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  • Mucoviscidosis
  • CF

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  • 277.0 Cystic fibrosis
  • Associated Physical Therapy Diagnoses
    • 780.7 Malaise and fatigue
    • 786.0 Dyspnea and respiratory abnormalities
    • 786.05 Shortness of breath

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  • E84 Cystic Fibrosis
  • E84.9 Cystic fibrosis, unspecified

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Description

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  • Genetic disease
  • Mucus buildup, blocks airways
  • Mutation on chromosome 7

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Essentials of Diagnosis

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  • Productive cough
  • Early diagnosis critical
  • Autosomal recessive heritability
  • This mucus
  • Salty sweat

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General Considerations

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  • Exocrine glands affected
  • Bacteria can grow in the built-up mucus
  • Mucus blocks pancreatic digestive enzymes from small intestines
    • Intestines cannot absorb fats, proteins, vitamins
    • Malnutrition
  • Affects lungs, pancreas, liver, intestines, sinuses

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Demographics

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  • Usually diagnosed in newborns
  • Children of Caucasian descent most commonly affected
  • Individuals can live to middle age with medical care

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Signs and Symptoms

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  • Baby’s skin tastes salty
  • Mucus in the lungs
  • Dehydration
  • Chest tightness
  • Fatigue
  • Malnutrition
  • Constipation
  • Clubbing of finger tips
  • Wheezing
  • Sinusitis
  • Chronic cough
  • Shoulder raised to allow increased air-flow to lungs
  • Shortness of breath
  • Tensed muscles from dyspnea
  • Anxiety
  • Depression

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Functional Implications

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  • Disabling dyspnea when performing simple tasks
  • Decreased exercise tolerance
  • Malnutrition causes fatigue

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Possible Contributing Causes

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  • Genetic: inherited gene from both parents
  • Each parent must be carrier of gene; may not be aware
  • Children have 25% chance of inheriting CF if both parents are carriers

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Differential Diagnosis

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  • Bronchiectasis
  • Fungal or parasitic infection
  • Lung abscess
  • Cat scratch fever
  • Sarcoidosis
  • Hodgkin’s lymphoma
  • Asthma
  • Central airway obstruction
  • Lung cancer

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Laboratory Tests

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  • Prenatal screening
  • Genetic test
  • Sputum culture
  • Blood test
  • Sweat chloride test

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Imaging

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Diagnostic Procedures

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  • Lung function or pulmonary function tests
    • Spirometry
    • Body plethysmography
    • Peak expiratory flow

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Medication

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  • For lung infection, antibiotics
  • Pancreatic enzyme

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  • To hospital for imaging, x-ray
  • To physician for medication
  • To pulmonologist for medical assessment
  • To respiratory therapist for disease management

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  • Exercise limitation
  • Excess loss of salts/electrolytes from sweating
  • Shortness of breath
  • Proximal upper-limb strength impairment
  • Limited endurance capacity
  • Infertility

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  • Chest physical therapy
    • Percussion
    • Postural positioning
  • Manual therapy to loosen ribcage, diaphragm, thoracic spine
  • Exercise (aerobic, strength, flexibility, posture, breathing)
  • Airway clearance
  • Pursed-lip breathing
  • Coughing techniques
  • Pulmonary rehabilitation (PR)
    • Improves exercise capacity
  • Inspiratory muscle training (IMT)

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  • Patient will be able to ambulate over 600 meters in a six-minute walk test
  • Patient will score above 80% of their predicted ...

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