Cystic Fibrosis Flashcards

1
Q

Cystic Fibrosis - Patho

A
  • Autosomal recessive trait
    > meconeum tested
  • Chronic multisystem disorder
    > GI, pancreas, bile ducts, reproductive,
  • Exocrine gland dysfunction leading to incrd viscosity of mucus
  • Secretion results in mechanical obstruction
  • Thick mucus accumulated, dilates, precipitates, and coagulates to form concretions in glands & ducts
  • Progressive & incurable
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2
Q

Cystic Fibrosis - Organs Affected

A
  • Respiratory
    > obstruction
  • Pancreas
    > affects absorption
  • Bile ducts
    > portal HTN
  • GI
    > mechanical obstruction
  • Reproductive dysfunction
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3
Q

Cystic Fibrosis - Diagnostics

A
  • Sweat Chloride Test
    > chloride concentration greater than 60 mEQ/L is a positive test result
    > higher than 40 mEq/L is diagnostic in infants younger than 3 months
  • Universal newborn screening
  • DNA identification of mutant genes
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4
Q

Cystic Fibrosis - Manifestations

A
  • Salty-tasting skin
  • Chronic respiratory problems
  • Lung infections
  • Poor growth/weight loss
  • Meconium ileus
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5
Q

Cystic Fibrosis - Respiratory System

A
  • Stagnation of mucus and bacterial colonization result in destruction of lung tissue
  • Tenacious secretions are difficult to expectorate: they obstruct bronchi/brinchioles
  • Emphysema and atelectasis occur as airways become incringly obstructed
  • Chronically in a hypoxic state which contraction & thickening of muscle fibers in pulmonary arteries/arterioles
  • This leads to pulmonary HTN & eventual cor pulmonale
  • Ruptured bullae leads to pneumothorax
  • Erosion of the bronchial wall leads to hemoptysis
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6
Q

Cystic Fibrosis - Respiratory Management

A
  • Airway clearance therapies
  • Bronchodilator medication
  • Physical exercise
  • Aggressive treatment of pulmonary infections
  • Aerosolized antibiotics
  • Home intravenous antibiotic therapy
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7
Q

Cystic Fibrosis - GI System

A
  • One of 1st manifs is a meconium ileus in newborn
  • Intestinal obstruction caused by thick intestinal secretions
  • Frothy & foul-smelling stools
  • Deficiency in fat-soluble vitamins
    > A, D, E, K
  • Malnutrition & failure to thrive
  • Pancreatic fibrosis which may incr risk for diabetes
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8
Q

Cystic Fibrosis - Integumentary System

A
  • Very high concentrations of sodium & chloride in sweat
  • Skin tastes salty
  • Dehydration & electrolyte imbalances occur
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9
Q

Cystic Fibrosis - Reproductive System

A
  • Can delay puberty in girls
  • Inhibits fertility due to thick mucus
  • Males are typically sterile due to blockage of vas deferens
  • Hard to maintain pregnancy
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10
Q

Prognosis of Cystic Fibrosis

A
  • Median survival is 42yrs
  • Progressive & incurable disease
  • Transplantation
    > incrs life expectancy
    > availability of ogans
    > surgical complications
  • Interprofessional care management
    > hospital care
    > home care
    > family support
    > transition to adulthood
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11
Q

Cystic Fibrosis - Family Support

A
  • Coping w/ emotional needs of child & fam
  • Need for treatments multiple times a day
  • Frequent hospitalization
  • Home care
  • Implications of genetic transmission of disease
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