Name: ___Dan Estadilla______ Date______6/12/2016_______

Disease Process:__________Cystic Fibrosis_________________

Anatomic Alterations
of the lungs

Etiology and
Epidemiology

Most common fatal inherited disorder in childhood

Autosomal recessive gene disorder caused by mutations in a pair of genes located in
chromosome 7.

Absence of three base pairs of codn 508 that codes for phenylalanine on
chromosome 7, 70-75% of patients with cystic fibrosis
Mucus blocks the passageways of pncreas, preventing enzymes from pncreas
from reaching the intestines.
Most men with cystic fribrosis are infertile
Fertility is decreased in women with CF secondary to thick cervical mucus.

Newborn Screening
Sweat Test
Molecular Diagnosis
Nasal Potential Differences
Prenatal Testing
Stool Fecal Fat Test

Diagnostic Tests

Appear normal at birth, but changes develop quickly.

Partial bronchial obstuction (mucus plugging)
Hyperinflation of the alveoli
Total bronchial obstruction (mucus plugging)
Atelectasis
Bronchiectasis
Exvessive production and accumulation of thick and tenacious mucus in
tracheobronchial tree secondary to indaqate hydration of the periciliary fluid
layer.
Impairment of normal mucociliary clearing mechanism ensues and many small
bronchi and bronchioles.

RESP 126 Clinical Medicine 1

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Clinical Manifestations

General Management

Wheezing
Chronic cough
Sputum production
Frequent respiratory infections
Digital clubbing
Gastrointestinal disorders
Delayed growth

Prevention of pulmonary infections, reduce amount of thick bronchial secretions,

improve air flow, provide adequate nutrition.
Pulmonary rehabilitation teams (RT, PT, OT, dietitian, psychologist
Regular medical checkups for general health, weight, height, pulmonary fucntion
abilities, and sputum culture results
Reccomended to heavily salt their food or take sodium supplements.

Oxygen therapy Protocol

Bronchopulmonary higiene thereapy
Lung expansin therapy
Aerosolized medication protocol
Mechanical ventilation protocol

Respiratory Care
Treatment Protocols

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Inhaled hypertonic saline-hydrate thick mucus in airways

Antibiotics-Tobramycin (TOBI) or aztreonam (Cayston) amikacin, colisitin,

Medical Treatments
(i.e. medications, etc)

Complications

National Education,
Initiative and
Prevention Programs

ciproflxacin, levofloxcain.
Ibuprofen-recommended in children and Young adolescent with mild CF-reduces
broncial inflmmation without hindering bacterial clearance decreases
neutrophil migration and inflammation in the lungsnot reccomended after the
age of 13
Inhaled corticosteriods/Systemic Glucocorticoids
Systemic glucorticoids are not recommended in children and adolescents-benefits
outweighed by adverse effects
Inhaled has not shown any clear benefits and therefore is not recommended

Coughing up blood
Diabetes
Infertility
Malnutrition
Chronic respiratory failure
Pancreatitis
Reoccuring pneumonia
pneumothorax

Lung transplant
Heart-lung transplant
Cystic fibrosis foundation

RESP 126 Clinical Medicine 1

DA

RESP 126 Clinical Medicine 1

DA