Cystic Fibrosis & Bronciectasis Flashcards
Define cystic fibrosis (CF)
- multisystem, childhood dz
- genetic disorder of the exocrine glands
- fibrotic dz with obstructive features
What does CF affect?
- plasma membrane Na/Cl channel of epithelial cells in lungs
What is the result of CF’s affect?
- excessive, viscous, sticky, mucous plug
What other systems are affected in CF?
- GI
- reproductive
- nearly all exocrine ducts
What is the MC, lethal, autosomal recessive inheritance in caucasians?
- CF
What is the affected gene in CF?
- CFTR
What is the pathophysiology of CF?
- aberrant CFTR protein allows too much influx of Na/Cl
- causes inadequate hydration of bronchotraceal epithelium
- impairs mucocilliary fxn
- allows oportunistic bacteria
What is the result of the pathophysiology of CF?
- thick, sticky mucous
- decreased mucocilliary movement
- dehydration of mucuous
- biofilm formation from mucous adhereing
- neutrophilic DNA
What is the main cause of death in CF?
- respiratory failure
What is linked to accelerated lung disease and death in CF?
- poor nutrition
What are the complications of CF?
- cysts
- bullae
- bronciectasis
- hemoptysis
- PNA
- PAH
- cor pulmonale
- clubbing
What are the non-pulmonary symptoms of CF?
- meconium ileus (intestinal blockage)
- pancreatic insufficiency
- constitutional symptoms
What are the lung S&S of CF?
- persistent cough
- persistent wheezing
- excessive sputum
- barrel chest
- recurrent/severe PNA
- digital clubing
What is the PNA bug that complicates CF?
- P. aerunginosa
How does the PNA bug complicate CF?
- adheres to mucoid biofilm
- highly resistant to abx
- progresses to function pulm decline and bronchiectasis
How is CF detected?
- universal screenings at birth: blood test to detect elevated trypsinogen
- Sweat Test
What will the sweat test show in CF?
- excessive NaCl
What is the main function of the CFTR protein?
- reabsorb Cl- and Na+ ions in sweat glands
What does a loss of CFTR proteins lead to in the sweat test?
- decreased reabsorption and NaCl
- hypertonic sweat
What diagnostic test are used for CF?
- sweat test
- if sweat test is inconclusive: nasal potential difference measurement or molecular CFTR DNA mutation test
- PFTs
- CXR
What do the PFTs show in CF?
- obstructive patter progressing to restrictive
What is the goal of tx in children with CF?
- delay P. aeruginosa
What is the goal of tx in adults with CF?
- suppression of established P. aeruginosa
What is the best tx approach to CF?
- multidiciplinary
What are the txs for CF?
- chest PT
- positive expiratory pressure mask
How is PNA treated in CF patients?
- need ID consult
- long term abx
How are complications of CF prevented?
- clearance of viscous, lower airway secretions via inhaled DNAase or hypertonic saline
- vaccinations
What is the definitive tx for CF?
- lung transplant
