens
- Biliary cirrhosis
What is the treatment for CF?
Refer to CF center
- chest physiotherapy is the standard of care
- pts => 6 should receive inhaled hypertonic saline and dornase alfa (mucolytic)
-Bronchodilators for airway clearance
- treat respiratory tract infections -> Inhaled tobramycin
-Pancreatic enzyme replacement
-Nutritional/psychosocial support
