Cystic Fibrosis CAT Flashcards
1
Q
What is cystic fibrosis?
A
- Inherited autosomal recessive genetic disorder
- Causes exocrine glands to overproduce thick mucus (causes obstruction), overproduce normal secretions, overproduce sodium and chloride
2
Q
Clinical presentation for CF
A
- More common in Caucasian kids
- Most common symptom is high concentrations of sodium and chloride in sweat
- Persistent cough
- Salty skin
- Sputum production
- Wheezing
- Poor weight gain
- Recurrent infections
3
Q
Lab or imaging used with CF
A
- Neonates’ meconium to screen for increased albumin
- Pilocarpine iontophoresis sweat test is SOLE DIAGNOSTIC TOOL
- Sodium and chloride >60mEq/L (40 mEq/L is normal)
- Perform test to ensure accuracy
4
Q
Most common complication w/ CF
A
Exacerbation of obstructive pulmonary disease
5
Q
Pulmonary function testing
A
- Decreased forced expiratory volume
- Decreased forced vital capacity
- Increased functional residual capacity
- Increased residual volume
6
Q
Common comorbidities w/ CF
A
- Hypoxemia
- Hypercapnia
- Chronic pulmonary infections
- Poor absorption
- Barrel chest
- Pectus carinatum
- Kyphosis
- Decreased pancreatic function
7
Q
Airway obstruction in CF causes __
A
- Pulmonary hypertension
- Atelectasis
- Pneumonia
- Lung abscess
8
Q
Severe complications include __
A
- Cirrhosis
- Diabetes
- Pneumothorax
- Cardiac pathology
- Pancreatitis
- Cor pulmonae
- Intenstinal obstruction
9
Q
Role of PT in patients w/ CF
A
- Airway clearance/postural drainage
- Percussion
- Vibration
- Breathing and assistive cough techniques
- Ventilatory muscle training
- Maximize overall function
- Train family for postural drainage and chest PT
10
Q
Long-term outcomes associated w/ CF
A
- Terminal disease
- Median age has increased due to improvements in management (well into forties)
- Kids who present initially w/ GI symptoms generally do better
- Males prognosis > female prognosis
11
Q
Most common cause of death for patients w/ CF
A
Respiratory failure
