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
asthma pathophysiology
- genetic component found, but des not account for all types and severity
- abnormal mucous accumulation
- hypertrophy of smooth muscle, increased # of goblet cells, and deposition of interstitial collagen found in some
astma diagnosis
- wheezing and rhonchi
- coughing, difficulty breathing and chest tightness are worse at night or early morning
- hyperexpansion of thorax
- decr use of diaphragm
acute asthma attack
- increased respiratory rate
- expiratory grunting
- intercostal muscle retractions
- nasal flaring
- altered inspiratory-expiratory ratios
- coughing
- bluish color of lips and nails
NIH classification system step 1:
- intermittent
- symptoms <1x/wk
- brief exacerbations
- nocturnal symptoms< 1x/mo
- asymptomatic with normal lung function between exacerbations
- FEV or PEFR>80% predicted
NIH classification system Step 2:
- mild persistent
- symptoms >1x/wk, <1x/day
- exacerbations affect activity an dsleep
- nocturnal symptoms >2x/mo
- FEV or PEFR >80%
NIH classification system step 3
- moderate persistent
- daily symptoms
- exacerbations affect activity and sleep
- nocturnal symptoms >1x/wk
- FEV or PEFR 60-80%
NIH classification step 4
- severe persistent
- cont symptomps
- frequent exacerbations
- frequent nocturnal symptoms
- physical activities limited by symptoms
- FEV or PEFR <60%
Pulmonary function tests
- compared to predicted values based on age, sex and height
- may test after use of bronchodilator
- incr residual volume and functional residual capacity
- PEFR can be measured at home with a peak flow meter
asthma impairments of infancy and early childhood
- diagnosis not typically made until 3-6 years of age
- PFT can usually be performed around age 6 with child cooperation
- Hx of wheezy bronchitis, croup, recurrent pneumonia, difficulty sleeping and RSV
- high correlation with very low birth weight and prematurity
asthma impairments in childhood
- PFT is easier and more effective as diagnostic tool
- major presenting sign is wheezing
- associated with chronic or recurrent otitis media with effusion and GERD
- some may exhibit symptoms only after exercise, at night or in cold air
- PFT may be normal
asthma impairments in adolescence
- sympyoms typically decr
- may have significant impairments revealed on PFT measures
asthma treatment
- avoidance of triggers
- bronchodilators: inhaled or injected
- self monitoring
- action plan shared with school
- relaxation techniques
- allergy shots
- pharmacologic agents
asthma management techniques
- hydration
- timing of medication with activity
- proper warm up with EIA
- breath control techniques
asthma PT interventions neuromuscular
- facilitate muscle recruitment for imporvedpatterns of stabilization and breathing
asthma PT intervention musculoskeletal
- Rib cage mobilization
- stretching and strnegthening