Cystic fibrosis Flashcards
Signs and symptoms
salty skin thick secretions in lungs wheezing breathlessness persistent cough producing thick mucus inflamed nasal passages or stuffy nose digestive issues
Digestive issues related to CF
severe constipation
foul smelling, greasy stools
poor weight gain
intestinal blockages
Diagnosis
Sweat test
genetic test or blood test
routine screening in newborns w/on 1st yr of life
Management
No cure but treatment can ease symptoms and reduce complications
Goals
Prevent and control infections in the lungs
Loosen mucus in the lungs
Treat and prevent intestinal blockages
Provide adequate nutrition
Prevent dehydration
Treat recurrent infections with antibiotics
Bronchodilators
Steroids
Vitamin replacement
Aerosols to help move mucociliary escalator
Physiotherapy - exercises and pulmonary rehabilitation
Lung rehabilitation
Lung transplant
Medications
bronchodilators
supplemental O2
Mucolytics
CF auscultations
Inspiratory and expiratory wheeze - obstruction
Fine crackles - secretions
X ray Cf findings
Tram track lines radiating from hilum
Ring shadows = clusters of cysts in upper lobes
Exacerbation = bilateral lower lobe infiltrates
Spirometry CF
Obstructive Pattern - decrease in:
Forced expiratory volume in 1 sec (FEV1)
Ratio from FEV1 to forced vital capacity
In CF they have a impaired/ missing …
CFTCR (CF transmembrane conductance regulator) chloride channel due to deleted genes
CFTCR effects transport of Chlorine and sodium resulting in
reduced water
Normal lungs Chlorine is…….and Sodium is…..
CL - secreted
Na and H20 is reabsorbed
In CF patients CL is …. Na channels are….
resulting…
Cl is trapped inside the cell
Na channels become hyperactive
Resulting in more H2O mvmt to follow the Na Mvmt
the increase in reabsorption of Na+ → increased reabsorption of H2O → lack of moisture in the lungs → thick secretions in the lungs
Dehydrated airways → difficult to move thick secretions
As anything that results in movement of secretion is impaired this results in….
More hypotonic secretions
Nasal polyps observed
Bronchiole pathways not working correctly → mucociliary system not working correctly because thick dehydrated mucus & not enough fluid to propel mucus up
This leads to infections → bronchiectasis
Average life expectancy and why
30
respiratory/ heart failure
heart becomes hypertrophic because pumping blood in the lungs = difficult
CF also causes other organ issues. Which organs
Lungs Pancreas Liver Intestines Kidneys Sex organs → Infertility
diabetes is common in CF
