cf and broch

  • pathophysiology of CF

    • autosomal recessive disease

    • defect in gene encoding the CFTR on chromosome 7

    • CFTR proteins exchange chloride and water across cell membranes

    • therefore dysfunction leads to water and salt accumulate in cells leading to thick and viscous secretions

  • cycle of CF slide

  • CF classes TBC

    • normal

    • class I

    • class II (most common): CFTR protein is created, but misfolds keeping it from moving to the cell surface

    • class III

    • class IV

    • class V

  • doesn't just affect the lungs it also affects

    • GI system: can have malnutrition and poor growth , also can have CF related diabetes

    • can create gallstones as bile created from liver can be thicker than normal

    • infertility , mainly in men, as absence of

    • bone issues (OP, OA etc)

  • how CF affects the lungs cycle

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  • CF clinical features

    • chronic cough

    • purulent sputum (colonisation → morbidity/mortality)

    • frequent chest infections

    • dyspnoea

    • chest pain

    • haemoptysis (blood in sputum) (associated with infection)

    • clubbing (fingers)

    • ausc: course inspiratory crackles

  • complication of CF

    • haemoptysis - blood in sputum

    • pneumothorax

    • urinary stress incontinence: from repeated stress on pelvic floor)

  • use of airway clearance techniques

    the main treatment for CF

    • ACBT

    • PEP / oscillating PEP

    • autogenic drainage

    • postural drainage

    • exercise

  • inhalation therapy

    • medications: antibiotics

    • saline: combined with AWCT’s (hypertonic saline + PEP)

  • management for infants

    • parental education

    • 5 modified postural drainage positions with percussion and vibrations

    • infant PEP

    • assisted autogenic drainage

    • active developmental play

  • management of young children

  • what is bronchiectasis

    resp condition characterised by abnormal, irreversible dilation/widening of the bronchi

    inflammation from infection

    • airway obstruction

    • reduced elasticity

    • impaired mucociliary clearance

  • diagnosis

    high resolution CT scan

    large vs small airways

    can be a feature of other chronic lung disease such as CF and COPD

  • different types

    • cylindrical: enlarged and cylindrical airways

    • varicose: irregular with areas of dilation and constriction

    • saccular or cystic: dilated bronchi form clusters of cysts (worst)

  • pathophysiology slide

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  • pathophysiology cycle

    • infection

    • inflammation

    • airway damage —> bronchiectasis

    • decreased mucociliary clearance and then cycle repeats

  • clinical features

    1. Recurrent infections Cough +/- sputum (purulent) +/- haemoptysis- frank or minor

    2. Chest pain

    3. Fatigue & malaise

    4. Sinusitis

    5. Dyspnoea

    6. +/- incontinence

    7. Ausc: localised or wide-spread insp and exp crackles

  • severity index includes

    Combination outcome measure

    • BMI

    • Spirometry (% of pred. FEV1)

    • Hx Hospital Admissions

    • Number of Severe exacerbations (last two years)

    • NMRC Breathlessness Score

    • Sputum Cultures

    • Radiology

  • bronchiectasis management

    1. Antibiotics (Regular vs Exacerbations)

    2. Bronchodilators

    3. Inhalation therapy: hypertonic saline and / or mannitol - incr ciliary transport by altering mucus rheology and hydration of airway surface

    4. Vaccinations

    5. Airway clearance

    6. Pulmonary Rehabilitation