Cystic fibrosis and asthma Flashcards
CF definition
- inherited disorder of ion transport in the exocrine glands
- affects manly the respiratory, digestive, and male reproductive systems
CF etiology
- 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
CF pathophysiology
- obstruction of mucous secreting exocrine glands by hyperviscous secretions
- progressive airway obstruction affecting both small an dlarge airways
- irreversible airway damage
CF diagnosis
- 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
CF clinical manifestation gastrointestinal
- 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
CF clinical manifestation pulmonary
- 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
CF clinical manifestation musculoskeletal
- 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
CF medical management
- 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
Malnutrition
- 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
lung transplantation
- first successful double lung transplant in 1987
- pre-op conditioning program should be placed to optimize. -functional ability, exercise tolerance, emotional well-being.
PT intervention
-airwayclearance
- 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
Parent education
- 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
infancy signs and symptoms of CF
- 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
infancy PT intervention
- 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
spirometry
-pulmonary function is assessed by spirometry if patient can perform voluntarily (usually >6 y/o)
cycle ergometer
- progressive protocol can be done to determine VO2 max and peak work capacity
- pros inexpensive, portable, safe, familiar exercise
pre-school and school-age period intervention goals
- improve exercise tolerance
- secretion clearance
- correction and mainatenance of posture
- promote self efficacy and adherence
HFCWO
- inflatable vest attached to pump that generates high freq oscillations applied to external chest wall
- creates a cough like expiratory flow bias that shears mucus form airway walls and encourages movement upward
adolescence CF management
- arrested sexual development and smaller than avg physical build can lead to incr feelings of isolation
- poor adhearence to conventional PT
autogenic drainage
- control of inspiratory and expiratory airflow to generate maximum airflow within bronchi
- 3 phases
- “unstick” mucus in distal airway
- ” collect” mucus in middle airways
- “evacuate” from cetral airways
CF ball therapy
- means of postural exercises
- means of WB exercises
- means of aerobic fitness
- improve balance
- improve coordination
- incr relaxation
asolescence intervention posture
- needed for efficient breathing mechnaics
- Postural changes in CF associated with hyper inflated lungs
- incr AP chest diameter
- shoulder elevation
- shoulder forward protraction
- abdominal flexion
- thoracic kyphosis
asthma pathology
- ocstructive lung disease caused by incr reaction of the airways to various stimuli
- chronic inflammatory condition with acute exacerbations characterized by complex biomechanical , autonomic, and immunologic factors
asthma 3 significant characteristics
- airway inflammation
- airway obstruction that is often reversible either spontaneously or with pharmacologic intervention
- bronchial hyperresponsiveness to stimuli that are either intrinsic or extrinsic