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
2
Q
Cystic Fibrosis - Organs Affected
A
-
Respiratory
> obstruction -
Pancreas
> affects absorption -
Bile ducts
> portal HTN -
GI
> mechanical obstruction - Reproductive dysfunction
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
4
Q
Cystic Fibrosis - Manifestations
A
- Salty-tasting skin
- Chronic respiratory problems
- Lung infections
- Poor growth/weight loss
- Meconium ileus
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
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
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
8
Q
Cystic Fibrosis - Integumentary System
A
- Very high concentrations of sodium & chloride in sweat
- Skin tastes salty
- Dehydration & electrolyte imbalances occur
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
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
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