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Condition/Disorder Synonyms

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  • Mucoviscidosis

  • CF

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ICD-9-CM Code

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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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ICD-10-CM Codes

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  • E84 Cystic Fibrosis

  • E84.9 Cystic fibrosis, unspecified

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Preferred Practice Patterns

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Key Features

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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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Clinical Findings

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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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Means of Confirmation or Diagnosis

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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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  • Chest radiograph

  • Chest CT, high resolution

  • Ventilation/perfusion lung scan

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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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Findings and Interpretation

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  • May show osteoporosis on x-ray

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Treatment

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Medication

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  • For lung infection, antibiotics

  • Pancreatic enzyme

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Referrals/Admittance

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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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Impairments

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  • Exercise limitation

  • Excess loss of salts/electrolytes from sweating

  • Shortness of breath

  • Proximal upper-limb strength impairment

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