CF Flashcards
What are the respiratory symptoms of CF?
Persistent cough with thick, purulent sputum
Wheezing & frequent respiratory infections (especially with Pseudomonas)
Bronchiectasis (chronic airway dilation & damage)
Airway obstruction & progressive respiratory failure
what are the GI symptoms of CF?
Steatorrhea (fatty, foul-smelling stools due to fat malabsorption)
Failure to thrive & malnutrition (due to pancreatic insufficiency)
Distal Intestinal Obstruction Syndrome (DIOS) (blockage due to thick intestinal mucus)
Cystic Fibrosis-Related Diabetes (CFRD) (insulin deficiency & resistance)
Liver disease (e.g., biliary cirrhosis, portal hypertension)
what are the reproductive symptoms of CF?
Males: Absent vas deferens → Infertility
Females: Thick cervical mucus → Menstrual irregularities & subfertility
what symptoms are in newborns with CF?
Meconium ileus (bowel obstruction at birth)
Failure to thrive & frequent respiratory infections
what is the cause of CF?
Mutation in the CFTR gene → Leads to thick, sticky mucus in lungs, pancreas, intestines, liver, and reproductive organs.
what are the respiratory complications?
Chronic lung infections (often Pseudomonas aeruginosa)
Bronchiectasis & airway obstruction
Respiratory failure & cor pulmonale
Pneumothorax, hemoptysis (life-threatening in severe cases
what are the GI complications?
Pancreatic insufficiency → Malabsorption (fat, protein, vitamins A, D, E, K)
Cystic Fibrosis-Related Diabetes (CFRD)
Liver disease (biliary cirrhosis, hepatic steatosis, portal hypertension)
what are the major complications?
Respiratory failure (leading cause of death)
Pneumothorax & hemoptysis
Cor pulmonale (right-sided heart failure due to chronic lung disease)
Severe malnutrition due to malabsorption
what does chest x-ray and CT scan show?
bronchiectasis, hyperinflation
what does pulmonary function test show
progressive airflow obstruction, decreased FEV1
what are the treatment goals for CF?
Clear mucus & maintain lung function
Prevent & manage infections
Optimize nutrition & pancreatic enzyme replacement
Manage complications (e.g., CFRD, liver disease, DIOS)
what is the respiratory management for CF?
Chest physiotherapy (CPT) (postural drainage, percussion, vibration)
Huff coughing (helps clear thick mucus)
High-frequency chest wall oscillation (vest therapy)
what does dornase alfa (pulmozyme) do?
thins mucus
what does hypertonic saline (%7)
hydrates airways and loosens mucus
what does albuterol do for CF?
Relaxes airway muscles before CPT
what are the antibiotics used for CF?
Inhaled tobramycin (for Pseudomonas aeruginosa infections)
Azithromycin (anti-inflammatory & antibacterial properties)
when is oxygen therapy used for CF?
Used for severe disease (Caution: CF patients have chronic CO₂ retention; excessive O₂ can suppress respiratory drive!)
when is lung transplantation used for CF?
For end-stage lung disease with severe respiratory failure
when is hospitalization required for CF?
Severe respiratory exacerbations
Worsening hypoxemia
Massive hemoptysis or pneumothorax
what is the nutrition management for CF?
High-calorie, high-fat diet (due to high energy expenditure)
Pancreatic enzyme replacement therapy (PERT) (e.g., pancrelipase) with every meal/snack
Fat-soluble vitamin supplementation (A, D, E, K) Increased sodium intake (especially during hot weather or fever)
how do you prevent infection in CF?
Frequent handwashing
Avoiding sick contacts
Vaccinations (influenza, pneumococcal, COVID-19)
Strict adherence to airway clearance therapy
why is hydration crucial for CF?
Thins mucus & prevents airway obstruction
what does invacaftor (kalydeco) do in CFTR therapy?
Improves chloride transport (specific mutations)
what does lumacaftor/lvacaftor (orkambi)
Helps CFTR proteins fold correctly
what does Tezacaftor/Ivacaftor (Symdeko), Elexacaftor-Tezacaftor-Ivacaftor (Trikafta) in CFTR therapy
Newer treatments for broader CF mutations
