Cystic fibrosis and asthma Flashcards
1
Q
CF definition
A
- inherited disorder of ion transport in the exocrine glands
- affects manly the respiratory, digestive, and male reproductive systems
2
Q
CF etiology
A
- caused by abnormal CF transmembrane conductance regulator protein
- epithelial cells have impermeability to Na+ and Cl- in abnormal amt of fluid being removed from the lumen
- accumulation of hyperviscous secretions
- impaired clearance
-primary organs affected are respiratory and GI
3
Q
CF pathophysiology
A
- obstruction of mucous secreting exocrine glands by hyperviscous secretions
- progressive airway obstruction affecting both small an dlarge airways
- irreversible airway damage
4
Q
CF diagnosis
A
- prenatal diagnosis is possible and screening of carriers through genetic counceling
- newborn screening: blood sample, IRT/DNA test , sweat test to look at na/Cl levels
5
Q
CF clinical manifestation gastrointestinal
A
- pancreatic insufficiency- viscous secretions begin to obstruct pancreatic duct in utero.
- loss of pancreatic exocrine function
- in infancy they may show sign of protein-calorie malnutrition
6
Q
CF clinical manifestation pulmonary
A
- chronic cough and sputum production
- child is unable to expectorate the mucus because of increased viscosity which is a perfect breeding ground for bacteria
- reduced oxygen can lead to cyanosis and hypoxia
7
Q
CF clinical manifestation musculoskeletal
A
- muscle pain frequently reported along with decreased bone mineral density
- hypertrophic pulmonary osteoarthritis occurs with increasing freq with age and results in clubbing of the fingers and toes
8
Q
CF medical management
A
- depends on the stage of disease and the organs involved
- antibiotics to address bacterial infections
- annual flu shot
- inhaled steroids
- bronchodilators
- corticosteroids to decr inflammation
- hypertonic saline
9
Q
Malnutrition
A
- decr pancreatic function resulting in malabsorption and risk of vitamin an dmineral deficiency
- frequent racking cough, the incr inmucous production and the incr work of breathing
10
Q
lung transplantation
A
- first successful double lung transplant in 1987
- pre-op conditioning program should be placed to optimize. -functional ability, exercise tolerance, emotional well-being.
11
Q
PT intervention
-airwayclearance
A
- improved ventilation and gas exchange
- limitation of tissue damage associated with chronic infection
- always include huffing and coughing in airway clearance
- percussion and postural drainage, positioning, active cycle of breathing etc
12
Q
Parent education
A
- instruction on administration of techniques
- assistance to make adjustment an dprovisions to ensure treatment adherence
- dangers of unnecessarily shielding the child
- infant with CF has typical physical and emotional development needs
13
Q
infancy signs and symptoms of CF
A
- lungs morphologically normal at birth
- within a few months, signs of impaired respiratory function may suggest bronchiolitis
- pronounced wheezing
- evidence of hyperinflation on x-ray
- airflow obstruction due to: inflammation, mucosal edema, copious mucous secretion, incr airway tone
14
Q
infancy PT intervention
A
- earlier intervention: allows for prevention of onset of progressive airway damage
- incr adherence to therapy regimen
- allows for early emphasis of preventon of repirtaory impairment
- initial goal is prevention of obstructive mucus plugging
15
Q
spirometry
A
-pulmonary function is assessed by spirometry if patient can perform voluntarily (usually >6 y/o)
